VOLUME 16, 2001

Volume 16 No 01 JANUARY 2001


Hydrometrocolpos (HMC) is accumulation of secretions in the vagina and uterus caused by one of two mechanisms: 1- excessive intrauterine stimulation of the infant's cervical mucous glands by maternal estrogen (secretory HMC), or 2- accumulation of urine (urinary HMC)in the presence of a vaginal obstruction. HMC can arise from congenital or acquired pathology. Acquired causes include vaginitis from Diphtheria or measles, senile vaginitis, from radiation therapy and corrosive vaginitis. Congenital vaginal obstruction causing HMC is due to imperforate hymen, transverse vaginal septum and persistence of urogenital sinus with complete distal vaginal obstruction. The child may present with a lower abdominal mass from the dilated vagina and uterus, urogenital sinus, obstructive uropathy (hydronephrosis), dribbling, respiratory distress, bowel obstruction and lower extremity venous congestion. HMC usually occurs in the neonatal period and the majority of cases are caused by vaginal occlusion by a transverse septum combined with cervical secretion. HMC can be associated with congenital adrenal hyperplasia when there is a long urogenital sinus. Physical exam (obvious vagina septum of imperforate hymen or a urogenital sinus), US (large cystic anechoic mass, anteriorly compress bladder and fluid-debris level), voiding cystogram, and sinoscopy can establish the cause of HMC. Vaginal decompression by catheter placement, endoscopic septotomy or vaginostomy is done initially followed later by opening of septum or vaginal pull-through.

1- Hahn-Pedersen J, Kvist N, Nielsen OH: Hydrometrocolpos: current views on pathogenesis and management. J Urol 132(3):537-40, 1984
2- Sawhney S, Gupta R, Berry M, Bhatnagar V: Hydrometrocolpos: diagnosis and follow-up by ultrasound--a case report. Australas Radiol 34(1):93-4, 1990
3- Arena F, Racchiusa S, Proietto F, Romeo C, Aloisi G, Cruccetti A, Visalli C, Zimbaro G, Romeo G: Urinary hydrometrocolpos by persistent urogenital sinus; prenatal diagnosis and neonatal management. Pediatr Med Chir 20(1):75-9, 1998
4- Bhatnagar V, Agarwala S, Mitra DK: Tubed vaginostomy: a new technique for preliminary drainage of neonatal hydrometrocolpos. Pediatr Surg Int 13(8):613-4, 1998
5- Amagai T, Ohkawa H, Kaneko M: Endoscopic septotomy: a new surgical approach to infantile hydrometrocolpos with imperforate hemivagina and ipsilateral renal agenesis. J Pediatr Surg 34(4):628-31, 1999
6- Arena F, Romeo C, Cruccetti A, Antonuccio P, Basile M, Romeo G: The neonatal management and surgical correction of urinary hydrometrocolpos caused by a persistent urogenital sinus. BJU Int 84(9):1063-8, 1999
7- Reynolds M: Neonatal Disorders of the External Genitalia and Vagina. Seminars Pediatr Surg 7(1): 2-7, 1998

Preduodenal Portal Vein

Preduodenal portal vein (PDPV) is an extremely rare vascular anomaly that could cause extrinsic obstruction of the second portion of the duodenum. This vascular anomaly is often symptomless, but in a few occasions can lead to intestinal obstruction requiring surgical correction. In 50% the  PDPV is combined with high intestinal obstruction and in one half of these is considered obstructive. Embryologically, the anomalous portal vein is the persistence of a preduodenal vitelline communicating vein and passes in front of the second portion of the duodenum. Most cases can be seen associated with duodenal atresia/stenosis, polysplenia, malrotation, annular pancreas, extrinsic adhesive bands, biliary atresia and cardiac defects. US can establish the diagnosis and UGIS will show a dilated stomach and first part of the duodenum with passage disturbance in the 2nd portion of the duodenum during fluoroscopy. PDPV is an extrinsic cause of duodenal obstruction that rarely needs bypass procedures.  Duodenoduodenal anastomosis anterior to the portal vein is the procedure of choice to manage this anomaly.

1- Georgacopulo P, Vigi V: Duodenal obstruction due to a preduodenal portal vein in a newborn. J Pediatr Surg 15(3):339-40, 1980
2- Esscher T: Preduodenal portal vein--a cause of intestinal obstruction? J Pediatr Surg 15(5):609-12, 1980
3- Fernandes ET, Burton EM, Hixson SD, Hollabaugh RS: Preduodenal portal vein: surgery and radiographic appearance. J Pediatr Surg 25(12):1270-2, 1990
4- Choi SO, Park WH: Preduodenal portal vein: a cause of prenatally diagnosed duodenal obstruction. J Pediatr Surg 30(10):1521-2, 1995
5- Nakada K, Kawaguchi F, Wakisaka M, Nakada M, Enami T: Digestive tract disorders associated with asplenia/polysplenia syndrome. J Pediatr Surg 32(1):91-4, 1997

Splenic Trauma

Spleen is the most common injured organ in blunt abdominal trauma. Hematologic and immunologic importance of the spleen has changed the attitude of trauma surgeons toward preservation of this organ whenever hemodynamics physiology permits. Massive hemorrhage (> 50 cc/kg weight) and hemodynamic instability are indications for surgery. CT-Scan continues to be the choice of imaging during blunt abdominal trauma to establish the diagnosis of solid organ rupture in blunt abdominal trauma and rule out other major abdominal injuries. Isolated splenic rupture can be managed conservative in almost 80-90% of cases reducing complications and post-splenectomy sepsis. Low velocity of injury, thicker capsule, ribs elasticity and transverse nature of the laceration explain propensity for spontaneous healing in children. Associated lesions are not a contraindication for conservative management. Should conservative management fails the next step is splenography or splenectomy. Child is admitted to intensive care for 48 hours, followed by in-hospital observation until stable to be discharge home. Vaccination (pneumococcus, hemophilus and meningococci) affords added protection. Sonography is helpful for sequential splenic imaging to show when the appearance returns to normal, though clinical exam suffices. Participation in body contact sports should be curtailed for at least three months after injury.

1- Adler DD, Blane CE, Coran AG, Silver TM: Splenic trauma in the pediatric patient: the integrated roles of  ultrasound and computed tomography. Pediatrics 78(4):576-80, 1986
2- Pearl RH, Wesson DE, Spence LJ, Filler RM, Ein SH, Shandling B, Superina RA: Splenic injury: a 5-year update with improved results and changing criteria for conservative management. J Pediatr Surg 24(1):121-4, 1989
3- Velanovich V, Tapper D: Decision analysis in children with blunt splenic trauma: the effects of observation, splenorrhaphy, or splenectomy on quality-adjusted life expectancy. J Pediatr Surg 28(2):179-85, 1993
4- Morse MA, Garcia VF: Selective nonoperative management of pediatric blunt splenic trauma: risk for missed associated injuries. J Pediatr Surg 29(1):23-7, 1994
5- Bond SJ, Eichelberger MR, Gotschall CS, Sivit CJ: Nonoperative management of blunt hepatic and splenic injury in children. Ann Surg 223(3):286-9, 1996
6-  Thaemert BC, Cogbill TH, Lambert PJ: Nonoperative management of splenic injury: are follow-up computed tomographic scans of any value? J Trauma 43(5):748-51, 1997

Volume 16 No 02 FEBRUARY 2001

Neuroblastoma Stage IV

Stage IV Neuroblastoma (metastatic NB) refers to high risk group of children with the primary tumor in the adrenal gland, mediastinum or pelvis associated with disease progression in other sites (bone marrow, cortical bone, liver, lymph node). Role of surgery in stage IV NB is controversial. Cure will require control of the primary tumor and elimination of metastatic disease. For infants with metastatic NB a more than 95% resection has been found adequate surgical treatment either initially or after effective chemotherapy. Adding ipsilateral lymph node dissection does not appear to affect survival. Delayed surgery after several courses of chemotherapy may be as effective as initial resection and is associated with fewer complications statistically. Resection without induction chemotx results in significant blood loss. High risk NB usually invades blood vessels and surrounding structures precluding resection. Intensive preop chemotherapy reduces tumor size and invasiveness allowing surgical removal. A fibrotic capsule forms with less blood supply to the tumor. Stage IV NB is best managed with initial chemotx until distant metastasis are controlled followed by primary gross tumor removal (even in the face of significant tumor reduction) and completion chemotx. Gross complete resection is best accomplished when a good partial response is obtained. Radiotx is added to unresectable lesions. Even when chemotx changes the tumor histology (Shimada) from unfavorable to favorable this does not improve overall outcome. Resection is not confounded by biology of the tumor (n-myc status). Survival is improved with kidney preservation during surgery. Local control of disease is a prerequisite for successful bone marrow transplantation.

1- DeCou JM, Bowman LC, Rao BN, Santana VM, Furman WL, Luo X, Lobe TE, Kumar M: Infants with metastatic neuroblastoma have improved survival with resection of the primary tumor. J Pediatr Surg 30(7): 937-941, 1995
2- La Quaglia MP, Kushner BH, Heller G, Bonilla MA, Lindsley KL, Cheung NKV: Stage 4 neuroblastoma diagnosed at more than 1 year of age: Gross total resection and clinical outcome. J Pediatr Surg 29(8): 1162-1166, 1994
3- Shamberger RC, Allarde-Segundo A, Kozakewich HPW, Grier HE: Surgical management of stage III and IV neuroblastoma: Resection before or after chemotherapy? J Pediatr Surg 26(9): 1113-1118, 1991
4- Haase GM, O'Leary MCO, Ramsay NKC, Romansky SG, Stram DO, Seeger RC, Hammond GD: Aggressive surgery combined with intensive chemotherapy improves survival in poor-risk neuroblastoma. J Pediatr Surg 26(9): 1119-1124
5- Shorter NA, Davidoff AM, Evans AE, Ross AJ 3rd, Zeigler MM, O'Neill,JA Jr: The role of surgery in the management of stage IV neuroblastoma: a single institution study. Med Pediatr Oncol 24(5):287-91, 1995
6- Tsuchida Y, Yokoyama J, Kaneko M, Uchino J, Iwafuchi M, Makino S, Matsuyama S, Takahashi H, Okabe I, Hashizume K, et al: Therapeutic significance of surgery in advanced neuroblastoma: a report from the study group of Japan. J Pediatr Surg 27(5):616-22, 1992

Incidental Appendectomy

Removing a normal appendix incidentally during a surgical procedure done for reasons other than abdominal pain is associated with a small but definite increase in adverse postoperative outcome. In this respect incidental appendectomy has been found to increase the incidence of postoperative septic complications (wound infection). It is neither cost-effective as an estimated 36 incidental procedures would be needed to prevent one case of appendicitis. As any procedure it increases adhesion formation from surgical manipulation in the right lower quadrant fossa. In potentially contaminated primary procedures the addition of incidental appendectomy does not increase operative morbidity or mortality. Incidental appendectomy is indicated in procedures where a potential diagnostic pitfall can occur such as Ladds procedure for malrotation, diagnostic laparoscopy for right quadrant pain and surgically reduced ileo-colic intussusception.

1- Wen SW, Hernandez R, Naylor CD: Pitfalls in non-randomized outcomes studies. The case of incidental appendectomy with open cholecystectomy. JAMA 274(21):1687-91, 1995
2- Miranda R, Johnston AD, O'Leary JP: Incidental appendectomy: frequency of pathologic abnormalities. Am Surg 46(6):355-7, 1980
3- Mulvihill S, Goldthorn J, Woolley MM: Incidental appendectomy in infants and children. Risk v rationale. Arch Surg 118(6):714-6, 1983
4- Sugimoto T, Edwards D: Incidence and costs of incidental appendectomy as a preventive measure. Am J Public Health 77(4):471-5, 1987
5- Addiss DG, Shaffer N, Fowler BS, Tauxe RV: The epidemiology of appendicitis and appendectomy in the United States. Am J Epidemiol 132(5):910-25, 1990
6- Ritchey M, Haase GM, Shochat SJ, Kelalis PP: Incidental appendectomy during nephrectomy for Wilms' tumor. Surg Gynecol Obstet 176(5):423-6, 1993

Currarino Triad

Congenital caudal anomalies that include anorectal malformation, sacral bony abnormality and a presacral mass is known as the Currarino Triad (CT); an autosomic dominant hereditary syndrome described in 1981 caused by abnormal separation of neuroectoderm from endoderm. The anorectal malformation associated with CT is stenosis (or agenesis) of the distal rectum causing intractable constipation (chief complaint of this triad) or intestinal obstruction. Sacral agenesis and abnormalities of the os sacrum (scimitar sacrum, hemisacrum with preserved first sacral vertebra) are the most common bony anomalies identified. Most frequently the presacral mass in CT is reported to be an anterior meningocele, a benign teratoma, enteric, dermoid cyst or a combination. Though prenatal diagnosis can be made, most cases are diagnosed postnatally in the first decade of life. Routine pelvic x-rays should be done in all cases of anorectal stenosis. Pelvic ultrasound and x-rays in patients with history of chronic constipation since early childhood will suggest the diagnosis. MRI is the study of choice detecting the presacral mass and any anomalies of the spinal canal (tethered cord syndrome caused by the presacral mass). Management consists of excision of the presacral mass and repair of the anorectal malformation. A gene associated with CT has been mapped to the terminal portion of the long arm of chromosome 7 (7q36).

1- Kirks DR, Merten DF, Filston HC, Oakes WJ: The Currarino triad: complex of anorectal malformation, sacral bony abnormality, and presacral mass. Pediatr Radiol 14(4):220-5, 1984
2- Kochling J, Pistor G, Marzhauser Brands S, Nasir R, Lanksch WR: The Currarino syndrome--hereditary transmitted syndrome of anorectal, sacral and presacral anomalies. Case report and review of the literature. Eur J Pediatr Surg 6(2):114-9, 1996
3- Lee SC, Chun YS, Jung SE, Park KW, Kim WK: Currarino triad: Anorectal malformations, sacral bony abnormality, and presacral mass - A review of 11 cases. J Pediatr Surg 32(1): 58-61, 1997
4- Gudinchet F, Maeder P, Laurent T, Meyrat B, Schnyder P: Magnetic resonance detection of myelodysplasia in children with Currarino triad. Pediatr Radiol 27(12):903-7, 1997
5- Zia-ul-Miraj M, Brereton RJ: Currarino's triad: an unusual cause of constipation in children. Pediatr Surg Int 13(5-6):437-9, 1998
6- Samuel M, Hosie G, Holmes K: Currarino triad-Diagnostic dilemma and a combined surgical approach.  J Pediatr Surg 35(12):1790-1794, 2000

Volume 16 No 03 MARCH 2001

Congenital Pouch Colon

Congenital pouch colon (CPC) is a significant pouch dilatation of a shortened colon associated with a high anorectal malformation (colo-vesical fistula). First described in 1959 most reported cases come from Northern India with male predominance (4:1). Plain films showing an enormous air-fluid level occupying more than half the width of the abdomen sometimes showing gas within the bladder is diagnostic. Subtypes described variate in the amount of colon involved, but patients can be classified into two types: (1) partial CPC - a segment of normal colon is present between the ileum and the sac, and (2) complete CPC where the ileum opens directly into the sac. A wide fistula with the bladder is usually found, though the CPC can end blindly or as a fibrous cord. Females are associated with a vesical or vestibular fistula. Management depends on the general condition of the child, associated anomalies and pouch anatomy during initial surgery. Ideally division of the colo-vesical fistula, tubular colorrhaphy and end colostomy should be done. With life threatening anomalies window colostomy with or without division of the fistula is best. An ischemic pouch should be removed. Those cases with less colon involvement can undergo a proximal end colostomy with pouch excision. Definite pull-through reconstruction is done later in life using the posterior sagittal approach. Long-term results of continence are not encouraging in children that have undergo tubular colorrhaphy.

1- Chadha R, Bagga D, Malhotra CJ, Mohta A, Dhar A: The embryology and management of congenital pouch colon associated with anorectal agenesis. J Pediatr Surg 29(3):439-46, 1994
2- Wakhlu AK, Wakhlu A, Pandey A, Agarwal R, Tandon RK, Kureel SN: Congenital short colon. World J Surg 20(1):107-14, 1996
3- Budhiraja S, Pandit SK, Rattan KN: A report of 27 cases of congenital short colon with an imperforate anus: so-called 'pouch colon syndrome'. Trop Doct 27(4):217-20, 1997
4- Chadha R, Bagga D, Mahajan JK, Gupta S: Congenital pouch colon revisited. J Pediatr Surg 33(10):1510-5, 1998
5- Chadha R, Gupta S, Mahajan JK, Bagga D, Kumar A: Congenital pouch colon in females. Pediatr Surg Int 15(5-6):336-42, 1999
6- Herman TE, Coplen D, Skinner M: Congenital short colon with imperforate anus (pouch colon). Report of a case. Pediatr Radiol 30(4):243-6, 2000

Adrenal Hemorrhage

Retroperitoneal adrenal Hemorrhage usually occurs at birth or during the first postnatal days the result of traumatic or breech delivery, large fetal size, disorders of hemostasis, perinatal asphyxia and fetal hypoxia producing anemia from blood loss accompanied by an enlarging flank mass. Other times adrenal Hemorrhage manifests as unexplained hyperbilirubinemia with a mass in the flank in a healthy infant. Most cases affect the right adrenal gland (75%). Differential diagnosis includes neuroblastoma, cortical renal cysts, adrenal abscess, obstructed upper cortical renal cyst, and obstructed upper excretory tract in duplicated kidney. Diagnosis of neonatal adrenal hemorrhage is based on ultrasound (echo-free mass superior to downward displaced normal kidneys with linear calcifications). Fine needle aspiration can be done to confirm the diagnosis. The hematoma resolves gradually under supportive management by three months of age as documented by serial sonography. Lesion evolution with progressive decrease in its size and development of calcifications on repeated sonographic follow-up studies may be the only reliable sign in preventing unnecessary surgery.

1- Pery M, Kaftori JK, Bar-Maor JA: Sonography for diagnosis and follow-up of neonatal adrenal hemorrhage. J Clin Ultrasound 9(7):397-401, 1981
2- Miele V, Patti G, Cappelli L, Calisti A, Valenti M: [Echography in the diagnosis of neonatal adrenal hemorrhage]. Radiol Med (Torino) 87(1-2):111-7, 1994
3- Chein CL, Chen WP, Yang LY, Fu LS, Lin CY: Early detection of neonatal adrenal hemorrhage by ultrasonography. Chung Hua Min Kuo Hsiao Erh Ko I Hsueh Hui Tsa Chih 37(2):128-32, 1996
4- Lin JN, Lin GJ, Hung IJ, Hsueh C: Prenatally detected tumor mass in the adrenal gland. J Pediatr Surg 34(11):1620-3, 1999

Neonatal Laparoscopy

Technical advances and new instrumentations have made possibly the use of minimal invasive procedures in smaller infants. Advantage includes the magnification by the loupes and video, the excellent illumination and exposure. Diagnostic laparoscopy can be used in the neonatal period for: 1) the evaluation of the cholestatic infant, 2) abdominal, pelvic (ovarian cysts) and retroperitoneal masses and 3) intersexual defects. At the therapeutic level laparoscopy can be utilized for Hirschsprung pull-through surgery and bowel malrotation. Other laparoscopic procedures done during the neonatal period include fundoplication, pyloromyotomy, placement of dialysis catheters, liver biopsy, gastrostomy and placement of VP shunts. From the physiologic point of view laparoscopic procedures in newborns can be associated with hypothermia and hypercapnia. Hypothermia (increased heat loss) is due to rapid insufflation of carbon dioxide, gas leak and multiple instrument changes. Hypercapnia is monitored using end-tidal CO2 and can be neutralized hyperventilating and maintaining high peak insufflation pressures during the procedure. No cardiac depression is noted when the intraabdominal pressure is maintained at 6 to 8 mm Hg. Transient arrhythmias have been observed in infants.

1- Fujimoto T, Segawa O, Lane GJ, Esaki S, Miyano T: Laparoscopic surgery in newborn infants. Surg Endosc 13(8):773-777, 1999
2- Esposito C, Garipoli V, Di Matteo G, De Pasquale M: Laparoscopic management of ovarian cysts in newborns. Surg Endosc 12(9):1152-4 , 1998
3- Schier F, Waldschmidt J: Experience with laparoscopy for the evaluation of cholestasis in newborns. Surg Endosc 4(1):13-4, 1990
4- Hay SA, Soliman HE, Sherif HM, et al: Neonatal jaundice: the role of laparoscopy. J Pediatr Surg 35(12):1706-9, 2000
5- Ure BM, Bax NM, van der Zee DC: Laparoscopy in infants and children: a prospective study on feasibility and the impact on routine surgery. J Pediatr Surg. 35(8):1170-3, 2000
6- Holland AJ, Ford WD: The influence of laparoscopic surgery on perioperative heat loss in infants. Pediatr Surg Int 13(5-6):350-1, 1998
7- Bozkurt P, Kaya G, Yeker Y, Tunali Y, Altintas F: The cardiorespiratory effects of laparoscopic procedures in infants. Anaesthesia 54(9):831-4, 1999

Volume 16 No 04 APRIL 2001

Dermoid Cysts

Dermoid cysts are among the commonest epithelial tissue malformation found in children. Histologically these cysts are subclassified into epidermoid, dermoid cysts and teratomas. Dermoid cysts occur most commonly among regions of embryologic fusion such as the top lateral corner of the eyebrows (angular dermoid), in the midline of the neck (confusing with thyroglossal duct cysts), over the midline of the nose, in the temporo-parietal and suprasternal area. Dermoids are non-tender, mobile, translucent, slowly growing tumors composed of keratin, hair follicles and sebaceous glands within a thin wall that contains epithelial elements. Midline dermoids are entrapped epithelium of branchial arch origin at the time of fetal midline fusion. Occasionally the cyst rests on the dura (intracranial extension) or on the orbital fascia causing bone erosion seen in x-rays. Intraglossal dermoids have also been described. Infection is the most common complication and is caused by repeated local trauma. Those with intracranial extension are at risk of aseptic meningitis. History and physical exam are the most important element in the evaluation of neck masses in children. Complete surgical excision  when the diagnosis is made is the treatment of choice.

1- Gold BC, Skeinkopf DE, Levy B: Dermoid, epidermoid and teratomatous cysts of the tongue and the floor of the mouth. J Oral Surg 32:107-111, 1974
2- McAvoy JM, Zuckerbraun L: Dermoid Cysts of the Head and Neck in Children. Arch Otolaryngol 102: 529-531, 1976
3- Park YW: Evaluation of neck masses in children. Am Fam Phys 51(8):1904-12, 1995
4- Rood SR, Johnson JT, Myers EN, Lipman S: Congenital masses of the head and neck. Postgrad Med 72(5):141-5, 148-9, 1982
5- Felder H: Benign congential neoplasms: dermoids and teratomas. Arch Otolaryngol 101(5):333-4, 1975
6- May M: Neck masses in children: diagnosis and treatment. Pediatr Ann 5(8):518-35, 1976
7- Crawford R: Dermoid cyst of the scalp: intracranial extension. J Pediatr Surg 25(3):294-5, 1990

Inoperable Wilms' Tumor

On occasion Wilms tumors (Nephroblastoma) grow to such massive size  that primary surgical excision poses a high risk of complications and mortality to the child. This type of tumor is either found unable to be resected during surgical exploration or judge inoperable by clinical evaluation or imaging studies. The inoperable criterias most commonly utilized are: huge size of the tumor,  involvement of adjacent vital structures, intra-caval involvement above the level of the hepatic veins and atrial tumor extension. This factors significantly increase the risk of surgical morbidity, principally hemorrhage, during initial nephrectomy. Solely relying on imaging studies for staging and deciding inoperability can lead to inaccurate decision. In such cases it is recommended to do initial exploration to determine operability and obtain tumor biopsy including suspicious lymph nodes or other metastatic foci. Preop chemotherapy has been found to almost always ease surgical resection by reducing tumor size decreasing the incidence of  tumor rupture. Histologic patterns are still recognized after preop chemotherapy, though there can be  initial biopsy sampling errors. Failure of tumor reduction can be followed by radiation therapy. Inoperability as a criterion is an adverse prognostic factor independent of stage and results in loss of important staging information. Once there is evidence of tumor size reduction and evidence of vena cava tumor regression definite resection should be completed.

1- Ritchey ML, Pringle KC, Breslow NE, et al: Management and Outcome of Inoperable Wilms Tumor: A Report of National Wilms Tumor Study-3. Ann Surg 220 (5): 683-690, 1994
2- National Wilms Tumor Study-5 Protocol, pags 13-14, 1997
3- Ritchey ML: The role of preoperative chemotherapy for Wilms' tumor: the NWTSG perspective. National Wilms' Tumor Study Group. Semin Urol Oncol 17(1):21-7, 1999
4- Graf N, Tournade MF, de Kraker J: The role of preoperative chemotherapy in the management of Wilms' tumor. The SIOP studies. International Society of Pediatric Oncology. Urol Clin North Am 27(3):443-54, 2000
5- Capra ML, Walker DA, Mohammed WM, Kapila L, Barbor PR, Sokal M, Robson K, Hewitt M, Stewart R: Wilms' tumor: a 25-year review of the role of preoperative chemotherapy. J Pediatr Surg 34(4):579-82, 1999
6- Ritchey ML, Shamberger RC, Haase G, Horwitz J, Bergemann T, Breslow NE: Surgical Complications after Primary Nephrectomy for Wilms' Tumor: Report from the National Wilms' Tumor Study Group. J Amer Coll Surg 192 (1): 63- 68, 2001
7- Broecker BH, Perlmutter AD: Management of unresectable Wilms tumor. Urology 24(2):170-4, 1984
8- Grosfeld JL, West KW, Weber TR: Second-look laparotomy for Wilms' tumor: indications and results in 19 patients. J Pediatr Surg 20(2):145-9, 1985

Ivemark's Syndrome

First described in 1959, Ivemark's syndrome refers to a uniformly fatal renal-hepatic-pancreatic dysplasia of unknown origin affecting infants in the first six-months of life. Prenatal diagnosis has been made as early as the 18 weeks of fetal development. An autosomal recessive pattern of inheritance has been proposed in this syndrome. The renal malformation consists of cystic dysplasia characterized by disturbance in glomerular differentiation, delay in tubular differentiation and abnormal expression of epithelial markers in glomeruli and tubules (multicystic kidneys). Hepatic abnormality consists of enlarged portal areas and elongated biliary ducts with a tendency to perilobular fibrosis. The intrahepatic ductal dilatation resembles Caroli's disease. The pancreatic abnormality consists of dilated duct, cysts and fibrosis with diminution of parenchymal tissue. Clinically the child develops during the neonatal period cholestatic jaundice followed by renal and pancreatic failure. Ivemark syndrome should not be confused with the asplenia-cardiac anomaly syndrome also bearing the same eponym. Ultrasound findings in the three affected organs suggest the diagnosis that unfortunately will be confirmed at autopsy. Management is supportive.

1- Strayer DS, Kissane JM: Dysplasia of the kidneys, liver, and pancreas: report of a variant of Ivemark's syndrome. Hum Pathol 10(2):228-34, 1979
2- Bernstein J, Chandra M, Creswell J, Kahn E, Malouf NN, McVicar M, Weinberg AG, Wybel RE: Renal-hepatic-pancreatic dysplasia: a syndrome reconsidered. Am J Med Genet 26(2):391-403, 1987
3- Carles D, Serville F, Dubecq JP, Gonnet JM: Renal, pancreatic and hepatic dysplasia sequence. Eur J Pediatr 147(4):431-2, 1988
4- Larson RS, Rudloff MA, Liapis H, Manes JL, Davila R, Kissane J: The Ivemark syndrome: prenatal diagnosis of an uncommon cystic renal lesion with heterogeneous associations. Pediatr Nephrol 9(5):594-8, 1995
5- Torra R, Alos L, Ramos J, Estivill X: Renal-hepatic-pancreatic dysplasia: an autosomal recessive malformation. J Med Genet 33(5):409-12, 1996
6- Abbi R, Daum F, Kahn E: Ontogeny of renal dysplasia in Ivemark syndrome: light and immunohistochemical characterization. Ann Clin Lab Sci 29(1):9-17, 1999

Volume 16 No 05 MAY 2001

Neck Adenopathy

Cervical adenopathies are the most common neck masses identified in children. Though they may occur in any location, the anterior cervical chain position is the most common presentation. Lymph node enlargement is usually preceded by a viral upper respiratory infection Nodes are soft, mildly tender and usually decrease in size after a short course of antibiotics. Acute suppurative adenitis occur during early childhood (six months to three years of age) most commonly in submandibular and deep cervical nodes preceded by pharyngitis. Nodes develop erythema, swelling and cellulitis. Infecting agents include staph aureus and group A streptococcus. Other less common organisms are fungi, atypical mycobacteria and Bartonella species. Antibiotics and drainage are curative. Chronic adenitis refer to nodes that persist enlarged for more than six-weeks. Tonsillar nodes (solitary, non-tender, mobile and soft) are most commonly affected by a reactive hyperplasia process and can be observed. Work-up should include complete blood count, ESR, skin (PPD) and Mono test, chest film and ultrasound looking for the perfusion patterns of affected lymph nodes. Indications for biopsy of chronic lymphadenopathies include older children (> eight years), rapid growth, nodes greater than two centimeters, supraclavicular position, cluster - hard - fixed non-tender characteristic, an abnormal chest film, history of  malignancy or very anxious parents. Biopsy is done of the largest node under general anesthesia as an outpatient procedure.

1- Bodenstein L, Altman RP: Cervical lymphadenitis in infants and children. Semin Pediatr Surg 3(3):134-41, 1994
2- Ramadan HH, Wax MK, Boyd CB: Fine-needle aspiration of head and neck masses in children. Am J Otolaryngol 18(6):400-4, 1997
3- Steinkamp HJ, Teichgraber UK, Mueffelmann M, Hosten N, Kenzel P, Felix R: Differential diagnosis of lymph node lesions. A semiquantitative approach with power Doppler sonography. Invest Radiol 34(8):509-15, 1999
4- Soldes OS, Younger JG, Hirschl RB: Predictors of malignancy in Childhood Peripheral Lymphadenopathy. J Pediatr Surg 34(10): 1447-1452, 1999
5- Slap GB, Brooks JSJ, Schwartz JS: When to perform biopsies of enlarged peripheral lymph nodes in young patients. JAMA 252:1321-1326, 1984

Familial Adenomatous Polyposis

Familial history of colon cancer is an important indicator of future risk for colorectal cancer. The more extensive and closer the affected relatives, the greater the risk. Highest risk is found in Familial Adenomatous Polyposis (FAP). Once the risk is appreciated screening for the disease must take place. This involves genetic analysis for members of syndrome families along with lower gastrointestinal endoscopy for the rest as polyps can occur throughout the gastrointestinal tract. FAP is a genetic (autosomal dominant) premalignant condition that will ultimately manifest with the development of colorectal carcinoma. FAP has been linked to germline mutations of the adenomatous polyposis coli (APC) gene. Initial presentation can be rectal bleeding. Besides multiple adenomatous polyps of the colon predisposing to malignancy at an early age, a variety of extra colonic manifestations are associated with this condition. Once the diagnosis of FAP is established endoscopic surveillance should be instituted. All polyps should be subjected to histopathological exam to determine presence of adenomatous epithelium. With the presence of dysplastic changes total colectomy with the creation of an ileorectal or ileoanal (pouch) anastomosis is recommended. Almost one-third of cases develops adenomas in the ileal pouch after proctocolectomy. Baseline small bowel enteroscopy should be done at the time of surgery and in the postop period in children with FAP and juvenile polyposis. With duodenal polyps enteroscopy should be done at the time of surgery. Biopsy and/or excision of larger polyps should be done as they may harbor a carcinoma.

1- Nelson RL, Orsay CP, Pearl RK, Abcarian H: The protean manifestations of familial polyposis coli. Dis Colon Rectum 31(9):699-703, 1998
2- Rodriguez-Bigas MA, Penetrante RB, Herrera L, Petrelli NJ: Intraoperative small bowel enteroscopy in familial adenomatous and familial juvenile polyposis. Gastrointest Endosc 42(6):560-4, 1995
3- Vaiphei K, Thapa BR: Juvenile polyposis (coli)--high incidence of dysplastic epithelium. J Pediatr Surg 32(9):1287-90, 1997
4- Ruttenberg D, Elliot MS, Bolding E: Severe colonic dysplasia in a child with familial adenomatous polyposis. Int J Colorectal Dis 6(3):169-70, 1991
5- Lal G, Gallinger S: Familial adenomatous polyposis. Semin Surg Oncol 18(4):314-23, 2000
6- O'Riordain DS, O'Dwyer PJ, Cullen AF, McDermott EW, Murphy JJ: Familial juvenile polyposis coli and colorectal cancer. Cancer 68(4):889-92, 1991
7- Poddar U, Thapa BR, Vaiphei K, Rao KL, Mitra SK, Singh K: Juvenile polyposis in a tropical country. Arch Dis Child 78(3):264-6, 1998
8- Iwama T, Mishima Y, Utsonomiya J: The Impact of familial Adenomatous Polyposis on the Tumorigenesis and Mortality at the Several Organs: Its Rational Treatment. Ann Surg 217(2): 101-108, 1993
9- Parc YR, Olscwang S, Desaint B, et al: Familial Adenomatous Polyposis: Prevalence of Adenomas in the Ileal Pouch after restorative Proctocolectomy. Ann Surg 233 (3): 360-364, 2001

Denys-Drash Syndrome

The Denys-Drash Syndromes (DDS), first described in 1967, encompass the combination of male pseudohermaphroditism (XY gonadal dysgenesis), early onset glomerulopathy caused by diffuse mesangial sclerosis, Wilms' tumor, and constitutional mutations in the WT1 suppressor gene.  WT1 gene, found in 11p13 chromosomal region, expresses a regulated transcription factor of the zinc-finger family proteins restricted to the genitourinary system, spleen, dorsal mesentery of the intestines, muscles, central nervous system and mesothelium. Mutations in  the WT1 gene have been found in less than 10% of Wilms tumors specimens examined and in greater than 95% of DDS patients. Most of the mutations described are dominant missense mutations. Wilms tumor associated with DDS occurs at a younger age. Since DDS patients eventually go into end stage renal failure, the diagnosis of DDS should be suspected in any child with Wilms tumor that develops renal failure. Patients with Wilms' tumor and aniridia or genitourinary malformation should be followed closely throughout life for signs of renal failure.

1- Sherbotie JR; van Heyningen V; Axton R; Williamson K; Finn LS; Kaplan BS: Hemolytic uremic syndrome associated with Denys-Drash syndrome. Pediatr Nephrol 14(12):1092-7, 2000
2- Grundy P, Coppes M: An Overview of the Clinical and Molecular Genetics of Wilms' Tumor. Med Pediatr Oncol 27: 394-397, 1996
3- Lugo-Vicente HL: Molecular Biology and Genetics Affecting Pediatric Solid Tumors. Boletin Asoc Med PR 92(4-5-6-7-8):72-82, 2000
4- Coppes MJ, Liefers GJ, Higuchi M, Zinn AB, Balfe JW: Inherited WT1 mutation in Denys-Drash syndrome. Cancer Res 52(21):6125-8, 1992
5- Little M, Wells C: A clinical overview of WT1 gene mutations. Hum Mutat 9(3):209-25, 1997
6- Breslow NE, Takashima JR, Ritchey ML, Strong LC, Green DM: Renal failure in the Denys-Drash and Wilms' tumor-aniridia syndromes. Cancer Res 60(15):4030-2, 2000

Volume 16 No 06 JUNE 2001

Mesenchyma Hamartoma

Hepatic mesenchymal hamartoma (MH) is a rare cystic tumor, the second most common  benign liver tumor of childhood, occurring more commonly in the right lobe. The infant or child, whose average age is 15 months, is born or develops progressive abdominal distension and palpable non-tender mass. The mass is large, (up to 2500 gm reported) frequently containing cysts filled with clear fluid or gelatinous material. Can be encapsulated, infiltrate or attached to the liver by a pedicle. MH can cause respiratory distress or heart failure from arteriovenous shunting. MH is considered as a proliferative malformation arising from ductal plates.  Histologic shows a mixture of loose mesenchymal tissue, bile ducts, connective tissue, and hepatocytes along with cysts formed either from degenerative areas of mesenchyme or dilated bile ducts and lymphatics. Increased levels of alpha-fetoprotein has been found in most cases. CT, US and MRI are diagnostic showing a large, predominantly cystic mass with internal septa. Surgical excision is the treatment of choice. Prenatal diagnosis is suggested when a multicystic mass is found in the fetal abdomen. Though there is evidence that MH regress spontaneously, management consists of complete removal of the tumor. Pedunculated tumors are easily resected. Simple enucleation is preferred for subcapsular lesions. Marsupialization is not recommended. Recurrence or malignant transformation (malignant mesenchymoma) is extremely rare, but careful follow-up of non-resectable tumors is mandatory.

1- Stocker JT, Ishak KG: Mesenchymal hamartoma of the liver: report of 30 cases and review of the
literature. Pediatr Pathol 1(3):245-67, 1983
2- Raffensperger JG, Gonzalez-Crussi F, Skeehan T: Mesenchymal hamartoma of the liver. J Pediatr Surg 18(5):585-7, 1983
3- Lack EE: Mesenchymal hamartoma of the liver. A clinical and pathologic study of nine cases. Am J Pediatr Hematol Oncol 8(2):91-8, 1986
4- Stanley P, Hall TR, Woolley MM, Diament MJ, Gilsanz V, Miller JH: Mesenchymal hamartomas of the liver in childhood: sonographic and CT findings. AJR Am J Roentgenol 147(5):1035-9, 1986
5- DeMaioribus CA, Lally KP, Sim K, Isaacs H, Mahour GH: Mesenchymal hamartoma of the liver. A 35-year review. Arch Surg 125(5):598-600, 1990
6- Hirata GI, Matsunaga ML, Medearis AL, Dixon P, Platt LD: Ultrasonographic diagnosis of a fetal abdominal mass: a case of a mesenchymal liver hamartoma and a review of the literature. Prenat Diagn 10(8):507-12, 1990
7- Luks FI, Yazbeck S, Brandt ML, Bensoussan AL, Brochu P, Blanchard H: Benign liver tumors in children: a 25-year experience. J Pediatr Surg 26(11):1326-30, 1991
8- Barnhart DC, Hirschl RB, Garver KA, Geiger JD, Harmon CM, Coran AG: Conservative management of mesenchymal hamartoma of the liver. J Pediatr Surg 32(10):1495-8, 1997
9- von Schweinitz D, Dammeier BG, Gluer S: Mesenchymal hamartoma of the liver--new insight into histogenesis. J Pediatr Surg 34(8):1269-71, 1999
10- Ramanujam TM, Ramesh JC, Goh DW, Wong KT, Ariffin WA, Kumar G, Taib NA: Malignant transformation of mesenchymal hamartoma of the liver: case report and review of the literature.  Pediatr Surg 34(11):1684-6, 1999
11- Meyers RL, Scaife ER: Benign liver and biliary tract masses in infants and toddlers. Semin Pediatr Surg 9(3):146-55, 2000

Femoral Hernias

Femoral hernias (FH) in the pediatric age are very rare accounting for less than 0.5% of all groin hernias in children. Children present with a recurrent groin lump that is usually reducible. The correct preoperative diagnosis is often overlooked. FH is defined as a protrusion of viscera, fat or omentum occurring through the femoral hiatus. Peak incidence occurs between five and ten years of age. Misdiagnosis includes inguinal hernia, lymphadenitis, and lymphangioma. Preoperative diagnosis is possible if the bulge appears in a location inferior and lateral to that of the commonly occurring indirect hernia. Early recurrence of a groin swelling after what seems to be an adequate inguinal herniorrhaphy should be suspected of having a missed femoral hernia. Mc Vay expressed that the etiology was a congenitally narrow posterior inguinal wall attachment into Cooper's ligament with a resultant enlarged femoral ring. Excision of the sac and repair of the femoral canal is curative. At surgery Cooper's ligament (Mc Vay) repair is the surgical treatment of choice, though some contend that  simple repair of the femoral ring carries good long-term results.

1- Mc Vay CB, Savage LE: Etiology of femoral hernias. Ann Surg 154: 25-32, 1961 (Suppl)
2- Marshall DG: Femoral hernias in children. J Pediatr Surg 18(2):160-2, 1983
3- Anderas P, Jona JZ, Glicklich M, Cohen RD: Femoral hernias in children. An infrequent problem. Arch Surg 122(8):950-1, 1987
4- Radcliffe G, Stringer MD: Reappraisal of femoral hernia in children. Br J Surg 84(1):58-60, 1997
5- Ollero Fresno JC, Alvarez M, Sanchez M, Rollan V: Femoral hernia in childhood: review of 38 cases. Pediatr Surg Int 12(7):520-1, 1997
6- Al-Shanafey S, Giacomantonio M: Femoral hernia in children. J Pediatr Surg 34(7):1104-6, 1999


Thoracic Empyema (TE) is an infection (pus) of the pleural cavity. TE develops after complications of bacterial pneumonia (most commonly), thoracic trauma or surgery. Three distinct phases of TE developments are recognized: exudative, fibrinopurulent and organizing. In the early exudative phase the fluid is thin with a low viscosity and cellular content. Intravenous antibiotics,  aspiration or chest tube drainage accomplishes successful management as the lung expands rapidly. This phase can be followed by bacterial invasion, deposition of fibrin, increase turbidity and cellular content with fixed, less expandable, lung tissue known as fibrinopurulent stage. Loculations form, effective closed pleural drains become impossible and antibiotics are less effective in this phase. If left untreated (two to four weeks after primary infection) the TE goes through a final organizing stage with thickening of the fibrinous peel and complete lung entrapment. In this final stage open decortication may be required. Success in management of TE consists in identifying its early phases followed by thorough debridement and lysing of the pleural space of all fibrinous material, adhesions and loculations during the fibrinopurulent phase before fibrosis begins. Indication for video-assisted thoracoscopic debridement includes lack of medical response, pulmonary air leakage, localized effusion, persistent respiratory distress and pleural thickening without resolution on imaging (US or CT Scan). Thoracoscopic debridement and irrigation have accomplished this goal in several series of children reducing complications from open thoracotomy and hospital stay. Early thoracoscopy facilitates removal of restrictive purulent debris, decreases parenchymal injury, promotes rapid recovery and has a high rate of success. Benefits include good visualization of the entire thoracic cavity for more effective debridement and efficient drainage, and subjectively diminished postoperative pain and associated morbidity.

1- Kern JA, Rodgers BM: Thoracoscopy in the management of empyema in children. J Pediatr Surg 28(9):1128-32, 1993
2- Silen ML, Weber TR: Thoracoscopic debridement of loculated empyema thoracis in children. Ann Thorac Surg 59(5):1166-8, 1995
3- Stovroff M, Teague G, Heiss KF, Parker P, Ricketts RR: Thoracoscopy in the management of pediatric empyema. J Pediatr Surg 30(8):1211-5, 1995
4- Davidoff AM, Hebra A, Kerr J, Stafford PW: Thoracoscopic management of empyema in children. J Laparoendosc Surg 6 Suppl 1:S51-4, 1996
5- Rizalar R, Somuncu S, Bernay F, Ariturk E, Gunaydin M, Gurses N: Postpneumonic empyema in children treated by early decortication. Eur J Pediatr Surg 7(3):135-7, 1997
6- Steinbrecher HA, Najmaldin AS: Thoracoscopy for empyema in children. J Pediatr Surg 33(5):708-10, 1998
7- Gandhi RR, Stringel G: Video-assisted thoracoscopic surgery in the management of pediatric empyema. J Soc Laparoendosc Surg 1(3):251-3, 1997
8- Patton RM, Abrams RS, Gauderer MW: Is thoracoscopically aided pleural debridement advantageous in children? Am Surg 65(1):69-72, 1999
9- Merry CM, Bufo AJ, Shah RS, Schropp KP, Lobe TE: Early definitive intervention by thoracoscopy in pediatric empyema. J Pediatr Surg 34(1):178-80, 1999
10- Carey JA, Hamilton JR, Spencer DA, Gould K, Hasan A: Empyema thoracis: a role for open thoracotomy and decortication. Arch Dis Child 79(6):510-3, 1998
11- Grewal H, Jackson RJ, Wagner CW, Smith SD: Early video-assisted thoracic surgery in the management of empyema. Pediatrics 103(5):e63, 1999
12- Rodriguez JA, Hill CB, Loe WA, Kirsch DS, Liu DC: Video-assisted thoracoscopic surgery for children with stage II empyema. Am Surg 66(6):569-72, 2000
13- Chan W, Keyser-Gauvin E, Davis GM, Nguyen LT, Laberge JM: Empyema thoracis in Children: A 26-year review of the Montreal Children's Hospital Experience. J Pediatr Surg 32(6): 870-872, 1997
14- Subramaniam R, Joseph VT, Tan GM, Gih A, Chay OM: Experience with video-assisted thoracoscopic surgery in the management of complicated pneumonia in children. J Pediatr Surg 36(2): 316-319, 2001 

Journal Club