VOLUME 12, 1999

Volume 12 No 01 JANUARY 1999

Biliary Hypoplasia

Biliary hypoplasia is a rare cause of persistent neonatal conjugated hyperbilirubinemia. Pathologically, affected children have absent or reduced number of bile ductules with normal distribution of branches of the portal vein and hepatic artery within the liver parenchyma. Biliary hypoplasia is also identified as paucity of interlobular bile ducts (PILBD). Two types of PILBD are recognized: 1) syndromic (arteriohepatic dysplasia or Alagille's syndrome) with characteristic extrahepatic abnormalities (fascial appearance, pulmonic artery stenosis, vertebral anomalies, embryotoxon and delayed weight-height development), and 2) non-syndromic biliary hypoplasia. Biliary hypoplasia is clinically indistinguishable from biliary atresia and can sometimes be confused. A definitive diagnosis is difficult to make in early infancy. Differentiation between biliary atresia, hypoplasia and neonatal hepatitis continues to require direct visualization of the biliary ducts. This mean laparoscopic or open intra-operative cholangiography and liver biopsy. The cholangiogram will show diminutive intra- and extra-hepatic biliary tree. Attempts to establish biliary flow by means of hepatic porto-enterostomy (Kasai procedures) in children with PILBD have been unsuccessful and contraindicated. Management is conservative and  include predigested formulas, ursodeoxycholic acids (10 mg/kg/day), phenobarbital and A,D,K,E vitamin replacement. Non-syndromic PILBD have better long-term prognosis. Children with syndromic PILBD identified in infancy because of cholestasis have a 50% probability of long-term survival without liver transplantation.

1- Cynamon HA, Powell GK, Isenberg JN, Lobe TE: Support for a conservative approach to mixed intrahepatic and extrahepatic biliary hypoplasia. J Pediatr Surg 22(11):1031-2, 1987
2- Schwartz MZ: An alternate method for intraoperative cholangiography in infants with severe obstructive jaundice. J Pediatr Surg. 20(4): 440-442, 1985
3- Kahn E, Daum F: Arteriohepatic dysplasia (Alagille's syndrome): a common cause of conjugated hyperbilirubinemia. Ann Clin Lab Sci 14(6):480-6, 1984
4- Kahn EI, Daum F, Markowitz J, Aiges HW, Schneider KM, So HB, Altman P, Chandra RS, Silverberg M: Arteriohepatic dysplasia. II. Hepatobiliary morphology. Hepatology 3(1):77-84, 1983
5- Lugo-Vicente HL: Biliary Atresia: An Overview. Bol Asoc Med PR 87(7-9): 147-153, 1995
6- Hoffenberg EJ, Narkewicz MR, Sondheimer JM, Smith DJ, Silverman A, Sokol RJ: Outcome of syndromic paucity of interlobular bile ducts (Alagille syndrome) with onset of cholestasis in infancy. J Pediatr 127(2):220-4, 1995

Lumbar Hernias

Congenital lumbar hernias are rare abdominal parietal defects in infants and children. Approximately 10% of all lumbar hernias are congenital and the vast majority are unilateral. They have been divided in three categories: 1) superior - occurring in the superior lumbar triangle (Grynfelt-Lesshaft), 2) inferior - occurring through the inferior lumbar triangle (Petit) or 3) a combination of them. They have a well-defined fascial defect. Acquired lumbar hernias outnumber congenital hernias and may result from surgery, infection and/or trauma. Since the hernia defect enlarges with growth or have the potential to incarcerate early operative repair is preferred. Lumbar hernias are associated to the lumbo-costo-vertebral syndrome (caudal regression  anomalies, diaphragmatic hernia, ureteropelvic junction obstruction, cloacal exstrophy and lipomeningocele). Repair of small defects can be accomplished by primary closure. Large and recurrent defects may need gortex patching. When they include a more extensive deficiency of the entire lateral abdominal wall extending to the rectus sheath and inguinal ligament closure may also need prosthetic material.

1- Somuncu S, Bernay F, Rizalar R, Ariturk E, Gunaydin M, Gurses N: Congenital lumbar hernia associated with the lumbocostovertebral syndrome: two cases. Eur J Pediatr Surg. 7(2)122-124, 1997
2- Fakhry SM, Azizkhan RG: Observations and current operative management of congenital lumbar hernias during infancy. Surg Gynecol Obstet. 172(6)475-479, 1991
3- Pul M, Pul N, Gurses N: Congenital lumbar (Grynfelt-Lesshaft) hernia. Eur J Pediatr Surg. 1(2)115-117, 1991
4- Hancock BJ, Wiseman NE: Incarcerated congenital lumbar hernia associated with the lumbocostovertebral syndrome. J Pediatr Surg. 23(8)782-783, 1988
5- Mehta MH, Patel RV, Mehta SG: Letter to the editor. J Pediatr Surg 27(9)1258-1259, 1992

Castleman's Disease

Castleman's disease (CD) also known as angiofollicular lymph node hyperplasia is a benign disorder characterized by enlarged hyperplastic lymph nodes that occurs very rarely in children.  Two classes of CD are identified in children: Hyaline-vascular (HV) type characterized by vascular proliferation and hyalinization, and the plasma cell (PC) type characterized by mature plasma cells in the interfollicular spaces with less vascular proliferation (this type is most common in pediatric age). Its clinical presentation is that of a slowly growing localized abdominal, chest (mediastinum or lung hilum), or neck  mass. Within the abdomen CD is located in the small bowel mesentery. May be either asymptomatic or present systemic symptoms of fever, malaise, increase ESR, thrombocytosis, anemia and hypergammaglobulinemia. The enlarged nodes may mimic a malignant tumor of the lymphoid system. Histopathological evaluation confirms the diagnosis. Surgical excision of the mass and surrounding nodes involved is necessary to affect a cure.

1- Shroff VJ, Gilchrist BF, DeLuca FG, McCombs HL, Wesselhoeft CW: Castleman's Disease Presenting as a Pediatric Surgical Problem. J Pediatr Surg 30 (5): 745-747, 1995
2- Patel U, Forte V, Taylor G, Sirkin W: Castleman's disease as a rare cause of a neck mass in a child. J Otolaryngol 27(3):171-3, 1998
3- Tuerlinckx D, Bodart E, Delos M, Remacle M, Ninane J: Unifocal cervical Castleman disease in two children. Eur J Pediatr 156(9):701-3, 1997
4- Bapat KC, Malde HM, Pandit AA, Mittal BV, Kedar RP, Bapat RD, Relekar RG: Solitary retroperitoneal angiofollicular lymph node hyperplasia. J Postgrad Med 38(2):90-4, 1992
5- Salisbury JR: Castleman's disease in childhood and adolescence: report of a case and review of literature. Pediatr Pathol 10(4):609-15, 1990
6- Powell RW, Lightsey AL, Thomas WJ, Marsh WL: Castleman's disease in children. J Pediatr Surg 21(8):678-82, 1986

Volume 12 No 02 FEBRUARY 1999

Airway Foreign Bodies

Below the age of three foreign body (FB) aspiration or ingestion is one of the leading causes of accidental death. Most FB that lodge in the airway tree creates a ball-valve phenomenon in the affected bronchus that allows bidirectional but unequal flow of air. Air flows preferentially into the bronchus with inspiration and less is allowed to flow out during expiration resulting in significant air-trapping and hyperinflation of the affected lobe or lung. The mediastinum shifts to the opposite site of the FB. Alternatively, with a total blockage of the bronchus there is loss of volume with atelectasis and shift of the mediastinum to the same side. Diagnosis relies on clinical judgment, history, physical exam and radiographic evaluation. The child present with an aspiration event followed by coughing, wheezing, dyspnea, fever, wheezing or decrease breath sounds over the affected hemithorax. After chest films confirmation management consists of rigid bronchoscopy for extraction of the FB. With history of choking crisis and mild symptoms bronchoscopy should also be done. Peanuts, corns, beans and seeds are the most common offending agents causing further damage by virtue of an associated inflammatory reaction. A forceps during bronchoscopy will help extracted most FB. Other times a fogarty retraction is needed in case of segmental bronchus position. Longstanding foreign body in the airway may be responsible for irreversible complications (bronchiectasia).

1- Silva AB, Muntz HR, Clary R: Utility of conventional radiography in the diagnosis and management of pediatric airway foreign bodies. Ann Otol Rhinol Laryngol 107(10 Pt 1):834-8, 1998
2- Cataneo AJ, Reibscheid SM, Ruiz Junior RL, Ferrari GF: Foreign body in the tracheobronchial tree. Clin Pediatr (Phila). 36(12):701-6, 1997
3- Barrios Fontoba JE, Gutierrez C, Lluna J, Vila JJ, Poquet J, Ruiz-Company S: Bronchial foreign body: should bronchoscopy be performed in all patients with a choking crisis? Pediatr Surg Int 12(2-3):118-20, 1997
4- Reilly J, Thompson J, MacArthur C, Pransky S, Beste D, Smith M; Gray S, et al: Pediatric aerodigestive foreign body injuries are complications related to  timeliness of diagnosis. Laryngoscope 107(1):17-20, 1997
5- Black RE, Johnson DG, Matlak ME: Bronchoscopic removal of aspirated foreign bodies in children. J Pediatr Surg 29(5):682-4, 1994
6- Menendez AA, Gotay Cruz F, Seda FJ, Velez W, Trinidad Pinedo J: Foreign body aspiration: experience at the University Pediatric Hospital. P R Health Sci J 10(3):127-33, 1991

Pulmonary Sequestration

Pulmonary sequestrations refer to masses of abnormal lung parenchyma with anomalous systemic blood supply not communicating with the normal tracheobronchial tree. The abnormal lung parenchyma may be Intralobar (IS) or Extralobar Sequestration (ES). Intralobar  is contained within the visceral pleural of a lower lobe receiving the blood supply from the abdominal aorta or other thoracic vessel. It is believed IS are acquired postinfectious process due to their association with chronic recurrent lung infection and reactive airway disease. ES  is a congenital malformation with variable ectopic blood supply (aorta) having its own pleural investment separate from normal lung, containing typical features of CCAM-2 (40%) and associated malformations (40%). Both types can have patent communication with foregut. Prenatal diagnosis can be obtained with real-time US with Doppler imaging (can cause fetal lung compression, mediastinal shifts and hydrops). Postnatally, contrast-enhanced CT may establish the diagnosis eliminating the need for more invasive imaging (arteriography). Most presents in early infancy with a soft tissue opacity in the posterior basal segments of the lung on simple chest films. Management consists of resection to alleviate symptoms and avoid complications. ES can be managed with resection alone, while IS needs lobectomy. Anecdotal cases of partial or total disappearance of these masses while asymptomatic has been reported.

1- Garcia-Pena P, Lucaya J, Hendry GM, McAndrew PT, Duran C: Spontaneous involution of pulmonary sequestration in children: a report of two cases and review of the literature. Pediatr Radiol 28(4):266-70, 1998
2- Plattner V, Haustein B, Llanas B, Allos N, Vergnes P, Heloury Y: Extra-lobar pulmonary sequestration with prenatal diagnosis. A report of 5 cases and review of the literature. Eur J Pediatr Surg 5(4):235-7, 1995
3- Nuchtern JG, Harberg FJ: Congenital lung cysts. Semin Pediatr Surg 3(4):233-43, 1994
4- Sade RM, Clouse M, Ellis FH Jr: The spectrum of pulmonary sequestration. Ann Thorac Surg 18(6):644-58, 1974

Pectus Carinatum

Pectus carinatum (pigeon breast, keel chest) is an infrequent chest wall deformity. Rarely produce cardio-respiratory derangements as the thoracic cavity enlarges symmetrically with the defect. The child (most commonly a boy in his early teens) will be brought by the parents because of cosmetic and psychologic concerns. Satisfactory subjective long-term results of most patients justify surgical correction. Repair is performed through a transverse or submammary incision with subperiosteal resection of the lower costal cartilages from sternum to costochondral junction bilaterally. The sternum is fractured to straighten it and there no need for sternal strut use in this deformity. Complications can include seroma, atelectasis and pneumothorax.  In a well-motivated, skeletally immature individual body cast bracing can be an effective treatment for cosmetically displeasing pectus carinatum.

1- Lacquet LK, Morshuis WJ, Folgering HT: Long-term results after correction of anterior chest wall deformities. J Cardiovasc Surg 39(5):683-8, 1998
2- Fonkalsrud EW, Salman T, Guo W, Gregg JP: Repair of pectus deformities with sternal support. J Thorac Cardiovasc Surg 107(1):37-42, 1994
3- Mielke CH, Winter RB: Pectus carinatum successfully treated with bracing. A case report. Int Orthop 17(6):350-2, 1993
4- Shamberger RC, Welch KJ: Surgical correction of pectus carinatum. J Pediatr Surg 22(1):48-53, 1987
5- Robicsek F, Cook JW, Daugherty HK, Selle JG: Pectus carinatum. J Thorac Cardiovasc Surg 78(1):52-61, 1979

Volume 12 No 03 MARCH 1999


The absence of the spleen (asplenia) occurs after surgical removal, following chronic conditions or congenital. Trauma is the most common cause of removing the spleen in children and sickle cell disease is the most common cause of functional asplenia in children. Congenital absence of the spleen is usually associated with serious malformations, primarily cardiovascular and abdominal heterotaxia. The spleen contributes importantly to the normal and pathologic removal of blood cells from the circulation and to defense against infection with encapsulated bacteria. Asplenia increases the risk of fulminant bacteremia (post-splenectomy sepsis) and mortality with these organisms. This risk is also increased by the underlying condition that caused the removal of the spleen, i.e., trauma, malignancy or hematologic disease. Several recommendations have been given when dealing with an asplenic individual. These are to vaccinate the child against pneumococcus (Pneumovax vaccine), hemophilus influenza type b and meningococcus. Regarding Pneumovax use revaccination after 3-5 years is recommended for children with asplenia who are 10 years of age or younger and for older children and adults who were immunized at least five years before. Duration of vaccine-induced antibodies is unknown but may be shorter than that in normal persons. Long-term antimicrobial prophylaxis is also used. This carries the problem of compliance and for how long. Significant febrile episodes should be managed aggressively, and probably most important, the patient and family should be carefully educated about this complication (name tag). Most deaths from hyposplenia-related septicemia are preventable.

1- 1997 Red Book: Report of the Committee on Infectious Disease of the American Academy of Pediatrics. 24th edition, pags. 56-58
2- Lane PA: The spleen in children. Curr Opin Pediatr 7(1):36-41, 1995
3- Sills RH: Splenic function: physiology and splenic hypofunction. Crit Rev Oncol Hematol 7(1):1-36, 1987
4-Phoon CK; Neill CA: Asplenia syndrome: insight into embryology through an analysis of cardiac and extracardiac anomalies. Am J Cardiol 15;73(8):581-7, 1994
5- Styrt B: Infection associated with asplenia: risks, mechanisms, and prevention. Am J Med 88(5N):33N-42N, 1990

Pancreas Divisum

Pancreas divisum (PD), believed the most common congenital anomaly of the pancreas, is an embryologic variation of pancreas development where the dorsal (Santorini) and ventral portions (Wirsung) ducts drain separately. Diagnosis is made with ERCP (short duct of Wirsung that does not communicate with main pancreatic duct of Santorini). Not everybody with this ductal anomaly develops pancreatitis. Likewise with the minor papilla draining the bulk of the pancreas in PD, a small orifice size (< 0.75 mm) plays a role in outflow obstruction and development of pancreatitis. Children with PD and recurrent episodes of pancreatitis will need endoscopic sphincterotomy of the minor and sometimes major papilla. If not feasible technically, surgical sphincteroplasty of both papillae along with cholecystectomy (bile stasis leads to gallstones) is indicated. Intraoperative pancreatogram will help determine if both papilla are stenotic. Once chronic pancreatitis is established, ductal drainage or resection may be necessary.

1-  O'Rourke RW, Harrison MR: Pancreas divisum and stenosis of the major and minor papillae in an 8-year-old girl: treatment by dual sphincteroplasty. J Pediatr Surg 33(5):789-91, 1998
2- Tagge EP, Tarnasky PR, Chandler J, Tagge DU, Smith C, Hebra A, Hawes RH, Cotton PB, Othersen HB Jr: Multidisciplinary approach to the treatment of pediatric pancreaticobiliary disorders. J Pediatr Surg 32(2):158-64, 1997
3- Sanada Y, Yoshizawa Y, Chiba M, Nemoto H, Midorikawa T, Kumada K: Ventral pancreatitis in a patient with pancreas divisum. J Pediatr Surg 30(5):665-7, 1995
4- Crombleholme TM, deLorimier AA, Way LW, Adzick NS: The modified Puestow procedure for chronic relapsing pancreatitis in children. J Pediatr Surg 25(7):749-54, 1990
5- Adzick NS, Shamberger RC, Winter HS, Hendren WH: Surgical treatment of pancreas divisum causing pancreatitis in children. J Pediatr Surg 24(1):54-8, 1989
6- Warshaw AL, Richter JM, Schapiro RH: The cause and treatment of pancreatitis associated with pancreas divisum. Ann Surg 198(4):443-52, 1983

Meconium Peritonitis

Meconium peritonitis (MP) is a chemical peritonitis that occurs following bowel perforation during fetal life. It is generally looked upon as benign, resulting in no long-term sequelae. The peritonitis occurs when the meconium leaves the bowel, enters the peritoneal cavity and spreads throughout causing a sterile inflammatory reaction. Most common site of bowel perforation is the distal ileum, and 50% of  babies with MP develop intestinal obstruction. Prenatal ultrasound findings include ascites, intraabdominal masses, bowel dilatation and the development of intraabdominal calcifications. Bowel disorders which lead to MP in utero are those resulting in bowel obstruction and perforation, such as small bowel atresias, volvulus and meconium ileus. MP can be divided into simple or complex. Cases with spontaneously healed perforation (simple MP) need observation as they rarely develop symptoms. Newborns with complex MP are born with bowel obstruction a/or pseudocyst formation (localized collection of meconium contained in a cyst made of fibrous granulation tissue). Complex MP needs surgical therapy.

1- Patton WL, Lutz AM, Willmann JK, Callen P, Barkovich AJ, Gooding CA: Systemic spread of meconium peritonitis. Pediatr Radiol 28(9):714-6, 1998
2- Dirkes K, Crombleholme TM, Craigo SD, Latchaw LA, Jacir NN, Harris BH, D'Alton ME: The Natural History of Meconium Peritonitis Diagnosed in Utero. J Pediatr Surg 30(7): 979-982, 1995
3- Miller JP, Smith SD, Newman B, Sukarochana K: Neonatal abdominal calcification: is it always meconium peritonitis? J Pediatr Surg 23(6):555-6, 1988
4- Olsen MM, Luck SR, Lloyd-Still J, Raffensperger JG: The spectrum of meconium disease in infancy. J Pediatr Surg 17(5):479-81, 1982
5- Fujioka M, Bowen A: Cystic meconium peritonitis. Radiology 140(2):380, 1981

Volume 12 No 04 APRIL 1999

Gastro-Esophageal Reflux Disease

Gastro-esophageal reflux disease (GERD) has two distinct forms in children. In infants, reflux causes delayed growth & development, recurrent respiratory infections and life-threatening situations. Most symptoms resolve when the valvular competence of the cardia develops in the second year of life. Older children manifest symptoms of dysphagia and substernal burning. We manage most children medically. Indications for surgery consist of failure to thrive, esophagitis, stricture, chronic aspiration pneumonia, life-threatening events and Barrett metaplasia changes. Children referred for surgery should have an esophagogram, endoscopy, and evaluation of gastric emptying mechanisms to document: magnitude of reflux, presence of pharyngeal incoordination, dysmotility, strictures, malrotation, and grade of esophagitis. pH studies and milk scans may farther find a cause and effect relationship between reflux and respiratory problems. Surgical options consist of partial (Thal, Boix-Ochoa, Toupe) or complete wrap (Nissen) fundoplasty reconstruction. The procedure can be done open or laparoscopic. Those kids with delayed gastric emptying will benefit from a gastric emptying procedure (pyloroplasty, antroplasty). Neurologically impaired children referred for feeding gastrostomy should undergo a similar work-up to identified potentially dangerous reflux problems. Alternatively the gastrotomy can be constructed percutaneously and the problems of GERD be assessed later in life. Neurologic status and gastric emptying are major predictors of operative success.

1- Bustorff-Silva J, Fonkalsrud EW, Perez CA, Quintero R, Martin L, Villeseñor E, Atkinson JB: Gastric Emptying Procedures Decrease the Risk of Postoperative Recurrent Reflux in Children with Delayed Gastric Emptying. J Pediatr Surg 34(1): 79-83, 1999
2- Chung DH, Georgeson KE: Fundoplication and gastrostomy. Semin Pediatr Surg 7(4):213-9, 1998
3- Jolley SG: Gastroesophageal reflux disease as a cause for emesis in infants. Semin Pediatr Surg. 4(3):176-89, 1995.
4- Hebra A, Hoffman MA: Gastroesophageal reflux in children. Pediatr Clin North Am 40(6):1233-51, 1993
5- Jolley SG: Current surgical considerations in gastroesophageal reflux disease in infancy and childhood. Surg Clin North Am 72(6):1365-91, 1992
6- Rode H, Millar AJ, Brown RA, Cywes S: Reflux strictures of the esophagus in children. J Pediatr Surg 27(4):462-5, 1992
7- Pearl RH, Robie DK, Ein SH, Shandling B, Wesson DE, Superina R, Mctaggart K, Garcia VF, O'Connor JA, Filler RM: Complications of gastroesophageal antireflux surgery in neurologically impaired versus neurologically normal children. J Pediatr Surg 25(11):1169-73, 1990

Laryngo-Treacheal Clefts

Laryngo-tracheal clefts (LTC) are rare congenital anomalies that can involve the larynx or the laryngo-tracheal and esophageal wall. Subtypes of LTC occur between the aerodigestive system and can be limited to the larynx up to involve all the way to reach the carina or the right main bronchus (Subtypes: type 1 to the cricoid, type 2 involving the cricoid, type 3 in cervical trachea and type 4 into the thoracic trachea). LTC arises from errors in chondrification and fusion of the laryngeal supporting cartilage or tracheo-esophageal folds. LTC are associate to the "G" and Pallister-Hall syndrome, to esophageal atresia and to anal malformations. As neonate they present a hoarse cry, inability to handle secretions, cyanosis, choking, coughing, stridor and recurrent pneumonia depending on the length of the cleft. Diagnosis of LTC is made by endoscopy. Management of type 1 is conservative or endoscopically depending on symptoms. For type 2 to 4 defects initial tracheostomy for securing airway and gastrostomy for feeding is needed. This is followed by repair of the LTC using an anterior laryngeal approach for type 2 & 3 and combined cervico-thoracic approach for type 4 and those associated with esophageal atresia. Morbidity (leaks, pharyngoesophageal incoordination and reflux) and mortality is very high. Early suspicion and diagnosis are crucial.

1- DuBois JJ, Pokorny WJ, Harberg FJ, Smith RJH: Current Management of Laryngeal and Laryngotracheal Clefts. J Pediatr Surg 25(8): 855-860, 1990
2- Parsons DS, Stivers FE, Giovanetto DR, Phillips SE: Type I posterior laryngeal clefts. Laryngoscope 108(3):403-10, 1998
3- Corbally MT, Fitzgerald RJ, Guiney EJ, Ward D, Blayney A: Laryngo-tracheo-oesophageal cleft: a plea for early diagnosis. Eur J Pediatr Surg 3(4):241-3, 1993
4- Robie DK, Pearl RH, Gonsales C, Restuccia RD, Hoffman MA: Operative strategy for recurrent laryngeal cleft: a case report and review of the literature. J Pediatr Surg 26(8):971-3, 1991
5- Myer CM 3d, Cotton RT, Holmes DK, Jackson RK: Laryngeal and laryngotracheoesophageal clefts: role of early surgical repair. Ann Otol Rhinol Laryngol 99(2 Pt 1):98-104, 1990

Wilms Genetics

Wilms tumor (WT) development involves at least three genes. The first of these identified as WT1 is a suppressor gene mapped to a deletion of chromosome 11p13 found in sporadic and heredofamilial cases. WT1 required for normal renal development encodes a zinc finger binding protein that is important in regulating the formation of the early nephron. Mutations of WT1 are found in WT associated with aniridia, genitourinary defects (hermaphroditism) and mental retardation. The second WT suppression gene is WT2, known to be involved in the Beckwith Wiedemann locus located in the 11p15 region. The WT3 locus is likely to be found in the long arm of chromosome 16q and is suspected of tumor progression rather than initiation. WT gemlike mutations will help determine if they are additional indicators of clinical behavior and outcome.

1- Fleming S: Genetics of kidney tumors. Forum 8(2):176-84, 1998
2- Haase GM; Ritchey ML: Nephroblastoma. Semin Pediatr Surg 6(1):11-6, 1997
3- Feinberg AP: Multiple genetic abnormalities of 11p15 in Wilms' tumor. Med Pediatr Oncol 27(5):484-9, 1996
4- Tay JS: Molecular genetics of Wilms' tumour. J Paediatr Child Health 31(5):379-83, 1995
5- Coppes MJ, Haber DA, Grundy PE: Genetic events in the development of Wilms' tumor. N Engl J Med 331(9):586-90, 1994
6- Slater RM, Mannens MM: Cytogenetics and molecular genetics of Wilms' tumor of childhood. Cancer Genet Cytogenet 61(2):111-21, 1992

Volume 12 No 05 MAY 1999

Müllerian Inclusions

Inguinal hernia repair is the most common procedure performed in the pediatric age group. Unintentional vas deferens injury has been reported in as much as 1.5% of cases. Occasionally the pathologist will report glandular or epithelial like structure in hernial sac tissue. These müllerian inclusions remnants found in hernial sacs are a great cause of concern since they can resemble and be confused with segments of vas deferens or epididymis leading to the erroneous conclusion that a functional reproductive structure has been disrupted. Müllerian inclusions can be identified in 6% of hernial sacs, mostly in the prepubertal age child. These structure are lined by ciliated columnar epithelial and surrounded by a rim of condensed fibrous tissue of variable thickness. They arise from paratesticular embryonal remnants. They are usually one or two, rounded, embedded in fibrous connective tissue and associated with small blood vessels. Masson trichrome staining will show that the connective tissue of the inclusion is composed of fibroblasts without a smooth muscle component as seen in normal vas deferens. Size is another factor as the mean remnant diameter is 0.17 to 0.19 mm, and does not change significantly with age. Normal vas deferens diameter goes from 0.69 to 1.5 mm. If the microscopic evaluation reports vas deferens the possibility of surgical injury or duplication should be considered. This issue should be discuss with the family and later evaluation for infertility done.

1- Lugo-Vicente HL: The pediatric inguinal hernia: is contralateral exploration justified? Bol Asoc Med P R 87(1-2):8-11, 1995
2- Walker AN, Mills SE: Brief Scientific Reports: Glandular Inclusions in Inguinal Hernial Sacs and Spermatic Cords. Am J Clin Pathol 82:85-89, 1984
3- Tolete-Velcek F, Leddomado E, Hansbrough F,Thelmo WL: Alleged resection of the vas deferens: medicolegal implications. J Pediatr Surg 23:21-3, 1988
4- Popek EJ: Embryonal remnants in inguinal hernia sacs. Hum Pathol 21(3):339-49, 1990
5- Gomez-Roman JJ, Mayorga M, Mira C, Buelta L, Fernandez F, Val-Bernal JF: Glandular inclusions in inguinal hernia sacs: a clinicopathological study of six cases. Pediatr Pathol 14(6):1043-9, 1994
6- Gill B, Favale D, Kogan SJ, Bennett B, Reda E, Levitt SB: Significance of accessory ductal structures in hernia sacs. J Urol 148(2 Pt 2):697-8, 1992
7- Binderow SR, Shah KD, Dolgin SE: True duplication of the vas deferens. J Pediatr Surg 28(2):269-70, 1993

Neuroblastoma Stage III

Neuroblastoma (NB) in early stages of development (stage I & II) benefits from surgical excision. The role of surgery in the management of neuroblastoma stage III tumor (tumor infiltrating across the midline with or without lymph node involvement) is controversial. Many variables enter the formula of determining risk of disease, i.e., age, site, stage, N-myc status, DNA diploidy and Shimada classification to mention a few of the most important. Some reports have independently found that stage III managed initially with chemotherapy and radiotherapy and is responding benefits from eventual complete tumor excision despite site, age or histology. Complete surgical excision as determine by free margin of tissue has a significant survival advantage overall. Preop chemotx converts a friable tumor into a firmer, more mature and easily resectable tumor. Surgical complications in advance stages are higher (bleeding, nephrectomy, adjacent organ removal, infection). Some have found that complete resection is not needed in biologically favorable children with NB less than one year of age. Biologically unfavorable patients one year of age or greater who undergo gross surgical resections has improved survival. Defining subgroups of patient with poor prognostic biologic markers and histology to decide whether surgery or bone marrow transplant is the next best option is pending trial randomization and study.

1- Matthay KK, Perez C, Seeger RC, etal: Successful treatment of stage III neuroblastoma based on prospective biologic staging: a Children's Cancer Group study. J Clin Oncol 16(4):1256-64, 1998
2- Haase GM, O'Leary MC, Ramsay NK, et al: Aggressive surgery combined with intensive chemotherapy improves survival in poor-risk neuroblastoma. J Pediatr Surg 26(9):1119-23, 1991
3- Haase GM, Wong KY, deLorimier AA, Sather HN, Hammond GD: Improvement in survival after excision of primary tumor in stage III neuroblastoma. J Pediatr Surg 24(2):194-200, 1989
4- Powis MR, Imeson JD, Holmes SJ: The effect of complete excision on stage III neuroblastoma: a report of the European Neuroblastoma Study Group. J Pediatr Surg 31(4):516-9, 1996
5- Tsuchida Y, Yokoyama J, Kaneko M, etal: Therapeutic significance of surgery in advanced neuroblastoma: a report from the study group of Japan. J Pediatr Surg 27(5):616-22, 1992
6-  Kaneko M, Iwakawa M, Ikebukuro K, Ohkawa H: Complete resection is not required in patients with neuroblastoma under 1 year of age. J Pediatr Surg 33(11):1690-4, 1998
7- Kaneko M, Ohakawa H, Iwakawa M: Is extensive surgery required for treatment of advanced neuroblastoma? J Pediatr Surg 32(11):1616-9, 1997

Piriform Sinus

Congenital piriform sinus fistulas or cysts are a cause of acute/ recurrent suppurative thyroiditis or adenitis. Derived from the fourth pharyngeal pouch (ultimobranchial body), almost all occur on the left side of the neck. They present as a lateral cervical cyst or sinus anterior to the sternocleidomastoid muscle. The fistulae arises from the hypopharynx and end in or next to the thyroid lobe. Esophagoscopy can help visualized the pyriform orifice connected to the cyst. CT-Scan suggests the diagnosis. In the acute situation they may need incision and drainage to convert it into a draining sinus, followed later by excision. As cysts they should be removed completely. The side wall of the piriform sinus is opened with the help of a laryngoscope and the bottom part of the mucosa of the sinus transected with the internal orifice of the fistula, after which the fistula is removed en bloc. Histology will show thyroid or thymic tissue in the wall of the cyst.

1- Mouri N, Muraji T, Nishijima E, Tsugawa C: Reappraisal of lateral cervical cysts in neonates: Pyriform Sinus Cyst as an Anatomy-based Nomenclature. J Pediatr Surg 33(7): 1141-1144, 1998
2-Edmonds JL, Girod DA, Woodroof JM, Bruegger DE: Third branchial anomalies. Avoiding recurrences.Arch Otolaryngol Head Neck Surg 123(4):438-41, 1997
3- Hashizume K, Kawarasaki H, Iwanaka T, Kanamori Y,Tanaka K, Utsuki T, Komuro H, Uno K: A new operational approach for the piriform sinus fistula. Surg Today 23(4):293-7, 1993
4- Boix-Ochoa J, Pumarola Segura F, Asensio Llorente M: Piriform sinus fistula, a new disease. An Esp Pediatr 36(6):467-9, 1992
5- Miyauchi A, Matsuzuka F, Kuma K, Katayama S: Piriform sinus fistula and the ultimobranchial body. Histopathology 20(3):221-7, 1992
6- Miyauchi A, Matsuzuka F, Kuma K, Takai S: Piriform sinus fistula: an underlying abnormality common in patients with acute suppurative thyroiditis. World J Surg 14(3):400-5, 1990

VOLUME 12 No 06 JUNE 1999


Tracheomalacia refers to a structural/functional generalized or localized weakness of the tracheal rings' support resulting in partial respiratory obstruction. Most cases are associated with esophageal atresia and as such flaccid tracheal development after external pressure from the dilated proximal blind esophageal segment has been proposed as pathogenetic mechanism. Vascular rings, prolonged ventilatory support and tracheotomy are secondary causes of tracheomalacia. Most cases develop expiratory obstruction since only the intrathoracic trachea if affected. The harsh barking cough is the most characteristic initial symptom. Nutritional problems are the result of difficulty breathing as cyanotic attacks might occur during feeding. Other incitatory elements are intercurrent respiratory infections and aspiration. Severe forms are characterized by life-threatening apneic spells, inability to extubate the airways, and episodic pneumonia. A cough and wheeze may progress to complete airway obstruction and cyanosis. Diagnosis is obtained with simple lateral thoracic films (narrow slit-like appearance), bronchoscopy during spontaneous breathing (antero-posterior narrowing in expiration), cinetracheobronchography (allows extent of tracheal collapse) or cine CT studies. Reflux must be rule out and manage aggressively. For mild to moderate symptoms no management is necessary as the child will improve with time. For severe life threatening tracheomalacia aortopexy must be undertaken.  Failed aortopexy may need tracheal reinforcement with autologous cartilaginous grafts.

1- Spitz L and Phelan PD: Chap. 22 Tracheomalacia, In Beasley SW, Myers NA and Auldist AW "Oesophageal Atresia", Chapman & Hall Medical Publishers, New York, 1991, pags 331-340
2- Kimura K, Soper RT, Kao SCS et al: Aortosternopexy for tracheomalacia following repir of esophageal atresia: Evaluation by cine-CT and technical refinement. J Pediatr Surg 25:769-72, 1990
3- Cacciaguerra S, Bianchi A: Tracheal ring-graft reinforcement in lieu of tracheostomy for tracheomalacia. Pediatr Surg Int 13(8):556-9, 1998
4- Greenholz SK, Karrer FM, Lilly JR: Contemporary surgery of tracheomalacia.   J Pediatr Surg 21(6):511-4, 1986
5- Messineo A, Filler RM: Tracheomalacia. Semin Pediatr Surg 3(4):253-8, 1994


Munchausen by proxy (MBP) syndrome refers to a behavioral affected parent or caretaker that fabricates or induces an illness in a child and persistently seeks medical care. MBP affects children less than six years of age. The working definitions for MBP are: illness in a child simulated or produced by a parent, persistent presentation of the child for medical care, denial of knowledge by the parent as to the etiology of the child's illness and acute symptoms and signs in the child that abates when he is separated from the instigator. The most common symptoms are: bleeding, seizures, central nervous system depression, apnea, diarrhea and vomiting.  The perpetrator (mostly the mother) uses a variety of methods to obtain this means: strangulation,  poisoning, laxative administration, etc. The most difficult issue is diagnostic confirmation, parental confrontation and optimal medicolegal disposition of involved children. Secret video telemetry, poisonous toxin screening and detailed past medical history collection are useful. Children might have an unnecessary diagnostic tests and operation (fundoplication, central venous catheter placement) before establishing the diagnosis of MBP. When faced with a patient with enigmatic signs and symptoms and a family with classic personality traits consider the diagnosis. Disappearance of symptoms after removal of the suspected perpetrator remains the key to diagnosis.

1- Lacey SR, Cooper C, Runyan DK, Azizkhan RG: Munchausen syndrome by proxy: patterns of presentation to pediatric surgeons. J Pediatr Surg 28(6):827-32, 1993
2- Rosenburg D: Web of deceit: A literature review of Munchausen syndrome by proxy. Child Abuse Neg 11:547-563, 1987
3- Feldman KW, Hickman RO: The Central Venous Catheter as a Source of Medical Chaos in Munchausen Syndrome by Proxy. J Pediatr Surg 33(4): 623-627, 1998
4- Senocak ME, Turken A, Buyukpamukcu N: Urinary Obstruction Caused by Factitious Urethral Stones: An amazing manifestation of Munchausen syndrome by proxy. J Pediatr Surg 30(12): 1732-1734, 1995

Blue Rubber Bleb Nevus Syndrome

Blue rubber bleb nevus (BRBN) syndrome (also Bean's syndrome) is a rare congenital condition characterized by the presence of multiple angiomatic lesions in the skin (soft, rubbery, and compressible). They are associated with similar lesions in other organs, namely the gastrointestinal tract and oral cavity causing anemia through chronic bleeding or intussusception. Skin or endoscopic biopsy reveals the lesions to be cavernous hemangiomas. Clinically the child presents with hematemesis, melena and has multiple bluish rubber bleb-like hemangiomas over the body, in the stomach, jejunum and colon. The syndrome is likely caused by a gene mapping to chromosome 9p and shows autosomal dominant inheritance. Alpha-2a interferon therapy has been found beneficial for relieving the life-threatening consumptive coagulopathy associated with BRBN. The GI hemangiomas can be managed with intermittent laser-steroid therapy. Therapy is mainly symptomatic directed to complications.

1- McKinlay JR, Kaiser J, Barrett TL, Graham B: Blue rubber bleb nevus syndrome. Cutis 62(2):97-8, 1998
2- Goraya JS, Marwaha RK, Vatve M, Trehan A: Blue rubber bleb nevus syndrome: a cause for recurrent episodic severe anemia. Pediatr Hematol Oncol 15(3):261-4, 1998
3- Kunishige M, Azuma H, Masuda K, Shigekiyo T, Arii Y, Kawai H, Saito S:  Interferon alpha-2a therapy for disseminated intravascular coagulation in a patient with blue rubber bleb nevus syndrome. A case report. Angiology 48(3):273-7, 1997
4- Dieckmann K, Maurage C, Faure N, Margulies A: Combined laser-steroid therapy in blue rubber bleb nevus syndrome: case report and review of the literature. Eur J Pediatr Surg 4(6):372-4, 1994
5- Oranje AP: Blue rubber bleb nevus syndrome. Pediatr Dermatol 3(4):304-10, 1986
6- Browne AF, Katz S, Miser J, Boles ET Jr: Blue Rubber Bleb Nevi as a cause of intussusception. J Pediatr Surg 18(1):7-9, 1983

Journal Club