PSU Volume 59 NO 01 JULY 2022

Trichobezoars

Bezoars are masses of solidified organic or non-biological material commonly found in the intestinal tract. Trichobezoar is a mass composed of hair that lodges in the proximal gastrointestinal tract, usually the stomach, causing symptoms of gastric outlet obstruction. Trichobezoars constitute 6% of all bezoars. Human hair is resistant to digestion as well as peristalsis due to its smooth surface accumulating between the mucosal folds of the stomach. With time continuous ingestion of hair leads to the impaction of hair with mucous and food causing the formation of a trichobezoar. Trichobezoars are almost exclusively seen in females between the ages of six and ten years, who have bizarre appetite, and emotional disturbances or mental retardation. Trichobezoars in young females with psychiatry comorbidity are usually the result of the urge to pull out hair (trichotillomania) and swallow it (trichophagia). Other psychiatry disorders such as mental disorders, abuse, pica, obsessive compulsive disorder, depression and anorexia nervosa may also be associated with trichobezoars. Many of the children also eat fabric fibers, paper napkins, plastic shopping bag pieces and cloth pieces. Trichobezoars produce multiple clinical manifestations such as: large firm movable epigastric mass, fullness, bloating, regurgitation, nausea, vomiting, epigastric pain, hematemesis, and tiredness. Originally the mass develops in the stomach and can move distally to the small bowel by fragmentation of a portion, extension, or total translocation. Children complain of early satiety and weight loss. This might reduce intake and develop failure to thrive and anemia. If left untreated the chronic obstruction might result in death from malnutrition, gastric mucosal erosion, ulceration, hemorrhage, or perforation. Due to significant size of the trichobezoar in most cases, the blood supply to the mucosa of the stomach and part of the affected bowel is reduced, which may cause ulceration and eventually perforation. In addition, intussusception, obstructive jaundice, protein-losing enteropathy, pancreatitis and even death has been reported as complications of trichobezoars.  Gastric bezoars with extension into the proximal small bowel or even colon is known as Rapunzel syndrome. The diagnosis is achieved using simple abdominal films or ultrasound and confirmed with CT scan or MRI. They appear as a well-defined intraluminal mass containing mottled gas. CT is superior as it not only identifies a heterogenous bezoar but can also define its extension distally. Direct visualization of the bezoar through upper GI endoscopy is the gold standard for imaging. The management of trichobezoar in the stomach or small intestine is associated with its size, and they can attain a significant size before causing symptoms. Trichobezoars can be managed with dissolution, suction, lavage, mechanical endoscopic fragmentation, or excision. Medical treatment and enzymatic degradation although attractive due to noninvasiveness have been reported as ineffective. Endoscopic removal if effective would be the best method. Unfortunately, due to the size, density and hardness of most of these gastric masses achieving endoscopic retrieval is effective in less than 5% of all cases. Removal of multiple fragments requires repeated introduction of the endoscope causing pressure ulceration, esophagitis end even perforation during retrieval. Hence endoscopy is only valuable as a diagnostic modality. The same occurs with laparoscopy which have been found inferior to laparotomy due to the size of these hair concretions that assume the form of the stomach. Longer operating time with laparoscopy and the risk of spilling contaminated hair fragments into the peritoneal cavity makes laparoscopy less attractive. Laparotomy is successful in most cases with almost 12% of children suffering from complications including wound infection, pneumonia, and paralytic ileus. Laparotomy with gastrostomy for manual removal is considered the treatment of choice for most trichobezoars. In case of a tail of the bezoar into the proximal bowel caution to avoid fragmentation should be exercise, otherwise a separate incision in the bowel might be needed for removal. Whenever a surgeon encounters a small or large bowel trichobezoar, they should look for a gastric trichobezoar. Beside trichobezoar removal, management should focus in preventing recurrence. Psychiatry follow-up is necessary to reduce the frequency of recurrence. The mortality is relatively low associated to complications and septicemia.

Vascular Access ESRD Children

The incidence and prevalence of end-stage renal disease (ESRD) in children have been increasing with time. Transplantation is the renal replacement therapy of choice for children with ESRD. Until kidney transplant is achieved, the child will rely in several methods of vascular or peritoneal access for ultrafiltration of blood. The most used modality of management while awaiting a kidney transplant is hemodialysis. There are three forms of vascular access available to provide hemodialysis: central venous catheter (CVC), arteriovenous fistula (AVF) and arteriovenous grafts (AVG) using prosthetic or biological material. The ideal vascular access delivers a flow rate adequate for the dialysis prescription, has a long use-life, and a low rate of complications (infection, stenosis, thrombosis, aneurysm, and limb ischemia). Most pediatric patients receiving hemodialysis have a CVC as primary access. CVC use is appropriate in those patients expected to receive a renal transplant within a short period of time and in exceptionally small children weighting less than 10 kg. The ideal vascular access is the arteriovenous fistula (AVF) due to its low complication rate and long-life span. The appropriate patient would be a child above 20 kgs in weight, in whom more than 12 months of hemodialysis is anticipated and has no cardiac dysfunction. The preferred sites for AVF placement include radial artery to cephalic vein, brachial artery to cephalic vein and brachial artery to basilic vein in the non-dominant forearm when a preferred minimum of 2.5 mm venous diameter is utilized. Potential benefits of AVF creation include a lower infection rate, lower thrombosis and stenosis rate, and greater freedom with regards to activities. Possible complications of AVF include stenosis, thrombosis, possible leg length discrepancy if placed in the lower extremity, and steal syndrome. The European Society of Pediatric Nephrology suggests that children requiring chronic hemodialysis start with a functioning AVF where appropriate, and reserve cuffed CVC for very small children or those requiring urgent unplanned hemodialysis. Place AVF in non-dominant arm at least three months before anticipated use, distally in the arm, and assessing maturation four to six weeks after AVF formation by clinical examination and duplex ultrasound. When waiting times for transplantation is significantly reduced, AVF formation rates significantly decreases. Arteriovenous grafts (AVG) are also an option for hemodialysis in children. They are placed in the forearm between the brachial artery and basilic or brachial vein. The PTFE graft is the most utilized due to fewest complication rates. Higher infection rates have been noted with thigh grafts than upper extremity grafts. Advantages of AVG include shorter time to first use, higher primary patency rate, and ease of technical creation. Access stenosis and infection rates are higher in AVGs than in AVFs, but episodes of thrombosis are similar. Disadvantages of AVG include thrombosis, stenosis, and infection. Infections are problematic as they may require graft removal. CVC are the most commonly used vascular access in children with ESRD. A CVC is first choice in children that require urgent hemodialysis or is near to receive a planned renal transplant. Two types of CVC: acute and chronic. Acute CVC are non-cuffed catheters utilized for immediate access and smaller in size. Chronic CVC are larger in size and contain a subcutaneous cuff for protection against infection. Disadvantages of using a CVC include short life span, thrombosis, infection, malfunction, and possible fibrin sheath formation. Median survival time of CVC for ESRD is between four and 10 months. In children with small vasculature and weighting less than 10 Kg, a CVC is the best temporary solution. This includes children with extremity contractures, bony deformities or other morbidity limiting condition to allow nursing personnel ready access. The order of placement of CVS should be sequential with this order of use: the right internal jugular vein, right external jugular vein, the left internal and external jugular veins, subclavian veins, femoral veins or translumbar access to the inferior vena cava. To achieve good flow the tip should be placed in the right atrium. Kinking is the most common reason for central line removal in uncuffed catheters, and second most common reason in cuffed catheters. The most common reason for cuffed CVC removal is infections with potential consequences of septic shock, subacute bacterial endocarditis, osteomyelitis, and epidural abscess. Chlorhexidine use for exit site care has the lowest rate of infection and bacterial colonization rate. Thrombus formation is another potential complication of long-term CVC use occurring in right atrial wall, vessel wall or completely occluding the vessel. Most CVC-related thromboembolism episodes remain asymptomatic and are associated with large catheters sized utilized, double lumen catheters, type of CVC, insertion site (subclavian vein is associated with a high risk of thrombosis), insertion technique, and patient related risk factors (prothrombotic state).  Formation of fibrin sheath around the catheter covers the intake and outflow holes leading to malfunction. CVC should be considered as a bridge to a more permanent, optimized, vascular access. The placement of peripheral inserted central catheters (PICC) should be avoided.  

ROTEM

Bleeding is a serious condition related to surgery, invasive procedures, childbirth, and trauma. Impaired hemostasis is a contributing factor to postoperative bleeding.  Rotational thromboelastography (ROTEM) is a rapid point of care viscoelastic test of hemostasis in whole blood which allows measurement of global clot development, stabilization, and dissolution in time. ROTEM reflects in vivo hemostasis assessing both thrombosis and fibrinolysis. Coagulopathy is a problem in pediatric surgical procedures, mostly of concern in cardiac, craniofacial oncologic operation and neonatal complex procedures. Other parameters that predict risk of coagulopathy are massive blood loss, prior coagulopathy, diffuse bleeding during surgery, sepsis, liver failure and prolonged operative time. Conventional hemostatic evaluation (platelets, PT, INR, PTT, and fibrinogen levels) has limitations directing blood component therapy. As ROTEM evaluates the whole process of coagulation from the beginning of clot formation to the need of fibrinolysis, the test promptly guides blood-component therapy in surgical patients. Patients with normal ROTEM have a low chance of having clinically significant coagulopathy. ROTEM provides information on platelets number/function and fibrinogen reserves. ROTEM provides test results within 10-15 minutes with very small samples appropriate for the neonatal surgery setting. Practical clinical uses of ROTEM include cardiac surgery (hemostatic resuscitation), trauma, obstetric (postpartum hemorrhage), liver transplantation (coagulopathy of cirrhosis, intraoperative replacement of large blood losses, changing metabolism of coagulant factors), hemophilia and burns. In adult and pediatric trauma ROTEM examines the diagnosis of coagulopathies, including hypocoagulation, hypercoagulation, platelet dysfunction and fibrinolysis. ROTEM can also be used to direct blood and blood-product transfusion. In trauma patients ROTEM can be used to diagnose coagulopathy, predict, and guide transfusion therapy and reduce unnecessary exposure to allogeneic blood products. TEG and ROTEM have several advantages to routine coagulation tests. They are easy to use, produce rapid graphical and numerical results of the hemostatic status, can detect the anticoagulant effect of acidosis, hypo-, or hyperthermia, and are able to detect and quantify the underlying cause of coagulopathy, such as thrombocytopenia, factor deficiency, heparin effect, hypofibrinogenemia and hyperfibrinolysis. Treatment for such identified disorders may include transfusion of blood products (FFP< platelets and cryoprecipitate), or specified drugs and the effect can be evaluated in vitro. ROTEM improved mortality in elective cardiac surgery, excision of burn wounds and liver transplantation, along with reduction in the need for RBC, FFP and platelet transfusion and combined treatment. TEG and ROTEM have the potential to reduce mortality, receiving unneeded transfusions and development of dialysis-dependent renal failure. ROTEM used showed a reduction in the time to the first transfusion of blood products (RBC) and reduced total number of a hospital stay. Acquired coagulopathy for pediatric trauma patients is predominantly characterized by poor fibrinogen polymerization, poor clot firmness, followed by hyperfibrinolysis and prolonged initiation of coagulation. ROTEM-guided algorithms implement the concept of personalized or precision medicine in perioperative bleeding management. Using ROTEM-guided algorithms in bleeding patients results in improved patient safety and outcome including perioperative morbidity and mortality. The use of visual assessment of bleeding using a bleeding score does not correlate well with ROTEM measurements to diagnose coagulopathy.  

PSU Volume 59 NO 02 AUGUST 2022

Perianal Crohn's Disease

Crohn's disease is a transmural bowel inflammatory disease affecting any age, involving all segments of the intestine including extraintestinal sites. Almost one-fourth of Crohn's cases occur in children younger than 18 years of age. Perianal Crohn's disease (PCD) accounts for 10-40% of children with Crohn's disease. PCD has a wide variety of clinical manifestations such as skin lesions, anal canal lesions, anoperineal abscesses or fistula, anovaginal fistulas and cancer (adenocarcinoma and squamous cell carcinoma). Skin lesions can be further subdivided into tags, hypertrophic papilla, or hemorrhoids, while the canal lesions include fissures, ulcers, or stricture/stenosis. Fissures and skin tags are the most common presentation of pediatric PCD. The symptoms of PCD include pain, itching, bleeding, purulent discharge, and incontinence of stools. Initial inflammation in the affected rectum forms ulcers or shallow fistulas, they subsequently extend into the deep or penetrating fistulas with persistent exposure to feces and pressure caused by defecation; infected anal glands penetrate the intrasphincteric space and progress to form fistulas or abscess. Manifestations of PCD have three categories: 1) tissue destruction (anal fissures, tags, deep ulcers lined with granulation tissue), 2) fistulas and abscess (multiple, complex, frequently related), 3) rectal stricture due to long standing disease. Spectrum of PCD can be mild or severe leading to constipation, fecal incontinence, recurrent infections, sepsis, compromise sexual function, diminished quality of life, which could end in diverting colostomy or ileostomy. PCD is usually painless and is diagnosed by physical exam. Endoanal US and MRI can further aid in diagnosis, rectoanal involvement, involvement of other adjacent structures and management. Most useful radiological study to evaluate abscess and fistulas is MRI. Evaluation of PCD starts with external/rectal examination followed by MRI, and exam under anesthesia performed by experienced surgeon. Presence and extent of intestinal disease can be further determined using a variety of endoscopic, anoscopy, ultrasound, and imaging tests (CT, MRI, fistulography). Skin tags do not resolve completely with treatment but remain present and benign. Fissures will often heal completely with minor nitrate-based therapy. Is clinically relevant to use Bell's classification of fistulas into simple and complex. Simple fistulas involve a low intersphincteric or trans-sphincteric location, single short tract, internal opening lower and closer to anal verge with the external opening near the anal verge without abscess. Complex fistulas involve sphincter muscle/anorectal ring, are multiple, branch with or without abscess, with internal opening of the fistulous tract above sphincter muscles and external opening further away from anal opening.  Simple fistulizing disease if superficial and confined to the anal canal healing spontaneously in 50% of cases. Rectovaginal and complex fistulas (high intersphincteric, high trans-sphincteric, extrasphincteric or suprasphincteric) rarely heal without therapy. Rectal strictures predict poor outcome, diagnosis is often delayed, most are in the distal rectum associated with proctitis, with most children responding to anal dilatation, preferably under anesthesia. Tight obstructive stricture should be considered for ostomy diversion. Medical management of PCD include local measures such as warm sitz baths, bowel regulation with fiber products, or antidiarrheal medication. Steroids should not be used during active disease. Antibiotics, immunomodulator and biologic agents are very beneficial to these patients. Antibiotics are first line therapy reducing fistula associated pain and drainage include metronidazole or ciprofloxacin, though symptoms usually recur immediately after antibiotic discontinuation. Immunomodulation using azathioprine or 6-mercaptopurine is effective, but slow and incomplete; leucocyte count and liver transaminase levels should be monitored. Biologic agents are the mainstay therapy for complex PCD. Biologics refer to anti-tumor necrosis factor antibodies which are potent anti-inflammatory agents such as infliximab and adalimumab. Anti-TNF biologic therapy using adalimumab without surgery is effective therapy for healing fistula in children with moderate to severe PCD in approximately half of the patients. The success of surgery management during acute tissue inflammation is limited, and fraught with complications. Limitation includes draining abscess and relieving acute symptoms. Perineal abscess cavities should be drained. Drain both internally and externally, keep communication between then with silk or silastic. Seton placement, which is reserved for the management of complex fistulas, can be left in place indefinitely, they usually deteriorate and fall in around one year, cause no harm and is well tolerated. Once inflammation subsides considerably, elective surgery to deal with fistula and stricture should followed. Combined surgical and biologic therapy produce better healing of perineal disease, longer effect duration and lower recurrence rate compared with either treatment alone. Stubborn fistulas that persist after medical therapy can be managed with fibrin plug or fibrin glue. Severe PCD can be managed with colostomy or ileostomy with a high risk of becoming permanents. With minimal colitis a sigmoid diversion is performed, with colonic involvement an ileostomy is utilized.

3D Printing

3D printing (3DP), also known as additive manufacturing, is the construction of a three-dimensional object from either a computer-aided designed or a digital 3D model. The final product is deposited, joined or solidified under computer control with materials being added together layer by layer to create a synthetic part. The 3DP process typically consists of depositing material layer-by-layer in a semiliquid, liquid or powder form and solidifying using light energy, an electron beam, chemical binders, or heat and allowing to solidify at room temperature. 3DP modeling can process a wide array of materials such as metals, polymers, or even ceramics into the desired shape. The technical pathway to create a physical 3D anatomical model starts with image acquisition, postprocessing segmentation, preprint preparation and the printing process itself. The key advantage of 3D printing in medicine is the ability to produce very complex shapes or geometrics that would be otherwise almost impossible to construct by hand including hollow parts or parts with internal truss structures such as body cavities. 3D printing technology can model a specific patient anatomy based on CT/MRI imaging using specific software for such purposes. The created model can be a complex congenital malformation in any part of the body. Three-dimensional visualization system provides surgeons with more in-depth knowledge of the topographic anatomy. This construction provides the potential for teaching and training after successfully producing accurate models of complex or rare anatomy.3DP technology is rapidly growing in many surgical fields such as maxillofacial reconstruction, craniofacial, ENT, urology, orthopedics, cardiac, cancer, hepatobiliary, plastic, and pediatric surgery. The 3D printed models created are highly fidelity to the original organs and capable of demonstrating spatial relationship of target area of organs involved in disease or malformations.3D printing can help better understand crucial surgical anatomy of solid organs not easily accessible through conventional imaging. Vivid demonstration of spatial relationship of three-dimensional objects is of great value in understanding complex surgical anatomy and subsequent operation planning. The use of 3D printing technology enables the creation of simulation models for teaching and practicing purposes such as learning to do laparoscopic or thoracoscopic procedures such as pyloromyotomy and repair of esophageal atresia respectively. Congenital heart diseases such as atrial septal defects, ventricular septal defects, Fallot tetralogy and transposition of the great vessels have benefited from 3DP modeling technology. Interventional cardiology has successfully used 3DP model to stimulate surgical stent placement and assessment of flow. In neurosurgery, surgeons use the 3DP models for comprehending the spatial relationship of brain lesions to the neural pathways, blood vessels, and functional anatomy. Orthopedic application of 3DP with MRI data is mainly used in resection planning for oncological tumors such as osteosarcomas and osteochondromas. Surgeons have reported more precise resections of bone tumors, with less blood loss, shorter operative time, and reduced radiation exposure during surgery. Urological application of 3DP includes kidney tumors resection planning and increase ability to plan for nephron-sparing procedures. Comparison between 3DP models of normal and abnormal anatomy is utilized to educate trainees and preoperative counseling of patient. Hence 3DP clinical applications include teaching, developing a diagnosis or plan (intervention and simulation), procedure performance using the models, and materials (shaping devices to mold growing anatomy or substitution prosthetics). Costs are still high.          

Sea Urchin Injuries

Saltwater aquatic animals can cause significant injury and morbidity to humans, specially to children. Aquatic ecosystems are usually visited for commercial (fishing) and recreational (bathing and aquatic sports) purposes. Trauma and envenomation can be caused by sea urchin spines. Sea urchins are invertebrates and member of the echinoderm's family with radial symmetry. There are over 700 known species of sea urchins with approximately 80 species toxic to humans. As slow moving non-aggressive and bottom dwellers sea animals they are found in deep water, rocky inclines, and coral reefs. Spines of calcium carbonate and pedicellaria are attached to the tegument. The spines can be hollow or solid with toxins associated with them or their thin epithelial layer. The spines can be short and thick, or long and fine penetrating skin through footwear or bathing suit. The toxin substance identified in the pedicellaria, or spines include steroids, serotonin, glycosides, cholinergic substances, histamine, and bradykinin. Primary injury of sea urchin is through spine penetration with the most common affected areas being feet (80%), ankles and hands (15%). Diagnosis is usually based on clinical findings and history. Injuries by urchin spines are usually seen in divers, snorkelers, bathers, and fishermen. Reactions related to sea urchin can be classified as immediate and delayed. Spine penetration causes immediate intense pain, bleeding, erythema, edema, and local myalgia. Should a joint be penetrated synovitis may occur. With more than fifteen spines injury systemic symptoms can occur such as paresthesia, radiated pain, hypotension, muscular weakness, dyspnea, aphonia, deafness and even death. Spine fragment retention can cause further infection, granulomas and foreign body reaction with pain and edema lasting for weeks with vesicular reaction due to delayed hypersensitivity. Sea urchin granulomas are believed to be delayed reaction as a result of the spine remnant in the wound. The granulomas develop due to foreign body reaction against inorganic substances in the spine. Clinically it manifests as a firm, painless, nonsuppurative nodule with a central depression and hyperkeratotic surface, especially if localized in the hand. They are pathologically described as sarcoid type. Tenosynovitis can occur if joints or tendons sheaths are penetrated by the spine. Impairment of joint function and severe destructive effects with functional loss can result when the inflammation takes the form of granulomatous synovitis or chronic chemical synovitis. The spine fragments may or may not be seen in simple x-ray films. Ultrasound or MRI imaging can be helpful in doubtful cases. Treatment of sea urchin spine injury in most of the cases is conservative. The management of sea urchin injuries is immediate immersion of the affected area in warm water (110-115 F) for 30-90 minutes. This makes the toxin and proteinaceous irritants inactive relieving pain. Some sources recommend mixing water with vinegar in a 1:1 ratio. Spines and pedicellaria should be removed by irrigation with water and soft traction. Observation and supportive treatment for allergic reactions, muscular paralysis and respiratory distress is recommended with more than 15 spine injuries at a time. Surgical exploration is indicated when fragments are affecting joints, tendons, or neurovascular structures to prevent delayed complications. Local or systemic antibiotics is recommended for secondary infections. Difficult to extract spines can be destroy using erbium:YAG laser ablation. Laser utilization is preferred in areas such as the heel of the foot.             

PSU Volume 59 NO 03 SEPTEMBER 2022

Duplex Kidneys

Duplex kidney also known as duplex collecting system is the most frequent congenital anomaly of the urinary tract carrying an incidence of approximately 1%. The diagnosis is defined as a renal unit comprised of two pelvicalyceal system. Duplex kidneys are further classified as either complete or partial. The complete variety consist of two separate pelvicalyceal system arising from two ureteral buds from the mesonephric duct. The two ureters fuse separately to the mesenchyme and leads to independent draining sections of the kidney described as upper or lower moiety ureters. The upper moiety ureter tends to insert more caudally and medial into the bladder than the normally inserting lower moiety ureter (Meyer-Weigert rule). The lower moiety ureter is often affected by vesicoureteral reflux (VUR) In the partial variety there occurs a range of incomplete duplications within a kidney and ureter leading to bifid moieties and ureter, but a single ureteric insertion distally. Duplex kidneys are found to be two times more common in females and bilateral in less than 20% of children. Most duplex abnormalities are asymptomatic carrying no clinical significance. A minority of duplex kidneys can be associated with main clinical manifestations such as urinary tract infection, urinary incontinence, dysuria, and the presence of an abdominal mass. Some of the most frequent complications are associated with ectopic insertion of the upper moiety ureter in the complete duplex kidney, often occurring with an ureterocele. In females this can cause incontinence since the insertion often occurs below the urethral sphincter. In males, fluid slowly drain into an accessory sexual structure producing a pelvic cystic mass. Another complication of duplex kidneys is VUR of the lower moiety caused by lateral displacement of the ureters insertion into the bladder predisposing the patient to recurrent urinary tract infections and renal scarring with permanent functional impairment of the kidney. VUR is regarded as the most common abnormality associated with complete ureteral duplication. VUR which occurs in 30% of cases is much more likely to occur into the lower pole ureter because it inserts laterally into the bladder and has a shortened intramural segment. Still, other complications of duplex kidneys include a multicystic dysplastic moiety and pelvic-ureteric junction (PUJ) obstruction. PUJ obstruction occurs most commonly in the lower moiety. The complication that occurs in duplex kidneys is specific to the moiety involved. Upper moiety complications are the most common being ectopic ureteric insertion, with or without ureterocele and multicystic dysplastic moiety. Lower moiety complications are VUR, renal scarring and PUJ obstruction. Any ureter that does not insert onto the trigone may be considered ectopic. If the ectopic ureter is located in the proximal female urethra, the result is usually obstruction, reflux or both presenting with urinary tract infection. If the location of the ectopic ureter is distal to the external sphincter, the child presents with continuous incontinence despite a normal voiding pattern. An ectopic ureter can also insert into the vestibule, vagina, or uterus. An ectopic ureter in male inserts most commonly into the prostatic urethra, though may insert into the ejaculatory duct, seminal vesicles, or vas deferens presenting with urinary tract infection or epididymitis. The first imaging modality to diagnose a duplex kidney is abdominal ultrasound whether prenatally or postnatally. Further imaging needed to characterize the anomaly includes nuclear medicines studies (DMSA), voiding cystourethrograms, CT-scan, MRI and cystoscopy. A MAG3 renogram may confirm the diagnosis of a duplex kidney. VUR is the most common anomaly associated with renal duplex systems and may occur in both moieties but is more frequent in the lower moiety. CT angiography can be performed to offer detailed information on renal vessels in case a partial nephrectomy is needed. The goals of management of duplex kidneys is the preservation of functional renal parenchyma, elimination of infection, obstruction and VUR with maintenance of urinary continence. Reflux is managed with antibiotics and frequent radiological follow-up with spontaneous resolution in 50% of children. Recurrent infection or renal scarring are indications for surgery. In patients with ectopic ureter treatment depends on function of the upper pole segment. With poor or absent function upper pole heminephrectomy and ureterectomy is indicated. Partial or heminephrectomy in a duplex kidney is a procedure performed for the complicated clinical course of the disease. The most common indications for surgery in children are recurrent urinary tract infections, ureterocele with hypo- or a functioning moiety and ectopic ureter causing incontinence in girls. An upper urinary tract approach (heminephroureterectomy) is described for a poorly functioning upper moiety or an ectopic upper moiety ureter. A lower approach (bladder reconstructive surgery) with excision of ureterocele and ureteric re-implantation has been described for children with good lower moiety function and high grade VUR. Ureterocele management has tended toward transurethral endoscopic incision. A more complete ureterocele excision is necessary following transurethral incision or if an ureterocele is very large or ectopic causing bladder outflow obstruction.         

Congenital Cystic Lung Lesions

A lung cyst is a cystic parenchymal airspace measuring one centimeter or more in diameter with a well-defined thin wall usually less than 2 to 4 mm thick. The term cavity is used for air-containing lesion with a relatively thick wall greater than 4 mm. Cystic lung lesions may contain either air or fluid or a combination of both, often with an air-fluid level. Most children with congenital cystic lung (CCL) lesions do not present complications such as respiratory failure, pneumonia, or lung abscess, immediately after birth or for the first few months of life. Indications for lung resection in patients with CCL lesions should be determined by the risk of developing infectious complications, respiratory distress, pneumothorax, hemorrhage, or malignant transformation. The cystic congenital lung lesions encountered in children include bronchogenic cyst, congenital pulmonary airway malformation (CPAM), congenital lobar emphysema, bronchial atresia, and pulmonary sequestration. Bronchial atresia and congenital lobar emphysema are not truly cystic lesions but have a focal overinflated lung that mimics cystic lung changes. Most CCL lesions are thought to be caused by in utero airway obstruction, often with an aberrant, stenotic, or atretic supplying airway. Most CCL lesions are initially discovered by prenatal US which can be further evaluated by prenatal MRI. CCL lesions are largest in size in the second trimester and tend to decrease in size or remain stable in the third trimester. Large or rapidly expanding lesions may cause hydrops fetalis and demise related to central venous compression by the lesion. When imaging is performed soon after birth, CCL lesions can be fluid filled, dense opacities that mimic a solid mass and there is delayed clearance of fetal lung fluid and replacement with air due to an obstructed or abnormal airway connection. Postnatally, CT angiography should be used to further characterized CCL lesions including any infradiaphragmatic feeding artery, as seen in pulmonary sequestration. Differential diagnosis for lesions discovered postnatally include diaphragmatic hernia, infection, lymphatic, vascular or neoplastic cystic lesions. Bronchogenic cysts arise due to abnormal budding of the foregut at 26-40 days gestation, content is serous fluid to thick mucus or blood. Imaging reveals mediastinal (most common) or less commonly intrapulmonary mass with smooth or lobulated borders and smooth enhancing wall. Air trapping or atelectasis distal to the lesion with airway compression can result in recurrent pneumonia. The most frequent symptoms is pain, cough, fever, respiratory distress or dyspnea. They are found in the mediastinum or pulmonary parenchyma, neck, pericardium, pleura, diaphragm, or abdominal cavity. Intrapulmonary cysts are 20% of all bronchogenic cyst occurring in the lower lobes. CPAM accounts for 25% cases of lung cyst and consist of a group of macrocystic or microcystic pulmonary malformations due to early lung maldevelopment. They are divided into large-cyst type 1 (2 to 10 cm cysts), small-cyst type 2 (cysts 5mm to 2cm), and microcystic or solid type 3 (cysts < 5 mm). CPAM occurs equally in either lung, usually unilobar, very rarely multilobar or bilateral. Congenital lobar emphysema (CLE) is characterized by over-distension and air-trapping in the affected lobe, concomitant compression of the remaining lung tissue, and displacement of the mediastinum by herniation of the emphysematous lobe across the anterior mediastinum into the opposite side of the chest. Deficiency of the cartilage wall of the affected bronchi suggest a developmental cause. Hyperlucency of the affected lobe and herniation to the contralateral side are the most common radiographic findings. Pulmonary sequestration is defined as lung tissue with no normal connection to the tracheobronchial tree associated with aberrant systemic arterial blood supply from the lower thoracic or lower abdominal aorta reaching the lung via the inferior pulmonary ligament. Is divided into intralobar or extralobar types. Venous drainage is via the pulmonary veins in the intralobar and the systemic veins in the extralobar sequestration. Extralobar sequestration accounts for 25% cases of pulmonary sequestration, left lower lobe involvement is more common, has its own pleural layer and may occur in ectopic locations including mediastinum, pericardium and upper abdomen, almost invariably left suprarenal, and is more often associated with other malformations such as diaphragmatic hernia or scimitar syndrome. Intralobar sequestration has the highest risk for infection and is an indication for removal. Congenital bronchial atresia results from local obliteration or stenosis of a segmental, subsegmental or lobar bronchus at or near its origin. Usually involve the left lobe and segment bronchi of right lobe, middle lobe, and occasionally lower lobe. Imaging features include a hilar mass and overinflation of the peripheral lung often associated with an opaque round mucocele in the bronchial tree just distal to the obstruction. Cystic lung neoplasms include lymphangioma, histiocytosis and pleuro-pulmonary blastoma. Pleuropulmonary blastoma has three pathological features: type 1 is entirely cystic lesion, type 2 is characterized by cystic lesion and solid mass, while type 3 is a purely solid high-grade sarcoma. Less than 25% of children with CCL lesion develop symptoms early in life defined as respiratory compromise (stridor, excessive crying, coughing, failure to thrive, need of supplemental oxygen) or infection. Recurrent chest infection occurs in one-third of all patients. Symptomatic neonates require urgent surgery after diagnostic imaging and cardiac assessment. Symptoms of respiratory distress or episodic infection of the abnormal lung will need lung resection. Late symptoms can occur any time after life. Asymptomatic children with CCL lesions are managed expectantly and should undergo lung resection after six months of age as the surgery is less invasive, safer, and more beneficial. Most small asymptomatic lung lesions present sometime during life with intractable infective complications or pneumonia. In stratifying lesions from higher to lower risk of infection and symptoms, CPAM and bronchogenic cysts are categorized as medium to higher risk, while bronchial atresia, microcystic CPAM and extralobar sequestration are considered lower risk. Bilateral lesions signify a higher risk for malignancy and further workup is required.

Circumcision

PSU Volume 59 NO 04 OCTOBER 2022

Pharyngeal Arches, Cleft and Pouches

The head and neck develop during the 4-5th intrauterine week. The connective tissue growth of mesenchymal tissue in the cranial region of the fetus results in the formation of arches separated by cleft. At the same time out pocketing on the lateral wall of the pharynx known as the pharyngeal pouches develops. They separate the arches on the endodermal (internal) surface while cleft separate the arches on the ectodermal (external) surface. The term branchial arches are also known as pharyngeal arches since it more accurately describes human anatomy. Each branchial arch is lined externally by an ectoderm-lined recess, referred as a pharyngeal/branchial cleft and internally by a layer of endoderm, referred to as pharyngeal/branchial pouch. Initially there are four pharyngeal clefts, but only the 1st cleft gives rise to a permanent structure, the external auditory meatus, the space created by the 2nd, 3rd and 4th pharyngeal cleft give rise to the cervical sinus. The fetus develops six pharyngeal arches. The 5th regress immediately. Each arch has own innervation, a muscular component, cartilaginous or skeletal support and a vascular component. The branchial (or pharyngeal) arches represent the embryological precursor of the face, neck, and pharynx. Refer to Table 1 regarding the component's distribution of the arches.

Pharyngeal pouches are endodermal out-pockets occurring between the pharyngeal arches. The pouches give rise to several tissues responsible for the formation of permanent structures. Of six pairs of pouches, only four give rises to a structure since the 5th and 6th pouch blends within the 4th pharyngeal pouch. See Table 2 regarding the structure associated with each pharyngeal pouch. Pharyngeal pouches produce tissue necessary for hearing, calcium homeostasis and adequate immune response.

Failure of branchial arches to properly develop may result in a congenital malformation. Anomalies of the branchial arches are the second most common congenital lesions of the head and neck in children. Thyroglossal duct cyst is the most common congenital lesion of the neck in children. Pharyngeal arch anomalies may present as cysts, sinus tracts, fistula, or cartilaginous remnants and present with typical clinical and radiological patterns dependent on which arch is involved. Most branchial cleft malformations in children involve the 1st and 2nd arches. Second branchial arch anomalies are the most common, accounts for 95% of cases and most commonly present as cysts. 1st branchial anomalies occur 4% of cases and 3rd and 4th branchial arch anomalies are very rare. Branchial cysts present in older children/young adults, while fistulas present in infants/young children. Surgical management involves complete surgical excision encompassing the external sinus opening with dissection of the sinus tract. First branchial anomalies present as cystic masses adjacent to the external auditory canal and submandibular area, are often misdiagnosed, and often managed inadequately. Since they have propensity for infection, surgical excision is definitive treatment. There is potential risk to the facial nerve during resection. Third branchial cleft anomalies are associated to the superior laryngeal nerve, arise from the rostral end of the pyriform fossa, usually present as fistula/sinus tract in the posterior cervical space, posterior to the sternocleidomastoid muscle (posterior triangle of the neck). Fourth branchial cleft fistula/sinus tracts arise from the pyriform sinus apex, are left-sided, and most often present as sinus tract coursing from the apex of the pyriform fossa to the upper aspect of the left thyroid lobe. They are a cause of purulent thyroiditis. Cleft lip and palate, auricular atresia and micrognathia are examples of 1st branchial arch malformation. A branchial cleft cyst is an example of a 2nd branchial arch malformation.

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References:
1- Waldhausen JH: Branchial cleft and arch anomalies in children. Semin Pediatr Surg. 15(2):64-9, 2006
2- Miles B, Srinivasan VN: Embryology, Pharyngeal Pouch. In: StatPearls [Internet]. Treasure Island (FL): StatPearls Publishing; 2022 Jan.2022 Apr 5.
3- Shen LF, Zhou SH, Chen QQ, Yu Q: Second branchial cleft anomalies in children: a literature review. Pediatr Surg Int. 34(12):1251-1256, 2018
4- Bajaj Y, Ifeacho S, Tweedie D, et al:  Branchial anomalies in children. Int J Pediatr Otorhinolaryngol. 75(8):1020-3, 2011
5- Li W, Xu H, Zhao L, Li X: Branchial anomalies in children: A report of 105 surgical cases. Int J Pediatr Otorhinolaryngol. 104:14-18, 2018
6- Adams A, Mankad K, Offiah C, Childs L: Branchial cleft anomalies: a pictorial review of embryological development and spectrum of imaging findings. Insights Imaging. 7(1):69-76, 2016
7- Li Y, Lyu K, Wen Y, et al: Third or fourth branchial pouch sinus lesions: a case series and management algorithm. J Otolaryngol Head Neck Surg. 48(1):61, 2019

Electromagnetic Navigation Bronchoscopy

Electromagnetic navigation bronchoscopy (ENB) is a promising technology that increases the diagnostic accuracy of peripheral lung and mediastinal lesion, and is taken as a supplement to traditional bronchoscopy, endotracheal ultrasound, and endotracheal biopsy techniques into consideration. ENB has been designed as a localizing and guide endoscopic tool through the airway to a target lesion. The system consists of an electromagnetic tracking system that detects a position sensor incorporated within a flexible catheter which can be advanced through a bronchoscope. An electromagnetic field is generated by a localization system which consists of a processor, amplifier, and location board. When the sensor is placed within the electromagnetic field, its position and orientation can be identified. This information is superimposed on a previously acquired high-resolution CT data creating a 3-dimensional virtual bronchoscopic image. ENB-guided transbronchial needle aspiration or biopsy has shown high accuracy and lower complication rate, such as a pneumothorax or hemothorax, than the conventional percutaneous core needle biopsy. The rate of successful ENB-guided biopsy can reach 92%. Transbronchial ENB-guided lung biopsy is feasible and safe, provides larger samples and has a higher diagnostic yield than transbronchial lung biopsy only. The advantages of this method are no pain with localization procedure, complete control of ventilation, simplified workflow, high accuracy, and immediate transition to operation, which eliminates the consequences of complications. Small, deep or subsolid pulmonary nodules can be difficult to find during either thoracoscopy or even thoracotomy. Larger lung resections are performed when the nodule is hard to find or the need to convert to thoracotomy to do bimanual palpation. Criteria for using ENB preoperative localization include nodule diameter of 5 mm or less, ratio of maximum diameter of nodule to minimum distance between pleural space and inferior border of nodule of 0.5 mm or less, and for nodules with low density on CT.  Small pulmonary nodules can be identified using ENB-guided transbronchial dye-marking. Dye utilized include methylene blue, indigo carmine or Indocyanine green. Indigo carmine can be visible at least three days after marking. While fluorescein is dissipated after several hours of staining. This localization technique can help resect the pulmonary lesion during thoracoscopic exploration. An accurate localization is very important for these sublobar or non-anatomical resections. Nodules should be resected with negative resection margins. There are several potential benefits to applying ENB for the preoperative localization of pulmonary nodules prior to thoracoscopy. It can be safely and effectively implemented in the same OR, and VATS resection can be performed immediately following the localizing procedure. Long delay after staining results in difficult to locate nodule due to excessive diffusion and fading of methylene blue. No concern after immediate surgery for developing pneumothorax or hemothorax. The ENB localization success rate is around 79-100%. ENB has been recently utilized in pediatric oncology patients needing a lung biopsy or resection for nodule and found to be safe and efficacious. The technique is performed in the same operating room as the biopsy. It can be able to localize lesions as small as 2-3 mm identified on CT. ENB can be used for multiple biopsies as well as for bilateral biopsies. There are reports that ENB can interfere with cardiac monitoring.

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References:
1- Abbas A, Kadakia S, Ambur V, Muro K, Kaiser L: Intraoperative electromagnetic navigational bronchoscopic localization of small, deep, or subsolid pulmonary nodules. J Thorac Cardiovasc Surg. 153(6):1581-1590, 2017
2- Cho HJ, Roknuggaman M, Han WS, Kang SK, Kang MW: Electromagnetic navigation bronchoscopy-Chungnam National University Hospital experience. J Thorac Dis. 10(Suppl 6):S717-S724, 2018
3- Qian K, Deng Y, Shen C, Feng YG, Deng B, Tan QY: Combination of electromagnetic navigation bronchoscopy-guided biopsy with a novel staining for peripheral pulmonary lesions. World J Surg Oncol. 17(1):158, 2019
4- Wang LL, He BF, Cui JH, et al: Electromagnetic navigational bronchoscopy-directed dye marking for locating pulmonary nodules. Postgrad Med J. 96(1141):674-679, 2020
5- Harris K, Schaefer E, Rosenblum J, Stewart FD, Arkovitz MS: Intraoperative electromagnetic navigation bronchoscopy (IENB) to localize peripheral lung lesions: A new technique in the pediatric oncology population. J Pediatr Surg. 57: 179-182, 2022
6- Song JW, Park IK, Bae SY, et al: Preoperative electromagnetic navigation bronchoscopy-guided one-stage multiple-dye localization for resection of subsolid nodules: A single-center pilot study. Thorac Cancer. 13(3):466-473, 2022

PUCAI

The Pediatric Ulcerative Colitis Activity Index (PUCAI) is a non-invasive objective measure of clinical outcome developed to standardize the reporting of ulcerative colitis disease activity in children. PUCAI is based on six quantifiable items obtained from the child history without need for an abdominal exam, blood testing or endoscopy results. The six items recollected within the activity index include the presence of abdominal pain, rectal bleeding amount, stool consistency, number of stools, whether there are nocturnal stools and the activity bedrest of the child. The highest score is 85, with 65 or above used as an assessment of severe disease. Moderate disease is between 35-64 and mild disease is between 10 and 34 points score. Less than 10 means the child is probably in remission. Clinically significant response is defined by a PUCAI change of at least 20 points. PUCAI has been mostly used to characterized disease activity and effect of medical therapy. The high feasibility and validity of the PUCAI score in clinical practice provide strong support for the use of this instrument as a clinical tool serving as a basis for inpatient and outpatient care algorithms. PUCAI is not an objective measure of inflammation. PUCAI can be used with confidence as the sole outcome measure in pediatric UC without the need for endoscopy. Several colitis from UC is initially managed with systemic steroids. Effectiveness of steroid can be measured using PUCAI on day 3 and day 5 of treatment. Should the score be above 45, steroid-refractory disease should be considered, and second-line treatment should be started. Second-line medical management includes agents such as infliximab, tacrolimus, and cyclosporine. Infliximab is a monoclonal antibody to human TNF-alpha which has been shown to be effective in modulating intestinal inflammation in UC. Infliximab has a short-term response rate of 75%, and a long-term response rate of 64%. Infliximab has significantly reduced the colectomy rate in children with acute colitis. Tacrolimus is a macrolide immunosuppressant that inhibits calcineurin, which subsequently prevents the production of pro-inflammatory cytokines such as interleukin-2. Most restorative proctocolectomy children has received tacrolimus as a bridge to surgery and there is a significant improvement in the PUCAI score of patients receiving preoperative tacrolimus therapy. There is a strong correlation between the preoperative PUCAI score and the likehood than an ulcerative colitis patient needs a staged restorative proctocolectomy. Children with severe UC, bowel dilatation, uncontrolled pain, toxic appearance, significant anemia, or hypoalbuminemia should be hospitalized and started in systemic steroids. The mortality of acute severe UC is 1%, mainly from perforation, toxic megacolon, and infectious complications. Ominous signs in UC include severe abdominal pain or tenderness, vomiting, fever, significant weight loss and bowel dilatation. PUCAI scores in days 3 and 5 of corticosteroid therapy can predict patients failing therapy with steroid in need of salvage therapy with infliximab. PUCAI score does not significantly correlate with the likehood of developing surgical complications. Emergent colectomy is performed infrequently with medical therapy constituting first-line therapy in severe UC. If an infectious etiology is suspected (i.e., C Difficile) antibiotics should be used empirically. Oral feedings should be held if toxic megacolon is suspected. Persistency elevated PUCAI score in the face of steroid therapy is a call for second-line treatment. Therapeutic options include surgical intervention, infliximab and calcineurin inhibitors. Second-line medical therapy can induce a response in 70% of children. Surgical options can be elective or emergent. Children with UC more often undergo colectomy for medically refractory disease. Elective surgery is done in children unresponsive to medical management, in those with active, or steroid-dependent, UC despite optimized medical therapy or with evidence of dysplasia or malignancy on endoscopy. Restorative proctocolectomy with ileal pouch-anal anastomosis (J pouch) and a covering loop ileostomy is the recommended elective surgery for pediatric UC. Emergent surgery is indicated in children with colonic perforation, massive hemorrhage, toxic megacolon, or sepsis. Children with fulminant disease without adequate control and PUCAI > 65 despite intense medical therapy should be referred for surgery. In the emergent situation a subtotal colectomy, end-ileostomy and blind rectal pouch stump is recommended (Three-stage procedure). A minimally invasive laparoscopic approach is recommended in children as there are equivalent outcomes to open surgery both for urgent and elective cases, and possibly superior outcomes regarding fertility in girls. Functional outcomes and surgical complications are comparable after hand-sewn and stapled anastomosis. Colectomy should be preferably performed 4-6 weeks after the last infliximab infusion if it can be safely postponed. Children that undergo emergent surgery have a higher incidence of postoperative complications compared with those undergoing elective colectomy.   

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References:
1- Turner D, Hyams J, Markowitz J, et al: Pediatric IBD Collaborative Research Group. Appraisal of the pediatric ulcerative colitis activity index (PUCAI). Inflamm Bowel Dis. 15(8):1218-23, 2009
2- Gray FL, Turner CG, Zurakowski D, et al: Predictive value of the Pediatric Ulcerative Colitis Activity Index in the surgical management of ulcerative colitis. J Pediatr Surg. 48(7):1540-5, 2013
3- Siow VS, Bhatt R, Mollen KP. Management of acute severe ulcerative colitis in children. Semin Pediatr Surg. 26(6):367-372, 2017
4- Turner D, Ruemmele FM, Orlanski-Meyer E, et al: Management of Paediatric Ulcerative Colitis, Part 1: Ambulatory Care-An Evidence-based Guideline From European Crohn's and Colitis Organization and European Society of Paediatric Gastroenterology, Hepatology and
Nutrition. J Pediatr Gastroenterol Nutr. 67(2):257-291, 2018
5- Turner D, Ruemmele FM, Orlanski-Meyer E, et al: Management of Paediatric Ulcerative Colitis, Part2: Acute Severe Colitis- An Evidence-based Consensus Guideline From the European Crohn's and Colitis Organization and the Auropean Society of Paedeiatric Gastroenterology, Hepatology and Nutrition. J Pediatr Gastroenterol Nutr 67(2); 291-310, 2018
6- Tan Tanny SP, Yoo M, Hutson JM, Langer JC, King SK. Current surgical practice
in pediatric ulcerative colitis: A systematic review. J Pediatr Surg. 54(7):1324-1330, 2019

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PSU Volume 59 NO 05 NOVEMBER 2022

Testicular Seminoma

Pediatric germ cell tumors are rare, with 80% benign and 20% malignant. Ovarian and testicular site accounts for 40% of all cases. Testicular germ cell tumors are the most common tumors which usually occur in males between 20-40 years old. The incidence has risen markedly over the years. Testicular germ cell tumors have two main histology: seminoma and non-seminomatous. Non-seminomatous GCT includes embryonal carcinoma, choriocarcinoma, teratoma and yolk sac tumor. Teratoma, both mature and immature, are the most common histologic non-seminomatous variety. Germinomas, also term seminoma in males and dysgerminoma in females, are undifferentiated germ cell tumors typical of adolescents. Testicular germ cell tumors have two age peaks: children under three years may experience both mature teratoma and malignant yolk sac tumor, while adolescents may harbor seminomas or other mixed tumors often with delayed diagnosis and more advanced disease. The occurrence of germ cell tumors increases in children with undescended testes, and the risk is higher with Intraabdominal testes. The most common histological subtype in this situation is seminoma, which occur in adolescent and young adults. Patients with Trisomy 21 are 50 times more likely to have testicular cancer. Testicular seminoma originates in the germinal epithelium of the seminiferous tubules. The disease is thought to result from the proliferation of immature spermatogonia. There are three main pathologic categories of testicular seminoma: classical, spermatocytic and seminoma with syncytiocytotrophoblastic cells. This last type is associated with elevated serum beta-HCG levels. A seminoma with a high mitotic index (>3 mitotic figures/HPF) is designated an anaplastic seminoma, a more aggressive tumor. Main clinical findings with testicular seminoma are a painless scrotal mass which may be associated with infertility, though the tumor may appear as an emergent situation with pain and acute inflammatory characteristics and hydrocele. On physical exam there is a unilateral, firm to hard palpable mass in the scrotum localized to the testis. In a patient with a testicular mass, serum markers measurements represent the first diagnostic step to verify a possible malignant germ cell tumor. These include AFP, beta-HCG and LDH. AFP elevation indicates non-seminomatous disease, LDH is used to follow the overall seminomatous tumor burden, and beta-HCG is present in 5-10% of seminoma patents usually associated with metastatic disease. In addition, Placental alkaline phosphatase (PALP) produced by the placenta, is also produced by cancer cells. PALP is not specific for testicular tumors, it can also be found in pulmonary, digestive system, breast, and female reproductive organ cancer. PALP is commonly employed as a routine diagnostic marker for seminoma/germinoma. PALP can be detected in 98% of seminomas, 85% yolk sac tumor and 97% embryonal carcinomas, but not in teratomas. OCT4 is an ectomere binding transcription factor detected in tumor germ cells which has pluripotent potential. It is mostly detected in seminomas, embryonal carcinomas, dysgerminomas, and germ cell component of gonadoblastoma. OCT4 is positive in 100% seminomas and negative in normal testicular tissue. OCT4 is more specific and sensitive that PALP. Ultrasound of the scrotum is the preferred initial imaging investigation to evaluate any suspected testicular mass growth. Diagnostic work-up includes a thoraco-abdominal CT-scan to evaluate possible metastatic spread, especially on lungs and on the retroperitoneal lymph nodes. Should the suspected lesion suggest a neoplasm, a surgical approach on the tumor is recommended as soon as possible. Surgery is the main therapy for testicular tumors. This includes an inguinal approach with vascular control before mobilization of the testis. If malignancy is proven by frozen section examination of a biopsy of the mass, en-bloc resection of testis and spermatic structures with high ligation of the cord at the internal inguinal ring is needed (radical inguinal orchiectomy). Patients with scrotal skin involvement by the tumor and those operated or biopsied through a scrotal approach should undergo hemiscrotectomy to ensure local disease control. Retroperitoneal lymph node dissection is required when enlarged nodes remain after chemotherapy. Inguinal nodes exploration is indicated only in patients with scrotal involvement. Seminomatous and non-seminomatous tumors are notable for their responsiveness to chemotherapy. Seminoma is one of the most treatable cancer with a survival rate of 98% in early-stage disease. Testicular seminoma is also sensitive to radiation therapy. Radiation therapy planning is based on the results of a contrast-enhanced CT of the chest, abdomen, and pelvis.

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References:
1- Cecchetto G: Gonadal germ cell tumors in children and adolescents. J Indian Assoc Pediatr Surg. 19(4):189-94, 2014
2- Zhang T, Ji L, Liu B, Guan W, Liu Q, Gao Y: Testicular germ cell tumors: a clinicopathological and immunohistochemical analysis of 145 cases. Int J Clin Exp Pathol. 11(9):4622-4629, 2018
3- Singh AP, Tanger R, Mishra D, Ansari M, Gupta AK, Shukla AK: Testicular Mixed Germ Cell Tumor in a Newborn Child: A Rare Case. Journal Indian Association of Pediatric Surgeons. 24(2): 144-146, 2019
4- Sanguesa C, Veiga D, Llavador M, Serrano A: Testicular tumours in children: an approach to diagnosis and management with pathologic correlation. Insight into imaging, 11(1), 74, 2020
5- Caballero Mora FJ, Munoz Calvo MT, Garci-a Ros M, et al: [Testicular and paratesticular tumors during childhood and adolescence]. An Pediatr (Barc). 78(1):6-13, 2013
6- Bahrami A, Ro JY, Ayala AG: An overview of testicular germ cell tumors. Arch Pathol Lab Med. 131(8):1267-80, 2007

Choriocarcinoma

Pediatric germ cell tumors most commonly develop within the gonad, though extragonadal primary tumors can involve the anterior mediastinum, retroperitoneum, or the central nervous system. Choriocarcinoma is a very rare and aggressive germ cell neoplasm of trophoblastic origin. Choriocarcinoma is a disease seen predominantly in women, but men can also be affected as part of a mixed germ cell tumor. Two types of choriocarcinoma have been described: gestational and non-gestational. Gestational choriocarcinoma is the most common form, affect only females and arises following a hydatidiform mole, normal pregnancy or most commonly, spontaneous abortion. Non-gestational choriocarcinoma is rare, arises from pluripotent germ cell, can affect young males or females, in the gonads, or midline structures with pluripotent germ cells. The pathogenesis of choriocarcinoma start with cytotrophoblastic cells functioning as stem cells and undergoing malignant transformation. Choriocarcinoma Histologic demonstrate syncytiotrophoblasts, these are large eosinophilic multinucleated cells with known large hyperchromatic nuclei, intermixed with cytotrophoblasts, polygonal cells with distinct borders and single irregular nuclei. Choriocarcinoma show absence of chorionic villi and presence of abnormal intermediate trophoblast and cytotrophoblast, rimmed with syncytiotrophoblasts with areas of necrosis and hemorrhage. Choriocarcinoma is a extremely vascular neoplasm characterized by necrosis and absence of chorionic villi. In mixed germ cell tumors, choriocarcinoma will show mixtures of syncytiotrophoblasts and cytotrophoblasts with varying component of other germ cell tumors. Apart from the uterus, it can be found in tubes, ovaries, lung, liver, spleen, kidneys, bowel, and brain. Spontaneous abortions and molar pregnancy increase the risk of developing choriocarcinoma. Due to elevation of HCG, patients can present with abnormal uterine bleeding, gynecomastia in men and hyperthyroidism. Males can show symptoms of metastatic disease with hemoptysis, involved liver, gastrointestinal tract, and brain. Choriocarcinoma secrete human chorionic gonadotropin (HCG). HCG is an excellent biomarker of disease progression, response, and subsequent post-treatment surveillance. After diagnosis of choriocarcinoma evaluation for malignancy should be undertaken. The lung is the most common site of metastatic disease. Brain, chest, abdomen, and pelvis should be evaluated by CT or MRI imaging. Management of choriocarcinoma entails multimodal therapy with drugs, radiation, and surgery. Low risk choriocarcinoma can be managed with single agent chemotherapy. High-risk disease is managed with multi-agent chemotherapy, adjuvant radiation and surgery. Removal of the uterus or metastatic foci is performed in conjunction with chemotherapy. The prognosis of non-gestational choriocarcinoma is worse than his gestational counterpart due to low sensitivity to chemotherapy. Low risk choriocarcinoma has a 100% survival in women managed with chemotherapy, while high-risk tumors have a 90% survival with multiagent therapy with or without radiation and surgery. Primary infantile or neonatal choriocarcinoma is extremely rare with most cases involving metastatic disease to the fetus from an intraplacental choriocarcinoma. Is associated with a poor prognosis and high mortality for the baby. Most of these mother with choriocarcinoma were asymptomatic during pregnancy. Most babies show anemia, developmental delay, hepatomegaly, hemoptysis, or respiratory failure. Ultrasound shows a tumor with rich vascularization. Beta-HCG is extremely high. Management is multiagent chemotherapy. Infantile choriocarcinoma represent a metastatic focus from primary maternal or placental gestational trophoblastic tumor.  

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References:
1- Ngan HYS, Seckl MJ, Berkowitz RS, et al: Update on the diagnosis and management of gestational trophoblastic disease.  Int J Gynaecol Obstet. 143 Suppl 2:79-85., 2018
2- Keenan C, Ramirez N, Elijovich L, et al: A rare manifestation of choriocarcinoma syndrome in a child with primary intracranial germ cell tumor and extracranial metastases: A case report and review of the literature. Pediatr Blood Cancer. 68(6):e29000, 2021
3- Rzanny-Owczarzak M, Sawicka-Metkowska J, Jonczyk-Potoczna K, et al: Simultaneous Occurrence of Choriocarcinoma in an Infant and Mother. Int J Environ Res Public Health. 18(4):1934, 2021
4- Shah R, Weil BR, Weldon CB, Amatruda JF, Frazier AL: Neonatal Malignant Disorders: Germ Cell Tumors. Clin Perinatol. 48(1):147-165, 2021
5- Ngan HYS, Seckl MJ, Berkowitz RS, et al: Diagnosis and management of gestational trophoblastic disease: 2021 update.  Int J Gynaecol Obstet.155 Suppl 1(Suppl 1):86-93, 2021

Microaggression in Surgery

Originally described by Chester Pierce in 1970, microaggression referred to a minor damaging humiliation and indignity toward African-Americans. Subtle comments, slights and insults directed toward minorities, as well as to women and other stigmatized groups that engender hostility. Microaggresion is a brief and commonplace verbal, behavioral or unintentional environmental discrimination against a personal characteristic such as gender, sexual orientation, race, or ethnicity. Microaggressions are associated with a negative emotional response which may be associated with worsening mental health. They have a negative effect and unpleasant psychological impact on recipients. The term microaggression expanded in 2007 to include all minorities groups as well as marginated communities. Microaggressions can be a conscious or unconscious microprejudice. Four subtypes of microaggression have been described: microassault, microinsult, microinvalidations, and environmental microaggressions. Microassault are "old fashioned" discriminatory statements, often intentional, characterized by verbal or nonverbal attacks clearly intended to offend the recipient. Microassaults are toward individuals rather than a group with racism motivation. Microinsults are subtle snubs or humiliations that convey a demeaning message to the recipient in a way that may be unintentional to the perpetrator. It happens when women or minority members of society are confused for a nurse, janitor, or interpreter, because they are not seen as a traditional physician. Ignoring medical students in the operating room or doing rounds, making comments that suggest people obtained their current position because of affirmative action rather than knowledge, skills, or abilities. Microinvalidations are performed to exclude, negate, dismiss the personal thoughts, feelings, or experiment reality of a person. They deny concern about fairness by insisting that the workplace in a meritocracy or invalidating a woman or minority student experience of inequality by calling them oversensitive. Environmental microaggressions occur when microassault, microinsult and microinvalidation are reflected in the cultural processes and climate of the workplace. An example is a hallway decorated with pictures of white male surgeons, when medical schools and departments of surgery unintentionally exclude and minimize the identity of minorities and women by excluding accomplishments and portrait of members of some racial, ethnic, and cultural background. Microaggressions adversely affect the psychological and physical health of the recipient. Microaggressions produce depression, anxiety, traumatic response, increase use of alcohol and hypertension in the recipient. Racism and sexism that manifest as microaggressions in the workplace present dilemmas for individuals from minorities groups. Microaggressions can be responded by raising a voice of concern and resistance, relying on social networks for support, or developing self-protective mechanisms such as desensitization. A structured response from the recipient can start with a conversation with the perpetrator to observe, think, feel and desire how the comment was interpreted. Next the victim can use action by asking clarifying questions, tell what you observed, discuss the impact of the comment, and express your own thought and feeling about the situation. A third strategy of response is to focus on what was observed and the recipientsÕ resulting thoughts or feelings to decrease the potential for defensiveness and encourage dialogue. Subtle racial comments, actions, and assumptions are witnesses, are experiences by minority medical students contributing significantly to feelings of burnout, invalidation, and insecurity in them. Bias is the prejudice in favor of or against one thing, person, or group. When bias is unconscious, uncontrollable, or as result of an irrational process, the bias is implicit. Implicit bias in medical education can result in inaccurate evaluations that affect promotion and disproportionate hiring and representation. Microaggression and implicit bias can extend bidirectionally from patient to physician as well as from physician to patient. Environmental invalidations are the most common microaggression reported, suggesting women in surgery face ongoing microaggressions at the systemic level creating many barriers to advancement. Implicit bias can manifest in microaggressive actions such as overlooking women for positions of power or offering challenging cases to male colleagues who are believed to be more capable than their female counterpart. Women experience more microaggressions than men during virtual residency interviews, with the most commonly experienced microaggression type being environmental. As response, women lower the ranking and stand out negatively toward those programs committing microaggressions.            

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References:
1- Torres MB, Salles A, Cochran A: Recognizing and Reacting to Microaggressions in Medicine and Surgery. JAMA Surg. 154(9):868-872, 2019
2- Chisholm LP, Jackson KR, Davidson HA, Churchwell AL, Fleming AE, Drolet BC: Evaluation of Racial Microaggressions Experienced During Medical School Training and the Effect on Medical Student Education and Burnout: A Validation Study. J Natl Med Assoc. 113(3):310-314, 2021
3- Turner J, Higgins R, Childs E: Microaggression and Implicit Bias. Am Surg. 87(11):1727-1731, 2021
4- Sprow HN, Hansen NF, Loeb HE, et al: Gender-Based Microaggressions in Surgery: A Scoping Review of the Global Literature. World J Surg. 45(5):1409-1422, 2021
5- Hackworth JM, Kotagal M, Bignall ONR, Unaka N, Matheny Antommaria AH: Microaggressions: Privileged Observers' Duty to Act and What They Can Do. Pediatrics. 148(6):e2021052758, 2021
6- Hoi KK, Kana LA, Sandhu G, et al: Gender Microaggressions During Virtual Residency Interviews and Impact on Ranking of Programs During the Residency Match. J Grad Med Educ. 14(4):398-402, 2022

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PSU Volume 59 NO 06 DECEMBER 2022

Nephroptosis

Nephroptosis, floating kidneys or renal ptosis, is defined as a significant descent of the kidney, of more than 5 cm or two vertebral bodies, when the patient moves from supine to upright. This is different to an ectopic kidney where it remains in a constant abnormal position. The downward displacement of the kidney give rise to symptoms either due to effects on the ureter or the renal vessels. Nephroptosis is asymptomatic in most patients (80-90%) and occurs more commonly in thin women. The condition is rare in the pediatric age, while it manifest during the second to fourth decade with most being women. The right side is affected in 70% of cases, the left in 10%, and 20% are bilateral. It is believed that nephroptosis is caused by excessive mobility due to deficient support from the perinephric structure. This can cause stretching, torsion or kinking of the hilar vessels and proximal ureter. The diagnosis of nephroptosis requires a high index of suspicion and imaging confirmation. Major symptoms are pain, nausea/vomiting, transient hematuria, and orthostatic hypertension. Pain in the flank or abdomen is the most common symptom (90%), and typically occurs when upright, and relieved by recumbency. Nephroptotic pain can be associated with intermittent ureteric obstruction causing hydronephrosis, ischemia, narrowing or kinking of the renal artery or vein causing stasis, traction and stimulation of the visceral nerves, and symptoms due to secondary pathology. The hematuria originates from the calyceal and renal pelvis veins due to compression. Hypertension is caused by activation of the renin-angiotensin-aldosterone system. Nephroptosis is a diagnosis of exclusion after ruling out renal calculi, PUJ obstruction and pyelonephritis. All suspected cases should undergo both supine and upright studies such as US with Doppler, intravenous urography, contrast CT and radionuclide scans (dynamic or static). The Whitaker test, though invasive can also be used as a diagnostic tool. The affected ptotic kidney will return to its normal position when the patient is supine. Radionuclear scans (DTPA renogram) can demonstrate vascular flow impairment, abnormal tubular secretion, irregular distribution of the tracer, reduced glomerular filtration rate and outlet obstruction. Management should be considered in symptomatic patients with more than three months of symptoms duration and evidence of no other pathology associated, with nuclear medicine and Doppler imaging showing descent of the symptomatic kidney with obstruction or diminished flow to the symptomatic side. Treatment is surgical, namely nephropexy. Nephropexy can be performed open, laparoscopic, or percutaneously. Optimal nephropexy should include complete nephrolysis and release of the attachments to the peritoneum, mobilization of the kidney to a more cephalad retroperitoneal position, relief of associated urinary obstruction, and fixation of the renal axis without tension. The percutaneous nephropexy relies in the scar formation after access and placing a drain for several days. The laparoscopic nephropexy has a significant successful outcome in more than 90% of patients, excellent cosmetic results, less postoperative pain, less hospital stay, lower morbidity and faster recovery. Is more time-consuming and expensive than the open procedure.

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References:
1- Srirangam SJ, Pollard AJ, Adeyoju AA, O'Reilly PH: Nephroptosis: seriously misunderstood? BJU Int. 103(3):296-300, 2009
2- Bansal D, Defoor WR Jr, Noh PH: Pediatric robotic assisted laparoscopic nephropexy: case study. Springerplus. ;2:321, 2013
3- Chan VSH, Lam TPW, Lam WWM: Nephroptosis: The wandering kidney. Kidney Res Clin Pract. 37(3):306-307, 2018
4- Grauer R, Gray M, Schenkman N: Modified Whitaker test: a novel diagnostic for nephroptosis. BMJ Case Rep. 13(4):e235108, 2020
5- Philippou P, Michalakis A, Ioannou K, Miliatou M: Laparoscopic Nephropexy: The Sliding Clip Technique. J Endourol Case Rep. 17;6(3):224-227, 2020
6- Kahle J, Lakhani J: A disappearing abdominal mass in a teenage female. SAGE Open Med Case Rep. 2020 Jun 3;8:2050313X20927965. doi: 10.1177/2050313X20927965. eCollection 2020

Pleuropulmonary Blastoma

Pleuropulmonary blastoma (PPB) is considered the most common primary malignancy of the lung in children. 25-50 new cases are seen per year in the USA. PPB arise initially as a pulmonary cyst. Three pathologic stages of PPB have been described: type 1, purely cystic PPB; type 2 cystic/solid PPB; and type 3 purely solid PPB. The median age at diagnosis and the pathologic type of PPB: type 1 at 8 months; type 2 at 35 months; type 3 at 44 months. A progression from type 1 to 3 can occur, though not all cases progress to a more malignant transformation. Regression of the purely cystic form is called type 1r. PPB type 1 develops at a much younger age than type 2 or 3, with 97% presenting before the age of 3 years. Type 1 PPB appears as multiloculated, air-filled cysts with thin septa. The cysts are lined with benign respiratory epithelium and mesenchyme, with an underlying component of malignant mesenchymal cells that may have rhabdomyoblastic differentiation. Type 1 are most often unilateral, unifocal, peripheral, over 5 cm in size, and occur with a slight male predominance. Lung cysts can be seen prenatally between gestational age 23 to 35 weeks. Type 1, purely cystic PPB can be mistaken for another congenital cystic lung lesion. Type 1r (regression type) was originally recognize in older relatives of PPB, though cysts with these features can be found in very young children. Type 1r PPB have the same multilocular cystic appearance as type 1, but without the interspersed primitive malignant cells. Type 1r have a median age of diagnosis of 47 months compared to 8 months for type 1.  Type 1r age range is larger, and a lung cyst in an older individual with DICER1 or a relative with PPB patient is most likely this type. Type 2 PPB account for approximately one-third of cases, with an equal male-to-female ratio, and present later than type 1 at a median age of 35 months, very rarely seen prior to 12 months of age. Type 3 PPB has the worse prognosis, is entirely comprised of tumor cells without intervening cystic space, present at a more advanced age, with a median age-of-diagnosis of 44 months, and do not appear to be seen before 12 months of age. Type 2 and 3 PPB are histologically similar, displaying a mixed sarcomatous pattern, are diagnosed at an older age, and have metastatic potential to the brain, bone, and rarely liver. Chest/abdominal CT and brain MRI with bone scan are required. Both type 2 and 3 are aggressive malignancies that require chemotherapy soon after the first diagnostic surgery. PPB is associated with an unique set of disorders, and the genetic basis of the PPB familial syndrome is the heterozygous loss-of-function mutation of DICER1 found in 70-80% of children who develop PPB. The presence of such a germline mutation defines DICER1 PPB familial tumor predisposition syndrome. Germline DICER1 mutations are inherited as an autosomal dominant fashion in 80% of cases and arising the novo in the rest. In addition to PPB, DICER1 mutation includes cystic nephroma, ovarian Sertoli-Leydig cell tumors, ciliary body medulloepithelioma, nodular hyperplasia and differentiated carcinoma of the thyroid gland, pituitary blastoma, pineoblastoma, nasal chondromesenchymal hamartoma, and ERMS. Due to the rarity of PPB, screening for the general population is not needed. In children with DICER1 mutation only 4% of infants would develop a PPB. DICER1 germline testing should be performed in all pediatric patients with lung cysts early in life. P53 mutations in the cystic epithelial cells also have an important role in PPB type progression. Clinical presentation is usually with nonspecific respiratory complaints such as difficulty in breathing, dyspnea, chest pain, hemoptysis. Fever, malaise, and anorexia are associated with type 2 and 3 PPB. On imaging, chest simple films are the first modality used for evaluation. PPB is most commonly seen on the right side. It appears as hemi-opaque thorax with contralateral tracheal and mediastinal deviation. Diagnosis needs CT of chest and metastatic workup for type 2 and 3, since metastasis is unknown in type 1. Pleural effusion and pneumothorax con be commonly seen. Calcification is not a common finding. Management of PPB is surgical resection. Type 1 and 1r should be managed via complete resection with widely negative margins. An open approach is advocated to minimize chance of tumor spillage. Adjuvant chemotherapy is not typically given for type 1 PPB unless there is intraoperative tumor spill, incomplete resection, or local invasion of adjacent structures. In older asymptomatic patients with type 1r removal is not explicitly indicated and observation might be appropriate. For type 2 and 3 PPB, both systemic chemotherapy and surgical resection are critical component of management. Chemotherapy is typically based on sarcoma regimens. Surgical resection of type 2 and 3 PPB may require from a wedge resection to lobectomy or pneumonectomy to achieve negative margins. Involved pleural space should also be resected en-bloc with the primary tumor and involved pulmonary lobe. Radiation therapy is ineffective in general. For brain metastasis all three treatment modalities, surgery, chemotherapy, and radiation therapy is recommended. The type of PPB is the strongest prognostic factor, with outcomes better for type 2 over type 3. Correlation between the PPB type and survival is type 1 -94%; type 2, 71%; type 3, 53%. Distant metastasis at diagnosis had a statistically significant detrimental effect on survival. Anaplasia is common in both type 2 and 3 without significant prognostic effect. Gross total resection for adequate local control has important favorable prognostic implication. The PPB type and presence of distant metastasis at diagnosis are the most important prognostic factors related to treatment outcome.

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References:
1- Messinger YH, Stewart DR, Priest JR, et al: Pleuropulmonary blastoma: a report on 350 central pathology-confirmed pleuropulmonary blastoma cases by the International Pleuropulmonary Blastoma Registry. Cancer. 121(2):276-85, 2015
2- Knight S, Knight T, Khan A, Murphy AJ: Current Management of Pleuropulmonary Blastoma: A Surgical Perspective. Children (Basel). 6(8):86, 2019
3- Madaan PK, Sidhu HS, Girdhar S, Mann KK: Pleuropulmonary blastoma: A report of three cases and review of literature. Radiol Case Rep. 16(10):2862-2868, 2021
4- Gonzalez IA, Stewart DR, Schultz KAP, Field AP, Hill DA, Dehner LP: DICER1 tumor predisposition syndrome: an evolving story initiated with the pleuropulmonary blastoma. Mod Pathol. 2022 Jan;35(1):4-22. doi: 10.1038/s41379-021-00905-8.
5- Kunisaki SM, Lal DR, Saito JM, et al: Pleuropulmonary Blastoma in Pediatric Lung Lesions. Pediatrics. 2021 Apr;147(4):e2020028357. doi: 10.1542/peds.2020-028357. Epub 2021 Mar 24.
6- Bownes LV, Hutchins SC, Cardenas AM, Kelly DR, Beierle EA: Pleuropulmonary blastoma in an adolescent. J Pediatr Surg Case Rep. 59:101482, 2020
7- Sparber-Sauer M, Tagarelli A, Seitz G, et al: Children with progressive and relapsed pleuropulmonary blastoma: A European collaborative analysis. Pediatr Blood Cancer. 68(12):e29268, 2021

Sternal Cleft

Sternal cleft is a very rare congenital midline malformation resulting from failure of fusion of the sternum. It represents 0.15% of all patients presenting with a chest wall malformation in life. Sternal cleft arise from failure of development or ventral fusion of the sternal bars between the 6th and 9th weeks of gestation. The resultant anatomical defect produces and concave defect in the sternum covered by skin, with an orthotopic heart and intact pericardium. The shape of the defect can vary from a narrow  V' to a wider  U'-shaped cleft. Sternal cleft is classified within three major groups: cleft sternum without associated anomalies, thoracic or true ectopia cordis with varying degree of cleft sternum with the heart outside the chest wall, and thoracoabdominal ectopia cordis, also referred as Cantrell's pentalogy. Sternal cleft without associated anomalies is further classified as either partial (superior or inferior) or complete. With partial sternal cleft there is skin coverage of the midline defect with an intact pericardium and a normal diaphragm. The superior cleft is often associated with malformations like facial hemangioma or abdominal raphe. The partial inferior form is often associated with ectopia cordis alone or as part of the pentalogy of Cantrell. Sternal cleft is easily diagnosed at birth. Sternal cleft causes paradoxical respiratory movements and increases the risk of harmful events on mediastinal viscera. Surgical correction is recommended in the neonatal period. The thoracic wall has a higher compliance at this age. In older patients the rib cage is stiff and repair is difficult. Sternal cleft are usually diagnosed during the neonatal period, can also be diagnosed prenatally, females are affected more commonly than males and the most represented form is the partial superior type appearing in two-third of all cases. One-third develop symptoms such as dyspnea, respiratory distress, and recurrent respiratory tract infections. Two-thirds has an associated defect, mainly cardiac and vascular. The PHACE syndrome (posterior fossa brain abnormalities, hemangiomas, cranial vascular abnormalities, aortic coarctation, cardiac defects, eye abnormalities) and sternal malformations are seen together. Pectus excavatum can also be seen associated with sternal cleft. If not treated surgically patients with sternal cleft can have impaired gas exchange, present respiratory symptoms such as dyspnea and cough, or develop chest infection. Contrast enhanced CT of the chest with 3D reconstruction and/or MRI is utilized as imaging of choices when the defect is discovered. Echocardiogram is needed to determine cardiac associated structural defects. Correction by surgery is indicated to protect the heart and great vessels from direct injury, maintain the growth potential of the chest wall, avoid use of prosthetic material when possible, improve respiratory dynamics, and restore a good cosmetic appearance. Correction during early life permits easy direct closure to be effected due to flexibility of the chest resulting in a lower risk of cardiac compression. Primary closure with or without chondrotomies, periosteal flaps or cartilage resection is the preferred management. Alternatives procedures include bone graft interposition, prosthetic closure, and muscle flap interposition. Autologous cartilage from the resected inferior part of the sternal defect can be used to obliterate space at the superior part of the sternal defect as it can decrease tension between the costal ridge during closure. In certain circumstances a partial or complete thymectomy might be necessary to reduce the risk of mediastinal compression. Breast development in female with sternal cleft is normal, but special care must be taken during repair in neonates not to damage the breast buds. Complications during surgery include pneumothorax, massive blood loss, cardiac injury or compression and arrhythmia. 

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References:
1- Yamanaka K, Higuma T, Watanabe K, Okada Y, Ichida F, Yoshimura N: Congenital sternal cleft. J Pediatr Surg. 47(11):2143-5, 2012
2- Torre M, Rapuzzi G, Carlucci M, Pio L, Jasonni V: Phenotypic spectrum and management of sternal cleft: literature review and presentation of a new series. Eur J Cardiothorac Surg. 41(1):4-9, 2012
3- Harijee A, Vijayaraghavan S, Marathi AR, et al: Complete Sternal Cleft Repair. Indian J Plast Surg. 53(3):419-422, 2020
4- Temur B, Mete S, Beken S, Onalan MA, Erek E: Complete sternal cleft treatment in a low birth weight patient. Turk Gogus Kalp Damar Cerrahisi Derg. 28(4):684-687, 2020
5- Tanriverdi HI, Doganeroglu F, Genc A, Yilmaz O: Primary closure of superior partial sternal cleft in a 2-month-old girl: case report. Ann Pediatr Surg. 2021;17(1):43. doi: 10.1186/s43159-021-00113-8.
6- Mongkornwong A, Kongpanichakul L, Tawaranurak N, Chansanti O, Chitithavorn V, Chuangsuwanich A: Autologous tissue reconstruction sternal cleft: a rare congenital malformation.  J Surg Case Rep. 4;2022(3):rjab550. doi: 10.1093/jscr/rjab550, 2022

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