PSU Volume 53 No 01 JULY 2019

TPN Peritoneal Extravasation

Umbilical venous catheters (UVC) in newborns provide intravascular access for administration of intravenous fluids, parenteral nutrition (TPN), drugs, transfusions and central venous monitoring especially for management of low birth weight infants. Improper placement of umbilical catheters  is associated with complications that may lead to morbidity and mortality. The tip of the umbilical venous catheter should be positioned at the junction of the inferior vena cava and right atrium just at or above the level of the diaphragm (T7 to T9). Also, adequate blood return must ne obtained before use. A normal positioned UVC between T7 and T9 can stay in use up to 14 days if intravenous access is in need, otherwise is advised to discontinue the UVC by day 10 and replace it with a percutaneously place intravenous central catheter. Improper placement of UVC can lead to intraperitoneal extravasation of TPN. This may also lead to vessel perforation or liver capsule disruption due to hepatic necrosis. The complication of intraperitoneal spillage has an insidious onset. The infant will develop abdominal distension, tenderness associated with acute pain, ascites, dehydration, hemoconcentration, characteristic induration of the abdominal wall due to infiltration of the soft tissue and acute renal failure from hypovolemia and hypertonicity of TPN. Abdominal ultrasound will demonstrate ascites. Paracentesis if performed reveals a cloudy fluid high in glucose, triglycerides and protein consistent with TPN. The differential diagnosis is chylous ascites with high chylomicrons and lymphocytes in a peripheral smear of fluid sample. Intraperitoneal TPN extravasation has also been reported after placement of femoral central a venous catheter. Management of TPN peritoneal extravasation should consist of catheter removal, paracentesis or peritoneal exploration to accomplished peritoneal lavage of the offending fluid. Prognosis is usually good if not associated with serious liver laceration with uncontrollable hemorrhage.

Alpha-1-Antitrypsin Deficiency

Alpha-1-antitrypsin is a glycoprotein produced primarily in the liver secreted under the influence of proinflammatory cytokines forming several globulins whose roles are to suppress additional tissue damage by neutralizing granulocyte elastase and proteinase primarily in the lung during infective and inflammatory process. Is responsible for protecting tissues against proteolytic damage by enzymes like neutrophil elastase and proteinase. It is also a tissue reparation inductor. Deficiency of alpha-1-antitrypsin (A1ATD) is a rare autosomal recessive co-dominant disorder most often seen in Northern European ancestry populations. A1ATD is the most common genetic cause of pediatric liver disease and transplantation. The defect in alpha-1-antitrypsin expression in the hepatocyte is followed by damage to the liver and/or lungs primarily. A1ATD can be asymptomatic or long-lasting and even permanently symptomatic depending in expression on the genotype and numerous exogenous factors such as infection, toxic or other damages to the liver and lungs. Liver damage occurs due to intrahepatocyte retention of alpha-1-antitrypsin and in the lung due to the lack of its protective effect. Early in life A1ATD causes a cholestatic syndrome, conjugated hyperbilirubinemia beyond the second week of life, associated with low birth weight and poor weight gain sometimes difficult to differentiate from biliary atresia. The liver damage is severe in only 1-2% of affected patients, most often has a slowly progressive character and juvenile cirrhosis develops in 15% of cases. In other organs A1ATD can cause pulmonary emphysema (chronic obstructive pulmonary disease), cytoplasmic anti-neutrophil cytoplasmic vasculitis and inflammatory necrotizing panniculitis in the skin. A1ATD individuals screened at birth who smoke develop COPD by age of 40 years. A1ATD is also associated with other diseases such as rheumatoid arthritis, sinusitis, nasal polyps, inflammatory bowel disease, peptic ulcer disease and diabetes. Management of A1ATD is limited. A diagnosis of A1ATD may have important implications including testing of family members, genetic counseling, smoking avoidance, avoidance of high risk occupations and consideration of augmentation therapy.

Thromboelastography

Coagulation disorders can lead to intra- or postoperative bleeding associated with increased morbidity and mortality. The transfusion of blood products to correct coagulopathy disorders is guided by clinical judgement, standard laboratory testing (PT, PTT, platelets, bleeding time, etc.), and thromboelastography testing. Thromboelastography (TEG) is a viscoelastic hemostatic assay, a functional test of clot formation and degradation performed on whole blood, at a point of care (emergency department, intensive care units or operating room) for clotting system deficiency that analyzes each phase of the coagulation process. Blood from a patient is mixed with citrate and placed in a cup of the TEG machine connected to a computer. The cup oscillates and the coagulation process occurs. This testing rapidly generates numeric and graphic results that can lead to guided-directed intervention for correct of coagulation disorders. Five variables are measured using thromboelastography. They are: R (reaction time) quantify as the time until measurable clot is formed, usually between five and 10 minutes. Should the patient have a prolonged R time then management is with fresh frozen plasma transfusion. K time - time since initial clot formation until it has a 20 mm fixed strength, normally one to 3 minutes. An abnormal K-time means low fibrinogen levels and is managed with cryoprecipitate. Alpha angle - is the speed of fibrin accumulation, similarly affected by fibrinogen levels. Normal alpha angle is 53-72 degrees. Again, an abnormal alpha angle is managed with cryoprecipitate. Maximum amplitude (MA) - consist of the highest vertical amplitude of the TEG tracing. MA reflects the strength of the clot normally reaching 50-70 mm. A narrow maximum amplitude is managed with platelets infusion. LY30 percentage - represent 30% decrease in the amplitude of the TEG tracing thirty minutes after maximum amplitude is obtained. It is a measure of fibrinolysis normally being zero and 8%. A higher than normal LY30 is indicative of a hypercoagulable state and may be managed with antifibrinolytics such as aminocaproic acid. TEG is superior to conventional coagulation testing in detecting early trauma coagulopathy reducing mortality. TEG should be available in trauma level I and  II centers.     

PSU Volume 53 No 02 AUGUST 2019

Xanthogranulomas

Xanthogranulomas are rare benign nodules that usually arise in the subcutaneous tissue. When they appear in early infancy or childhood they are called juvenile xanthogranulomas (JXG). Approximately 20% are present at birth and most arise in the first year of life. The anatomic predilection of appearance of juvenile xanthogranuloma in children occurs in the head and neck region, though appearance on the trunk, extremities and extracutaneous locations has also been reported. Skin lesions are self-limited and can vary in size; they can be solitary or multiple. In the eye, particularly the uveal tract, is the most frequent site of extracutaneous involvement. JXG are composed of collections of histiocytes, foamy cells and Touton giant cells. The diagnosis of JXG requires histologic analysis of a biopsy specimen or the nodule itself.  JXG is a benign cutaneous fibrohistiocytic lesion with a type of granulomatous process. Clinically the lesion is papulonodular with a tan to orange color and several millimeters in size. Children with multiple skin lesions or younger than two years of age are at a greater risk for ocular involvement. Systemic JXG which involves the visceral organs is rare occurring in less than 5% of cases. Systemic JXG can appear in the lung, heart, gastrointestinal tract, CNS, adrenal gland, pituitary gland, bones or kidney. There is a sex male predilection for JXG. Spontaneous regression of the cutaneous lesion is frequent. Differential diagnosis includes spitz nevi, mastocytomas, and dermatofibromas. Should the cutaneous nodule keep growing or cause functional impairment it should be removed surgically. Removal must be complete to avoid recurrence of the lesion. JXG generally carries a good prognosis.  Congenital giant JXG is a rare subtype most commonly affecting newborn females.

Cinematic Rendering

Detailed surgical anatomy provided by today imaging technology is essential in planning preoperative surgical decisions, surgical approach and ultimate management of many conditions. Virtual 3 dimensional (3-D) reconstruction techniques have been developed to help surgeons have a more detailed situation of the anatomy of the disease. Two types of 3-D rendering techniques are commonly utilized to visualize volumetric 3-D data: surface shade display and volume rendering. These techniques provide a global overview of volumetric data along with detailed anatomical and pathological information which is usually more difficult to discern in conventional 2-D images. In 2016 a new technique of 3-D visualization of cross sectional image data called cinematic rendering (CR) was developed. Cinematic rendering is a physically based volume-rendering method that works with random sampling computational algorithms. Multiple light maps and transfer function are used to generate a realistic depiction of medical imaging data. This computer animation program was first used by the entertainment industry. Data is retrieved from either conventional CT and MRI scans to produce the cinematic rendering images. CR helps to transfer complex surgical anatomical information to physicians. CR visualization improves the correctness of image interpretation. Moreover, CR enables the display of CT image data in a colored fashion. Also, a 3-D printed anatomical model can be reconstructed before the surgical procedure enabling the surgeon to study the precise anatomic relationship of the tumor with its vascular supply, an integral part of a surgical resection. Resident surgeons benefit more than attending surgeons when using CR imaging as it enhances anatomic education. CR imaging speeds up and improves the understanding of complex anatomical situations compared with conventional CT Scanning in the chest, abdomen and pelvis.   

ERAS

Enhanced recovery after anesthesia (ERAS) is a recently developed program of standardization of perioperative care driven by clinical pathways impacting both surgical outcomes and efficiency of care in high-volume centers. ERAS pathways have been shown to reduce length of stay, reduce complication rates, reduce providers communication errors and improve patient satisfaction in some adults surgical procedures such as colorectal surgery. Few studies have investigated the applicability of this paradigm to pediatric populations. The ERAS society initially developed in Europe organized the protocol into three contexts of care, namely preoperative, intraoperative and postoperative. This include a multidisciplinary team approach encompassing patient education, increased preop volume loading, optimize nutrition, appropriated antibiotic prophylaxis, short-acting anesthetics, maintenance of normothermia, minimally invasive surgical techniques, avoiding drains, using epidural for chest procedures, minimizing opioids analgesics, limiting intra- and postop fluid resuscitation, early removal of drains and catheters, prevention of nausea and vomiting, early oral nutrition, early postop mobility and reporting outcomes. ERAS programs in adults have reduced length of stay by one to two days without increasing readmission rates, decrease postop complications by 30% and shortened return of bowel function by one day. ERAS protocols offer an easy way to improve the cost of care. ERAS protocol applied to the appropriate pediatric surgical populations may be associated with decreased length of stay, decreased narcotic use, and no detectable increase in complications. Pediatric studies using ERAS have demonstrated less intervention than the 20 components suggested for adults. These few pediatric studies have demonstrated that ERAS implementation reduces length of stay by one day with no increase in complications or readmissions, less use of IV fluid in the operating room, less narcotic during hospitalization with early tolerance of feedings. Shorter stay becomes a patient safety benefit as errors and nosocomial infections are reduced upon early discharge. ERAS protocol is well accepted by patients and families. Recent poll demonstrated that pediatric surgeons are already executing several individual ERAS interventions outside of an official protocol.

PSU Volume 53 NO 03 SEPTEMBER 2019

Aspergillus Appendicitis

Fungi in very rare situations, mostly immunosuppressed children, can cause appendicitis. Aspergillus  is a widespread fungus identified in the environment and usually enter the human body by airborne transmission colonizing the nasal cavities or fascial sinuses. Isolated gastrointestinal aspergillosis arises from ingestion of food contaminated with Aspergillus and colonization by Aspergillus of gastrointestinal ulcers which arise from previous chemotherapy. The diagnosis of isolated (primary) aspergillus appendicitis is very rare, is delayed and associated with profound immunosuppression. This last factor of immunosuppression causes the delay in the clinical features of appendicitis. The very few cases have demonstrated a clinical pentad associated with this disease, namely: clinically-suspected appendicitis, profound neutropenia, recent chemotherapy, acute leukemia (either AML or ALL), and poor clinical course if managed solely with antibiotics or anti-candida medication. Aspergillus fumigatus is the most common and frequent species that causes infection in humans followed by A. Flavus and A. terreus. As mentioned, ingested contaminated food or mucosal ulcers from chemotherapy give rise to Aspergillus infestation. Aspergillus invade the appendicial mucosal wall due to immunosuppression from neutropenia and acute leukemia. The child develops persistent right quadrant pain, fever and systemic GI signs unresponsive to antibacterial or anti-candidal therapy. Ultrasound or with greater accuracy a CT-Scan will show appendicitis. The appendix should be removed promptly and the specimen microscopically examined for Aspergillus with special stains. Anti-Aspergillus therapy with voriconazole or amphotericin should be instituted after removal of the appendix since the child will continue to be colonized with the organism. Surgery is crucial for removing the inflamed appendix, clearing the infection and producing tissue sampling. Positive galactomannan levels guide the decision to change the antifungal therapy regime.  

Eosinophilic Cellulitis

Eosinophilic cellulitis, also known as Well's syndrome, is a rare inflammatory skin disorder of unknown etiology.  Its categorized as a relapsing eosinophilic dermatitis with variable clinical appearance. Cutaneous lesions are variable in appearance and may be similar to cellulitis, urticaria, insect bites, morphea or contact dermatitis. During the acute phase the child develops tender urticarial plaques, vesicles, bullae or nodules in the skin. This is usually accompanied with peripheral blood eosinophilia. Papulovesicular blistering and nodular lesions which are often painful or pruritic can also be seen. Later the skin lesions become indurated with morphealike appearance resolving without significant scarring. Histopathologic findings of the lesion include eosinophilic granulomatous infiltration of the dermis and formation of flame figures without signs of vasculitis. Hypersensitivity due to different triggers such as insect bites, or stings, drugs, infections, atopic dermatitis and contact dermatitis have been proposed. Causative medications include antibiotics, anticholinergic agents, anesthetics, non-steroidal anti-inflammatory agents, thyroid medications, chemotherapeutic agents, thiomersal containing vaccination, anti-tumor necrosis factor agents and thiazide diuretics. Children with sensitivity to thiomersal found in certain vaccines (such as influenza) can develop eosinophilic cellulitis. Diagnosis of the condition might need biopsy for histopathological identification. Other times the lesions coalesces and cause a subcutaneous abscess which need to be drained. Well's syndrome is initially managed successfully with topical or systemic corticosteroids and calcineurin inhibitors but it often relapse upon tapering. Systemic glucocorticosteroid are the most common and effective treatment modality reported.   

Appendicitis in the Neutropenic Child

With advancement in chemotherapy many children now survived to leukemia, lymphoma and other malignancies. The use of chemotherapeutic agents is associated with bone marrow toxicity and development of neutropenia along with infectious complications in the gastrointestinal tract. Right quadrant pain in the neutropenic child can be the direct consequence of acute appendicitis, neutropenic typhlitis, pseudomembranous colitis or obstructive ileus. Both appendicitis and neutropenic colitis are the most common surgical complications in children with leukemia. The use of steroids in these patients can masquerade acute appendicitis since the blunt the classic signs of appendicitis such as abdominal tenderness, rebound tenderness, involuntary guarding and abdominal wall rigidity. This causes confusion in the clinician trying to determine of the child has an acute abdominal condition or his symptoms are the result of side effects of chemotherapy. The incidence of appendicitis in the population of chemotherapy-induced neutropenia children is the same to the general pediatric population (2% - 4%). The clinical presentation and findings on physical exam of neutropenic children diagnosed with appendicitis are often vague and atypical. Since the most important decision is to differentiate acute appendicitis from neutropenic colitis children with right lower abdominal pain, fever and CT Scan findings of cecal wall thickness carries a presumptive diagnosis of typhlitis. In the absence of these findings acute appendicitis should be suspected and managed accordingly. Limited operative morbidity in neutropenic children with appendicitis leads to favor surgery over medical antibiotic therapy with delayed intervention. Medical management of appendicitis in the child with neutropenia increase hospital stay and use of pain medication. The majority of the children managed nonoperatively had recurrence of their RLQ pain on subsequent rounds of chemotherapy. Early appendectomy once the diagnosis is confirmed is safe and eliminates the course of the disease in addition to avoiding the concern for uncontrolled intraperitoneal contamination in the immunosuppressed child.

PSU Volume 53 NO 04 OCTOBER 2019

Earlobes Keloids

Keloids and hypertrophied scars are benign fibroproliferative cosmetic deformities of a wound original border after injury or trauma. Keloids are the result of excessive collagen deposition in genetically and/or environmental predisposed populations. Almost 70% of keloids or hypertrophied scars occur in children. Keloids occur in dark skin individuals (African American), are associated with familiar history and related to hormonal changes caused by puberty. The earlobe is the most common location for keloid development after injury caused by piercing. Incidence of earlobe keloid development after ear piercing is around 2.5%. During development and afterward keloids causes pain, pruritus, tenderness, and cosmetic disfigurement impairing the quality of life of the patient. The child with a keloid is affected socially and psychologically. Earlobe keloids affect both genders equally after ear piercing. The recurrence rate after simply excising keloids is as high as 80%, the reason for using other adjuvant therapies such as corticosteroid injection (Kenalog), cryotherapy, cryosurgery, laser therapy and radiotherapy. The majority of these treatment managements are associated with frequent recurrences. The median time for recurrence after excision is six months, meaning that long follow-up is needed to identify those cases that will eventually recur. Intralesional corticosteroid placement is considered a main stay of therapy of keloids owing to the ease of use and high degree of endurance. Mechanisms of action of steroids include suppression of inflammation, promotion of collagen degeneration and inhibition of further collagen production. Side effects of intralesional steroid therapy include cutaneous atrophy, skin hypopigmentation and pain associated with the injection. Radiotherapy, though expensive, claims local control after excision in more than 80% of patients. Radiation reduces recurrence after excision by reducing cellular proliferation. The preferred approach to pediatric earlobe keloids is excision with closure using absorbable sutures and concomitant steroid injection.      

CBD Dilatation After Cholecystectomy

With the current use of US for different intraabdominal symptoms physician has found that common bile duct dilatation (CBD) can occur after a cholecystectomy. Whether this finding is found in patients who are asymptomatic or with symptoms it brings the difficult situation if the dilatation found incidentally is pathological or not. This brings further imaging studies to elucidate this controversy such as MRCP or even ERCP in these patients. Studies that are either expensive or invasive. It is believed this dilatation of the CBD in post cholecystectomy patients is a physiologic response caused after the reservoir ability of the gallbladder with its removal is lost. Also, an increase in the intraluminal pressure of the CBD can be an etiological factor. Many cross-sectional studies reveal that the CBD diameter increases in gallbladder resected patients compared with normal people. The dilatation of the CBD can be more than 6 mm observed in 80% in the proximal part and 60% in the distal part of the CBD. This dilatation after cholecystectomy seems to increase with the increase in age of the patient. Eastern populations have shown that the CBD dilatation is frequently accompanied by ampullary diverticulum, biliary sludge or abnormal pancreatico-biliary anatomy. Most cases of CBD dilatation after cholecystectomy are asymptomatic and have normal liver function tests and total bilirubin. The patient sex, body weight and height, diagnosis and bile duct diameters do not have an effect on the postoperative common bile duct dilatation. A CBD dilatation within 10 mm in asymptomatic adult patients after cholecystectomy is permissible, can be regarded as normal physiologic changes and does not warrant further studies. This data has not been elucidated in post-cholecystectomy children yet. In children the normal diameter of the CBD depends on the age of the patient. Should the child present with an asymptomatic dilatation of the CBD an MRCP should be performed.

Urogenital Sinus

Urogenital sinus (UGS) refers to persistent anatomic defect where there is a normally placed anus, but the bladder and vagina share a common channel orifice. The vagina and urethra might join anywhere along a spectrum from the bladder neck, with a common channel (high confluence) to those that join near the perineum with a short common UGS (low confluence). Urogenital sinus is a common feature of a variety of congenital anomalies such as congenital adrenal hyperplasia (most common), isolated malformation unrelated to masculinization or rectal malformation and persistence of a cloacal malformation. The vast majority of children with UGS abnormalities have genital ambiguity and are thus identified as neonates. Hydrocolpus is relatively common and affected infants may demonstrate other syndromic features. This ambiguity is most commonly secondary to congenital adrenal hyperplasia (CAH). The virilization of children with CAH cause clitoral enlargement (clitoromegaly) and UGS. The larger the clitoris the longer the UGS confluence. In order to provide management, the child with a UGS need to undergo specific and detail imaging of the anatomy of the defect. This includes pelvic US, MRI (including evaluation of spinal cord anomalies), CT-Scan and VCUG. An endoscopy of the sinus tract is imperative before embarking on repair. The goal of surgical correction of the UGS is the creation of separate openings in the vulva for the urethra and vagina with preservation of the function of both organs. The relationship of the vagina to the bladder neck is the most critical determining factor in the type of vaginoplasty to be performed. Using a perineal approach either posterior vaginoplasty for short confluence defects is utilized or partial/total urogenital mobilization for high confluence defects is required to achieve such goals. Timing of surgery is controversial. Cases with recurrent urinary tract infection and significant Hydrocolpus should be reconstructed before the age of one year. Complications include urinary incontinence, vaginal stenosis and fistula between the urethra and the vagina. Fortunately, these complications occur infrequently. 

PSU Volume 53 No 05 NOVEMBER 2019

Intercostal Cryoanalgesia

Intercostal cryoanalgesia refers to the localized freezing of intercostal peripheral nerve through the application of a cryoprobe which achieves freezing temperatures. Intercostal cryoanalgesia achieved by application of a cryoprobe at -60 degree C for two minutes to the intercostal nerve to induce axonotmesis. When the intercostal nerve axon is frozen, the transmission of an electrical signal along the axon is prevented providing analgesia to that dermatome. Thereafter Wallerian degeneration of axons occurs starting at the point of injury and moving toward the nerve endings. Axonal regeneration eventually occurs since the fibrous neural structures including the perineurium and epineurium are preserved. Axonal regeneration occurs at a rate of one to 3 mm/day. This regeneration process is completed in approximately 4-6 weeks after freezing injury. Intercostal cryoanalgesia has been utilized for the acute and chronic control of pain after thoracotomy. In children intercostal cryoanalgesia is used during bar placement for the Nuss procedure of pectus excavatum deformities. The greatest advantage of using peripheral nerve freezing for pain control during the Nuss procedure is the long period of analgesia provided to the child. Cryoanalgesia provides pain control for a maximum of two months until axonal regeneration occurs. The clinical experience to date demonstrates a significant reduction in length of hospital stay and decrease postoperative opioid requirements in pectus excavatum patients managed with intercostal cryoanalgesia when compared to thoracic epidurals. Transient post-cryoanalgesia intercostal neuralgia is reported to occur in up to 20% of patients and may be the result of an incomplete cold temperature axonal injury. A minority of patients does complain about persistent paresthesia long term. Ultrasound-guided percutaneous intercostal analgesia for mastectomy has also demonstrated a potential benefit of long-term pain control for patients.

Bronchopleural Fistula

Bronchopleural fistula (BPF) is a sinus tract communication between the main stem, lobar or segmental bronchus and the pleural space. BPF is considered a serious complication after pneumonectomy or any other pulmonary resection. Other common causes of BPF include after pulmonary infection causing necrosis, persistent spontaneous pneumothorax, chemotherapy or radiotherapy from malignancy or tuberculosis. BPF are classified as early if it develops during the first seven days after the insult, intermediate if they occur between eight and 30 days and late if they present after 30 days. The list of causes of BPF is numerous but most cases occur after lung resection (pneumonectomy, lobectomy or segmentectomy). Postoperative BPF is classified as acute, subacute or chronic. Symptoms can include tension pneumothorax, asphyxiation, dyspnea, hypotension, subcutaneous emphysema, cough with expectoration, tracheal or mediastinal shift, persistent air leak and a reduction in pleural effusions in chest films. Chronic cases develop more insidious symptoms such as failure to thrive, malaise and fever. If a BPF occurs early in the postop period it is usually caused by mechanical failure of bronchial stump closure and requires urgent surgery as symptoms can be catastrophic. Most children with BPF present symptoms in the first two weeks after lung resection. Diagnosis of BPF is made using clinical, radiographic and bronchoscopic findings that confirm air leak from a bronchus to the pleural space. CT Scan can reveal the fistulous tract between the bronchus and pleura. Initial management of BPF is chest tube drainage of air and fluid from the pleural cavity. Suture closure of the opened bronchial stump using a vascularized flap coverage with video-assisted thoracoscopic approach is curative for the BPF presenting in the first two weeks. Other times the BPF can be closed using a muscle flap to fill the pleural space through a formal thoracotomy. Children with mechanical ventilation have a more complicated disease course due to the persistent air leak and incomplete lung expansion. BPF cause significant morbidity, prolonged hospitalization and mortality.     

Appendiceal Stump Bleeding

Lower gastrointestinal bleeding arising from the appendix is an extremely rare condition. Several pathologic conditions have been reported to be related to bleeding from the appendix such as Crohn's disease, appendicitis, intussusception, angiodysplasia, neoplasm, endometriosis, erosion of the appendiceal mucosa and stump granuloma. The use of CT-Scan in conjunction with colonoscopy may aid in making an accurate diagnosis with regard to appendiceal bleeding. Gastrointestinal bleeding from the appendiceal stump after appendectomy is also extremely rare. The bleeding may drain into the peritoneal cavity, the retroperitoneum or into the lumen of the bowel presenting as hematochezia or melena. The cause of the bleeding is usually a small intramural branch of the appendiceal artery at the appendiceal stump. Appendiceal bleeding may be managed by endoscopic clipping, arterial embolization or surgical cecal resection if massive. Granulomatous appendicitis characterized by appendicular granulomas can also present with lower gastrointestinal bleeding. Granulomas are chronic inflammatory lesions consisting of clusters of epithelioid histiocytes accompanied by multinucleated giant cells and lymphocytes. This condition accounts for less than 2% of all appendicitis and only 5010% of them develop Crohn's disease. Subacute appendicitis produces granulomatous reaction in relation to a secondary inflammatory response to acute appendicitis or phlegmonous process managed conservatively with postponed appendectomy. The definitive management of granulomatous appendicitis is appendectomy with a good long term prognosis. Appendiceal stump granuloma bleeding can be managed expectantly since as soon as the inflammatory process subsides the bleeding will stop.  

PSU Volume 53 NO 06 DECEMBER 2019

Choledocholithiasis in Sickle Cell Disease

Sickle cell disease (SCD) is a vaso-occlusive hemolytic anemia that causes pigmented gallbladder and common bile duct stones (choledocholithiasis) due to increase red blood cell destruction. Elevated serum bilirubin is produced from the breakdown of heme release from hemolyzed erythrocytes. Choledocholithiasis can occur concomitantly with gallstones (secondary) or years after removal of the gallbladder (primary). Most cases (95%) of bile ducts stones in SCD are from migration of gallstones from the gallbladder. Diagnosis of primary common bile duct stones depends on the following criteria: 1) previous removal of the gallbladder with or without bile duct exploration, 2) at least two year asymptomatic period after surgery and 3) no evidence of biliary stricture from surgery. Once cholelithiasis is diagnosed cholecystectomy in children with SCD should be performed to avoid the morbidity associated such as cholecystitis, biliary colic, pancreatitis, bile duct obstruction and cholangitis. The SCD child with gallstones and bile duct obstruction, bile duct dilatation in US, pancreatitis, elevated total bilirubin and alkaline phosphatase or cholangitis should undergo an MRCP to rule out choledocholithiasis. If bile duct stones are diagnosed then an ERCP with sphincterotomy and stone extraction is the next step in management. The advantage of the preoperative ERCP is that the bile ducts obstruction is cleared reducing chance of biliary infection. This is then followed by laparoscopic cholecystectomy.  Should ERCP fails to clear the common bile duct of stones and the obstruction persists laparoscopic cholecystectomy with exploration of the bile duct and stone retrieval is in order. Recurrent biliary tract disease is a frequent complication of SCD (20% by age 4 years) and often presents as common bile duct obstruction by stone despite cholecystectomy. Increased perioperative complications may result from vaso-occlusion after transient hypoxia, hypothermia, dehydration and acidosis. Inadequate postoperative pain management may reduce respirator effort leading to poor pulmonary toilet, hypoxia and acute chest syndrome from intrapulmonary vaso-occlusive crisis.

Congenital Uterovaginal Prolapse

Uterovaginal prolapse (UVP) occurring in the neonatal period is very rare. Uterovaginal prolapse refers to the downward descent with protrusion of the uterus and vagina to the exterior through the introitus. Uterovaginal prolapse occurs due to weakness of the cardinal ligaments and uterosacral ligaments that provide support to the uterus and vagina. Most cases are associated with congenital anomalies of the spine such as spina bifida occulta or myelomeningocele due to maldevelopment of sacral innervation to the levator ani muscle (perineal branch of the sacral nerve). Congenital UVP is also seen in babies with congenital cutis laxa. Congenital UVP manifest symptoms at birth or within the first few days of life. A few cases are caused by abnormal stress such as prolonged labor in breach condition, birth trauma or abnormality of the cervix. Diagnosis is clinical: pink-fleshy mass is seen protruding from the introitus with circumferential prolapse of the vaginal wall. The external cervical os is usually seen at the upper end of the lesion with a normal appearing urethral orifice. Differential diagnosis includes vaginal polyp, urethral prolapse, paraurethral cysts and rhabdomyosarcoma botryoides. CT or MRI is diagnostic and helps clear the differential diagnosis. Management of congenital uterovaginal prolapse is conservative with single or repeated manual reduction of the defect. Hypertonic saline packs or vaginal pessaries have also been utilized. As genital edema subsides the prolapse is usually maintained in place. Surgical intervention is indicated when conservative management fails, when the prolapse becomes recurrent after repeated reductions or when there is evidence of vaginal mucosal hypertrophy or ulceration. These include fixation of the uterus anteriorly to the urinary bladder with nonabsorbable sutures using a small suprapubic incision, sling or sacral cervicopexy. Inflated Foley catheter into the vaginal orifice after reduction for a period of two weeks can also successfully correct the uterovaginal prolapse.     

Cushing's Syndrome

Cushing syndrome refers to the constellation of signs and symptoms associated with excessive production of cortisol. Cushing syndrome (CS) is caused by an ACTH producing pituitary or ectopic tumor (ACTH-dependent) or an adrenal adenoma/carcinoma (ACTH-independent). The child with CS develops hypertension, diabetes mellitus, hypokalemia, alkalosis, bone loss, fractures and psychiatric disturbances. CS patients suffer from infection, myocardial infarction or venous thrombosis. The primary source of ACTH should be eliminated as two-thirds of deaths are related to CS cortisol excess. Effective management includes normalization of cortisol levels or inhibiting its receptor to eliminate signs and symptoms of CS even if there is no established diagnosis. It also includes normalizing comorbidities using adjunctive therapy. Medical therapy with steroidogenesis inhibitors (metyrapone and ketoconazole) can reduce plasma cortisol levels.  It is recommended that initial resection of primary lesions underlying Cushing disease, ectopic and adrenal etiology be performed to reduce glucocorticoid excess. These include resecting unilateral adrenal disease, ectopic ACTH-secreting tumors with node dissection, transsphenoidal selective removal of pituitary tumors and surgical resection of bilateral adrenal disorders. Most cases (90%) of pediatric CS are due to pituitary adenomas and they are primarily managed with trans-sphenoidal surgery. ACTH-independent causes of CS in children are less than 10% and comprises adrenal cortex adenomas or hyperplasia. Laparoscopic surgical resection of disease adrenal gland whether unilateral or bilateral is highly and rapidly effective in eliminating the source of cortisol. Infantile ACTH-independent CS is very rare and usually reported as part of Mc-Cune Albright (large adrenal glands due to macro-nodular hyperplasia) or Carney complex disease (pigmented nodular hyperplasia). Failed pituitary management of CS or selected patients with ACTH-dependent CS from unmanageable primary source with reasonable life expectancy may benefit from medical therapy with inhibitors plus bilateral removal of the adrenal glands for control of symptoms.