VOLUME 15, 2000

Volume 15 No 01 JULY 2000

Overwhelming Post-Splenectomy Infections

Splenectomy impairs the immune response to bacterial infections. Such impaired immunologic functions include: formation of antibodies, deficiency of opsonization, lower IgM levels, deficiency in bacterial clearing and tuftsin deficient phagocytosis. Overwhelming post-splenectomy infection (OPSI) refers to a constellation of fast-developing symptoms (high fever, hypotension, rigor, bacteremia, leucocytosis) that leads to death in patients that have undergo removal of the spleen. Mortality rates after OPSI is established are 50%. When obtainable, blood cultures grow encapsulated organisms (pneumococcus, meningococcus, hemophilus, etc.). The vulnerability of OPSI is greatest within the first two years after the splenectomy, and it persists throughout life. The clinical appearance of OPSI can go from a mild event to death from sepsis with pulmonary complications as the most common morbidity. OPSI is more commonly identified after spleen removal for Hodgkin and trauma. Immunization against pneumococcus, H. Influenza and meningococcus should be given to all children who undergo splenectomy since these are the most common organisms associated with OPSI. In the elective situation the vaccine should be given two weeks prior to removal of the spleen. In setting of trauma it should be given as soon as possible, Though several studies have found better functional antibody responses with delayed (14-day) vaccination in the setting of trauma we will continue to administer the vaccine as soon as possible until well-randomized trials are done.

1- Jugenburg M, Haddock G, Freedman MH, Ford-Jones L, Ein SH: The morbidity and mortality of pediatric splenectomy: does prophylaxis make a difference? J Pediatr Surg 34(7):1064-7, 1999
2- Camel JE, Kim KS, Tchejeyan GH, Mahour GH: Efficacy of passive immunotherapy in experimental postsplenectomy sepsis due to Haemophilus influenzae type B.J Pediatr Surg 28(11):1441-4, 1993
3- Green JB, Shackford SR, Sise MJ, Powell RW: Postsplenectomy sepsis in pediatric patients following splenectomy for trauma: a proposal for a multi-institutional study.  J Pediatr Surg 21(12):1084-6, 1986
4-  Hays DM, Ternberg JL, Chen TT, Sullivan MP, Tefft M, Fung F, Gilchrist G, Fryer C, Gehan EA: Postsplenectomy sepsis and other complications following staging laparotomy for Hodgkin's disease in childhood. J Pediatr Surg 21(7):628-32, 1986
5- Reihner E, Brismar B: Management of splenic trauma--changing concepts. Eur J Emerg Med 2(1):47-51, 1995
6- Shatz DV; Schinsky MF; Pais LB; Romero-Steiner S; Kirton OC; Carlone, GM: Immune responses of splenectomized trauma patients to the 23-valent pneumoccal polysaccharide vaccine at 1 versus 7 versus 14 days after splenectomy. J Trauma 44(5):760-5, 1998
7- Schreiber MA, Pusateri AE, Veit BC, Smiley RA, Morrison CA, Harris RA: Timing of vaccination does not affect antibody response or survival after pneumococcal challenge in splenectomized rats. J Trauma 45(4):692-7, 1998
8- Caplan ES, Boltansky H, Snyder MJ, Rooney J, Hoyt NJ, Schiffman G,Cowley RA: Response of traumatized splenectomized patients to immediate vaccination with polyvalent pneumococcal vaccine. J Trauma 23(9):801-5, 1983

Esophageal Hernias

Two types of esophageal hernia recognized are the hiatal and paraesophageal hernia. Diagnosis is made radiologically always and in a number of patients endoscopically. The hiatal hernia (HH) refers to herniation of the stomach to the chest through the esophageal hiatus. The lower esophageal sphincter also moves. It can consist of a small transitory epiphrenic loculation (minor) up to an upside-down intrathoracic stomach (major). HH generally develops due to a congenital, traumatic or iatrogenic factor. Most disappear by the age of two years, but all forms of HH can lead to peptic esophagitis from Gastroesophageal reflux. Repair of HH is determined by the pathology of its associated reflux (causing failure to thrive, esophagitis, stricture, respiratory symptoms) or the presence of the stomach in the thoracic cavity. In the paraesophageal hernia (PH) variety the stomach migrates to the chest and the lower esophageal sphincter stays in its normal anatomic position. PH is a frequent problem after antireflux operations in patients without posterior crural repair. Small PH can be observed. With an increase in size or appearance of symptoms (reflux, gastric obstruction, bleeding, infarction or perforation) the PH should be repaired. The incidence of PH has increased with the advent of the laparoscopic fundoplication.

1- Bettex M, Oesch I: The Hiatus Hernia Saga. Ups and Downs in Gastroesophageal Reflux: Past, Present, and Future Perspectives. J Pediatr Surg 18(6): 670-680, 1983
2- Avansino JR, Lorenz ML, Hendrickson M, Jolley SG: Characterization and management of paraesophageal hernias in children after antireflux operation. J Pediatr Surg 34(11):1610-4, 1999
3- Kim SH; Hendren WH; Donahoe PK: Gastroesophageal reflux and hiatus hernia in children: experience with 70 cases. J Pediatr Surg 15(4):443-51, 1980
4- Bernhard UA, Shmerling DH: Follow-up examinations of conservatively and surgically treated children with hiatus hernia. Prog Pediatr Surg 18:118-31, 1985
5- Alrabeeah A, Giacomantonio M, Gillis DA:  Paraesophageal hernia after Nissen fundoplication: a real complication in pediatric patients. J Pediatr Surg Aug;23(8):766-8, 1988
6- Basso N, De Leo A, Genco A, Rosato P, Rea S, Spaziani E, Primavera A: 360 degrees laparoscopic fundoplication with tension-free hiatoplasty in the treatment of symptomatic gastroesophageal reflux disease. Surg Endosc 14(2):164-9, 2000

Rectal Duplication

Rectal duplications are very rare encompassing 5% of all GI duplications. They can be cystic or tubular (hindgut), small or involve a significant portion of the proximal recto-sigmoid colon. Most are cystic arising in a retrorectal position and 90% do not communicate with the rectum. Presentation depends on size (mass effect), fistulization (drainage of mucous or pus from the anus or a fistula is a frequent presenting sign), infection, the presence of ectopic gastric mucosa (causing ulceration & bleeding), prolapse, bladder outlet obstruction or malignant degeneration (adenocarcinoma). Epithelial lining of the duplication is usually colonic, other types being squamous, epithelium, gastric mucosa or urothelial. Barium enema, fistulogram, US, CT and MRI are helpful in localizing the anatomy and extent. Management of the duplication depends on location and size. Surgical excision through a transanal, transcoccygeal or posterior sagittal approach is warrant in retrorectal cysts. Anterior duplications or those associated with a genitourinary malformation require a laparotomy. High index of suspicion is needed to avoid delay and multiple operations. Complete excision is curative.

1- La Quaglia MP, Feins N, Eraklis A, Hendren WH: Rectal Duplications. J Pediatr Surg 25(9): 980-984, 1990
2- Rajah S, Ramanujam TM, Anas SR, et al: Duplication of the rectum: report of four cases and review of the literature. Pediatr Surg Int 13: 373-376, 1998
3- Poenaru KA, Soboleski D, Hurlbut D, Kamai I: Anterior Rectal Duplication: A Diagnostic Challenge. J Pediatr Surg 35(4): 613-614, 2000
4- Okur H, Kerkin E, Zorludemir U, Olcay I: Tubular Duplication of the Hindgut with Genitourinary Anomalies. J Pediatr Surg 27(9): 1239-1240, 1992
5-  Gibson TC, Edwards JM, Shafiq S: Carcinoma arising in a rectal duplication cyst. Br J Surg 73(5):377, 1986
6- Rauch MK, Martin EL, Cromie WJ: Rectal duplication as a cause of neonatal bladder outlet obstruction and hydronephrosis. J Urol 149(5):1085-6, 1993
7- Mboyo A, Monek O, Massicot R, Martin L, Destuynder O, Lemouel A, Aubert D: Cystic rectal duplication: a rare cause of neonatal intestinal obstruction. Pediatr Surg Int 12(5-6):452-4, 1997
8- Delarue A, Garcia-Meric P, Martin C, Piguet C, Andre N, Galli G, Guys JM: Antenatal rupture of a diverticular rectal duplication with neonatal perineal fistulization. Pediatr Surg Int 13(4):288-9, 1998

Volume 15 No 02 AUGUST 2000

Carcinoid Syndrome

The carcinoid syndrome (fascial flushing, diarrhea, tricuspid regurgitation, pulmonic stenosis, valvular fibrosis and wheezing) is the result of serotonin overproduction by a carcinoid tumor. Carcinoid tumors arise from enterochromaffin cells (APUD cells from the neural crests), occur in virtually every organ, could be multiple, metastatic and associated with a second malignancy. Patients are diagnosed biochemically from increased urinary excretion of 5-hydroxyindoleacetic acid (5-HIAA). Platelet serotonin levels are more sensitive for detecting carcinoids that secrete small amounts of serotonin. Jejunum-ileum, bronchus and appendix are the most common sites of origin. Carcinoid of the appendix is the most common neoplasm of the GI tract in childhood. Metastasis to liver of midgut carcinoids produce the syndrome. Tumors greater than 2 cm are more prone to metastasis needing aggressive surgical management. Octreotide scan and I-131 MIBG are useful in determination of location and extent of some carcinoid tumors, particularly those of midgut origin. A positive scan may predict the ability of Octreotide therapy to control symptoms of hormonal hypersecretion. Scans provide localization of the primary tumor that should be widely excised including lymph nodes. Higher survival rates are found for patients with midgut lesions who undergo intra abdominal debulking procedures excluding the liver. For single liver lesion resection is justified, otherwise with multiple diffuse disease hepatic artery ligation or embolization has been tried. Symptomatic metastasis should be managed with Octreotide. Prognosis is associated with the presence of liver metastasis, syndrome development and level of tumor markers (chromogranin A).

1- Hoberock TR, Knutson CO, Polk HC Jr: Clinical aspects of invasive carcinoid tumors. South Med J 68(1):33-7, 1975
2- Hanson MW, Feldman JM, Blinder RA, Moore JO, Coleman RE: Carcinoid tumors: iodine-131 MIBG scintigraphy. Radiology 172(3):699-703, 1989
3- Feldman JM: Carcinoid tumors and the carcinoid syndrome. Curr Probl Surg 26(12):835-85, 1989
4- Soreide O, Berstad T, Bakka A, Schrumpf E, Hanssen LE, Engh V, Bergan A, Flatmark A: Surgical treatment as a principle in patients with advanced abdominal carcinoid tumors. Surgery 111(1):48-54, 1992
5- Janson ET, Holmberg L, Stridsberg M, Eriksson B, Theodorsson E, Wilander E, Oberg K: Carcinoid tumors: analysis of prognostic factors and survival in 301 patients from a referral center.  Ann Oncol 8(7):685-90, 1997
6- Moertel CL, Weiland LH, Telander RL: Carcinoid tumor of the appendix in the first two decades of life. J Pediatr Surg 25(10):1073-5, 1990
7- Lamberts SW, Bakker WH, Reubi JC, Krenning EP: Somatostatin-receptor imaging in the localization of endocrine tumors. N Engl J Med 323(18):1246-9, 1990

Left Hypoplastic Colon Syndrome

Colonic obstruction in the newborn child could be the result of necrotizing enterocolitis, atresia, meconium plug syndrome, duplication cyst, Hirschsprung disease or the small left colon syndrome. The left (small) hypoplastic colon syndrome (LHCS) is a very rare cause of colonic obstruction identified in newborns with characteristic roentgenographic features resembling those of Hirschsprung's disease. Manifesting in the first 24-48 hours of life, LHCS is a functional disturbance related to immaturity of the intrinsic innervation of the colon that is especially common in low birth weight neonates or of diabetic mothers. Intestinal perforation, sepsis, hypoglycemia and death may occur. The diagnosis is suggested in a barium enema when the caliber of the left colon is small with a transitional zone at the splenic flexure. Management consists of hypoglycemia correction, antibiotics, nasogastric decompression and observation. In most babies the obstruction clears in 48-72 hours. When the clinical diagnosis is not readily apparent a rectal biopsy and sweat chloride test should be done to differentiate LHCS from Hirschsprung disease and cystic fibrosis respectively. The narrowed left colon remains narrow in follow-up.

1- Woodhurst WB, Kliman MR: Neonatal small left colon syndrome: report of two cases. Am Surg 42(7):479-81, 1976
2- Davis WS, Campbell JB: Neonatal small left colon syndrome. Occurrence in asymptomatic infants of diabetic mothers. Am J Dis Child 129(9):1024-7, 1975
3- Stewart DR, Nixon GW, Johnson DG, Condon VR: Neonatal small left colon syndrome. Ann Surg 186(6):741-5, 1977
4- al-Salem AH, Khwaja S, Wood BP: Radiological case of the month. Neonatal small left colon syndrome. Am J Dis Child 144(11):1273-4, 1990
5- Philippart AI, Reed JO, Georgeson KE: Neonatal small left colon syndrome: Intramural not intraluminal obstruction. J Pediatr Surg 10: 733, 1975

Beckwith-Wiedemann Syndrome

The Beckwith-Wiedemann Syndrome (BWS), first described in 1964, is characterized by the presence of macrosomia (gigantism), macroglossia, omphalocele and unusual linear fissures in the lobules of the external ear. One of the more frequent metabolic changes is transient neonatal hypoglycemia, the result of pancreas cell hyperplasia. Inheritance of the syndrome remains uncertain. Most cases are sporadic, but a number of familial cases have been reported. BWS is associated with a predisposition to embryonal tumors, most commonly Wilms' Tumor.  Genetic abnormalities found in these tumors affect the same chromosome region (11p15), which has been implicated in the etiology of BWS. Routine abdominal ultrasound screening every six months up to the age of eight years is recommended for children with BWS.

1- Wiedemann HR: Complexe malformatif familial avec hernie ombilicale et macroglossie--un "syndrome nouveau"? J Genet Hum 13:223, 1964
2- Engstrom W, Lindham S, Schofield P: Wiedemann-Beckwith syndrome. Eur J Pediatr 147(5):450-7, 1988
3- Lodeiro JG, Byers JW 3d, Chuipek S, Feinstein SJ: Prenatal diagnosis and perinatal management of the Beckwith-Wiedeman syndrome: a case and review. Am J Perinatol 6(4):446-9, 1989
4- Weksberg R, Squire JA: Molecular biology of Beckwith-Wiedemann syndrome. Med Pediatr Oncol 27(5):462-9, 1996
5- Li M, Squire JA, Weksberg R: Molecular genetics of Beckwith-Wiedemann syndrome. Curr Opin Pediatr 9(6):623-9, 1997
6- Steenman M, Westerveld A, Mannens M: Genetics of Beckwith-Wiedemann syndrome-associated tumors: common genetic pathways. Genes Chromosomes Cancer 28(1):1-13, 2000
7-Lugo-Vicente HL:  Molecular Biology and Genetics affecting Pediatric Solid Tumors. Bol Asoc Med PR (in press).

Volume 15 No 03 SEPTEMBER 2000

Askin Tumor

Askin tumor (synonyms are primitive neuroectodermal tumor or Ewing's sarcoma) is a malignant small round cell tumor of mesenchymal origin affecting the thoracopulmonary region of children and young adults with a tendency to recur locally. The rib is the most common site of primary tumor development. Establishing an accurate preoperative diagnosis of Askin's tumor is difficult. Microscopy and immunohistological stain of the specific marker - neuron-specific enolase, is essential. CT scan is valuable for evaluating tumor extension at diagnosis, the effects of chemotherapy and assessing recurrence after surgery, but can overestimate pleural, lung or diaphragmatic infiltration. MRI can determine chest wall muscle and marrow involvement. Neither is adequate for adjacent lung invasion. Bone, bone marrow and lung are the most frequent sites of metastasis. Treatment includes radical surgical resection (including affected lung tissue), neoadjuvant (local control of disease is critical) and adjuvant chemotherapy plus radiation. Surgical resection, with en bloc removal of involved structures and chest wall reconstruction, provides excellent local control of malignant chest wall tumors. Human dura, prostethic material (Gortex, Marlex, Vicryl) and myocutaneous flaps have been used for reconstruction. Patients with Askin tumors treated with aggressive pre-resection chemotherapy have smaller tumors to resect (less than 100 cc by volume) with improved survival. Overall the prognosis is poor.

1- Dang NC, Siegel SE, Phillips JD: Malignant chest wall tumors in children and young adults. J Pediatr Surg 34(12):1773-8, 1999
2- Sawin RS, Conrad EU 3rd, Park JR, Waldhausen JH: Preresection chemotherapy improves survival for children with Askin tumors. Arch Surg 131(8):877-80, 1996
3- Walton JM, Bass J, Sambey E, Rubin SZ: Use of human dura in pediatric chest wall reconstruction after tumor resection. J Pediatr Surg 29(9):1189-91, 1994
4- Shamberger RC, Tarbell NJ, Perez-Atayde AR, Grier HE: Malignant small round cell tumor (Ewing's-PNET) of the chest wall in children. J Pediatr Surg 29(2):179-84, 1994
5- Bourque MD, Di Lorenzo M, Collin PP, Russo P, Laberge JM, Moir C: Malignant small-cell tumor of the thoracopulmonary region: 'Askin tumor'. J Pediatr Surg 24(10):1079-83, 1989
6- Shamberger RC, Grier HE, Weinstein HJ, Perez-Atayde AR, Tarbell NJ: Chest wall tumors in infancy and childhood. Cancer 15;63(4):774-85, 1989
7- Parikh PM, Charak BS, Banavali SD, Advani SH, Saikia TK, Gopal R, Borges AM, Chinoy RF, Desai PB: Treatment of Askin Rosai tumor--need for a more aggressive approach. J Surg Oncol 39(2):126-8, 1988
8- Grosfeld JL, Rescorla FJ, West KW, Vane DW, DeRosa GP, Provisor AJ, Weetman R: Chest wall resection and reconstruction for malignant conditions in childhood. J Pediatr Surg 23(7):667-73, 1988
9- Askin FB, Rosai J, Sibley RK, Dehner LP, McAlister WH: Malignant small cell tumor of the thoracopulmonary region in childhood: a distinctive clinicopathologic entity of uncertain histogenesis. Cancer 43(6):2438-51, 1979

Gastric Duplication

Gastric duplications cysts account for less than 5% of all enteric duplications (the rarest form). As a duplication it is attached to its origin, has a well developed smooth muscle coat and gastric epithelial lining. Prenatal ultrasound finding of a cyst with peristaltic activity within the right upper quadrant of the fetal abdomen suggest the diagnosis. The most common site of origin of the duplication cyst is the greater curvature. Most are closed spherical cysts. Most cases are diagnosed during the first two years of life and are more common in females. The usual presentation is an abdominal mass with vomiting. Complications reported consist of recurrent pancreatitis, hemorraghe, perforation, peritonitis, torsion and malignant change in gastric mucosa. Contrast studies, US or CT-Scan suggests the diagnosis. Management of a gastric duplication cyst is surgical excision that can be accomplished laparoscopically. Gastric mucosal along heterotopic pancreatic tissue can be found in the cyst wall.

1- Bidwell JK, Nelson A: Prenatal ultrasonic diagnosis of congenital duplication of the stomach. J Ultrasound Med 5(10):589-91, 1986
2- Sieunarine K, Manmohansingh E: Gastric duplication cyst presenting as an acute abdomen in a child. J Pediatr Surg 24(11):1152, 1989
3- Bajpai M, Mathur M, Duplications of the alimentary tract: clues to the missing links. J Pediatr Surg 29(10):1361-5, 1994
4- Blais C, Masse S: Preoperative ultrasound diagnosis of a gastric duplication cyst with ectopic pancreas in a child. J Pediatr Surg 30(9):1384-6, 1995
5- Koumanidou C, Montemarano H, Vakaki M, Pitsoulakis G, Savvidou D, Kakavakis K: Perforation of multiple gastric duplication cysts: diagnosis by sonography. Eur Radiol 9(8):1675-7, 1999

Bile Duct Rhabdomyosarcoma

The botryoid variety of embryonal Rhabdomyosarcoma (RMS) is the most common tumor of bile ducts presenting during early life. Peak incidence at three to four years with a slight female predominance. The tumor is characterized by multiple polypoid grape-like projections into the lumen of the common bile duct with plate-like thickening of the common bile duct wall. It is characterized by a high risk of local recurrence to adjacent lymph nodes and a low risk of remote metastasis. Obstructive jaundice, cachexia, pain and abdominal mass are the usual presentation, often with fever and hepatomegaly. Attribution of these symptoms to hepatitis commonly delays definitive treatment. Other times the preoperative diagnosis is mistaken for a choledochal cyst. US defines the relationship of the tumor with portal vessels and biliary tract while CT-Scan and MRI determine operability. Aggressive surgery combined with the new adjuvant therapies (chemotherapy and radiotherapy) appears to provide the best chance for a longer survival. Intra-operative cholangiography is a valuable technique in establishing the level of biliary tree obstruction and verifying a functioning drainage procedure. The prognosis is poor and death is usually due to the effects of local invasion by the tumor.

1- Taira Y, Nakayama I, Moriuchi A, Takahara O, Ito T, Tsuchiya R, Hirano T, Matsushita T: Sarcoma botryoides arising from the biliary tract of children. A case report with review of the literature. Acta Pathol Jpn 26(6):709-18, 1976
2- Lack EE, Perez-Atayde AR, Schuster SR: Botryoid rhabdomyosarcoma of the biliary tract. Am J Surg Pathol 5(7):643-52, 1981
3- Martinez-F LA, Haase GM, Koep LJ; Akers DR: Rhabdomyosarcoma of the biliary tree: the case for aggressive surgery. J Pediatr Surg 17(5):508-11, 1982
4- Ruymann FB, Raney RB Jr, Crist WM, Lawrence W Jr: Rhabdomyosarcoma of the biliary tree in childhood. A report from the Intergroup Rhabdomyosarcoma Study. Cancer 56(3):575-81, 1985
5- Geoffray A, Couanet D, Montagne JP, Leclere J: Ultrasonography and computed tomography for diagnosis and follow-up of biliary duct rhabdomyosarcomas in children. Pediatr Radiol 17(2):127-31, 1987
6- von der Oelsnitz G, Spaar HJ, Lieber T, Munchow B, Booss D:  Embryonal rhabdomyosarcoma of the common bile duct. Eur J Pediatr Surg 1(3):161-5, 1991
7- Sanz N, de Mingo L, Florez F, Rollan V: Rhabdomyosarcoma of the biliary tree. Pediatr Surg Int 12(2-3):200-1, 1997
8- Balkan E, Kiristioglu I, Gurpinar A, Sinmaz K, Ozkan T, Dogruyol H: Rhabdomyosarcoma of the biliary tree. Turk J Pediatr 41(2):245-8, 1999

Volume 15 No 4 OCTOBER 2000


Effusion of lymph (chyle) into the pleural cavity is known as chylothorax. Chyle is clear-milky fluid with an elevated total protein and albumin level, a specific gravity above 1.012, the presence of WBC with lymphocyte predominance (80%), and elevated triglyceride (chylomicrons). In children is a potentially life-threatening disorder that has profound respiratory, nutritional (hypoalbuminemia), electrolyte (hyponatremia) and immunologic (lymphopenia, hypogammaglobulinemia, T-cell depletion) effects. Chylothorax has a congenital (mediastinal lymphangiomatosis), acquired or idiopathic origin. Acquired chylothorax is most commonly found; the result of a direct lesion of the thoracic duct or lymphatic vessels by trauma (thoracotomy, central venous catheters or chest tubes insertions), during cardiac surgery, mediastinal malignancy (neuroblastoma) or infection, repair of a diaphragmatic hernia or associated with superior vena cava obstruction (thrombosis). Initial management consists of: 1- chest tube drainage after failed thoracentesis (pleural space tamponade), 2- medium-chain triglyceride enriched formula for a week (lymphatic decompression), 3- TPN if chylothorax increases or persists. More protracted course (4 week medical tx) will require surgery to locate and suture ruptured subpleural lymphatics, ligate the thoracic duct, do chemical pleurodesis or place a pleuroperitoneal shunt. Those associated with venous obstruction or increase right sided cardiac pressure produce more volume, persist longer and are more difficult to manage.

1- Puntis JW, Roberts KD, Handy D: How should chylothorax be managed? Arch Dis Child 62(6):593-6, 1987
2- Jalili F: Medium-chain triglycerides and total parenteral nutrition in the management of infants with congenital chylothorax. South Med J 80(10):1290-3, 1987
3- Easa D, Balaraman V, Ash K, Thompson B, Boychuk R: Congenital chylothorax and mediastinal neuroblastoma. J Pediatr Surg 26(1):96-8, 1991
4- Le Coultre C, Oberhansli I, Mossaz A, Bugmann P, Faidutti B, Belli DC: Postoperative chylothorax in children: differences between vascular and traumatic origin. J Pediatr Surg 26(5):519-23, 1991
5- Allen EM, van Heeckeren DW, Spector ML, Blumer JL: Management of nutritional and infectious complications of postoperative chylothorax in children. J Pediatr Surg 26(10):1169-74, 1991
6- van Straaten HL, Gerards LJ, Krediet TG: Chylothorax in the neonatal period. Eur J Pediatr 152(1):2-5, 1993
7- Bond SJ, Guzzetta PC, Snyder ML, Randolph JG: Management of pediatric postoperative chylothorax. Ann Thorac Surg 56(3):469-72, 1993
8- Kavvadia V, Greenough A, Davenport M, Karani J, Nicolaides KH: Chylothorax after repair of congenital diaphragmatic hernia--risk factors and morbidity. J Pediatr Surg 33(3):500-2, 1998
9- Engum SA, Rescorla FJ, West KW, Scherer LR 3rd: The use of pleuroperitoneal shunts in the management of persistent chylothorax in infants. J Pediatr Surg 34(2):286-90, 1999
10- Beghetti M, La Scala G, Belli D, Bugmann P: Etiology and management of pediatric chylothorax. J Pediatr 136(5):653-8, 2000

Multiple Endocrine Neoplasia Type 1

Multiple endocrine neoplasia type 1 (MEN-1) is an autosomal dominant disorder characterized by the combined occurrence of parathyroid, pancreatic islet and anterior pituitary tumors. A single inherited locus on chromosome 11, band q13, causes MEN-1. Primary hyperparathyroidism (HPT) is the most common lesion of MEN-1. The albumin corrected total calcium is usually normal, but the ionized calcium and PT hormone is elevated. Once elevated the patient develops insidious complication from hypercalcemia (pancreatitis, peptic ulcer disease, muscle pains, weakness, neuropsychiatry disorders and nephrolithiasis). Surgical management of primary HPT in MEN-1 is controversial. The salient features of HPT in MEN-1 are a high incidence, if not universal occurrence of multiglandular disease, an operative failure rate because of failure of both to identify all four glands and to perform a radical resection, and a significant incidence of recurrent disease, sometimes cause by supernumerary or ectopic gland involvement. Surgical principles should be (1) identification of all four glands, (2) subtotal resection to ensure cure and facilitate possible reoperation, and (3) excision of supernumerary thymic glands. Extirpation of a single gland as a general primary procedure is inadequate causing recurrence. Although many patients with primary HPT and MEN syndrome have multiple abnormal parathyroid glands, two populations of patients exist; one population has solitary or double adenomas and recurrence is uncommon, whereas the other population of patients has hyperplasia and persistent or recurrent disease is common.

1- Trump D, Farren B, Wooding C, et al: Clinical studies of multiple endocrine neoplasia type 1 (MEN1). QJM 89(9):653-69, 1996
2-  Kraimps JL, Duh QY, Demeure M, Clark OH: Hyperparathyroidism in multiple endocrine neoplasia syndrome. Surgery. 112(6):1080-6, 1992
3- Thakker RV, Bouloux P, Wooding C, et al: Association of parathyroid tumors in multiple endocrine neoplasia type 1 with loss of alleles on chromosome 11. N Engl J Med 321(4):218-24, 1989
4- Tonelli F, Spini S, Tommasi M, Gabbrielli G: Intraoperative parathormone measurement in patients with multiple endocrine neoplasia type I syndrome and hyperparathyroidism. World J Surg 24(5):556-62, 2000
5- O'Riordain DS, O'Brien T, Grant CS, et al: Surgical management of primary hyperparathyroidism in multiple endocrine neoplasia types 1 and 2. Surgery 114(6):1031-7, 1993
6- Hellman P, Skogseid B, Juhlin C, et al: Findings and long-term results of parathyroid surgery in multiple endocrine neoplasia type 1. World J Surg 16(4):718-22, 1992


The word ranula comes from the Latin: rana, frog. Ranula is a large sessile cyst of the sublingual salivary gland in the floor of the mouth under the tongue. The lesion can be as small as a pea-sized cyst to one side or the other of the frenulum, or an enormous blue-gray translucent swelling that fills the mouth and cause respiratory problems. Two ranula varieties are described: a superficial, epithelial lined cyst resulting from ductal obstruction, and a cervical pseudocyst without epithelial lining resulting from extravasation of saliva (plunging) that dissects through the tissue planes of the neck and appear as a neck mass. In both cases management consists of excision of the thin-walled sac and sublingual gland if possible, or marsupialization to the oral cavity.

1- Quick CA, Lowell SH: Ranula and the sublingual salivary glands. Arch Otolaryngol 103(7):397-400, 1977
2- Parekh D, Stewart M, Joseph C, Lawson HH: Plunging ranula: a report of three cases and review of the literature. Br J Surg 74(4):307-9, 1987
3- de Visscher JG, van der Wal KG, de Vogel PL: The plunging ranula. Pathogenesis, diagnosis and management. J Craniomaxillofac Surg 17(4):182-5, 1989
4-Baurmash HD: Marsupialization for treatment of oral ranula: a second look at the procedure. J Oral Maxillofac Surg 50(12):1274-9, 1992
5- Morton RP, Bartley JR: Simple sublingual ranulas: pathogenesis and management. J Otolaryngol 24(4):253-4, 1995

Volume 15 No 5 NOVEMBER 2000

Hepatic Hemangioendothelioma

Hepatic hemangioendothelioma (HHE) is a rare, benign tumor that appears during the first six-months of life. Considered the most common vascular tumor of the liver in children is associated with a high mortality rate. HHE can be associated with congestive heart failure, anemia, thrombocytopenia (Kasabach-Merritt syndrome), hepatomegaly and cutaneous hemangiomas. Prenatal diagnosis has been associated with hydrops fetalis. Postnatal diagnosis is established with US, CT-Scan and MRI. Alpha-fetoprotein levels should be obtained to differentiate from hepatoblastoma. Mortality results from high-output cardiac failure secondary to arteriovenous shunting within the tumor (up to 50% of the cardiac output can be diverted), respiratory compromise, hepatic failure, intraperitoneal hemorrhage and consumptive coagulopathy. The younger the age at diagnosis, the more severe the cardiac symptoms. Natural history of asymptomatic HHE is spontaneous involution. Symptomatic lesions need aggressive management. Radiotherapy and chemotherapy have not shown consistently good results. Steroid and alpha-interferon are used as initial treatment to inhibit proliferation of endothelial and smooth muscle cells. Symptomatic solitary lesions can be managed with resection. Severe bilobar disease might need hepatic artery embolization or transplantation. Hepatic artery ligation or embolization should not be done in patients with shunting from the portal vein to the hepatic vein and minimal systemic arterial collateral circulation since it can result in hepatic necrosis.

1- Holcomb GW 3d, O'Neill JA Jr, Mahboubi S, Bishop HC: Experience with hepatic hemangioendothelioma in infancy and childhood. J Pediatr Surg 23(7):661-6, 1988
2- Becker JM, Heitler MS: Hepatic hemangioendotheliomas in infancy. Surg Gynecol Obstet 168(2):189-200, 1989
3- Gonen R, Fong K, Chiasson DA: Prenatal sonographic diagnosis of hepatic hemangioendothelioma with secondary nonimmune hydrops fetalis. Obstet Gynecol 73(3 Pt 2):485-7, 1989
4- McHugh K, Burrows PE: Infantile hepatic hemangioendotheliomas: significance of portal venous and systemic collateral arterial supply. J Vasc Interv Radiol 3(2):337-44, 1992
5- Davenport M, Hansen L, Heaton ND, Howard ER: Hemangioendothelioma of the liver in infants. J Pediatr Surg 30(1):44-8, 1995
6-  Samuel M, Spitz L: Infantile hepatic hemangioendothelioma: the role of surgery. J Pediatr Surg 30(10):1425-9, 1995
7- Daller JA, Bueno J, Gutierrez J, Dvorchik I, Towbin RB, Dickman PS, Mazariegos G, Reyes J: Hepatic hemangioendothelioma: clinical experience and management strategy. J Pediatr Surg 34(1):98-105, 1999


Candida species (Albicans, Parapsilosis, Tropicalis and Krausei) systemic infection has steadily increased in the neonatal intensive care units during the past years. Associated factors for this type of infection are: prolonged use of broad-spectrum antibiotics, parenteral hyperalimentation, intravenous fat emulsions and placement of a central-venous catheters (CVC). Fungal infections are particularly common when TPN is administered through CVC. Candida can be cultured from the skin, urine, blood and mouth of affected patients. Fever, not-doing-well, and abdominal distention are the most common presentations. Infants who are found to have systemic candidiasis should be treated by removing all factors that predispose to systemic candidiasis (eg., indwelling catheters, broad-spectrum antibiotics) as persistent fungemia, morbidity and mortality are associated with attempts to maintain the CVC in the presence of Candidemia. Early initiation of systemic antifungal therapy (amphotericin, fluconazole) is imperative, along with searching for additional foci of disease. Endophthalmitis, venous thrombosis and endocarditis are complications of CVC associated Candidemia. Once the disease is recognized mortality rates are 20% in infants.

1- Lacey SR, Zaritsky AL, Azizkhan RG: Successful treatment of Candida-infected caval thrombosis in critically ill infants by low-dose streptokinase infusion. J Pediatr Surg 23(12):1204-9, 1988
2- Leibovitz E, Iuster-Reicher A, Amitai M, Mogilner B: Systemic candidal infections associated with use of peripheral venous catheters in neonates: a 9-year experience. Clin Infect Dis 14(2):485-91, 1992
3-  Johnson DE, Thompson TR, Green TP, Ferrieri P: Systemic candidiasis in very low-birth-weight infants (less than 1,500 grams). Pediatrics 73(2):138-43, 1984
4- MacDonald L, Baker C, Chenoweth C: Risk factors for candidemia in a children's hospital. Clin Infect Dis 26(3):642-5, 1998
5- Rose HD: Venous catheter-associated candidemia. Am J Med Sci 275(3):265-9, 1978
6- Stamos JK, Rowley AH: Candidemia in a pediatric population. Clin Infect Dis 20(3):571-5, 1995
7- Eppes SC, Troutman JL, Gutman LT: Outcome of treatment of candidemia in children whose central catheters were removed or retained. Pediatr Infect Dis J 8(2):99-104, 1989
8- Dato VM, Dajani AS: Candidemia in children with central venous catheters: role of catheter  removal and amphotericin B therapy. Pediatr Infect Dis J 9(5):309-14, 1990

Congenital Lobar Emphysema

Congenital lobar emphysema (CLE) is an unusual lung bud anomaly characterized by massive air trapping in the lung parenchyma that nearly always occurs in infancy and affects males more commonly (2:1). Lobar over distension causes compression of adjacent lung tissue, mediastinal shift and decrease in venous return. When this occurs persistent progressive respiratory distress (dyspnea, tachypnea, wheezing, cough and cyanosis) develops requiring lobectomy. Asymptomatic CLE exists, more commonly beyond infancy and associated with an acute viral respiratory infection. Lobar hyperinflation, flat diaphragms and retrosternal air, mediastinal shift in simple films suggests the diagnosis. CT scan depicts the abnormal anatomy (lung herniation) and the morphology of the remaining lung. V/Q scans confirm the non-functioning nature of the affected lobe. Upper and middle right lobes are more commonly affected. Etiology centers in a combination of bronchial (flap/valve) obstruction with congenital cartilage dysplasia. Most common associated defect is cardiovascular (VSD, PDA). Symptomatic patients nearly always require lobectomy. Asymptomatic children do not benefit from surgical treatment but need close follow-up. Prenatally diagnosed cases need referral to surgery centers.

1- Murray GF: Congenital Lobar Emphysema. Surg Gynec & Obstet.  124: 611-625, 1967
2- Haller JA, Golladay ES, Pickard LR et al: Surgical Management of Lung Bud Anomalies: Lober Emphysema, Bronchogenic Cyst, Cystic Adenomatoid Malformation, and Intralobar Pulmonary Sequestration. Ann Thorac Surg 28(1): 33-43, 1879
3-  Markowitz RI, Mercurio MR, Vahjen GA; Gross I, Touloukian RJ: Congenital lobar emphysema. The roles of CT and V/Q scan. Clin Pediatr 28(1):19-23, 1989
4- Nuchtern JG, Harberg FJ: Congenital lung cysts. Semin Pediatr Surg 3(4):233-43, 1994
5- Schwartz MZ, Ramachandran P: Congenital malformations of the lung and mediastinum--a quarter century of experience from a single institution. J Pediatr Surg 32(1):44-7, 1997
6- Karnak I, Senocak ME, Ciftci AO, Buyukpamukcu N: Congenital lobar emphysema: diagnostic and therapeutic considerations. J Pediatr Surg 34(9):1347-51, 1999
7- Al-Bassam A, Al-Rabeeah A, Al-Nassar S, et al: Congenital cystic disease of the lung in infants and children (experience with 57 cases). Eur J Pediatr Surg 9(6):364-8, 1999
8- Olutoye OO, Coleman BG, Hubbard AM, Adzick NS: Prenatal diagnosis and management of congenital lobar emphysema. J Pediatr Surg 35(5):792-5, 2000

Volume 15 No 06 DECEMBER 2000

Abdominal Compartment Syndrome

Elevation of intra-abdominal pressure (IAP) may impair physiology and organ function producing what is known as Abdominal Compartment Syndrome (ACS). The physiological consequences of increased intraabdominal pressure consist of cardiac output reduction, pulmonary ventilation restriction (increasing peak inspiratory pressure and hypercapnia), renal function (oliguria) and visceral perfusion diminution (gut mucosal acidosis), and increased in cerebro-spinal pressure. ACS can be the result in abdominal wall defect closures (gastroschisis and omphalocele), inflammatory bowel conditions, trauma and intraabdominal infections (enterocolitis, appendicitis, bowel perforation). Vesical and inferior vena cava pressure recording have good correlation with IAP. Gastric, rectal, superior vena cava, femoral/brachial artery, and rectus compartment pressure are poor indicators of actual IAP. An elevated abdominal compartment pressure is considered as greater than 25 mm Hg. The bowel is the most sensitive organ to ACS and it develops evidence of end-organ damage before the development of classic renal, pulmonary and cardiovascular signs. Management consists of abdominal decompression. Reopening the abdominal wound is a lifesaving intervention prompted usually by cardiovascular deterioration. Use of delayed wound closure (staged celiotomy) may prevent development of this condition in high-risk surgical patients. Timely decompression of the ACS results in improvements in cardiopulmonary and renal function. Failure to recognize and treat ACS is inevitably fatal.

1-DeCou JM, Abrams RS, Miller RS, Gauderer MW: Abdominal compartment syndrome in children: experience with three cases. J Pediatr Surg 35(6):840-2, 2000
2- Bendahan J, Coetzee CJ, Papagianopoulos C, Muller R: Abdominal compartment syndrome. J Trauma 38(1):152-3, 1995
3- Lacey SR, Bruce J, Brooks SP, Griswald J, Ferguson W, Allen JE, Jewett TC Jr, Karp MP, Cooney DR: The relative merits of various methods of indirect measurement of intraabdominal pressure as a guide to closure of abdominal wall defects. J Pediatr Surg. 22(12):1207-11, 1987
4- Burch JM, Moore EE, Moore FA, Franciose R: The abdominal compartment syndrome. Surg Clin North Am 76(4):833-42, 1996
5- Williams M, Simms HH: Abdominal compartment syndrome: case reports and implications for management in critically ill patients. Am Surg 63(6):555-8, 1997
6- Watson RA, Howdieshell TR:  Abdominal compartment syndrome. South Med J 91(4):326-32, 1998
7- Neville HL, Lally KP, Cox CS Jr: Emergent abdominal decompression with patch abdominoplasty in the pediatric patient. J Pediatr Surg 35(5):705-8, 2000
8- Schein M, Wittman DH, Aprahamian CC, et al: The abdominal compartment syndrome: The physiological and clinical consequences of leveated intra-abdominal pressure. J Am Coll Surg 180: 745-753, 1995

Fetal Abdominal Wall Defects

Most common abdominal wall defects (AWD) are gastroschisis, omphalocele and hernia of the umbilical cord. Referral to tertiary centers with available neonatal intensive care is necessary in prenatally diagnosed cases. Changing the route of delivery does not affect outcome for either defect. Omphalocele has a high incidence of associated anomalies (cardiac, neurogenic, genitourinary, skeletal, chromosomal syndromes) that are the cornerstones of mortality. Detailed search for associated anomalies, fetal echocardiogram and karyotyping should be performed always. Cesarean section is justified in large omphaloceles (> 5 cm) to avoid liver damage, sac rupture and dystocia. Gastroschisis prenatal US appearance  depends on gestational age and condition of extruded bowel. Fetal karyotyping testing is less important. Intestinal atresia complicates the defect, the result of an intrauterine vascular accident. Intestinal obstruction due to atresia or luminal constriction may cause polyhydramnios, fetal growth retardation and preterm labor, findings that can be monitored with serial US. No benefit has been found in recommending routine c-section for most cases of gastroschisis. Preterm deliveries by c-section have been found to prevent bowel damage in fetus with progressive bowel dilatation  and thickening, a finding that has not been corroborated by others. Abnormal US appearance of fetal bowel is associated with more bowel edema, longer operative time and a higher incidence of postoperative complications.

1- Langer JC: Fetal Abdominal Wall Defects. Semin Pediatr Surg 2(2):121-128, 1996
2- Dykes EH: Prenatal diagnosis and management of abdominal wall defects. Semin Pediatr Surg. 5(2):90-4, 1996
3- Sipes SL, Weiner CP, Sipes DR 2d, Grant SS, Williamson RA: Gastroschisis and omphalocele: does either antenatal diagnosis or route of delivery make a difference in perinatal outcome? Obstet Gynecol 76(2):195-9, 1990
4- Lewis DF, Towers CV, Garite TJ, Jackson DN, Nageotte MP; Major CA: Fetal gastroschisis and omphalocele: is cesarean section the best mode of delivery? Am J Obstet Gynecol 163(3):773-5, 1990
5- Lurie S, Sherman D, Bukovsky I: Omphalocele delivery enigma: the best mode of delivery still remains dubious. Eur J Obstet Gynecol Reprod Biol 82(1):19-22, 1999
6- Raynor BD, Richards D: Growth retardation in fetuses with gastroschisis. J Ultrasound Med 16(1):13-6, 1997
7- Bethel CA, Seashore JH, Touloukian RJ: Cesarean section does not improve outcome in gastroschisis. J Pediatr Surg 24(1):1-3, 1989
8- Langer JC, Khanna J, Caco C, Dykes EH, Nicolaides KH: Prenatal diagnosis of gastroschisis: development of objective sonographic criteria for predicting outcome. Obstet Gynecol 81(1):53-6, 1993
9- Lenke RR, Persutte WH, Nemes J: Ultrasonographic assessment of intestinal damage in fetuses with gastroschisis: is it of clinical value? Am J Obstet Gynecol 163(3):995-8, 1990
10- Alsulyman OM, Monteiro H, Ouzounian JG, Barton L, Songster GS; Kovacs BW: Clinical significance of prenatal ultrasonographic intestinal dilatation in fetuses with gastroschisis. Am J Obstet Gynecol 175(4 Pt 1):982-4, 1996
11- Adra AM, Landy HJ, Nahmias J, Gomez-Marin O: The fetus with gastroschisis: impact of route of delivery and prenatal ultrasonography. Am J Obstet Gynecol 174(2):540-6, 1996


Cholecystokinin (CCK) is a naturally occurring octapeptide hormone that has several utilities in children. Secreted in the proximal small bowel, increases bile flow, causes contraction of the gallbladder and promotes GI and colonic motility. As diagnostic source is used in determining the ejection fraction of the biliary tree. Therapeutically, CCK has been used to increase bile flow in cases of TPN cholestasis. CCK is associated with functional and histologic improvement in the periportal area of the liver as well as preservation of gallbladder emptying ability. CCK significantly diminishes direct bilirubin levels in infants with TPN cholestasis, effectively clears the biliary tree from sludge and stones. CCK use can be associated with cramping abdominal pain, feeding intolerance, flushing and rarely hypotension. Children with clinical liver failure have no response to CCK. If conjugated hyperbilirubinemia from TPN does not resolve after three weeks of full enteral feedings and stools remain acholic, CCK therapy should be considered. Evidence-based data that CCK prevents TPN cholestasis is not conclusive. Stopping TPN and resuming enteral feeding is the only effective management in TPN cholestasis.

1- Lugo-Vicente HL: Gallbladder Dyskinesia in Children. Journal of Society of Laparoendoscopic Surgeons 1(1):61-65, 1997
2- Curran TJ, Uzoaru I, Das JB, Ansari G, Raffensperger JG: The effect of cholecystokinin-octapeptide on the hepatobiliary dysfunction caused by total parenteral nutrition. J Pediatr Surg 30(2): 242-247, 1995
3- Teitelbaum DH, Han-Markey T, Schumacher RE: Treatment of parenteral nutrition-associated cholestasis with cholecystokinin-octapeptide. J Pediatr Surg 30(7): 1082-1085, 1995
4- Moss L, Amii LA: New Approaches to understanding the etiology and treatment of total parenteral nutrition-associated cholestasis. Sem Pediatr Surg 8(3): 140-147, 1999
5- Rintala RJ, Lindahl H, Pohjavuori M: Total parenteral nutrition-associated cholestasis in surgical neonates may be reversed by intravenous cholecystokinin: A preliminary report. J Pediatr Surg 30(6): 827-830, 1995

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