PSU Volume 63 No 01 JULY 2024

Tracheostomy

Tracheostomy in children is a crucial surgical procedure increasingly performed as advancements in neonatal and pediatric intensive care improve the survival rates of children with severe medical conditions. This intervention is generally reserved for those requiring long-term airway management, often due to congenital anomalies, chronic respiratory failure, or extensive trauma. The procedure involves creating a direct airway through an incision in the neck into the trachea and is critical for patients who require prolonged mechanical ventilation or have obstructive airway disorders.

The indications for pediatric tracheostomy have evolved over recent years, reflecting a broader understanding of its benefits and risks. Historically, tracheostomies were often a last resort for managing airway obstructions or chronic pulmonary conditions. However, with better survival rates of premature infants and advancements in surgical techniques and postoperative care, the procedure is now more commonly indicated for a variety of conditions. These include severe upper airway anomalies, long-term ventilation due to neuromuscular diseases, and airway management following extensive surgeries.

Pediatric tracheostomy, unlike its adult counterpart, is predominantly a surgical procedure due to the specific anatomical and physiological needs of children. The smaller size and more delicate tissues of pediatric patients require meticulous surgical planning and precision. Surgeons must choose the appropriate type and size of the tracheostomy tube and carefully manage the timing of the initial placement and subsequent tube changes. This careful planning is essential to minimize perioperative complications, which can include hemorrhage, infection, subglottic stenosis, and accidental decannulation.

Postoperative care for pediatric tracheostomy patients is intensive and requires a multidisciplinary approach. Immediately following surgery, patients need constant monitoring to manage potential complications like bleeding, airway obstruction, or infection. Long-term management involves routine care of the tracheostomy site, regular changes of the tracheostomy tube, and management of complications such as granulation tissue formation and tracheomalacia. The care team typically includes pediatric otolaryngologists, pulmonologists, specialized nurses, speech and language therapists, and respiratory therapists.

Complications are a significant concern in pediatric tracheostomy care. Immediate postoperative complications can affect the overall success of the procedure and the long-term well-being of the patient. Long-term complications might involve damage to the tracheal wall, persistent fistulas, or recurrent infections. Each of these requires specific management strategies that can include surgical interventions, adjustments in the type or size of the tracheostomy tube, and intensive local care.

The decision to decannulate, or remove the tracheostomy tube, is a complex and critical part of the care process. Decannulation is considered when the child's original medical condition has resolved or improved sufficiently to allow safe removal of the tube. The process requires a careful assessment of the airway patency, respiratory muscle strength, and the ability of the child to protect their airway. Successful decannulation involves a detailed protocol that may include downsizing the tube, capping the tube to assess the child's ability to breathe without it, and careful monitoring for respiratory distress.

Recent advancements in pediatric tracheostomy care include the development of new tracheostomy tube materials and designs that reduce the risk of complications. Additionally, the use of speaking valves and other devices has significantly improved the quality of life for these patients, facilitating speech and normal breathing patterns, which are crucial for young children's development.

In conclusion, pediatric tracheostomy is a life-saving procedure that involves complex decision-making and extensive care management. The procedure's necessity arises from a variety of severe medical conditions that compromise the airway and require long-term management. Advances in medical technology, surgical techniques, and comprehensive care approaches continue to improve outcomes for pediatric tracheostomy patients. However, the procedure still carries significant risks that require a coordinated effort from a dedicated multidisciplinary team to ensure the best possible outcomes for these vulnerable pediatric patients.

Percutaneous Tracheotomy

Tracheostomy, particularly percutaneous tracheostomy (PT), is a vital procedure in pediatric and adult critical care, facilitating long-term ventilation and airway management in severely ill patients. Recent literature provides a comprehensive overview of various techniques, their adaptations, and clinical outcomes associated with PT, reflecting significant advancements in both technology and procedural methodologies.

Percutaneous tracheostomy, often performed at the bedside in intensive care units (ICUs), operating theaters, or emergency departments, has become preferable over surgical tracheostomy due to its minimal invasiveness, reduced complication rates, and cost-effectiveness. The procedure typically involves using a needle to puncture the trachea under direct or ultrasound guidance, followed by serial dilations to place the tracheostomy tube. Innovations and modifications of the PT technique, such as the use of ultrasound and various dilation methods, aim to enhance safety and accuracy, reducing risks like bleeding, infection, and damage to surrounding structures.

A significant focus in pediatric PT is the accuracy of tracheostomy tube placement, crucial due to the smaller anatomical scale and the delicacy of pediatric patients? tracheal structures. Studies highlight the challenge of accurately identifying tracheal rings via palpation alone, with ultrasound emerging as a valuable tool to increase the precision of the insertion site, thereby minimizing potential complications. Research underscores the importance of precise anatomical identification to avoid complications such as subglottic stenosis and damage to nearby vascular structures, which are more pronounced in pediatric patients due to their less prominent anatomical landmarks and softer cartilage structures.

The introduction of ultrasound in PT procedures has been shown to significantly enhance the safety and efficacy of the technique. It allows for real-time visualization of the needle, dilators, and tracheostomy tube in relation to critical anatomical features such as the thyroid gland, tracheal rings, and vascular structures. This method not only increases the accuracy of the procedure but also reduces the risk of complications, which is particularly crucial in settings where anatomical abnormalities or obesity may complicate traditional palpation methods.

Cost considerations remain crucial in the widespread adoption of PT. Studies have documented the development of modified techniques that utilize more readily available or less expensive equipment, which is particularly relevant in resource-limited settings. These adaptations make PT more accessible and cost-effective while maintaining safety and effectiveness.

Moreover, longitudinal studies on PT emphasize the importance of long-term follow-up to monitor complications such as tracheal stenosis, tracheomalacia, or tracheoesophageal fistulas, which may develop as late complications. The need for ongoing care and assessment post-procedure underscores the necessity of a multidisciplinary approach involving surgeons, intensivists, respiratory therapists, and nurses to optimize patient outcomes.

In conclusion, percutaneous tracheostomy has evolved significantly, with advancements in technique and technology that enhance safety and efficiency. The integration of ultrasound has been a pivotal improvement, particularly in pediatric care, ensuring higher accuracy and reducing risks. Ongoing research and innovation are expected to further refine PT, reducing its cost, and making it accessible to a broader range of patients while continuing to minimize associated risks and complications.

Pancreas Divisum

Pancreas divisum is a congenital anomaly affecting approximately 10% of the population, resulting from the failure of the dorsal and ventral pancreatic ducts to fuse during embryonic development. This condition causes most of the pancreatic secretions to be drained through the minor papilla rather than the major papilla, which is the more common drainage route in individuals without the condition. There are three subtypes of pancreas divisum: Type 1, also known as classic, Type 2, characterized by the absence of the ventral duct, and Type 3, which is functional. While the majority of patients with this anomaly are asymptomatic, a subset experiences significant clinical issues, including abdominal pain and recurrent pancreatitis.

Various diagnostic techniques have been employed to identify pancreas divisum, each with differing accuracies. Magnetic Resonance Cholangiopancreatography (MRCP), Secretin-enhanced MRCP (S-MRCP), and Endoscopic Ultrasound (EUS) are among the primary non-invasive techniques used. MRCP is widely used due to its non-invasive nature and high specificity, which is reported to be 99%. However, its sensitivity is relatively lower at 59%. Studies suggest that MRCP's accuracy can be significantly improved with secretin stimulation, which increases the sensitivity to 83% while maintaining a specificity of 99%. S-MRCP enhances the visualization of the pancreatic ducts by stimulating the pancreas to secrete more fluid, thus improving diagnostic accuracy. The area under the hierarchical summary receiver-operating characteristic curve for S-MRCP is 0.99, making it a highly reliable diagnostic tool.

EUS provides detailed imaging of the pancreatic ducts and has a sensitivity of 85% and specificity of 97%. Although slightly less sensitive than S-MRCP, EUS is superior to MRCP alone. The HSROC curve for EUS is 0.97, indicating its efficacy in diagnosing pancreas divisum. Despite these advancements, the traditional gold standard for diagnosing pancreas divisum has been Endoscopic Retrograde Cholangiopancreatography (ERCP), which is invasive and comes with potential complications. Nevertheless, ERCP remains crucial for both diagnostic and therapeutic purposes, particularly in symptomatic patients.

Therapeutic interventions are primarily considered for symptomatic patients, especially those suffering from recurrent pancreatitis. Various surgical and endoscopic procedures have been developed to manage symptoms and improve pancreatic drainage. ERCP is a common therapeutic intervention for pancreas divisum, particularly in patients with recurrent pancreatitis. It involves sphincterotomy, stone extraction, and stenting to relieve ductal obstruction. Studies have shown significant clinical improvement post-ERCP, with a reduction in the frequency of acute pancreatitis episodes.

In pediatric patients, ERCP has also proven to be effective and safe. A study involving children with symptomatic pancreas divisum treated with ERCP indicated a significant decrease in the median number of acute pancreatitis episodes post-procedure. The clinical remission rate was notably high, although the incidence of post-ERCP pancreatitis (PEP) was observed to be 7.9%. Female sex, stone extraction, and genetic mutations were identified as potential risk factors for PEP. Despite these risks, the benefits of ERCP in managing pediatric pancreas divisum are substantial, contributing to improved clinical outcomes and quality of life for these young patients.

In cases where traditional ERCP is not feasible due to anatomical challenges or severe inflammation, the EUS-guided rendezvous technique has been successfully employed. This technique involves puncturing the pancreatic duct using a therapeutic echoendoscope, followed by ductal stenting or dilation. The rendezvous technique has shown promising results in managing complicated cases of pancreas divisum, offering a viable alternative when standard ERCP approaches are inadequate.

Genetic factors also play a significant role in the pathogenesis of pancreatitis in patients with pancreas divisum. Mutations in genes such as SPINK1, PRSS1, and CFTR are commonly associated with increased susceptibility to pancreatitis. These genetic factors not only influence the severity of symptoms but also impact the response to therapeutic interventions. Understanding the genetic underpinnings of pancreas divisum can aid in identifying at-risk individuals and tailoring personalized treatment strategies.

Recent advancements in diagnostic imaging and therapeutic techniques have greatly enhanced the management of pancreas divisum. The comparison of diagnostic accuracies among MRCP, S-MRCP, and EUS revealed that S-MRCP is the most reliable, followed closely by EUS, while MRCP alone is less sensitive. These findings underscore the importance of selecting the appropriate diagnostic modality based on individual patient characteristics and clinical presentation.

In clinical practice, a combination of these diagnostic tools may be employed to ensure accurate diagnosis and effective management of pancreas divisum. For instance, an initial MRCP can be followed by S-MRCP or EUS if the diagnosis remains unclear. This stepwise approach can help avoid unnecessary invasive procedures while ensuring that symptomatic patients receive timely and appropriate intervention.

Therapeutic strategies continue to evolve, with minimally invasive techniques gaining prominence. The use of ERCP, particularly in pediatric populations, has demonstrated substantial benefits, including reduced episodes of pancreatitis and improved nutritional status. The introduction of EUS-guided techniques further expands the therapeutic options available, particularly for complex cases where traditional methods may fall short.

The ongoing research into the genetic aspects of pancreas divisum is also promising. Identifying specific genetic mutations associated with the condition can lead to more targeted therapies and preventative measures. For example, patients with known genetic predispositions may benefit from earlier intervention and more frequent monitoring to prevent complications.

In conclusion, pancreas divisum is a congenital anomaly with significant clinical implications for a subset of patients. Advances in diagnostic imaging, particularly the use of S-MRCP and EUS, have improved the accuracy of diagnosis, while therapeutic interventions such as ERCP and EUS-guided techniques offer effective management options for symptomatic individuals. Understanding the genetic factors associated with pancreas divisum further enhances the ability to tailor treatment and improve outcomes. Continued research and clinical studies are essential to refine these strategies and ensure the best possible care for patients with this condition.

PSU Volume 63 No 02 AUGUST 2024

Probiotics for NEC

Necrotizing enterocolitis (NEC) is a severe gastrointestinal disease primarily affecting preterm infants, characterized by intestinal inflammation and necrosis, which leads to significant morbidity and mortality. The exploration of probiotics as a preventative measure against NEC has gained substantial attention due to their potential benefits in enhancing intestinal health and reducing disease incidence. Probiotics are live microorganisms that, when administered in adequate amounts, confer health benefits to the host. These benefits are mediated through several mechanisms, including the enhancement of the intestinal barrier, production of short-chain fatty acids such as butyrate, competition with pathogenic bacteria, and modulation of the immune system by downregulating pro-inflammatory genes and upregulating cytoprotective genes.

The routine use of probiotics in preterm infants began gaining traction in the late 1990s. A seminal study by Dr. Angela Hoyos in 1999 highlighted that daily administration of Lactobacillus acidophilus and Bifidobacterium infantis to neonates in a neonatal intensive care unit significantly reduced the incidence of NEC and associated mortality. This pioneering research paved the way for numerous clinical trials and studies that followed, involving over 10,000 preterm infants, to evaluate the efficacy of probiotics in preventing NEC. The general consensus from these studies supports the beneficial role of probiotics in reducing the risk of NEC, mortality, and late-onset sepsis.

Randomized controlled trials (RCTs) have provided substantial evidence on the effectiveness of probiotics in preventing NEC. For example, Bin-Nun et al. conducted an RCT in 2005 which demonstrated that a mixture of B. infantis, S. thermophilus, and B. bifidus significantly reduced the incidence of NEC. Similarly, Dani et al. found in their 2013 study that supplementation with Lactobacillus rhamnosus GG (LGG) led to a reduction in NEC among preterm infants. Another notable trial, the ProPrems Trial conducted in Australia and New Zealand, involved very low birth weight infants, and found that supplementation with a combination of B. infantis, S. thermophilus, and B. lactis reduced the incidence of NEC by over half. Despite some heterogeneity in trial designs and probiotic strains used, systematic reviews and meta-analyses have consistently shown that probiotic supplementation favors the prevention of NEC, mortality, and late-onset sepsis.

Observational studies further support the findings from RCTs. A comprehensive review of 30 non-randomized studies involving over 77,000 infants from 18 different countries reported that probiotic supplementation significantly reduced the risk of NEC. This body of evidence underscores the potential of probiotics as a preventative strategy against NEC in preterm infants.

Several professional societies have issued recommendations regarding the use of probiotics in preterm infants, reflecting the growing acceptance and cautious optimism within the medical community. The Canadian Pediatric Society (CPS) advises caution and suggests considering probiotics for preterm infants at risk for NEC, particularly those with birth weights over 1 kilogram. The European Society for Pediatric Gastroenterology, Hepatology and Nutrition (ESPGHAN) emphasizes that probiotics should be produced under stringent manufacturing practices and that clinicians should discuss potential risks and benefits with parents. In contrast, the American Academy of Pediatrics (AAP) does not recommend universal probiotic administration for preterm infants, citing concerns about safety, particularly the lack of FDA-approved products and the potential risk of probiotic-associated sepsis.

The implementation of probiotics in clinical practice varies widely across different regions and institutions. For instance, Sunnybrook Health Sciences Centre in Canada reported a decrease in NEC rates from 4.4% to 1.7% after implementing probiotic supplementation with Lactobacillus reuteri DSM 17938. Similarly, Oregon Health & Science University in the United States observed a reduction in NEC incidence from 11% to 2.7% with the use of B. infantis EVC001. The University of Utah Medical Center also reported a decrease in NEC rates from 7% to 2% with their probiotic protocol, which included multiple strains of Bifidobacteria and Lactobacillus. Emory University Midtown in the United States saw a reduction in NEC incidence from 13.2% to 5.6% after implementing a regimen involving Lactobacillus reuteri.

Despite the promising results, there are safety concerns associated with probiotic use, particularly regarding the risk of contamination and probiotic-associated sepsis. Cases of sepsis linked to probiotics have been reported, underscoring the importance of stringent safety protocols and high-quality manufacturing practices. It is crucial for facilities implementing probiotic supplementation to develop comprehensive guidelines to ensure safety and to monitor the effects of this therapy closely.

In conclusion, the use of probiotics to prevent NEC in preterm infants has been extensively studied and generally supported by clinical evidence. While more clinical trials may not significantly alter the pooled outcomes given the existing body of research, the decision to implement routine probiotic supplementation remains complex and multifaceted. Factors such as a center?s baseline NEC incidence, the quality of the probiotic products available, and the effectiveness of other preventive measures, like promoting a human milk diet, play critical roles in this decision. Engaging multiple stakeholders, including families, in the decision-making process is essential to ensure informed choices and optimize outcomes for preterm infants. Although concerns about product quality and safety persist, the substantial evidence supporting the benefits of probiotics suggests that their use can be a valuable strategy in the fight against NEC, particularly in high-risk populations.

Hepatoblastoma

Hepatoblastoma (HB) is the most common primary liver malignancy in children, predominantly affecting those under three years of age. Over the past few decades, the overall survival rates have significantly improved from 30% to approximately 80%, thanks to advancements in chemotherapy and surgical techniques, including liver transplantation (LT). The disease stage and current treatment protocols are heavily influenced by the extent of the tumor, assessed using the PRETEXT (pre-treatment extent of disease) and POST-TEXT (post-treatment extent of disease) systems, which help in determining the surgical resectability and the need for LT.

Innovations in chemotherapy, particularly with cisplatin-based regimens, have rendered many initially unresectable tumors resectable. However, the curability of chemotherapy-resistant or metastatic HB relies heavily on achieving radical surgical resection. Recent studies indicate that aggressive surgical management of lung metastases, which are common in HB, can lead to favorable prognoses even when complete remission is not achieved through chemotherapy alone.

Preoperative imaging technologies have advanced considerably, with 3D imaging and virtual simulation of hepatectomy providing detailed insights into the anatomical relationships between tumors and vascular structures. These technologies, including 3D printing, have not only enhanced surgical planning but also improved patient and parent education. Additionally, diffusion-weighted MRI and the use of hepatobiliary MRI contrast agents like gadoxetate disodium have proven effective in detecting satellite lesions and evaluating the relationship of tumors to critical vascular structures.

Intraoperatively, augmented reality navigation systems and indocyanine green (ICG) fluorescence imaging have improved the precision of tumor localization and resection. ICG, in particular, has shown high sensitivity in identifying liver tumors during surgery, although it has limitations in detecting tumors deeper than 10 mm from the organ surface.

Minimally invasive surgical approaches, such as laparoscopic and robotic-assisted liver resections, have been increasingly applied in pediatric HB cases. These techniques, when performed by experienced surgeons, offer reduced postoperative complications and shorter recovery times. The use of radiofrequency-assisted pre-coagulation and advanced hemostatic techniques has further enhanced the safety and efficacy of these minimally invasive procedures.

The associating liver partition and portal vein ligation for staged hepatectomy (ALPPS) procedure has emerged as a valuable technique for increasing the future liver remnant (FLR) in patients with extensive liver tumors. Although initially associated with high morbidity, modified ALPPS procedures have shown promising results in selected pediatric HB cases, facilitating successful resections that might otherwise necessitate LT.

Liver transplantation remains a critical option for patients with unresectable HB. Primary LT is often recommended for advanced HB cases, especially those classified as central POST-TEXT III or IV. Advances in perioperative care and surgical techniques have significantly improved the outcomes of LT, with five-year survival rates now approaching 90%. Living donor liver transplantation (LDLT) offers a valuable alternative, particularly in regions with organ shortages, allowing for optimal timing and reduced delays in treatment.

Management of metastatic HB, particularly lung metastases, continues to be challenging. Neoadjuvant chemotherapy has been effective in achieving complete remission of lung metastases in over 50% of cases. For residual or recurrent metastatic lesions, surgical resection remains a critical component of treatment. Techniques such as ICG navigation surgery and CT-guided localization have enhanced the precision of metastasectomy, contributing to improved outcomes.

Relapse in HB patients, although less common with modern treatment protocols, still poses a significant challenge. Factors such as age, PRETEXT stage, and initial metastatic status are important predictors of recurrence. Despite this, a substantial proportion of relapsed patients can achieve long-term survival through repeat surgical interventions and chemotherapy. The feasibility of re-transplantation in selected cases of intragraft recurrence further underscores the importance of achieving complete tumor resection whenever possible.

In conclusion, the management of hepatoblastoma has seen remarkable advancements over the years, with significant improvements in imaging, surgical techniques, and transplantation strategies. Continued research and technological innovation hold promise for further improving the prognosis and quality of life for children affected by this malignancy. Integrating these advancements into a comprehensive, multidisciplinary approach remains key to optimizing outcomes and achieving long-term remission for hepatoblastoma patients.

Artificial Intelligence in Pediatric Surgery

The integration of artificial intelligence (AI) into pediatric surgery is rapidly evolving, bringing about significant transformations in healthcare. Artificial intelligence, defined as the capability of a machine to imitate intelligent human behavior, is making remarkable strides in the medical field. In pediatric surgery, AI's applications range from diagnostics to intraoperative assistance and postoperative care.

One of the critical areas where AI has shown significant potential is in the diagnosis of acute appendicitis in children. Acute appendicitis is the most common pediatric surgical emergency worldwide, and early diagnosis is crucial to prevent complications such as perforation and peritonitis. Traditional diagnostic methods rely on clinical signs, laboratory tests, and imaging, which can be subjective and variable in accuracy. AI algorithms, however, can analyze a combination of clinical, laboratory, and imaging data to provide more accurate and consistent diagnoses. For instance, a systematic review found that AI models achieved diagnostic accuracy rates above 90%, highlighting their potential to outperform traditional diagnostic methods.

AI's role extends into the operating room, where it assists surgeons with real-time data and procedural guidance. Technologies like augmented reality (AR) and machine learning algorithms are increasingly being integrated into surgical procedures. AR can overlay critical information onto the surgeon's field of view, enhancing precision and reducing errors. Machine learning models can analyze electro-neurophysiological data to distinguish between different tissue types during complex surgeries like selective dorsal rhizotomy, thereby improving outcomes.

Postoperative care is another area where AI can make a substantial impact. AI algorithms can predict postoperative complications by analyzing patient data, enabling early interventions and personalized care plans. This predictive capability is particularly valuable in pediatric patients, who may have different physiological responses compared to adults. AI-driven predictive analytics can optimize recovery strategies, reduce hospital stays, and improve overall patient outcomes.

While the benefits of AI in pediatric surgery are substantial, they come with a host of ethical, legal, and societal challenges. These challenges must be addressed to ensure that AI technologies are used responsibly and equitably. One of the primary ethical concerns with AI in healthcare is the potential for algorithmic bias. AI systems learn from historical data, which may contain biases that can be perpetuated or even amplified by the algorithms. This is particularly concerning in pediatric surgery, where biased algorithms could disproportionately affect vulnerable populations. Ensuring fairness and transparency in AI algorithms is critical to prevent discriminatory outcomes.

Patient privacy is another significant concern. AI systems require large amounts of data to function effectively, raising questions about data security and patient consent. Robust safeguards must be implemented to protect patient data and ensure that it is used ethically and transparently.

The legal implications of AI in pediatric surgery revolve around accountability and liability. When AI systems are used in clinical decision-making, it becomes challenging to determine who is responsible for errors the machine, the developer, or the healthcare provider. Clear guidelines and regulations are needed to define accountability and ensure that patients have recourse in case of malpractice.

From a societal perspective, the deployment of AI in healthcare must transcend socio-economic boundaries to ensure equitable access to these advanced technologies. This involves not only making the technology available but also providing the necessary training and education to healthcare providers to use AI tools effectively.

For AI to be successfully integrated into pediatric surgery, it is essential to build trust among healthcare providers, patients, and their families. This involves transparent communication about how AI systems work, their benefits, and their limitations. Education and training programs for healthcare professionals are crucial to ensure they are well-equipped to leverage AI technologies effectively and ethically.

Artificial intelligence is poised to revolutionize pediatric surgery by enhancing diagnostic accuracy, improving surgical precision, and optimizing postoperative care. However, the successful integration of AI into pediatric healthcare requires addressing ethical, legal, and societal challenges. Ensuring fairness, transparency, and patient privacy, while establishing clear accountability frameworks and providing comprehensive education and training, are essential steps towards harnessing the full potential of AI in pediatric surgery. As we navigate this transformative era, the continuous evolution of AI holds the promise of a future where technology becomes an indispensable ally in delivering optimal pediatric care.

PSU Volume 63 No 03 SEPTEMBER 2024

Lymphatic Duct Embolization

Lymphatic duct embolization (LDE) has emerged as a significant intervention for pediatric patients suffering from complex lymphatic disorders. This procedure, which involves blocking abnormal lymphatic vessels, is particularly beneficial in treating conditions like chylothorax, Kaposi form lymphangiomatosis (KLA), and other central lymphatic flow disorders. The following discussion delves into the clinical applications, methodologies, outcomes, and advancements in LDE for children, drawing from ten detailed studies.

Lymphatic disorders in children can present significant clinical challenges, often requiring innovative and multidisciplinary approaches to treatment. Among these disorders, chylothorax and Kaposi form lymphangiomatosis (KLA) are particularly notable due to their complexity and the severity of their symptoms. Chylothorax, characterized by the accumulation of lymphatic fluid in the pleural cavity, can be either congenital or acquired and often leads to respiratory distress. Traditional management of this condition includes dietary modifications and drainage, but these measures are not always effective. Lymphatic duct embolization (LDE) offers a minimally invasive alternative with promising results.

Kaposi form lymphangiomatosis (KLA) is a rare and aggressive lymphatic anomaly that typically presents with nonspecific symptoms such as respiratory distress, thrombocytopenia, and pleural effusions. The complexity of KLA often necessitates multimodal treatment approaches. For instance, an 11-year-old girl with KLA and recurrent chylous pericardial effusions, refractory to medical therapy and pericardial drainage, was successfully treated with thoracic duct embolization and sclerotherapy, resulting in significant clinical improvement and a prolonged symptom-free period.

The methodologies employed in LDE have seen significant advancements, particularly in imaging techniques. Dynamic Contrast-Enhanced MR Lymphangiography (DCMRL) and Intranodal Lymphangiography are two such techniques that have significantly improved the diagnosis and treatment planning for lymphatic disorders. These imaging techniques allow for detailed visualization of the lymphatic system, aiding in the precise targeting of abnormal vessels during embolization procedures.

The embolization procedure itself typically involves accessing the thoracic duct or other lymphatic vessels under ultrasound and fluoroscopic guidance. Various embolic agents can be used in the procedure, including coils, n-butyl cyanoacrylate glue, and lipiodol. For instance, in a study involving neonatal chylothorax, lipiodol embolization demonstrated high effectiveness with minimal adverse effects. The success rates of LDE in treating pediatric lymphatic disorders are promising. In one study, LDE achieved clinical success in 90% of patients with persistent chylothorax, highlighting its efficacy as a treatment option. Additionally, combining LDE with sclerotherapy has shown enhanced outcomes in cases of extensive lymphatic malformations.

The long-term prognosis for children undergoing LDE varies depending on the underlying condition and the extent of lymphatic involvement. For example, the aforementioned case of KLA treated with LDE, and sclerotherapy remained symptom-free for 15 months, suggesting that LDE can provide durable relief in some patients.

Recent advancements in therapeutic approaches have also contributed to the efficacy of LDE. Novel systemic therapies, such as mTOR inhibitors and MEK inhibitors, are being explored to complement LDE in treating lymphatic disorders. These therapies target the underlying molecular pathways involved in lymphatic anomalies, potentially improving outcomes when used in conjunction with embolization procedures.

The management of pediatric lymphatic disorders often requires a multidisciplinary approach, involving pediatricians, interventional radiologists, surgeons, and other specialists. Collaborative care ensures comprehensive treatment planning and follow-up, addressing both the immediate and long-term needs of the patient. This holistic approach is essential in managing the complex nature of lymphatic disorders in children.

The success rates of LDE in pediatric patients are particularly noteworthy. For instance, in a study of 88 patients with persistent chylothorax, LDE was clinically successful in 90% of the cases, underscoring its effectiveness. The procedure offers a valuable, minimally invasive treatment for a challenging and often morbid condition. Additionally, the combination of LDE with regional doxycycline sclerotherapy has shown enhanced outcomes, particularly in preventing recurrent chylopericardium by sclerosing the presumed nidus for lymphatic drainage.

The role of imaging in the success of LDE cannot be overstated. Techniques like Dynamic Contrast-Enhanced MR Lymphangiography (DCMRL) and Intranodal Lymphangiography have revolutionized the field by providing detailed images of the lymphatic system. These imaging modalities enable clinicians to pinpoint the exact location of lymphatic leaks and other abnormalities, facilitating targeted and effective treatment. For example, DCMRL involves the injection of gadolinium contrast into inguinal lymph nodes, allowing for clear visualization of lymphatic flow and potential obstructions.

Furthermore, the development of novel imaging techniques has paved the way for better classification and prognostication of lymphatic disorders. These advancements have led to a deeper understanding of the pathophysiological mechanisms underlying these conditions, thereby allowing for more precise and individualized treatment plans. For instance, isolated neonatal chylothorax, a condition characterized by respiratory distress due to pleural effusion, can now be effectively treated with oil-based contrast (lipiodol) embolization. This relatively simple and minimally invasive technique has transformed the treatment landscape for this condition, offering high success rates with minimal adverse events.

The integration of systemic therapies with LDE represents another significant advancement in the treatment of lymphatic disorders. mTOR inhibitors, such as sirolimus, have shown promising results in managing vascular and lymphatic anomalies. These inhibitors work by targeting the mammalian target of rapamycin (mTOR), a kinase involved in cell proliferation and angiogenesis. By inhibiting this pathway, mTOR inhibitors can reduce the volume of lymphatic tissue and alleviate symptoms. In a study involving patients with lymphatic anomalies, including KLA, sirolimus therapy resulted in significant clinical improvements and enhanced quality of life.

MEK inhibitors have also emerged as a potential therapeutic option for lymphatic disorders. These inhibitors target the MAPK/ERK pathway, which is involved in cell growth and differentiation. By inhibiting this pathway, MEK inhibitors can potentially reduce the progression of lymphatic anomalies and improve clinical outcomes. The integration of these systemic therapies with LDE offers a multifaceted approach to managing complex lymphatic disorders, addressing both the immediate symptoms and the underlying molecular mechanisms.

The importance of a multidisciplinary approach in managing pediatric lymphatic disorders cannot be overstated. The complexity of these conditions often requires the expertise of various specialists, including pediatricians, interventional radiologists, surgeons, and other healthcare professionals. Collaborative care ensures that all aspects of the patient?s condition are addressed, from diagnosis and treatment planning to follow-up and long-term management. This comprehensive approach is crucial in optimizing outcomes and improving the quality of life for children with lymphatic disorders.

In conclusion, lymphatic duct embolization represents a significant advancement in the treatment of pediatric lymphatic disorders. Its minimally invasive nature, combined with high success rates and the potential for durable outcomes, makes it a valuable option for conditions like chylothorax and Kaposi form lymphangiomatosis. The integration of novel imaging techniques and therapeutic agents continues to enhance the efficacy and safety of LDE, promising a brighter future for pediatric patients with complex lymphatic anomalies. Ongoing research and multidisciplinary care are essential to further optimize these interventions and improve the quality of life for affected children.

Cardiac Tamponade

Cardiac tamponade is a critical condition characterized by the accumulation of fluid in the pericardial space, leading to increased intrapericardial pressure and restricted cardiac function. This review examines cardiac tamponade in children, focusing on its etiology, clinical presentation, diagnosis, and management, based on a synthesis of multiple studies and case reports.

Cardiac tamponade is a medical emergency that can be fatal without prompt intervention. It involves the accumulation of fluid, blood, or gas in the pericardial sac, which compresses the heart and impairs its ability to pump blood effectively. While often associated with adult conditions, cardiac tamponade can also occur in children, stemming from various etiologies including infections, trauma, malignancies, and iatrogenic causes.

The causes of cardiac tamponade in children are diverse and can be broadly categorized into infectious, neoplastic, traumatic, and iatrogenic origins. Infections leading to cardiac tamponade often include bacterial, viral, and fungal pathogens. For instance, bacterial infections such as Staphylococcus aureus, Streptococcus pneumoniae, and Mycobacterium tuberculosis have been implicated in pediatric cases??.

Malignancies, particularly acute lymphoblastic leukemia, and other cancers are also significant contributors to pericardial effusion and subsequent tamponade in children. Traumatic causes, though less common, include blunt or penetrating chest injuries that result in hemopericardium. Iatrogenic causes are increasingly reported, especially related to central venous catheter placement, as evidenced by multiple case reports and studies.

The clinical presentation of cardiac tamponade in children can vary but typically includes symptoms such as dyspnea, tachycardia, and chest pain. Physical examination findings may reveal hypotension, jugular venous distension, distant heart sounds, and pulsus paradoxus (a decrease in systolic blood pressure during inspiration). In severe cases, signs of shock and hemodynamic instability are present??.

A specific case involving a 13-year-old girl with multisystem inflammatory syndrome in children (MIS-C) associated with COVID-19 presented with symptoms of myopericarditis and acute pericardial tamponade, highlighting the variability and complexity of clinical manifestations??.

Diagnosis of cardiac tamponade in children involves a combination of clinical assessment and imaging techniques. Echocardiography is the gold standard for diagnosing pericardial effusion and tamponade, as it can visualize the fluid accumulation and assess its impact on cardiac function. Other imaging modalities such as chest X-ray and CT scans can provide additional information but are secondary to echocardiography.

Electrocardiography (ECG) often shows low voltage QRS complexes and electrical alternans (variation in QRS complex amplitude), which are suggestive of significant pericardial effusion and tamponade.

Management of cardiac tamponade in children requires prompt intervention to relieve the pressure on the heart. The primary treatment is pericardiocentesis, which involves the insertion of a needle into the pericardial space to aspirate the excess fluid. This procedure can be performed under echocardiographic guidance to increase its safety and efficacy.

In cases where pericardiocentesis is not feasible or unsuccessful, surgical options such as pericardial window or pericardiectomy may be necessary. These procedures create a continuous drainage route for the pericardial fluid, preventing reaccumulation??.

Several case studies provide insights into the diverse presentations and management strategies for cardiac tamponade in children. For instance, a case involving a 7-year-old girl who developed tamponade following Hickman catheter insertion for bone marrow transplantation illustrates the risks associated with central venous catheter placement and the need for prompt surgical intervention??.

Another report described a 9-year-old boy and an 11-year-old girl presenting with acute cardiac tamponade as the first manifestation of systemic lupus erythematosus (SLE). These cases underscore the importance of considering autoimmune disorders in the differential diagnosis of pericardial effusion in children.

Cardiac tamponade in children is a life-threatening condition that requires immediate recognition and intervention. The etiology is varied, encompassing infections, malignancies, trauma, and iatrogenic causes. Clinical presentation can be subtle, necessitating a high index of suspicion and prompt use of diagnostic imaging. Management primarily involves pericardiocentesis, with surgical options reserved for refractory cases. Continuous monitoring and follow-up are essential to prevent recurrence and manage underlying conditions. Further research and case documentation are needed to enhance understanding and improve outcomes for pediatric patients with cardiac tamponade.

Isolated Fallopian Tube Torsion

Isolated Fallopian Tube Torsion (IFTT) is an exceptionally rare gynecological emergency, particularly affecting adolescent females. The condition involves the twisting of the fallopian tube without involvement of the ovary, leading to acute abdominal pain and, if not treated promptly, can result in severe complications such as ischemia, necrosis, infertility, or infection. The incidence of IFTT is estimated at approximately 1 in 1,500,000, highlighting its rarity and the diagnostic challenges associated with it due to nonspecific symptoms. This review synthesizes findings from eight detailed reports spanning several years, offering a comprehensive understanding of IFTT's clinical presentation, diagnostic hurdles, management strategies, and outcomes.

Patients with IFTT typically present with acute lower abdominal pain, often accompanied by nausea and vomiting. The pain may be intermittent or constant, and it can radiate to the thigh or groin, exacerbating the diagnostic challenge. The nonspecific nature of these symptoms often leads to a wide differential diagnosis, including appendicitis, ovarian torsion, ruptured ovarian cysts, urinary tract infections, and gastrointestinal disorders. This overlap necessitates a high degree of clinical suspicion and often extensive diagnostic workup to identify IFTT accurately.

Imaging plays a crucial role in the diagnostic process, although it is often inconclusive. Ultrasound is usually the first-line imaging modality used to evaluate acute abdominal and pelvic pain. Specific ultrasound findings suggestive of IFTT include a tubular structure with thick echogenic walls, internal debris, and a narrowed end, known as the beak sign. Doppler ultrasound can also reveal reduced or absent blood flow in the torted tube, although these signs are not definitive. MRI and CT scans can provide additional detail but are often employed when ultrasound findings are ambiguous. Ultimately, diagnostic laparoscopy is frequently necessary to confirm the diagnosis, as it allows direct visualization of the twisted fallopian tube and excludes other potential causes of the symptoms.

The management of IFTT primarily involves surgical intervention. The preferred approach is laparoscopy, which is minimally invasive and allows for both diagnosis and treatment. During surgery, the torted tube is detorted, and any associated pathology, such as hydrosalpinx or paratubal cysts, is addressed. Hydrosalpinx, a condition where the fallopian tube is filled with serous fluid, is a common finding associated with IFTT and complicates its management. The decision to preserve or remove the fallopian tube depends on its viability post-detorsion and the presence of any irreversible damage. If the tube appears viable after detorsion, conservative management with tube preservation is preferred to maintain fertility. However, if there are signs of necrosis or if the tube does not recover its normal appearance, salpingectomy (removal of the fallopian tube) is performed to prevent further complications.

The outcomes of IFTT management vary based on the timing of intervention and the condition of the fallopian tube at diagnosis. Early intervention generally results in better outcomes, with higher chances of tube preservation and subsequent fertility. Delayed diagnosis and treatment, on the other hand, often necessitate salpingectomy due to irreversible damage to the fallopian tube. Studies have shown that even when the fallopian tube appears ischemic, there can be significant recovery if detorsion is performed promptly. This underscores the importance of maintaining a high index of suspicion for IFTT in young females presenting with acute abdominal pain and pursuing early surgical evaluation.

The literature reveals that IFTT often occurs on the right side more frequently than the left. This could be due to the cushioning effect of the sigmoid colon on the left side, which potentially reduces the likelihood of torsion. Moreover, right-sided abdominal pain is more commonly investigated surgically due to the differential diagnosis that includes appendicitis, leading to more frequent identification of right-sided IFTT.

In pediatric populations, managing IFTT requires special consideration due to the implications for future fertility. Conservative surgical approaches are generally preferred to preserve the fallopian tube and maintain reproductive potential. However, in cases where hydrosalpinx is present, conservative management can be challenging. Hydrosalpinx increases the risk of recurrence of torsion, and patients often require multiple surgeries if the tube is initially preserved. Thus, a balance must be struck between preserving fertility and preventing recurrent torsion and associated complications.

Case studies and retrospective reviews have highlighted the varied presentations and management outcomes of IFTT. In a series of cases, patients ranged from 6 to 16 years old, with most presenting with acute right lower quadrant pain and some exhibiting additional symptoms like thigh pain or dysuria. Diagnostic imaging often revealed adnexal masses or hydrosalpinx, leading to surgical exploration. The intraoperative findings confirmed isolated fallopian tube torsion in all cases, with varied management strategies based on the viability of the fallopian tube and associated pathologies.

One notable case involved a 15-year-old girl who presented with four days of intermittent abdominal pain exacerbated by movement. Imaging revealed bilateral paratubal cysts, and diagnostic laparoscopy confirmed right IFTT. The right fallopian tube was detorted and preserved, and the paratubal cyst was enucleated. Follow-up showed no recurrence of symptoms, illustrating the potential for successful conservative management in select cases.

Another case involved an 11-year-old girl with acute right lower quadrant pain and emesis. Imaging suggested bilateral paraovarian cysts, and surgery revealed right fallopian tube torsion with ischemia. Despite detorsion, the tube was non-viable and was resected. This case underscores the need for prompt surgical intervention to assess and manage the viability of the fallopian tube.

In conclusion, Isolated Fallopian Tube Torsion is a rare but significant cause of acute abdominal pain in adolescent females, with important implications for fertility if not promptly diagnosed and treated. High clinical suspicion, appropriate imaging, and timely surgical intervention are critical to managing this condition effectively. While conservative management with tube preservation is ideal, especially in pediatric patients, the presence of associated conditions like hydrosalpinx may necessitate more aggressive surgical approaches to prevent recurrence and ensure patient well-being. Future research should focus on refining diagnostic criteria and management protocols to improve outcomes for patients with this rare condition.

PSU Volume 63 No 04 OCTOBER 2024

Laparoscopic Retroperitoneal Lymph Node Dissection

Laparoscopic Retroperitoneal Lymph Node Dissection (RPLND) is a crucial surgical technique primarily used in the management of testicular cancer and certain pediatric malignancies such as paratesticular rhabdomyosarcoma. Over the years, the technique has evolved significantly with advancements in minimally invasive surgery and the advent of robotic assistance, offering improved outcomes and reduced morbidity for patients. This review synthesizes information from multiple studies to provide a comprehensive understanding of the efficacy, safety, and advancements in laparoscopic and robot-assisted RPLND.

The earliest use of laparoscopic RPLND was reported in 1992, marking a significant shift from the traditional open surgical approach. The primary advantage of laparoscopic RPLND over open surgery lies in its minimally invasive nature, which translates to reduced postoperative pain, shorter hospital stays, and quicker recovery times. A study from 2010 highlighted the successful use of laparoscopic RPLND in high-risk pediatric patients with paratesticular rhabdomyosarcoma (PTRMS). The study reported that laparoscopic RPLND is a safe and effective diagnostic and therapeutic procedure for children over ten years of age with primary tumors larger than 5 cm. The average operative time was 382 minutes, with minimal blood loss and no significant postoperative complications, allowing for rapid commencement of adjuvant chemotherapy.

Robot-assisted RPLND (RA-RPLND) emerged as a significant advancement in the early 2000s, offering enhanced precision and visualization through robotic technology. The da Vinci Surgical System, for instance, has been instrumental in performing complex dissections with improved dexterity and control. A 2017 study documented the use of the da Vinci Xi system for RA-RPLND in adolescent patients. The study included two cases: a 17-year-old male with a mixed non-seminomatous germ cell tumor and a 15-year-old male with ectomesenchymoma. Both cases reported successful outcomes with no intraoperative complications and minimal blood loss. The robotic approach allowed for precise nerve-sparing dissections, preserving ejaculatory function in the patients.

Further supporting the efficacy of RA-RPLND, a 2012 study presented two adolescent cases involving paratesticular rhabdomyosarcoma (PT-RMS) and testicular germ cell tumor (T-GCT). The study emphasized that RA-RPLND is not only feasible but also provides excellent oncologic outcomes with low morbidity. The enhanced three-dimensional visualization and precise instrumentation afforded by robotic systems contribute to better surgical outcomes, including reduced risk of complications such as vascular and bowel injuries.

Despite the advantages, the learning curve associated with RA-RPLND is steep, requiring significant expertise in both laparoscopic and robotic surgery. A comprehensive review of robotic-assisted surgeries highlighted that proficiency in RA-RPLND could take up to 200 cases. However, the benefits, including reduced operative times and enhanced safety through robotic training modules, justify the initial learning curve. Additionally, the use of proctors and robotic training modules can further mitigate the risks associated with the learning curve, ensuring safer and more efficient surgeries.

Long-term oncologic outcomes of RA-RPLND have been encouraging. Studies have shown that RA-RPLND offers comparable, if not superior, results to traditional open and laparoscopic approaches in terms of disease-free survival rates. For instance, patients undergoing RA-RPLND for clinical stage I non-seminomatous germ cell tumors have reported high safety and early oncologic effectiveness, with minimal long-term complications. Moreover, the minimally invasive nature of the procedure reduces the likelihood of postoperative complications such as chylous ascites, ileus, and small bowel obstruction, which are more common in open surgeries.

The role of RA-RPLND in pediatric populations, although limited, has shown promising results. Pediatric patients, due to their smaller anatomical structures, present unique challenges in surgical management. The precision and enhanced visualization provided by robotic systems are particularly beneficial in this demographic, allowing for meticulous dissection and preservation of vital structures. Studies have reported that RA-RPLND in pediatric patients results in shorter hospital stays, quicker recovery times, and lower incidence of complications compared to open surgery.

However, despite its advantages, RA-RPLND is not without its limitations. The high cost of robotic systems and the need for extensive training are significant barriers to widespread adoption. Additionally, the lack of long-term data in pediatric populations necessitates further research to fully understand the long-term outcomes and potential late effects of the procedure. Ongoing studies and patient registries are crucial in addressing these gaps and providing more comprehensive data on the efficacy and safety of RA-RPLND.

In conclusion, laparoscopic and robot-assisted RPLND represent significant advancements in the surgical management of retroperitoneal lymph node dissections. The transition from open surgery to minimally invasive and robotic-assisted techniques has resulted in improved patient outcomes, reduced morbidity, and quicker recovery times. While the learning curve and high costs associated with robotic systems remain challenges, the benefits offered by RA-RPLND, particularly in terms of precision and safety, make it a valuable option in the surgical armamentarium. Continued research and long-term studies will further elucidate the role of RA-RPLND in both adult and pediatric populations, ensuring that patients receive the most effective and least invasive treatment options available.

Anaplastic Thyroid Cancer

Anaplastic Thyroid Cancer (ATC) is an exceedingly rare and aggressive form of thyroid malignancy. It accounts for less than 2% of all thyroid cancers but is responsible for a disproportionate number of thyroid cancer deaths due to its rapid progression and poor prognosis.

Anaplastic Thyroid Cancer predominantly affects older adults, typically presenting in the sixth to seventh decade of life. However, there are rare instances of ATC in younger populations, including children. According to a 2018 case report, ATC in children is extremely uncommon, with one of the youngest reported cases being a five-year-old child. Most thyroid malignancies in younger patients are well-differentiated papillary and follicular variants, with ATC being a rare progression from these types.

The clinical presentation of ATC often includes a rapidly enlarging neck mass, pain, dysphagia, and symptoms related to airway obstruction. The aggressive nature of the tumor leads to early metastasis, primarily to the lungs, bones, and brain. Diagnostic imaging such as CT and MRI are essential in evaluating the extent of local invasion and distant metastasis. A case reported in 2018 described a young child with a rapidly progressing thyroid mass that necessitated an emergency tracheostomy.

Histologically, ATC is characterized by a high degree of cellular atypia and the absence of the typical nuclear features seen in differentiated thyroid cancers. DICER1 mutations are increasingly recognized in poorly differentiated thyroid carcinoma, a precursor to ATC, particularly in younger patients?. Immunohistochemical staining plays a crucial role in the diagnosis, with markers such as EMA and cytokeratin being positive in ATC cells, while markers like TTF-1 and thyroglobulin are usually negative.

Molecular studies have identified several genetic mutations associated with ATC. The BRAFT1799A mutation is common, particularly in cases arising from previously differentiated thyroid cancers?. Other mutations frequently seen include TP53 and TERT promoter mutations. The identification of these mutations has implications for targeted therapy, although the aggressive nature of ATC often limits the effectiveness of such treatments.

The management of ATC is challenging due to its aggressive nature and poor response to conventional therapies. Surgery, when feasible, is the primary treatment modality. Total thyroidectomy is recommended to achieve local control, although the extensive local invasion often limits the completeness of surgical resection. Adjuvant therapies, including radiation and chemotherapy, have limited efficacy.

Recent advances in molecular biology have opened new avenues for targeted therapies. Inhibitors targeting specific genetic mutations, such as BRAF and MEK inhibitors, have shown promise in early studies. Immunotherapy, particularly checkpoint inhibitors, is also being investigated as a potential treatment option for ATC. However, the rarity of the disease poses significant challenges in conducting large-scale clinical trials to validate these approaches.

The prognosis for ATC remains poor, with a median survival of less than six months from diagnosis. Early diagnosis and aggressive treatment are critical to improving outcomes. Multimodal therapy, including surgery, radiation, and chemotherapy, may offer some benefit in locoregionally confined disease. Patients who received aggressive treatment had a median survival of 13 months, compared to less than six months for those who received less aggressive management.

In conclusion, Anaplastic Thyroid Cancer is a highly aggressive malignancy with a poor prognosis. The rarity of the disease, particularly in younger patients, underscores the need for increased awareness and early diagnosis. Advances in molecular genetics offer hope for targeted therapies, although their clinical utility remains to be fully established. Continued research and clinical trials are essential to develop more effective treatments and improve survival outcomes for patients with this devastating disease.

US for Necrotizing Enterocolitis

Ultrasound has increasingly become a vital tool in diagnosing and managing necrotizing enterocolitis (NEC) in neonates, particularly premature infants. NEC is a significant cause of morbidity and mortality in this vulnerable population, and early, accurate diagnosis is crucial for effective treatment. Traditional abdominal radiographs (KUB) have long been the standard for diagnosing NEC, but their limitations have led to the exploration of ultrasound as a complementary or alternative imaging modality.

One of the primary advantages of ultrasound over radiography is its ability to provide real-time, dynamic imaging of the bowel and surrounding structures. This capability allows for the assessment of bowel wall thickness, perfusion, and the presence of free fluid or air in the abdomen, which are critical indicators of NEC progression??. Moreover, ultrasound can detect pneumatosis intestinalis, portal venous gas, and pneumoperitoneum with higher sensitivity than radiographs, especially in the early stages of NEC?.

Radiographs, while useful, have significant limitations in sensitivity, often failing to detect early signs of NEC. Studies have shown that radiographs may miss up to 50% of early NEC cases, as their sensitivity ranges from 13% to 25% for detecting key features such as free air, which is identified in only 44% of perforated NEC cases?. This underlines the importance of more sensitive imaging techniques, such as ultrasound, which can identify subtle changes in bowel perfusion and wall thickness before they become apparent on radiographs?.

The technical aspects of performing abdominal ultrasound for NEC diagnosis are well-documented. Techniques such as gray-scale and Doppler ultrasound are employed to evaluate bowel wall integrity and blood flow, respectively?. For instance, intramural gas (pneumatosis) appears as echogenic dots within the bowel wall, while portal venous gas shows up as echogenic particles in the liver parenchyma?. These features are often detectable earlier and more clearly with ultrasound than with radiographs.

Several studies have highlighted the diagnostic and prognostic value of ultrasound in NEC. In a prospective study by Dordelmann et al., routine ultrasound screening of premature infants identified portal venous gas as a specific indicator of NEC, absent in other clinical conditions?. Similarly, a meta-analysis by Cuna et al. demonstrated that ultrasound features such as free air, absent peristalsis, and complex ascites were strongly associated with the need for surgical intervention or death?.

Despite its advantages, the use of ultrasound for NEC diagnosis is not without challenges. Overlying bowel gas can obscure underlying structures, making it difficult to obtain clear images. Additionally, the skill and experience of the sonographer and radiologist play a crucial role in accurately interpreting ultrasound findings?. There is also variability in the availability of ultrasound in neonatal intensive care units (NICUs), particularly outside regular hours, which can limit its use in urgent situations??.

The integration of ultrasound into NEC diagnosis protocols offers several benefits. For example, it can expedite the diagnosis, allowing for earlier intervention and potentially reducing the severity of the disease and its complications. Ultrasound also aids in stratifying patients who may fail medical management and require surgical intervention?. This stratification is crucial as the outcomes for surgically treated NEC are generally poorer compared to medically managed cases?.

Further research and quality improvement studies are needed to establish standardized protocols for the use of ultrasound in NEC. Advances in ultrasound technology, such as contrast-enhanced ultrasound, hold promise for even more accurate detection of bowel perfusion and other critical parameters?. Such advancements could significantly improve the sensitivity and specificity of ultrasound, making it an even more valuable tool in the fight against NEC.

In conclusion, while abdominal radiographs remain the standard imaging modality for NEC diagnosis, the addition of ultrasound can enhance diagnostic accuracy and patient outcomes. The real-time, detailed imaging capabilities of ultrasound provide critical insights into bowel health and disease progression, offering a more comprehensive approach to managing NEC. Continued research, training, and technological advancements are essential to fully integrate ultrasound into standard NEC diagnostic protocols, ultimately improving care for neonates affected by this severe condition.

PSU Volume 63 No 05 NOVEMBER 2024

Medihoney

Medihoney, a brand of medical-grade honey derived from the Leptospermum species (commonly known as Manuka honey), has gained significant attention in the field of wound care and surgical applications due to its potent antibacterial, anti-inflammatory, and wound healing properties. Over the years, extensive research has been conducted to explore the efficacy of Medihoney in treating various types of wounds, including chronic ulcers, burns, and post-surgical wounds. This essay delves into the scientific basis for the surgical use of Medihoney, its mechanisms of action, clinical applications, and its potential advantages over conventional treatments.

Medihoney's antibacterial activity is one of its most well-documented properties, making it particularly valuable in surgical settings where infection control is paramount. The honey is produced by bees that forage on the Leptospermum plant, which imparts unique phytochemical properties to the honey, enhancing its antimicrobial effectiveness. The antibacterial action of Medihoney is attributed to several factors. First, its high osmolarity creates a hyperosmolar environment that draws moisture out of bacterial cells, leading to their desiccation and death. Additionally, Medihoney's low pH, typically around 3.5, is sufficiently acidic to inhibit the growth of many pathogenic bacteria. Another significant factor is the production of hydrogen peroxide through the enzyme glucose oxidase, which contributes to Medihoney's broad-spectrum antibacterial activity. Finally, Methylglyoxal (MGO), a compound found in high concentrations in Manuka honey, has been shown to possess potent antibacterial properties, particularly against antibiotic-resistant strains such as MRSA. These properties make Medihoney an effective agent for preventing and treating infections in surgical wounds, particularly in cases where antibiotic resistance is a concern.

Beyond its antibacterial properties, Medihoney has several mechanisms that promote wound healing, which are particularly relevant in a surgical context. Medihoney facilitates the autolytic debridement of necrotic tissue through its osmotic effect, which draws lymphatic fluid into the wound, softening and helping to remove dead tissue without the need for surgical intervention. Furthermore, the high viscosity of Medihoney creates a moist wound environment conducive to the healing process by promoting the migration of epithelial cells and reducing scab formation. Medihoney also reduces inflammation by drawing out excess fluid from the wound, which helps to decrease swelling and pain?an essential benefit in post-operative wound care where controlling inflammation is crucial for proper healing. By reducing bacterial colonization, Medihoney also helps control wound odor, which can be distressing for patients and caregivers.

The application of Medihoney in surgical settings has been explored in various studies, demonstrating its efficacy in both preventing and treating wound infections, as well as in promoting faster healing. Post-operative infections are a significant concern in surgical practice, often leading to complications such as delayed healing, prolonged hospital stays, and in severe cases, sepsis. Medihoney has been used effectively in the management of surgical wounds, particularly in preventing infection and promoting healing in contaminated wounds. Studies have shown that when applied to surgical sites, Medihoney reduces the bacterial load and facilitates faster healing, often with less scarring compared to conventional treatments.

Chronic wounds, such as pressure ulcers and diabetic foot ulcers, present a significant challenge in clinical practice due to their resistance to conventional treatments and their propensity for infection. Medihoney has been particularly effective in these cases, as demonstrated by its use in patients with spinal cord injuries who developed chronic pressure ulcers. In one study, 90% of patients showed complete wound healing within four weeks of treatment with Medihoney, with no negative effects reported. This underscores its potential as a non-surgical therapy for chronic wounds, reducing the need for more invasive procedures.

Burns are another area where Medihoney has shown considerable promise. Burns often become infected, complicating healing and increasing the risk of scarring. Medihoney has been used to treat both superficial and deep burns, with studies showing that it helps to control infection, reduce pain, and promote faster healing. Its ability to maintain a moist wound environment is particularly beneficial in burn care, as it helps to prevent the formation of dry, hard scabs that can impede healing.

The use of Medihoney in preventing infections related to peritoneal dialysis is another important application in surgical care. A study published in The Lancet reported that Medihoney was effective in preventing catheter-related infections in patients undergoing peritoneal dialysis. The study highlighted that Medihoney did not induce bacterial resistance, a significant advantage over traditional antibiotics like mupirocin, which have been shown to lead to resistance even when used topically.

Medihoney offers several advantages over conventional treatments, particularly antibiotics, in the management of surgical wounds. One of the most significant concerns in modern medicine is the growing problem of antibiotic resistance. Medihoney, with its broad-spectrum antibacterial activity, offers an alternative that does not contribute to this issue. This makes it particularly valuable in the treatment of wounds infected with multi-drug-resistant organisms. Medihoney is a natural product with a long history of use in traditional medicine. Its safety profile is well-documented, with very few adverse effects reported in clinical studies, contrasting with the potential side effects and complications associated with synthetic antibiotics and antiseptics. In many cases, Medihoney can be a more cost-effective option compared to conventional treatments. Its ability to promote faster healing and reduce the need for surgical interventions can lower overall treatment costs. Many patients prefer treatments that are perceived as natural or less invasive. Medihoney fits this preference, which can improve patient compliance and overall satisfaction with treatment.

The use of Medihoney in surgical applications represents a significant advancement in wound care, combining ancient knowledge with modern medical practice. Its potent antibacterial properties, coupled with its ability to promote wound healing and reduce inflammation, make it an invaluable tool in the management of surgical and chronic wounds. As the threat of antibiotic resistance continues to grow, the role of Medihoney and other natural therapies in surgical care is likely to expand, offering clinicians effective alternatives to traditional treatments. Continued research and clinical trials will be essential in further defining the scope of its applications and optimizing its use in surgical practice.

Inguinal Hernia Repair in Preterm Infants

Inguinal hernia is a frequent condition in preterm infants, and deciding when to repair it is one of the most crucial aspects of management. Timing of repair involves balancing the risk of hernia-related complications, such as incarceration, with the risks associated with anesthesia in a population prone to respiratory and neurological complications. This updated review will focus extensively on the timing of repair, integrating the latest evidence, while also covering surgical approaches and anesthesia considerations.

The optimal timing for inguinal hernia repair in preterm infants is heavily debated among neonatologists, pediatric surgeons, and anesthetists. This is because the decision directly affects the risks of incarceration, which can lead to bowel necrosis, and anesthesia-related complications, particularly respiratory failure, and neurodevelopmental outcomes. In preterm infants, the incidence of inguinal hernia varies widely, with rates as high as 30% in some populations. The thinness of the hernia sac, the fragility of the abdominal wall, and the vulnerability of associated tissues make this group particularly susceptible to complications if the condition is left untreated.

One of the primary drivers of early hernia repair is the risk of incarceration. Studies have shown that the risk of incarceration in preterm infants can be significantly higher than in term infants, with estimates of up to 30% of preterm infants experiencing incarceration. Incarceration of the hernia can lead to bowel obstruction, ischemia, or testicular atrophy, making prompt repair necessary in many cases. Data from Vaos et al. demonstrated that delaying hernia repair beyond the first week after diagnosis dramatically increases the risk of incarceration by nearly fivefold?. Infants with incarcerated hernias not only face an emergency situation but also have poorer overall surgical outcomes and increased perioperative risks.

For this reason, many surgeons advocate for repairing the hernia as early as possible, often before the infant is discharged from the neonatal intensive care unit (NICU). This approach minimizes the risk of emergency surgery for incarcerated hernia, which is associated with higher morbidity.

However, early repair also comes with significant risks, particularly related to anesthesia. Preterm infants, especially those with bronchopulmonary dysplasia or other respiratory disorders, have an increased risk of perioperative complications. Studies have shown that infants undergoing surgery under general anesthesia are at a greater risk for postoperative apnea, respiratory failure, and the need for prolonged mechanical ventilation. This has led some experts to recommend delaying surgery until the infant is older and better able to tolerate the anesthetic, typically at a post-conceptional age of around 60 weeks.

Crankson et al. reported that delaying surgery until the infant reaches at least 47 weeks of post-conceptional age can reduce the incidence of postoperative apnea and other respiratory complications, making surgery safer for the infant. This delay allows time for the infant's respiratory system to mature, potentially decreasing the risks associated with anesthesia. Additionally, some studies suggest that waiting until the infant is older can reduce the likelihood of surgical complications, such as hernia recurrence, particularly in infants who required mechanical ventilation during their NICU stay.

Given the risks of both early and delayed repair, many experts advocate for an individualized approach based on the infant's overall clinical status, gestational age, and comorbidities. Some surgeons argue for a middle ground, performing surgery shortly before the infant is discharged from the NICU to avoid the risks of delaying surgery too long but still allowing time for some respiratory maturation.

A survey of members of the American Pediatric Surgery Association showed that 63% of surgeons would prefer to repair the hernia just before the infant's NICU discharge. In these cases, the hernia repair is delayed long enough to reduce anesthesia risks but is performed while the infant is still in the hospital, minimizing the need for emergency readmissions for incarcerated hernias.

A critical factor in deciding when to repair a hernia is the infant's post-conceptional age (PCA). Research consistently shows that infants with a PCA of less than 46 weeks are at a significantly higher risk for postoperative apnea and other respiratory complications. For this reason, some clinicians recommend delaying hernia repair until the infant reaches a PCA of at least 60 weeks.

However, this delay increases the risk of hernia incarceration, particularly as the infant begins to grow and move more, placing additional pressure on the abdominal wall. Data from Lautz et al. revealed that for every month hernia repair is delayed, the risk of incarceration increases by more than twofold. Thus, while waiting for the infant's PCA to reach a safer threshold for anesthesia is ideal, this delay must be carefully weighed against the increasing risk of incarceration.

The decision about when to repair an inguinal hernia also varies significantly based on institutional practices and available resources. Some centers have developed protocols for early repair, using spinal or caudal anesthesia to minimize the risks of general anesthesia, especially in infants with a high risk of respiratory complications. In centers where regional anesthesia expertise is available, early repair can be safely performed, minimizing the risk of incarceration while reducing the perioperative risks associated with general anesthesia.

Other institutions, particularly those without ready access to regional anesthesia techniques for infants, may opt to delay surgery and focus on close monitoring of the hernia, allowing the infant's respiratory system to mature before surgery. The lack of consensus on the optimal timing of surgery reflects the complexity of balancing these risks in a highly vulnerable population.

The choice of surgical technique can also influence the timing of repair. Open surgery is traditionally performed and remains the gold standard for many surgeons due to its well-established outcomes and lower risk of complications. Laparoscopic surgery, although less invasive, typically requires general anesthesia and is associated with an increased risk of pneumoperitoneum, which can exacerbate respiratory problems in preterm infants.

Some evidence suggests that laparoscopic surgery, while technically more demanding, may offer the advantage of inspecting both inguinal canals during the procedure, reducing the risk of missing a contralateral hernia. However, the risk of iatrogenic complications, such as injury to the spermatic cord, is higher in laparoscopic procedures. Consequently, the choice of surgical approach must consider the infant's overall condition and the surgeon's experience with both techniques.

Anesthesia is a major consideration in the timing of inguinal hernia repair. As previously mentioned, general anesthesia carries significant risks for preterm infants, particularly those with respiratory conditions. Regional anesthesia, including spinal and caudal blocks, has been shown to reduce these risks, leading some centers to prefer early repair using regional techniques.

However, regional anesthesia is not always feasible, particularly for more complex cases, such as bilateral hernias or when laparoscopic surgery is planned. In such cases, general anesthesia may still be necessary, and surgery is often delayed until the infant is older and better able to tolerate the anesthetic. For infants with a PCA of less than 46 weeks, some surgeons recommend preoperative caffeine administration and close postoperative monitoring to reduce the risk of apnea.

The timing of inguinal hernia repair in preterm infants is one of the most challenging decisions in neonatal surgery. Early repair reduces the risk of hernia incarceration but exposes the infant to the risks of anesthesia-related complications. Delaying surgery allows for respiratory maturation but increases the risk of hernia-related complications, including incarceration. An individualized approach, based on the infant's clinical status, PCA, and the availability of anesthesia techniques, is crucial for optimizing outcomes.

Central Venous Catheters in Pediatric Oncology

Central venous catheters (CVCs) are indispensable tools in the management of pediatric oncology patients, providing reliable venous access for the administration of chemotherapeutic agents, blood products, and parenteral nutrition. Despite their essential role, the use of CVCs in this vulnerable population presents significant risks, including infections, thrombosis, and mechanical complications. This review examines the latest findings on the safety, efficacy, and complications of CVCs in pediatric oncology patients, focusing on issues such as catheter-related bloodstream infections (CRBSIs), venous thromboembolism (VTE), and emerging management strategies.

Several types of CVCs are utilized in pediatric oncology, each with specific advantages and risks. The most common types include peripherally inserted central catheters (PICCs), non-tunneled CVCs, and tunneled catheters such as Hickman or Broviac lines. PICCs are often favored due to their ease of insertion and lower risk of mechanical complications compared to non-tunneled CVCs. PICCs can remain in situ for extended periods, which is advantageous for children undergoing long-term chemotherapy. A study analyzing 258 PICCs in pediatric oncology patients found a median catheter life of 102 days, highlighting their suitability for prolonged treatment.

However, the choice of catheter type depends on several factors, including the duration of use, the patient's clinical condition, and the risk of infection or thrombosis. In high-risk cases, such as stem cell transplantation or intensive chemotherapy, tunneled catheters may be preferred due to their lower risk of infection and ability to be in place for longer durations. Non-tunneled catheters, typically used in emergency settings, are associated with higher complication rates, and are usually replaced with tunneled catheters or PICCs once the patient is stabilized.

Catheter-related bloodstream infections (CRBSIs) are among the most frequent and severe complications associated with CVCs in pediatric oncology patients. Immunosuppression due to chemotherapy significantly increases the risk of infections, with bacteria such as Staphylococcus aureus, Staphylococcus epidermidis, and Enterobacterales being the most common culprits. The incidence of CRBSIs in pediatric oncology patients is reported to be 1.51 per 1,000 catheter-days, and infections can lead to serious outcomes, including sepsis, prolonged hospital stays, and even death.

Management of CRBSIs involves a combination of systemic antibiotic therapy and, in some cases, catheter removal. Recent studies have explored the possibility of catheter salvage using antibiotic lock therapy (ALT) and systemic antibiotics, especially for infections caused by organisms such as Enterobacterales. However, the success of catheter salvage remains variable, and in cases of persistent bacteremia or severe sepsis, immediate catheter removal is often recommended. A study conducted in pediatric oncology patients found that the cumulative incidence of ICU admission and death following CRBSIs was 16% and 5%, respectively, underscoring the severity of these infections.

Venous thromboembolism (VTE) is another significant complication associated with CVCs in pediatric oncology patients. CVCs disrupt normal blood flow and cause endothelial injury, increasing the risk of thrombosis. Studies have reported a high incidence of catheter-related thrombosis (CRT), with one study documenting a rate of 35.69 cases per 1,000 CVC-days. CRT can be asymptomatic in many cases, but symptomatic thrombosis, including pulmonary embolism, can occur and poses a life-threatening risk.

Several factors contribute to the development of CRT, including the duration of catheter placement, the method of insertion, and underlying conditions such as malignancy or infection. Prolonged catheter use has been identified as a major risk factor for CRT, with studies demonstrating that the risk of thrombosis increases with the number of catheters days. Interestingly, catheter malfunction and infection have also been associated with a higher risk of CRT, suggesting a possible link between infection, catheter occlusion, and thrombosis.

The management of CRT often involves anticoagulation therapy, although there are no specific guidelines for pediatric oncology patients. In cases of symptomatic thrombosis, catheter removal may be required, particularly if anticoagulation therapy is unsuccessful. Prophylactic anticoagulation is not routinely used in pediatric patients, and its role in preventing CRT remains controversial.

Mechanical complications such as catheter dislodgement, occlusion, and migration are also common in pediatric oncology patients. A study on PICCs in children found that catheter-related complications, including occlusion and breakage, occurred at a rate of 1.75 per 1,000 catheter-days. Catheter occlusion, often caused by fibrin sheath formation or thrombosis, can lead to treatment delays, and necessitate catheter replacement.

Catheter migration, although less common, can have serious consequences. For example, migration into the pleural space has been reported, leading to pleural effusion and respiratory distress. Proper catheter positioning, confirmed by imaging, and regular monitoring are essential to prevent these complications.

Given the high risk of complications associated with CVCs in pediatric oncology patients, several strategies have been proposed to minimize these risks. Infection prevention measures, including strict adherence to aseptic techniques during catheter insertion and maintenance, are critical. The use of antimicrobial-impregnated catheters and antibiotic lock solutions has shown promise in reducing the incidence of CRBSIs, although further studies are needed to confirm their efficacy in pediatric populations.

For thrombosis prevention, routine screening for CRT using ultrasonography has been recommended in high-risk patients, particularly those with prolonged catheter use or a history of thromboembolic events. Anticoagulation therapy, while not routinely used, may be considered in certain high-risk patients, although the benefits must be weighed against the risk of bleeding.

Mechanical complications can be minimized by ensuring proper catheter placement and securement techniques. Regular monitoring for signs of occlusion or migration is essential, and prompt intervention is required if complications arise. In some cases, replacing a malfunctioning catheter with a different type, such as switching from a non-tunneled CVC to a PICC, may be beneficial.

Central venous catheters are vital in the treatment of pediatric oncology patients, providing essential venous access for the administration of life-saving treatments. However, their use is fraught with risks, including infections, thrombosis, and mechanical complications. The management of these complications requires a multidisciplinary approach, involving infection control measures, anticoagulation therapy, and careful monitoring of catheter function. Advances in catheter technology, coupled with better prevention and management strategies, have the potential to improve outcomes for pediatric oncology patients requiring long-term venous access. Further research is needed to refine these strategies and develop evidence-based guidelines tailored to the unique needs of this vulnerable population.