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PEDIATRIC SURGERY UPDATE VOLUME 36, 2011

PSU Volume 36 No 01 JANUARY 2011
Laparoscopic Rectopexy
Full-thickness rectal prolapse is a relatively comm on and distressing condition in children that fortunately is usually self-limiting in the life of the affected child and family. Peak incidence is between one and three years of age. Medical management improves more than 80% of all cases of rectal prolapse. Patients are managed for the underlying cause of the prolapse such as chronic constipation or acute diarrhea, while cystic fibrosis is rule-out. Failed medical therapy and recurrent prolapse will be managed with surgery. Before surgical intervention the rectum should be evaluated with imaging studies (Barium Enema) and flexible endoscopy. Therapeutic surgical procedures include submucosal injection of sclerosant, Thiersch cerclage, open abdominal rectopexy with or without sigmoid resection and perineal rectopexy. Rectopexy alone for megarectum or megasigmoid is not appropriate and will eventually need resection of the dilated bowel. With the use of minimally invasive techniques, laparoscopic suture rectopexy namely, full posterior mobilization and fixation to the sacrum, has emerged as an alternative surgical management for recurrent rectal prolapse in children. No preoperative bowel preparation is required while the procedure can be done ambulatory with a low morbidity, low recurrence rate and excellent cosmetic results. Laparoscopic rectopexy has also been found to improve constipation and fecal incontinence.
References: 1- Demirbas S, Akin ML, Kalemoglu M, Ogn I, Celenk T: Comparison of laparoscopic and open surgery for total rectal prolapse. Surg Today. 35(6):446-52, 2005 2- Vijay V, Halbert J, Zissimopoulos A, Siddiqi S, Warren S: Day case laparoscopic rectopexy is feasible, safe, and cost effective for selected patients. Surg Endosc. 22(5):1237-40, 2008 3- Byrne CM, Smith SR, Solomon MJ, Young JM, Eyers AA, Young CJ: Long-term functional outcomes after laparoscopic and open rectopexy for the treatment of rectal prolapse. Dis Colon Rectum. 51(11):1597-604, 2008 4- Ismail M, Gabr K, Shalaby R: Laparoscopic management of persistent complete rectal prolapse in children. J Pediatr Surg. 45(3):533-9, 2010 5- Shalaby R, Ismail M, Abdelaziz M, Ibrahem R, Hefny K, Yehya A, Essa A: Laparoscopic mesh rectopexy for complete rectal prolapse in children: a new simplified technique. Pediatr Surg Int. 26(8):807-13, 2010 6- Dean Potter D, Bruny JL, Allshouse MJ et al: Laparoscopic suture rectopexy for full-thickness anorectal prolapse in children: an effective outpatient procedure. J Pediatr Surg 45(10): 2103-2107, 2010

Renal Leiomyoma
Leiomyomas are characterized as benign mesenchymal tumor that arise from smooth muscle cells. There most common location is the uterus and gastrointestinal tract, though they can rarely arise anywhere smooth muscle cells exist. Wilms tumor is still the most common renal tumor in children. Renal leiomyoma is a very rarely found in children. The leiomyoma of kidney can arise from the renal capsule (most common site), renal pelvis and calyces, or the renal cortical vasculature. Most cases of renal leiomyoma are found in adults at a median age of 40 years with female
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predominance. In children the rare cases have been found incidentally. When symptomatic the tumor has enlarged significantly causing pain, a palpable mass and hematuria. Alport syndrome has been associated with diffuse leiomyomatosis, including the genitourinary tract. Diagnostic studies include abdominal US (solid mass) and CT-Scan (well circumscribed homogenous enhanced mass). When very large they are undistinguishable from nephroblastomas. Managements consist of total nephrectomy. An association between Epstein-Barr virus and smooth muscle tumors such as renal leiomyoma in immunocompromised patients has been recognized recently.
References: 1-Tawfik OW, Moral LA, Richardson WP, Lee KR: Multicentric bilateral renal cell carcinomas and a vascular leiomyoma in a child. Pediatr Pathol. 13(3):289-98, 1993 2- Belis JA, Post GJ, Rochman SC, Milam DF: Genitourinary leiomyomas. Urology. 13(4):424-9, 1979 3- Anker MC, Arnemann J, Neumann K, Ahrens P, Schmidt H, Knig R: Alport syndrome with diffuse leiomyomatosis.Am J Med Genet A. 119A(3):381-5, 2003 4- Dionne JM, Carter JE, Matsell D, MacNeily AE, Morrison KB, de Sa D: Renal leiomyoma associated with Epstein-Barr virus in a pediatric transplant patient. Am J Kidney Dis. 46(2):351-5, 2005 5- Gupta A, Chandra N, Sharma A, Husain N, Kureel SN: Renal leiomyoma in a child: a rare renal tumor. J Pediatr Surg. 45(9):1900-3, 2010

Acquired Undescended Testis

Undescended testes (UT) are recently categorized into congenital and acquired forms. In the congenital form the child is born with an undescended testis, while the acquired form is characterized for later development in life of cryptorchidism. The acquired form of undescended testes comprises the high rate of orchidopexies performed later in life in children. Is a condition in which a previously fully descended testis can no longer be manipulated into a stable scrotal position. Acquired UT are mostly situated in a superficial inguinal pouch, of normal size, with a normal attachment of the gubernaculum, and in half of the cases associated with an open processus vaginalis They are not associated with epididymal deformities or abnormal attachment of the gubernaculum. Acquired UT can be secondary to failure of natural elongation of cord structures in proportion to body growth due to complete disappearance of the processus vaginalis. In contrast the congenital variety of UT is proximal to the external spermatic ring (intracanalicular), a complete hernial sac is present, and is associated with epididymal deformities and abnormal attachment of the gubernaculum. Acquired UT has also been reported in children with cerebral palsy due to spasticity of the cremasteric muscle. Both forms of UT should be managed by orchiopexy to avoid the adverse effects on germ cell development and fertility potential. For congenital UT orchiopexy is recommended at 6-12 months of age. Postoperative results are better for the acquired form compared with the congenital variety.
References: 1- Barthold JS, Gonzlez R: The epidemiology of congenital cryptorchidism, testicular ascent and orchiopexy. J Urol. 170(6 Pt 1):2396-401, 2003 2- Meijer RW, Hack WW, van der Voort-Doedens LM, Haasnoot K, Bos SD: Surgical findings in acquired undescended testis. J Pediatr Surg. 39(8):1242-4, 2004 3- Hutson JM, Hasthorpe S: Testicular descent and cryptorchidism: the state of the art in 2004. J Pediatr Surg. 40(2):297-302, 2005 4- Hutson JM, Hasthorpe S: Abnormalities of testicular descent. Cell Tissue Res. 322(1):155-8, 2005 5- Sijstermans K, Hack WW, Meijer RW, van der Voort-Doedens LM: The frequency of undescended testis from birth to adulthood: a review. Int J Androl. 31(1):1-11, 2008 6- Meij-de Vries A, Hack WWM, Heij HA, Meijer RW: Perioperative Surgical Findings in Congenital and Acquired Undescended Testis. J Pediatr Surg 45(9): 1874-1881, 2010

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PSU Volume 36 NO 02 FEBRUARY 2011

Ethanol Sclerotherapy for Lymphangiomas
Lymphatic malformations are very difficult lesions to manage and eradicate in children. They constitute 6% of all benign lesions in infants and children. Head and neck are the more commonly affected regions. Most lesions will appear within the first two years of life of the child. The cystic lymphatic lesions are classified as macrocystic (> 1 cm), microcystic (< 1 cm) and mixed variety. Lymphatic malformations do not have a familial tendency and they do not become malignant with time. Surgical excision is the standard method of choice to manage lymphatic lesions that are localized and do not involve vital surrounding structures. Mutilating surgery is avoided at all costs. Diagnosis of the anatomic arrangements that these lymphatic malformations have with vital structure is obtained with the help of CT-Scan and MRI imaging. For unresectable or residual lymphatic cystic lesions the use of sclerotherapy has been advocated. Sclerosing agents include OK-432, Bleomycin, Fibrin glue, Doxycycline, 50% dextrose, Ethibloc (alcoholic solution of corn protein) and recently 98% Ethanol. Some agents are very difficult to obtained due to FDA regulation. Others cause toxic, allergic and irritant effects to the child. Sterile 98% ethanol produces minimal side effect, is easy to purchase and has a low cost. Ethanol sclerotherapy can be affected using CT-guided technology with the child under sedation or general anesthesia. Th volume of ethanol used for sclerotherapy should be no more than 1.0 ml/kg of weight.
References: 1- Saba C, Bossi MC, Barletta A: Therapy of subcutaneous cystic lymphangioma with ultrasound-guided puncture and alcoholization. Radiol Med. 83(3):270-2, 1992 2- Puig S, Aref H, Brunelle: Double-needle sclerotherapy of lymphangiomas and venous angiomas in children: a simple technique to prevent complications. AJR Am J Roentgenol. 180(5):1399-401, 2003 3- Alomari AI, Karian VE, Lord DJ, Padua HM, Burrows PE: Percutaneous sclerotherapy for lymphatic malformations: a retrospective analysis of patient-evaluated improvement. J Vasc Interv Radiol. 17(10):1639-48, 2006 4- Shiels WE 2nd, Kenney BD, Caniano DA, Besner GE: Definitive percutaneous treatment of lymphatic malformations of the trunk and extremities. J Pediatr Surg. 43(1):136-9, 2008 5- Shiels WE 2nd, Kang DR, Murakami JW, Hogan MJ, Wiet GJ: Percutaneous treatment of lymphatic malformations. Otolaryngol Head Neck Surg. 141(2):219-24, 2009 6- Impellizzeri P, Romeo C, Astra Borrut F, et al: Sclerotherapy for cervical cystic lymphatic malformations in children. Our experience with computed tomography-guided 98% sterile ethanol insertion and review of the literature. J Pediatr Surg. 45(12): 2473-2478, 2010

Ogilvie Syndrome
Acute colonic pseudo-obstruction also known as Ogilvie's syndrome is a massive colonic dilatation associated with signs and symptoms of colonic obstruction without an evident mechanical cause. Ogive syndrome is observed predominantly in the elderly population with few cases reported in children. Predisposing factors for Ogilvie's syndrome in children includes postoperative state, trauma, infections, Sickle cell disease, cardiac diseases and chemotherapy for malignancy. Symptoms include constipation, abdominal pain, nausea, vomiting and abdominal distension. Diagnosis is suggested in flat simple abdominal films. Findings on CT-Scan are diagnostic showing massive colonic dilatation with diameters of eight to 12 cm and without evidence of overt mechanical obstruction. If left untreated, this dilatation can lead to colonic perforation and peritonitis in 10% of children with high mortality rates. Initial management consists of nasogastric decompression, bowel rest, hydration, electrolyte correction, along with discontinuation of drugs
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affecting bowel motility. If symptoms fail to improve with initial management then rectal tube or colonoscopy decompression is utilized. Neostigmine, an acethycholinesterase inhibitor which increases parasympathetic tone, has been found to be very effective in managing patients with Ogilvie syndrome. Neostigmine is slowly titrated in increments up to a total of 0.05mg/kg of weight. Also, oral erythromycin therapy has been used to manage this condition. Surgery will be needed if the child develops perforation or signs of bowel ischemia.
References: 1- Hyman PE: Chronic intestinal pseudo-obstruction in childhood: progress in diagnosis and treatment. Scand J Gastroenterol Suppl. 213:39-46, 1995 2- Gmora S, Poenaru D, Tsai E: Neostigmine for the treatment of pediatric acute colonic pseudo-obstruction. J Pediatr Surg. 37(10):E28, 2002 3- Jiang DP, Li ZZ, Guan SY, Zhang YB: Treatment of pediatric Ogilvie's syndrome with low-dose erythromycin: a case report. World J Gastroenterol. 13(13):2002-3, 2007 4- Kim TS, Lee JW, Kim MJ, Park YS, Lee DH, Chung NG, Cho B, Lee S, Kim HK: Acute colonic pseudo-obstruction in postchemotherapy complication of brain tumor treated with neostigmine. J Pediatr Hematol Oncol. 29(6):420-2, 2007 5- Khosla A, Ponsky TA: Acute colonic pseudoobstruction in a child with sickle cell disease treated with neostigmine. J Pediatr Surg. 43(12):2281-4, 2008 6- Lee JW, Bang KW, Jang PS, Chung NG, Cho B, Jeong DC, Kim HK, Im SA, Lim GY: Neostigmine for the treatment of acute colonic pseudo-obstruction (ACPO) in pediatric hematologic malignancies. Korean J Hematol. 45(1):62-5, 2010

Colostomy Closure
Colostomy closure is a common and important surgical procedure performed in children which carries a significant risk of morbidity and mortality. Some complication associated with closure of a colostomy includes wound infection, anastomotic dehiscence, bleeding, anastomotic stricture, incisional hernia and death. The periostoma lymphatics are colonized with bacteria, reason why surgery site infection rises during this procedure. For colostomy closure most children should be admitted the day before surgery for mechanical cleansing of the proximal and distal bowel. Systemic antibiotics during anesthesia induction are necessary. Infection rate is not affected by the use of oral antibiotics. Meticulous surgical technique including packing of proximal stoma, use of plastic drapes for surgical field immobilization, correct dissection, careful hemostasis avoiding contamination and performing an anastomosis in well vascularized limbs are essential to reduce complications. Peritoneal irrigation, fascial closure in layers, adequate hemostasis and avoidance of dead spaces are also essential issues to watch for. Postoperative nasogastric tubes are not necessary. Early feeding is encouraged.
References: 1- Weber TR, Tracy TF Jr, Silen ML, Powell MA: Enterostomy and its closure in newborns. Arch Surg. 130(5):534-7, 1995 2- Sangkhathat S, Patrapinyokul S, Tadyathikom K: Early enteral feeding after closure of colostomy in pediatric patients. J Pediatr Surg. 38(10):1516-9, 2003 3- Chandramouli B, Srinivasan K, Jagdish S, Ananthakrishnan N: Morbidity and mortality of colostomy and its closure in children. J Pediatr Surg. 39(4):596-9, 2004 4- Breckler FD, Rescorla FJ, Billmire DF: Wound infection after colostomy closure for imperforate anus in children: utility of preoperative oral antibiotics.J Pediatr Surg. 45(7):1509-13, 2010 5- Bischoff A, Levitt MA, Lawal TA, Pena A: Colostomy closure: how to avoid complications. Pediatr Surg Int. 26(11):108792, 2010

PSU Volume 36 NO 03 MARCH 2011

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Intramural Duodenal Hematoma

In children, an intramural duodenal hematoma is a rare cause of proximal bowel obstruction and most commonly arises after blunt abdominal trauma. Common mechanisms include bicycle handlebar injuries, play or athletic injuries, child abuse and motor vehicle accidents. Other causes of intramural duodenal hematoma (IDH) are after endoscopic intestinal biopsy and spontaneously in children suffering from hemophilia, Henoch-Schnlein purpura and Glanzmann's thrombasthenia. The child develops epigastric abdominal pain, persistent vomiting with high output bowel obstruction and abdominal tenderness. Associated traumatic pancreatitis is found in 30% of cases due to the direct blow or associated with obstruction of the ampulla of Vater. CT contrast or MRI imaging will allow early and definite diagnosis. Treatment of IDH is conservative with bowel rest, nasogastric suction and parenteral nutrition. Ultrasound and UGIS can be used to follow resolution of the hematoma. Stricture formation, lief-threatening hemorrhage or perforation is extremely rare sequelae in children. Surgery drainage may be needed in case of complications or if prolonged nonsurgical management fails. Scattered report of effective laparoscopically surgical drainage, internal incision and drainage by endoscopy and ultrasonically guided aspiration drainage has been published in the literature. In general the prognosis after resolution is good.
References: 1- Thoms CA, Ricketts RR: Intramural duodenal hematoma in children: reappraisal of current management. South Med J. 81(8):985-8, 1988 2- Chittmittrapap S, Chandrakamol B, Chomdej S: Intramural haematoma of the alimentary tract in children. Br J Surg. 75(8):754-7, 1988 3- Voss M, Bass DH: Traumatic duodenal haematoma in children. Injury. 1994 May;25(4):227-30 4- Lin YC, Chen Y, Yeh SJ: Traumatic intramural hematoma of the duodenum: report of one case. Acta Paediatr Taiwan. 45(6):343-5, 2004 5- Iuchtman M, Steiner T, Faierman T, Breitgand A, Bartal G: Post-traumatic intramural duodenal hematoma in children. Isr Med Assoc J. 8(2):95-7, 2006 6- Banieghbal B, Vermaak C, Beale P: Laparoscopic drainage of a post-traumatic intramural duodenal hematoma in a child. J Laparoendosc Adv Surg Tech A. 18(3):469-72, 2008 7- Chien JH, Ho TY, Shih-Peng L, Lee CL, Ou SF: Acquired duodenal obstruction in children. Pediatr Neonatol. 49(5):193-6, 2008 8- Kwon CI, Ko KH, Kim HY, Hong SP, Hwang SG, Park PW, Rim KS: Bowel obstruction caused by an intramural duodenal hematoma: a case report of endoscopic incision and drainage. J Korean Med Sci. 24(1):179-83, 2009

Atypical Fibroxanthoma
Atypical fibroxanthoma (AFX) is an uncommon skin/subcutaneous tissue neoplasm generally found in elderly Caucasian men with sun-damaged skin. It also rarely appears in children. AFX arising in children tends to occur on the limb and trunk areas. AFX typically begins as a solitary firm erythematous nodule that grows rapidly and may become ulcerated. Most lesions are less than 2 cm. AFX follows a benign clinical course and it rarely metastasize. Histologically, AFX presents as a dermal nodule composed of haphazardly arranged spindle cells with multinucleated giant and xanthomatous histiocytes scattered throughout the tumor. AFX is characterized for its cellularity, lack of organization, abnormal mitotic figures, pleomorphism and lymphocytic infiltrate around the edges. Management of AFX consists of complete excision. Recurrence occurs at a rate of 7% with the majority occurring within one year of excision. Poorly circumscribed or irregularly shaped tumors with infiltrative edges on histology may require larger clinical margins because they account for the majority of recurrences. AFX has been reported to metastasize to regional lymph nodes.
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References: 1- Mirza B, Weedon D: Atypical fibroxanthoma: a clinicopathological study of 89 cases. Australas J Dermatol. 46(4):235-8, 2005 2- Lum DJ, King AR: Peritoneal metastases from an atypical fibroxanthoma. Am J Surg Pathol. 30(8):1041-6, 2006 3- Melendez MM, Xu X, McClain SA, Huang SI: Atypical fibroxanthoma in a young woman: An unusual case presentation. Can J Plast Surg.15(3):169-72, 2007 4- Stefanato CM, Robson A, Calonje JE. The histopathologic spectrum of regression in atypical fibroxanthoma. J Cutan Pathol. 37(3):310-5, 2010 5- New D, Bahrami S, Malone J, Callen JP: Atypical fibroxanthoma with regional lymph node metastasis: report of a case and review of the literature. Arch Dermatol. 146(12):1399-404, 2010

Abdominal Pulmonary Sequestration

A pulmonary sequestration is a piece of nonfunctioning pulmonary tissue with no communication with the normal bronchial tree supplied by a systemic artery. Usually in the thorax (90%) they are classified as intralobar and extralobar. An extralobar sequestration can also be found in the abdomen of a child. Intraabdominal pulmonary sequestration is typically located in the left suprarenal area. The extralobar sequestration has a separate pleural lining. Most of these extralobar sequestrations are asymptomatic and appear as an infradiaphragmatic mass. The diagnosis can be suggested antenatally using ultrasound. Differential diagnosis includes neuroblastoma, duplication, teratomas and adrenal hemorrhage. MRI, color Doppler and angio-CT Scan can help define the mass and identify the feeder blood vessel. Associated conditions include diaphragmatic hernia and congenital heart disease. Management of abdominal pulmonary sequestration is surgical excision. Removal can be accomplished either open or preferably by laparoscopy. Laparoscopic excision has been demonstrated to be safe and better cosmetically.
References: 1- Gross E, Chen MK, Lobe TE, Nuchtern JG, Rao BN: Infradiaphragmatic extralobar pulmonary sequestration masquerading as an intra-abdominal, suprarenal mass. Pediatr Surg Int. 12(7):529-31. 1997 2- Danielson PD, Sherman NJ: Laparoscopic removal of an abdominal extralobar pulmonary sequestration. J Pediatr Surg. 36(11):1653-5, 2001 3- van der Zee DC, NMa Bax K: Laparoscopic resection of intra-abdominal extralobar pulmonary sequestration. Pediatr Surg Int. 21(10):841-2, 2005 4- Laje P, Martinez-Ferro M, Grisoni E, Dudgeon D: Intraabdominal pulmonary sequestration. A case series and review of the literature. J Pediatr Surg. 41(7):1309-12, 2006 5- Agayev A, Yilmaz S, Cekrezi B, Yekeler E: Extralobar pulmonary sequestration mimicking neuroblastoma. J Pediatr Surg. 42(9):1627-9, 2007 6- Joyeux L, Mejean N, Rousseau T, Couaillier JF, Piard F, Sapin E: Ectopic extralobar pulmonary sequestrations in children: interest of the laparoscopic approach. J Pediatr Surg. 45(11):2269-73, 2010

PSU Volume 36 No 04 APRIL 2011

Malignant Hyperthermia Syndrome
Malignant hyperthermia syndrome (MHS) is a pharmacogenetic disorder of skeletal muscle presenting as a hypermetabolic response to potent volatile anesthetic gases such as halothane, sevoflurane, desflurane, the depolarizing muscle relaxant succinylcholine and rarely after stress of vigorous exercise and heat. MHS is inherited in autosomal dominant pattern. A defect in the ryanodine receptor in chromosome 19. A receptor located in the calcium channels of the skeletal muscle sarcoplasmic reticulum. Classic signs of MHS include marked-degree hyperthermia,
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tachycardia, tachypnea, increased carbon dioxide production, increase oxygen consumption, acidosis, muscle rigidity and breakdown (rhabdomyolysis) and myoglobinuria. Elevations of endcarbon dioxide and temperature are the initial clues to consider MHS. MHS occur due to uncontrolled release of myoplasmic calcium which activates skeletal muscle to a hypermetabolic state causing ATP depletion, compromised muscle membrane integrity and release of potassium and rhabdomyolysis. Children comprise less than 20% of all cases. Diagnosis is established with the halothane/caffeine contracture test. Management consists of supportive measures, temperature control and Dantrolene, a specific antagonist of the pathophysiologic changes in MHS. The actual incidence of MHS has increased and the mortality is more than 15% in the Unites States. The risk of MHS in children undergoing muscle biopsy for suspected neuromuscular disease is less than 1%.
References: 1- Hall SC: General pediatric emergencies. Malignant hyperthermia syndrome. Anesthesiol Clin North America. 19(2):367-82, 2001 2- Brandom BW: The genetics of malignant hyperthermia. Anesthesiol Clin North America. 23(4):615-9, 2005 3- Flick RP, Gleich SJ, Herr MM, Wedel DJ: The risk of malignant hyperthermia in children undergoing muscle biopsy for suspected neuromuscular disorder. Paediatr Anaesth. 17(1):22-7, 2007 4- Larach MG, Gronert GA, Allen GC, Brandom BW, Lehman EB: Clinical presentation, treatment, and complications of malignant hyperthermia in North America from 1987 to 2006. Anesth Analg. 110(2):498-507, 2010 5- Sumitani M, Uchida K, Yasunaga H, Horiguchi H, Kusakabe Y, Matsuda S, Yamada Y: Prevalence of malignant hyperthermia and relationship with anesthetics in Japan: data from the diagnosis procedure combination database. Anesthesiology. 114(1):84-90, 2011

Tumor Lysis Syndrome

Tumor lysis syndrome (TLS) refers to a group of metabolic derangements caused during abrupt and massive release of cellular components into the blood after rapid lysis of malignant cells. This event occurs after the child receiving cytotoxic, cytolitic antibiotics or radiation therapy usually in malignancies such as leukemias, Burkitt's lymphoma and other tumors with high proliferative rate, large tumor burden or high sensitivity to chemotherapy. The release of such components and intracellular metabolites can lead to hyperuricemia, hyperkalemia, hyperphosphatemia, hypocalcemia and uremia known as TLS. The precipitation of uric acid can lead to impaired acute renal failure if not managed adequately. TLS can occur during an operation. Clinical manifestation includes nausea, vomiting, diarrhea, anorexia, lethargy, edema, fluid overload, hematuria, CHF, dysrhythmias, seizures, cramps, tetany and death. Management may consist of aggressive fluid hydration to enhance urine flow (considered the most effective strategy), alkalinization of the urine, blocking the conversion of xanthine and hypoxanthine to uric acid with allopurinol, and promoting the catabolism of uric acid with recombinant urate oxidase. Depending on risk stratification, the treatment above is instituted. Hyperphosphatemia, hyperkalemia and hypocalcemia should also be treated. The best management of TLS is prevention.
References: 1- Truini-Pittman L, Rossetto C: Pediatric considerations in tumor lysis syndrome. Semin Oncol Nurs. 18(3 Suppl 3):17-22, 2002 2- Lee MH, Cheng KI, Jang RC, Hsu JH, Dai ZK, Wu JR: Tumour lysis syndrome developing during an operation. Anaesthesia. 62(1):85-7, 2007 3- Coiffier B, Altman A, Pui CH, Younes A, Cairo MS: Guidelines for the Management of Pediatric and Adult Tumor Lysis Syndrome: An Evidenced-Based Review. J Clin Oncol 26(16): 2767-2778, 2008 4- Zonfrillo MR: Management of pediatric tumor lysis syndrome in the emergency department. Emerg Med Clin North Am. 27(3):497-504, 2009 5- Cairo MS, Coiffier B, Reiter A, Younes A; TLS Expert Panel: Recommendations for the evaluation of risk and prophylaxis
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of tumour lysis syndrome (TLS) in adults and children with malignant diseases: an expert TLS panel consensus. Br J Haematol. 149(4):578-86, 2010 6- Agrawal AK, Feusner JH: Management of tumour lysis syndrome in children: what is the evidence for prophylactic rasburicase in non-hyperleucocytic leukaemia? Br J Haematol. Jan 23, 2011

Branchio-Oto-Renal Syndrome
The Branchio-oto-renal (BOR) syndrome is a rare autosomal dominant disorder with incomplete penetrance and extremely variable phenotypic expressivity. Main clinical features are due to congenital abnormal development of the first and second branchial arches and urinary tract. Others anomalies include early hearing impairment, preauricular pits, deformity of pinna, external auditory canal stenosis, branchial fistula and renal anomalies. Renal anomalies are always present and consist of agenesis, hypoplasia or renal dysplasia, ureteropelvic junction obstruction, vesicoureteral reflux and calyceal diverticula. There also can be bifid kidneys with double ureters and calyceal anomalies. Renal agenesia and dysplasia are the causes of end-stage renal disease in these children. Bilateral renal agenesis is the extreme, leading to a miscarriage or immediate neonatal death. The syndrome gene maps to chromosome 8q13.3 called the EYA1 gene. The BOR syndrome should be included in the differential diagnosis of deafness and chronic renal failures in children.
References: 1- Pierides AM, Athanasiou Y, Demetriou K, Koptides M, Deltas CC. A family with the branchio-oto-renal syndrome: clinical and genetic correlations. Nephrol Dial Transplant. 17(6):1014-8, 2002 2- Schiff M, Parchoux B, Cochat P. Ear and kidney malformations with renal failure in an infant: what is the link? Nephrol Dial Transplant. 18(8):1673-4, 2003 3- Kulkarni ML, Deshmukh S, Kumar A, Kulkarni PM. Branchio-oculo-facial syndrome. Indian J Pediatr. 72(8):701-3, 2005 4- Li Y, Manaligod JM, Weeks DL. EYA1 mutations associated with the branchio-oto-renal syndrome result in defective otic development in Xenopus laevis. Biol Cell. 17;102(5):277-92, 2010 5- Morisada N, Rendtorff ND, Nozu K, Morishita T, Miyakawa T, Matsumoto T, Hisano S, Iijima K, Tranebjaerg L, Shirahata A, Matsuo M, Kusuhara K. Branchio-oto-renal syndrome caused by partial EYA1 deletion due to LINE-1 insertion. Pediatr Nephrol. 25(7):1343-8, 2010

PSU Volume 36 No 05 MAY 2011

Ehlers-Danlos Syndrome
Ehlers-Danlos syndrome (EDS), also known as cutis hyperelastica, is a group of inherited connective tissue disorders caused by a defect in the synthesis of collagen. Depending on the individual mutation the severity of the syndrome can vary from mild to severe. Several types of EDS have been categorized with type 4 considered the most dreadful considering the high propensity of these individuals to develop life-threatening arterial and digestive complications. EDS type 4 is an autosomic dominant defect (missense mutation in the COL3A1 gene) characterize by a fascial acrogeria appearance (large eyes, small chin, thin nose, lobeless ears), small stature with slim build, and thin pale translucent skin. Children with EDS have poor wound healing, hypermobile joints, clotting anomalies, spontaneous pneumothorax and recurrent hernias. Among the catastrophic events associated with type 4 EDS we can find arterial dissection or tear caused by deterioration of congenital fragile tissue leading to hematoma, false aneurysm and intracavitary bleeding. They are responsible for the majority of deaths. The next set of complications are spontaneous and recurrent perforation of the colon associated with a significant risk of leakage
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after anastomosis and spontaneous perforation or bleeding of the uterus. There is no cure for EDS and management is supportive.
References: 1- Liem MS, van der Graaf Y, Beemer FA, van Vroonhoven TJ: Increased risk for inguinal hernia in patients with EhlersDanlos syndrome. Surgery. 122(1):114-5, 1997 2- Iglesias JL, Renard T: Diaphragmatic hernia in an 8-year-old with Ehlers-Danlos syndrome. Pediatr Surg Int. 13(8):553-5, 1998 3- Lin IC, Ko SF, Shieh CS, Huang CF, Chien SJ, Liang CD: Recurrent congenital diaphragmatic hernia in Ehlers-Danlos syndrome. Cardiovasc Intervent Radiol. 29(5):920-3, 2006 4- Demirogullari B, Karabulut R, Demirtola A, Karabulut B, Gol IH, Aybay C, Symoens S, Sonmez K, Basaklar AC, Kale N: A novel mutation in the vascular Ehlers-Danlos syndrome: a case presenting with colonic perforations. J Pediatr Surg. 41(8):e27-30, 2006 5- Hingorjo MR: Ehlers-danlos syndrome--clinical presentation. J Pak Med Assoc. 58(5):279-81, 2008 6- Behjati S, Knight Y, Borgstein R, Goebells A, Myint F: A life-threatening complication of Ehlers-Danlos syndrome. Br J Hosp Med (Lond). 70(6):360, 2009

MALT Lymphoma
Marginal zone mucosa-associated lymphoid tissue (MALT) lymphomas comprised a group of indolent B-cell non-Hodgkin lymphomas which are rare to find in pediatric patients. The gastrointestinal tract is the predominant site for this type of MALT extranodal non-Hodgkin lymphoma. Almost one-third of the patient with malignant lymphomas has involvement of the stomach (most commonly), small intestine and large intestine. Other sites include salivary gland, tonsils, lungs, thyroid, conjunctiva and even skin. The main categories for MALT lymphomas are the low- and high-grade B-cell MALTomas with or without a low grade component. Small centrocyte-like cells, lymphoepithelial lesions, and reactive lymphoid follicles are the main specific histopathologic features of low-grade MALTomas. On the contrary, large high-grade cells, which usually infiltrate in sheets and between glands without forming lymphoepithelial lesions characterize the high-grade B-cell MALToma. The high-grade MALTomas have shown a worse prognosis than low-grade and mixed types MALTomas. Acquired MALTomas may develop as a reaction to autoimmune disease and infection. Helicobacter pylori infection predisposes to development of MALTomas in the stomach. Management of MALTomas includes surgical resection where anatomically feasible along with adjuvant chemotherapy.
References: 1- Kurugoglu S, Mihmanli I, Celkan T, Aki H, Aksoy H, Korman U: Radiological features in paediatric primary gastric MALT lymphoma and association with Helicobacter pylori.Pediatr Radiol. 32(2):82-7, 2002 2- Karabulut R, Snmez K, Trkyilmaz Z, Yilmaz Y, Akyrek N, Baaklar AC, Kale N: Mucosa-associated lymphoid tissue lymphoma in the appendix, a lead point for intussusception. J Pediatr Surg. 40(5):872-4, 2005 3- Dargent JL, Devalck C, De Mey A, Vandeweyer E, Lespagnard L, Heimann P: Primary cutaneous marginal zone B-cell lymphoma of MALT type in a child. Pediatr Dev Pathol. 9(6):468-73, 2006 4- Kojima M, Nakamura N, Shimizu K, Tamaki Y, Itoh H, Nakamura S: Marginal zone B-Cell lymphoma among primary B-Cell lymphoma of Waldeyer's ring: histopathologic and immunohistochemical study of 16 tonsillectomy specimens. Int J Surg Pathol. 16(2):164-70, 2008 5- Wang T, Gui W, Shen Q: Primary gastrointestinal non-Hodgkin's lymphoma: clinicopathological and prognostic analysis.Med Oncol. 27(3):661-6, 2010 6- Marte A, Sabatino MD, Cautiero P, Accardo M, Romano M, Parmeggiani P: Unexpected finding of laparoscopic appendectomy: appendix MALT lymphoma in children.Pediatr Surg Int. 24(4):471-3, 2008

Central Precocious Puberty

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Central precocious puberty (CPP) occurs with premature activation of the hypothalamic-pituitarygonadal axis. CPP is defined as the onset of secondary characteristics associated with increased linear growth velocity and accelerated bone maturation occurring before the age of seven to 8 years in girls, and nine years in boys. CPP is more common in girls than boys. If left untreated precocious puberty results in early epiphyseal closure and short final stature. The most common cause of CPP is idiopathic caused by early onset of luteinizing hormone, follicle stimulating hormone and estradiol secretion. The goal of therapy is to restore a prepubertal state attenuating the deleterious effect of early sex steroid exposure on physical development, skeletal maturation and ultimate adult height. This can be achieved with parenteral administration of long-acting gonadotropin releasing hormone agonists (GnRHa) which has been found to be effective in retarding progression of secondary sexual characteristics, preventing menses, slowing bone-age maturation and improving final height. Since GnRHa monthly injections are painful, a subcutaneous microporous hydrogel implant that release GnRHa on a daily basis has been developed and tested efficaciously in suppressing clinical and laboratory parameters of puberty for one year. The implant is placed in the inner aspect of the arm under local or general anesthesia as a minor procedure.
References: 1- Arrigo T, Cisternino M, Galluzzi F, Bertelloni S, Pasquino AM, Antoniazzi F, Borrelli P, Crisafulli G, Wasniewska M, De Luca F: Analysis of the factors affecting auxological response to GnRH agonist treatment and final height outcome in girls with idiopathic central precocious puberty. Eur J Endocrinol. 141(2):140-4, 1999 2- Hirsch HJ, Gillis D, Strich D, Chertin B, Farkas A, Lindenberg T, Gelber H, Spitz IM: The histrelin implant: a novel treatment for central precocious puberty. Pediatrics. 116(6):e798-802, 2005 3- Lazar L, Padoa A, Phillip M: Growth pattern and final height after cessation of gonadotropin-suppressive therapy in girls with central sexual precocity. J Clin Endocrinol Metab. 92(9):3483-9, 2007 4- Eugster EA, Clarke W, Kletter GB, Lee PA, Neely EK, Reiter EO, Saenger P, Shulman D, Silverman L, Flood L, Gray W, Tierney D: Efficacy and safety of histrelin subdermal implant in children with central precocious puberty: a multicenter trial.J Clin Endocrinol Metab. 92(5):1697-704, 2007 5- Carel JC, Eugster EA, Rogol A, Ghizzoni L, et al: Consensus statement on the use of gonadotropin-releasing hormone analogs in children. Pediatrics. 123(4):e752-62, 2009 6- Gab E, Barg E, Wikiera B, Grabowski M, Noczyska A: Influence of GnRH analog therapy on body mass in central precocious puberty. Pediatr Endocrinol Diabetes Metab. 15(1):7-11, 2009

PSU Volume 36 No 06 JUNE 2011

Venous Thromboembolism in Children
Venous thromboembolism (VTE) has a low risk of developing during childhood. Venous thromboembolism arises from stasis, vascular endothelial damage or a hypercoagulable state (Virchow's triad). Surgical risk factors include indwelling venous catheters, reduced lack of mobility after surgery, and intrinsic hematologic prothrombotic factors (factor V Leiden, antiphospholipid antibodies or prothrombin mutations). VTE disease in children is more common in neonates than older children and appears to affect mostly the upper limb veins, followed by iliac veins and inferior vena cava with the main danger being that of pulmonary embolism sometimes confused with a pneumonic process. Acquired inflammatory conditions, septicemia, staph infections, HIV are risk factors for VTE since they induce a hypercoagulable state due to transitory decrease of anticoagulant protein S and increase in procoagulant Factor VIII activity. Other risk factors in children are central venous catheter, blood dyscrasias, obesity and malignancy. Traumatic intimal tears after pelvic or orthopedic trauma and prolonged surgical procedures can also predispose to venous thromboembolism. Diagnosis of VTE is made by noninvasive radiologic imaging. Management of VTE in children in high risk scenarios or with established diagnosis consist of lowhome.coqui.net/titolugo/PSU36.html#3661 10/12

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molecular-weight heparin. Use of plasminogen activators is reserved for the management of thrombi that threaten life, limb or a specific organ.
References: 1- Tormene D, Gavasso S, Rossetto V, Simioni P: Thrombosis and thrombophilia in children: a systematic review. Semin Thromb Hemost. 32(7):724-8, 2006 2- Beardsley DS: Venous thromboembolism in the neonatal period. Venous thromboembolism in the neonatal period. Semin Perinatol. 31(4):250-3, 2007 3- Goldenberg NA, Bernard TJ: Venous thromboembolism in children. Pediatr Clin North Am. 55(2):305-22, 2008 4- Goldenberg NA, Bernard TJ: Venous thromboembolism in children. Hematol Oncol Clin North Am. 24(1):151-66, 2010 5-Long E, Pitfield AF, Kissoon N: Anticoagulation therapy: indications, monitoring, and complications. Pediatr Emerg Care. 27(1):55-61, 2011 6- Tshifularo N, Arnold M, Moore SW: Thromboembolism and venous thrombosis of the deep veins in surgical children-an increasing challenge? J Pediatr Surg. 46(3):433-6, 2011

Pancreatic Mediastinal Pseudocyst

Most pancreatic pseudocysts are located in the peripancreatic retroperitoneum. On very rare occasions active pancreatic secretions from a pancreatitis can gain access to the mediastinum causing a mediastinal pseudocyst. Access to the posterior mediastinum can occur through the esophageal hiatus most commonly, and also through extension through the aortic hiatus or direct erosion through the diaphragm. Children with pancreatic mediastinal pseudocysts can present with abdominal pain, anorexia, vomiting, dysphagia, respiratory distress and dyspnea. The etiology of the pancreatitis in such children can be a congenital ductal anomaly (pancreas divisum), a posttraumatic event or idiopathic. Diagnosis is made with CT Scan as it delineates location, extent and anatomic relationship to the adjacent mediastinal structures. The cyst contains elevated amylase levels. MRCP provides images of the pancreatic duct. The decision whether to wait for spontaneous resolution, cyst maturation or perform immediate intervention is dictated by the child symptoms. Increasing size, bleeding, infection, or compression symptoms, invasion or rupture warrants intervention. Intervention can consist of endoscopic transmural drainage (transpapillary, transgastric, transesophageal), internal surgical drainage (cystogastrostomy or cystoenterostomy), resection procedure or CT-guided percutaneous drainage.
References: 1- Sharma S, Puri S, Chaturvedi P, Kulshreshtha R, Baijal VN: Mediastinal pancreatic pseudocyst following traumatic rupture of diaphragm. Pediatr Radiol. 18(4):337, 1988 2- Crombleholme TM, deLorimier AA, Adzick NS, Longaker MT, Harrison MR, Cox KL, Heyman MB: Mediastinal pancreatic pseudocysts in children. J Pediatr Surg. 25(8):843-5, 1990 3- Bonnard A, Lagausie P, Malbezin S, Sauvat E, Lemaitre AI, Aigrain Y: Mediastinal pancreatic pseudocyst in a child. A thoracoscopic approach. Surg Endosc. 15(7):760, 2001 4- Snajdauf J, Rygl M, Kalousov J, Kucera A, Petr O, Pcha K, Mixa V, Keil R, Hrbal Z: Surgical management of major pancreatic injury in children. Eur J Pediatr Surg. 17(5):317-21, 2007 5- Gupta R, Munoz JC, Garg P, Masri G, Nahman NS Jr, Lambiase LR: Mediastinal pancreatic pseudocyst--a case report and review of the literature. MedGenMed. 11;9(2):8, 2007 6- Nuwayhid Z, Kassira N, Neville HL, Casillas J, Sola JE: Percutaneous retropleural drainage of a posttraumatic pancreatic mediastinal pseudocyst in a child. J Pediatr Surg. 46(3):585-7, 2011

Abdominal Pain in Sickle Cell Disease

Children with Sickle Cell Disease (SCD) present fairly commonly with acute abdominal pain. The pain must be differentiated between ischemia from a vasoocclusive crisis or another abdominal
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disease requiring surgery. Specific causes for abdominal pain in SCD include hepatic crisis, cholelithiasis, splenic sequestration and pancreatitis. Management consists of oxygenation, hydration and analgesia. When the child with SCD develops classic features of an acute surgical abdomen such as vomiting, tender distended abdomen with rigidity, involuntary guarding and rebound tenderness, the disease process might require surgery. Ultrasound & CT Scan imaging helps discover whether the child has bowel obstruction from infarction, a perforated viscus, appendicitis or cholecystitis. The incidence of appendicitis has been found to be lower in children with SCD. Close clinical monitoring is essential. Conservative therapy is warranted in the large majority of patients with SCD who present with acute abdominal pain. High pain scores, older age, increased polymorphonuclear count and homozygous SCD types are associated with prolonged hospital stay during vasoocclusive crisis. Surgical consultation is necessary if a surgical cause is suspected or the cause is not obvious after a thorough evaluation.
References: 1- Rogovik AL, Li Y, Kirby MA, Friedman JN, Goldman RD: Admission and length of stay due to painful vasoocclusive crisis in children. Am J Emerg Med. 27(7):797-801, 2009 2- Leung AK, Sigalet DL: Acute abdominal pain in children. Am Fam Physician. 67(11):2321-6, 2003 3- Antal P, Gauderer M, Koshy M, Berman B: Is the incidence of appendicitis reduced in patients with sickle cell disease? Pediatrics. 101(1):E7, 1998 4- Kudsk KA, Tranbaugh RF, Sheldon GF: Acute surgical illness in patients with sickle cell anemia. Am J Surg. 142(1):113-7, 1981 5- Bonadio WA: Clinical features of abdominal painful crisis in sickle cell anemia. J Pediatr Surg. 25(3):301-2, 1990 6- Ahmed S, Shahid RK, Russo LA: Unusual causes of abdominal pain: sickle cell anemia. Best Pract Res Clin Gastroenterol. 19(2):297-310, 2005

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