PSU Vol 12, 1999

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PEDIATRIC SURGERY UPDATE

VOLUME 12, 1999

Volume 12 No 01 JANUARY 1999

Biliary Hypoplasia

Biliary hypoplasia is a rare cause of persistent neonatal conjugated hyperbilirubinemia. Pathologically, affecte d children have absent or reduced number of bile d

distribution of branches of the portal ve in and hepatic artery within the liver parenchyma. Biliary hypoplasia is also identified as paucity of interlobular bile ducts

PILBD are recognized: 1) syndromic (arteriohepatic dysplasia or Alagille's syndrome) with characteristic extrahepatic abnormalities (fascial appearance, pulmoni

anomalies, embryotoxon and delayed weight-height development), and 2) non-syndromic biliary hypoplasia. Biliary hypoplasia is clinically indistinguishable from b

sometimes be confused. A definitive diagnosis is difficult to make in early infancy. Differentiation between biliary atresia, hypoplasia and neonatal hepatitis conti

visualization of the biliary ducts. This mean laparoscopic or open intra-operative cholangiography and liver biopsy. The cholangiogram will show diminutive intra-

tree. Attempts to es tablish biliary flow by means of hepatic porto-enterostomy (Kasai procedures) in children with PILBD have been unsuccessful and contraindic

conservative and include predigested formulas, ursodeoxycholic acids (10 mg/kg/day), phenobarbital and A,D,K,E vitamin replacement. Non-syndromic PILBD h

prognosis. Children with syndromic PILBD identified in infancy because of choles tasis have a 50% probability of long-term survival without liver transplantation.

References

1- Cynamon HA, Powell GK, Isenb erg JN, Lobe TE: Support for a co nse rvative ap proach to mixed intrahepatic and extrahepatic biliary hy poplas ia. 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 dys plasia (Alagille's sy ndrome): a common cause of co njugated hyperb ilirubinemia. Ann Clin Lab Sci 14(6):480-6, 1984

4- Kahn EI, Daum F, Markowitz J, Aiges HW, Schne ider KM, So HB, Altman P, Chandra RS, Silverberg M: Arteriohep atic dys plasia. II. Hepatob iliary morphology. Hepa tology 3(1):77-84, 19835- 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 Pe

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 major

been divided in three categories: 1) superior - occurring in the superior lumbar triangle (Grynfelt-Lesshaft), 2) inferior - occurring through the inferior lumbar tria

combination of them. They have a well-defined fascial defect. Acquired lumbar hernias outnumber congenital hernias and may res ult from surgery, infection and/o

defect enlarges with growth or have the potential to incarcerate early operative repair is preferred. Lumbar hernias are associated to the lumbo-costo-vertebral s

anomalies, diaphragmatic hernia, ureteropelvic junction obstruction, cloacal exstrophy and lipomeningocele ). Repair of small defe cts can be accomplished by pri

recurrent defects may need gortex patching. When they include a more extensive deficiency of the entire lateral abdominal wall extending to the rectus sheath an

may also need prosthetic material.

References1- Somuncu S, Bernay F, Rizalar R, Ariturk E, Gunay din M, Gurses N: Congenital lumbar hernia ass ociated with t he lumbocos tovertebral syn drome: two case s. Eur J Ped iatr Surg. 7(2)122-124, 1997

2- Fakhry SM, Azizkhan RG: Obse rvations and current o perative management of con genital lumbar hernias during infancy . Surg Gynecol Obs tet. 172(6)475-479, 1991

3- Pul M, Pul N, Gurses N: Congenital lumbar (Grynfelt-Less haft) h ernia. 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 dise ase (CD) also known as angiofollicular lymph node hyperplasia is a benign disorder characterized by enlarged hyperplastic lymph nodes that occ

Two classes of CD are identified in children: Hyaline-vascular (HV) type characterized by vascular proliferation and hyalinization, and the plasma cell (PC) type c

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 gro

chest (mediastinum or lung hilum), or neck mass. Within the abdomen CD is located in the small bowel mesentery. May be either asymptomatic or prese nt syste

malaise, increase ESR, thrombocytosis , anemia and hypergammaglobulinemia. The enlarged nodes may mimic a malignant tumor of the lymphoid syste m. Histop

confirms the diagnosis. Surgical excision of the mass and surrounding nodes involved is necessary to affect a cure.

References

1- Shroff VJ, Gilchrist BF, DeLuca FG, McCombs HL, Wess elhoeft CW: Cas tleman's Disease Pres enting as a Pediatric Surgical Prob lem. J Ped iatr Surg 30 (5): 745-747, 1995

2- Patel U, Forte V, Taylor G, Sirkin W: Cas tleman's diseas e as a rare cau se of a n eck mass in a child. J Otolaryng ol 27(3):171-3, 1998

3- Tuerlinckx D, Bodart E, Delos M, Remacle M, Ninane J: Unifocal cervical Cast leman d isease in two ch ildren. Eur J Ped iatr 156(9):701-3, 1997

4- Bapat KC, Malde HM, Pandit A A, Mitta l BV, Kedar RP, Bapat RD, Relekar RG: Solitary retroperitoneal angiofollicular lymph node hype rplasia. J Pos tgrad 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, Mars h WL: Castleman's disease in children. J Ped iatr 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 aaffected 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

significant air-trapping and hyperinflation of the affecte d lobe or lung. The mediastinum shifts to the opposite s ite of the FB. Alternatively, with a total blockage o

volume with atelectasis and shift of the mediastinum to the same side. Diagnosis relies on clinical judgment, history, physical exam and radiographic evaluation. T

aspiration event followed by coughing, wheezing, dyspnea, fever, wheezing or decrease breath sounds over the affected hemithorax. After chest films confirmatio

rigid bronchoscopy for extraction of the FB. With history of choking crisis and mild symptoms bronchoscopy should also be done. Peanuts, corns, beans and see ds

offending agents causing further damage by virtue of an associated inflammatory reaction. A forceps during bronchoscopy will help extracted most FB. Other tim

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needed in case of segmental bronchus position. Longstanding foreign body in the airway may be responsible for irreversible complications (bronchiectasia).

References

1- Silva AB, Mu ntz HR, Clary R: Utility of co nvent ional radiography in the d iagnos is and management of p ediatric airway foreign bod ies. An n Otol Rhinol Laryngol 107(10 Pt 1):834-8, 1998

2- Cataneo AJ, Reibsch eid SM, Ruiz Junior RL, Ferrari GF: Foreign bo dy in the tracheob ronchial tree. Clin Ped iatr (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):11

4- Reilly J, Thompso n J, MacArth ur C, Pransky S, Beste D, Smith M; Gray S, et al: Pediatric aerodigest ive foreign body injuries are co mplications related to timeliness o f diagnos is. Laryngoscope 107(1):

5- Black RE, Johnson DG, Matlak ME: Bronch oscopic removal of as pirated foreign bodies in children. J Pediatr Surg 29(5):682-4, 19946- Menend ez AA, Gotay Cruz F, Seda FJ, Velez W, Trinidad Pinedo J: Foreign body asp iration: experience at the Univers ity Pediatric Hosp ital. 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 tracheobronch

parenchyma may be Intralobar (IS) or Extralobar Seques tration (ES). Intralobar is contained within the visce ral pleural of a lower lobe receiving the blood supply

other thoracic vessel. It is believed IS are acquired postinfectious process due to their association with chronic recurrent lung infection and reactive airway diseas

malformation with variable ectopic blood supply (aorta) having its own pleural investment separate from normal lung, containing typical features of CCAM-2 (40

malformations (40%). Both types can have patent communication with foregut. Prenatal diagnosis can be obtained with real-time US with Doppler imaging (can ca

mediastinal shifts and hydrops). Postnatally, contrast-enhanced CT may establish the diagnosis e liminating the need for more invasive imaging (arteriography).

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 co

managed with resection alone, while IS needs lobectomy. Anecdotal cases of partial or total disappearance of these masses while asymptomatic has been reporte

References

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):2

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):23

3- Nuchtern JG, Harberg FJ: Congenital lung cys ts. Semin Ped iatr Surg 3(4):233-43, 1994

4- Sade RM, Clouse M, Ellis FH Jr: The s pectrum of p ulmonary seques tration. An n Tho rac 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 enlarge

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 lo

patients justify surgical correction. Repair is performed through a transverse or submammary incision with subperiosteal resection of the lower costal cartilages f

junction bilaterally. The sternum is fractured to straighten it and there no need for sternal strut use in this deformity. Complications can include seroma, atelectas

well-motivated, s kele tally immature individual body cast bracing can be an effective treatment for cosmetically displeasing pectus carinatum.

References

1- Lacquet LK, Morsh uis W J, Folgering HT: Long-term results after correct ion of an terior ches t wall deformities. J Cardiovas c Surg 39(5):683-8, 1998

2- Fonkalsrud EW, Salman T, Guo W, Gregg JP: Repair of pect us deformities with stern al sup port. J Thorac Cardiovas c Surg 107(1):37-42, 1994

3- Mielke CH, Winter RB: Pectus ca rinatum success fully treated with b racing. A case report. Int Orthop 17(6):350-2, 1993

4- Shamberger RC, Welch KJ: Surgical correct ion of p ectus carinatum. J Pediatr Surg 22(1):48-53, 19875- Robicsek F, Cook JW , Daughe rty HK, Selle JG: Pectus carinatum. J Tho rac Cardiovas c Surg 78(1):52-61, 1979

Volume 12 No 03 MARCH 1999

Asplenia

The absence of the spleen (asplenia) occurs after surgical removal, following chronic conditions or congenital. Trauma is the most common cause of removing the

cell disease is the most common cause of functional asplenia in children. Congenital absence of the spleen is usually associated with serious malformations, prima

abdominal heterotaxia. The spleen contributes importantly to the normal and pathologic removal of blood cells from the circulation and to defense against infectio

Asplenia increases the risk of fulminant bacteremia (post-splenectomy sepsis) and mortality with these organisms. This risk is also increased by the underlying co

removal of the spleen, i.e., trauma, malignancy or hematologic disease. Several recommendations have been given when dealing with an asplenic individual. Thes

against pneumococcus (Pneumovax vaccine), hemophilus influenza type b and meningococcus. Regarding Pneumovax use revaccination after 3-5 ye ars is recom

asplenia who are 10 ye ars of age or younger and for older children and adults who were immunized at least five years before. Duration of vaccine-induced antibod

shorter than that in normal persons. Long-term antimicrobial prophylaxis is also used. This carries the problem of compliance and for how long. Significant febrileaggressively, and probably most important, the patient and family should be carefully educated about this complication (name tag). Most deaths from hyposplenia

preventable.

References

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: p hys iology an d s plenic hyp ofunction . Crit Rev Oncol Hematol 7(1):1-36, 1987

4-Phoon CK; Neill CA: Asplenia sy ndrome: insight into embryology th rough an an alysis of cardiac an d extracardiac anomalies. Am J Cardiol 15;73(8):581-7, 19945- Styrt B: Infection as so ciated with as plenia: risks, mechanisms, an d prev ention. A m 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 (S

(Wirsung) ducts drain separately. Diagnosis is made with ERCP (short duct of Wirsung that does not communicate with main pancreatic duct of Santorini). Not evanomaly develops pancreatitis. Likewise with the minor papilla draining the bulk of the pancreas in PD, a small orifice s ize (< 0.75 mm) plays a role in outflow obs

pancreatitis. Children with PD and recurrent e pisodes of pancreatitis will need endoscopic sphincterotomy of the minor and sometimes major papilla. If not feas ibl

sphincteroplasty of both papillae along with cholecystectomy (bile stasis leads to gallstones) is indicated. Intraoperative pancreatogram will help determine if bot

chronic pancreatitis is established, ductal drainage or resection may be necessary.

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References

1- O'Rourke RW, Harrison MR: Pancreas divisum and s tenos is of the major and minor pap illae in an 8-year-old girl: treatment by dua l sphincte roplasty . 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 Pediat

3- Sanada Y, Yoshizawa Y, Chiba M, Nemoto H, Midorikawa T, Kumada K: Ventral pancreatitis in a p atient with p ancreas divisum. J Pediatr Surg 30(5):665-7, 1995

4- Crombleholme TM, deLorimier AA, Way LW, Ad zick NS: The modified Puesto w procedu re for chro nic relapsing pancreat itis in children. J Pediatr Su rg 25(7):749-54, 1990

5- Adzick NS, Shamberger RC, Winter HS, Hendren W H: Surgical treatment of pan creas divisum caus ing pan creatitis in ch ildren. J Pediatr Surg 24(1):54-8, 19896- 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 l

peritonitis occurs when the meconium leave s the bowel, enters the peritoneal cavity and spreads throughout causing a s terile inflammatory reaction. Most commo

the distal ileum, and 50% of babies with MP develop intestinal obstruction. Prenatal ultrasound findings include ascites, intraabdominal masses , bowel dilatation

intraabdominal calcifications. Bowel disorders which lead to MP in utero are those resulting in bowel obstruction and perforation, such as small bowel atresias , vo

MP can be divided into simple or complex. Cases with spontaneously healed perforation (simple MP) need observation as they rarely develop symptoms. Newbor

with bowel obstruction a/or pseudocyst formation (localized collection of meconium contained in a cys t made of fibrous granulation tissue). Complex MP needs sur

References

1- Patton WL, Lutz AM, W illmann JK, Callen P, Barkovich AJ, Gooding CA: Systemic spread of meconium peritonitis. Ped iatr Radiol 28(9):714-6, 1998

2- Dirkes K, Crombleholme TM, Craigo SD, Latchaw LA, Jacir NN, Harris BH, D'Alton ME: The Natu ral History o f Meco nium Peritonitis Diagnos ed in Utero. J Ped iatr Surg 30(7): 979-982, 1995

3- Miller JP, Smith SD, Newman B, Sukarochana K: Neonatal ab dominal calcification: is it always meconium peritonitis? J Pediatr Surg 23(6):555-6, 1988

4- Olsen MM, Luck SR, Lloyd-Still J, Raffensperg er JG: The sp ectrum of mecon ium disease in infancy. J Pediatr Su rg 17(5):479-81, 19825- 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 infec

situations. Most symptoms resolve when the valvular competence of the cardia develops in the second year of life. Older children manifest symptoms of dysphagi

manage most children medically. Indications for surgery consis t of failure to thrive, esophagitis, stricture, chronic aspiration pneumonia, life-threate ning events a

changes. Children referred for surgery should have an esophagogram, endoscopy, and evaluation of gastric emptying mechanisms to document: magnitude of refl

incoordination, dysmotility, strictures, malrotation, and grade of esophagitis. pH studies and milk scans may farther find a cause and effect relationship between r

problems. Surgical options consist of partial (Thal, Boix-Ochoa, Toupe) or complete wrap (Niss en) fundoplasty reconstruction. The procedure can be done open o

with delayed gastric emptying will benefit from a gastric emptying procedure (pyloroplasty, antroplasty). Neurologically impaired children referred for feeding gas

similar work-up to identified potentially dangerous reflux problems. Alternatively the gastrotomy can be constructed percutaneously and the problems of GERD b

Neurologic status and gastric emptying are major predictors of operative success.

References1- Bustorff-Silva J, Fonkalsrud EW, Perez CA, Quintero R, Martin L, Villese or E, Atkinson JB: Gast ric Emptying Procedures Decrease the Risk of Po sto perative Recurrent Reflux in Children with Delayed

34(1): 79-83, 1999

2- Chung DH, Georgeson KE: Fundop lication and g ast rosto my. Semin Pediatr Su rg 7(4):213-9, 1998

3- Jolley SG: Gast roeso phageal reflux diseas e as a cau se fo r emesis in infant s. 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 su rgical cons iderations in gas troesophag eal reflux diseas e 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 eso phagu s in children. J Pediatr Surg 27(4):462-5, 19927- Pearl RH, Robie DK, Ein SH, Shandling B, Wes son DE, Superina R, Mct aggart K, Garcia VF, O'Conno r JA, Filler RM: Complications o f gas troesophag eal antireflux surg ery in neu rologically impaired ve

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 bet

sys tem 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 t

trachea and type 4 into the thoracic trachea). LTC arises from errors in chondrification and fusion of the laryngeal s upporting cartilage or tracheo-e sophageal fol

"G" and Pallister-Hall syndrome, to esophageal atresia and to anal malformations. As neonate they present a hoarse cry, inability to handle secretions, cyanosis,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

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 larynge

and combined cervico-thoracic approach for type 4 and those as sociated with esophageal atresia. Morbidity (leaks, pharyngoes ophageal incoordination and reflu

Early suspicion and diagnosis are crucial.

References

1- DuBois JJ, Pokorny W J, Harberg FJ, Smith RJH: Current Management of Laryngeal an d Laryngo tracheal Clefts. J Ped iatr Surg 25(8): 855-860, 1990

2- Parsons DS, Stivers FE, Giovanett o DR, Phillips SE: Type I p os terior laryngeal clefts. Laryngoscope 108(3):403-10, 1998

3- Corbally MT, Fitzgerald RJ, Guiney EJ, Ward D, Blayney A: Laryngo-trach eo-oes ophageal cleft: a plea for ea rly diagnos is. Eur J Pediatr Surg 3(4):241-3, 1993

4- Robie DK, Pearl RH, Gons ales C, Restu ccia RD, Hoffman MA: Operative s trategy for recurrent laryngeal cleft: a cas e report and review of the literature. J Pediatr Surg 26(8):971-3, 19915- Myer CM 3d, Cotto n RT, Holmes DK, Jackson RK: Laryngeal and laryng otracheo eso phageal clefts: role of early s urgical repair. Ann Otol Rhinol Laryngo l 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 11p

heredofamilial cases . WT1 required for normal renal development encodes a zinc finger binding protein that is important in regulating the formation of the early n

are found in WT associated with aniridia, genitourinary defects (hermaphroditism) and mental retardation. The second WT suppression gene is WT2, known to be

Wiedemann locus locate d in the 11p15 region. The WT3 locus is like ly to be found in the long arm of chromosome 16q and is suspected of tumor progress ion rath

gemlike mutations will help determine if they are additional indicators of clinical behavior and outcome.

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References

1- Fleming S: Genetics of kidney tumors. Forum 8(2):176-84, 1998

2- Haase GM; Ritchey ML: Nephroblasto ma. Semin Ped iatr Surg 6(1):11-6, 1997

3- Feinberg AP: Multiple g enetic abnormalities of 11p15 in Wilms' tumor. Med Pediatr Onco l 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 ev ents 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

Mllerian 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

pathologist will report glandular or epithelial like structure in hernial sac tissue. These mllerian inclusions remnants found in hernial sacs are a great cause of co

resemble and be confused with segments of vas deferens or epididymis leading to the erroneous conclusion that a functional reproductive structure has been disr

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 co

variable thickness. They arise from paratesticular embryonal remnants. They are usually one or two, rounded, e mbedded in fibrous connective tissue and associa

Masson trichrome staining will show that the connective tissue of the inclusion is compose d of fibroblasts without a smooth muscle component as seen in normal v

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 t

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 infe

References

1- Lugo-Vicente HL: The p ediatric inguinal hernia: is contralateral exploration jus tified? Bol Aso c Med P R 87(1-2):8-11, 1995

2- Walker AN, Mills SE: Brief Scientific Reports : Glandular Inclus ions in Inguinal Hernial Sacs and Spermatic Cords. A m J Clin Path ol 82:85-89, 1984

3- Tolete-Velcek F, Leddomado E, Hansbro ugh F,Thelmo WL: Alleged res ection o f the vas deferens : medicolegal implications . J Pediatr Surg 23:21-3, 1988

4- Popek EJ: Embryonal remnants in inguinal h ernia s acs. Hum Pathol 21(3):339-49, 1990

5- Gomez-Roman JJ, Mayo rga M, Mira C, Buelta L, Fernandez F, Val-Bernal JF: Glandular inclusions in inguinal hern ia sac s: a clinicopathological s tudy of s ix case s. Pediatr Patho l 14(6):1043-9, 1994

6- Gill B, Favale D, Kogan SJ, Benne tt B, Reda E, Levitt SB: Significance of accessory ducta l struct ures in hernia sacs . J Urol 148(2 Pt 2):697-8, 19927- Binderow SR, Shah KD, Dolgin SE: True dup lication of th e vas deferens . J Ped iatr 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

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,

and Shimada class ification to mention a few of the most important. Some reports have independently found that stage III managed initially with chemotherapy an

responding benefits from eventual complete tumor excision despite site, age or histology. Complete surgical excision as determine by free margin of tissue has a

overall. Preop chemotx converts a friable tumor into a firmer, more mature and easily rese ctable tumor. Surgical complications in advance stages are higher (blee

organ removal, infection). Some have found that complete resection is not needed in biologically favorable children with NB less than one year of age. Biologicall

year of age or greater who undergo gross surgical resections has improved survival. De fining subgroups of patient with poor prognostic biologic markers and hist

surgery or bone marrow transplant is the next best option is pending trial randomization and study.

References

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, W ong KY, deLorimier AA , Sathe r HN, Hammond GD: Improvement in su rvival after excision of primary tu mor in st age III n euroblas toma. 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 resect ion is no t required in pat ients with neuroblast oma under 1 year o f age. J Pediatr Surg 33(11):1690-4, 19987- Kaneko M, Ohakawa H, Iwakawa M: Is extensive s urgery req uired for treat ment of adv anced neurob lastoma? J Ped iatr Surg 32(11):1616-9, 1997

Piriform Sinus

Congenital piriform sinus fistulas or cys ts are a cause of acute/ recurrent suppurative thyroiditis or adenitis. Derived from the fourth pharyngeal pouch (ultimobra

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 hypopharynxthyroid lobe. Esophagoscopy can help visualized the pyriform orifice connected to the cyst. CT-Scan suggests the diagnosis. In the acute situation they may need

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

bottom part of the mucosa of the s inus transected with the internal orifice of the fistula, after which the fistula is removed en bloc. Histology will show thyroid or t

cyst.

References

1- Mouri N, Muraji T, Nishijima E, Tsugawa C: Reappraisal of lateral cervical cys ts in neona tes : Pyriform Sinus Cys t as an A natomy-bas ed Nomenclature. J Ped iatr Surg 33(7): 1141-1144, 1998

2-Edmonds JL, Girod DA, Woo droof JM, Bruegger DE: Third branchial anomalies. Avo iding recurrences .Arch Otolaryn gol Head Neck Surg 123(4):438-41, 1997

3- Hashizume K, Kawarasaki H, Iwanaka T, Kanamori Y,Tanaka K, Uts uki T, Komuro H, Uno K: A new op erational app roach for the piriform sinus fistula. Surg Today 23(4):293-7, 1993

4- Boix-Ochoa J, Pumarola Segura F, Asens io Llorente M: Piriform sinus fistula, a new diseas e. An Esp Pediatr 36(6):467-9, 1992

5- Miyauchi A, Matsuzuka F, Kuma K, Katayama S: Piriform sinus fistula and the u ltimobranchial body. Histo patho logy 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

Tracheomalacia refe rs to a structural/functional generalized or localized weakness of the tracheal rings' support resulting in partial respiratory obstruction. Most

esophageal atresia and as such flaccid tracheal development after external pressure from the dilated proximal blind esophageal segment has been proposed as p

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Vascular rings, prolonged ventilatory support and tracheotomy are secondary causes of tracheomalacia. Most cases develop expiratory obstruction since only the

affecte d. The harsh barking cough is the most characteristic initial symptom. Nutritional problems are the res ult of difficulty breathing as cyanotic attacks might o

incitatory elements are intercurrent respiratory infections and aspiration. Severe forms are characterized by life-threatening apneic spells, inability to extubate th

pneumonia. A cough and wheeze may progress to complete airway obstruction and cyanosis. Diagnosis is obtained with simple lateral thoracic films (narrow slit-li

bronchoscopy during spontaneous breathing (antero-posterior narrowing in expiration), cinetracheobronchography (allows exte nt of tracheal collapse) or cine CT

out and manage aggressively. For mild to moderate symptoms no management is necessary as the child will improve with time. For severe life threatening trache

undertaken. Failed aortopexy may nee d tracheal reinforcement with autologous cartilaginous grafts.

References

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: Aortos ternopexy for tracheomalacia following repir of es ophag eal atresia: Evaluation by cine-CT and technical refinement. J Pediatr Surg 25:769-72, 1990

3- Cacciaguerra S, Bianchi A: Trach eal ring-graft reinforcement in lieu of tracheos tomy for t racheomalacia. Pediatr Surg Int 13(8):556-9, 1998

4- Greenholz SK, Karrer FM, Lilly JR: Contemporary s urgery of trach eomalacia. J Pediatr Su rg 21(6):511-4, 1986

5- Messineo A, Filler RM: Tracheomalacia. Semin Pediatr Surg 3(4):253-8, 1994

Munchausen

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

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

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. T

are: bleeding, se izures, central nervous system depression, apnea, diarrhea and vomiting. The perpetrator (mostly the mother) uses a variety of methods to obtai

poisoning, laxative administration, e tc. The most difficult issue is diagnostic confirmation, parental confrontation and optimal medicolegal disposition of involved c

telemetry, poisonous toxin screening and detailed past medical history collection are useful. Children might have an unnecessary diagnostic tests and operation (f

catheter placement) before e stablishing the diagnosis of MBP. When faced with a patient with enigmatic signs and symptoms and a family with classic personality

Disappearance of symptoms after removal of the suspected perpetrator remains the key to diagnosis.

References

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 Med ical Chaos in Munchausen Synd rome 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,

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 t

compress ible). They are associated with similar les ions in other organs, namely the gastrointestinal tract and oral cavity causing anemia through chronic bleeding

endoscopic biopsy reveals the lesions to be cavernous hemangiomas. Clinically the child presents with hematemesis, melena and has multiple bluish rubber bleb-l

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-

been found beneficial for relieving the life-threatening consumptive coagulopathy associated with BRBN. The GI hemangiomas can be managed with intermittent

Therapy is mainly symptomatic directed to complications.

References

1- McKinlay JR, Kaiser J, Barrett TL, Graham B: Blue rub ber bleb nevus syn drome. 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, 19983- Kunishige M, Azuma H, Masu da K, Shigekiyo T, Arii Y, Kawai H, Saito S: Int erferon alpha-2a therapy for disseminated intrav ascu lar coagulation in a patient with blue rubb er bleb nevus sy ndrome. A

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 syn drome. 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

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