VOLUME 20, 2003

Volume 20 No 01 JANUARY 2003

Desmoplastic Small Round Cell Tumor

Within the non-rhabdomyosarcoma soft tissue tumors in children, the Desmoplastic Small Round Cell Tumor (DSRCT) is an extremely locally aggressive and rare tumor with predilection for children and adolescent patients and predominant or exclusive intra-abdominal location. DSRCT is characterized by a generally diffuse pattern of growth of small round cells with hyperchromatic nuclei, scanty cytoplasm, patchy epithelial differentiation, immunohistochemical co-expression of keratin and desmin intermediate filaments and a focal but pronounced desmoplastic stromal component. This neoplasm exhibits a predominantly intra-abdominal serosal pattern with frequent
pelvic extension, less frequent retroperitoneal involvement and very rarely pulmonary and mediastinal spread. The child usually present with a palpable, painful mass which may cause abdominal distension, constipation, bowel or ureteral obstruction. On imaging bulky peritoneal soft-tissue masses without an apparent organ-based primary site are characteristic of intra-abdominal DSRCT. DSRCT can be mistaken for a rhabdomyosarcoma as it contains cells with rhabdoid features. Cytogenetic studies showed a t(11;22) translocation that differs from the Ewing's tumor translocation and seems to be specific to this entity. Management consists of dose intensive multimodal chemotherapy, aggressive surgery to resect visible disease, local radiotherapy, and myeloablative chemotherapy with stem-cell rescue in selected cases. Unfortunately the prognosis is very poor and most children succumb to widespread metastasis disease.

1- Yeoh G, Russell P, Wills EJ, Fleming S: Intra-abdominal desmoplastic small round cell tumor. Pathology  25(2):197-202, 1993
2- Kushner BH, LaQuaglia MP, Wollner N, Meyers PA, Lindsley KL, Ghavimi F, Merchant TE, Boulad F, Cheung NK, Bonilla MA, Crouch G, Kelleher JF Jr, Steinherz PG, Gerald WL: Desmoplastic small round-cell tumor: prolonged progression-free survival with aggressive multimodality therapy. J Clin Oncol  14(5):1526-31, 1996
3- Leuschner I, Radig K, Harms D: Desmoplastic small round cell tumor. Semin Diagn Pathol  13(3):204-12, 1996
4- Pickhardt PJ, Fisher AJ, Balfe DM, Dehner LP, Huettner PC: Desmoplastic small round cell tumor of the abdomen: radiologic-histopathologic correlation. Radiology  210(3):633-8, 1999
5- Quaglia MP, Brennan MF: The clinical approach to desmoplastic small round cell tumor. Surg Oncol 9(2):77-81, 2000

Juvenile Fibroadenoma

Juvenile or giant fibroadenoma of the breast is a benign lesion that can obtain a large formidable proportional size during breast development in female adolescent patients. Most cases in children  are seen between the ages of 10 and 15 years. The tumor is solitary in most affected children with a diameter of 4-6 centimeters. Multiple and bilateral involvement has been reported in a few cases. Differential diagnosis includes cystosarcoma phylloides, benign virginal hypertrophy (juvenile gigantomastia) or rhabdomyosarcoma. FNA or Tru-cut needle biopsy can establish a precise histological diagnosis. Growth is so fast that it can cause non-tender cellulitis of the skin by way of stretching. Microscopically the tumor is characterized by a rich cellular stroma and a prominent glandular epithelium. Juvenile adenofibromas regardless of size, should be excised so as to preserve as much breast tissue as possible. Management options include local excision with reconstruction, reduction mammoplasty, or simple mastectomy with reconstruction.

1- Musio F, Mozingo D, Otchy DP: Multiple, giant fibroadenoma. Am Surg  57(7):438-41, 1991
2- Morimoto T, Komaki K, Mori T, Sasa M, Miki H, Inoue H, Monden Y, Nakanishi H: Juvenile gigantomastia: report of a case. Surg Today  23(3):260-4, 1993
3- Remadi S, Ismail A, Karpuz V, Finci V, Zacharie S, Vassilakos P: Cellular (juvenile) fibroadenoma of the breast. A clinico-pathologic and immunohistochemical study of 7 cases. Ann Pathol  14(6):392-7, 1994
4- Boothroyd A, Carty H: Breast masses in childhood and adolescence. A presentation of 17 cases and a review of the literature. Pediatr Radiol  24(2):81-4, 1994
5- Herrera L, Lugo-Vicente HL: Primary Embryonal Rhabdomyosarcoma of the Breast in an Adolescent Female: A Case Report. J Pediatr Surg 33(10):1582-1584, 1998
6- Pacinda SJ, Ramzy I: Fine-needle aspiration of breast masses. A review of its role in diagnosis and management in adolescent patients. J Adolesc Health  23(1):3-6, 1998

Laparoscopic Bowel Resection

The enthusiasm brought by laparoscopic surgery results has expanded its capabilities to other intra-abdominal surgical procedures such as isolated or limited bowel resection in children. So far reported indications have included: resection of a Meckel's diverticulum, post-enterocolitis small bowel or colonic stricture, localized mesenteric cyst, inflammatory bowel disease (IBD), intussusception, intestinal lymphangioma and duplication. Technically the approach can include mobilization with extracorporeal resection and anastomosis (video-assisted), resection with intracorporeal stapled anastomosis or resection with hand-sewn end-to-end anastomosis depending on the lesion, mobility, size of the bowel and ability of the surgeon. Largest experience with lap bowel resection and anastomosis has been for inflammatory bowel disease, specially Crohn's disease children. Laparoscopically assisted colectomies can be performed safely in treating IBD. Laparoscopic-assisted ileocolic resection has also been found a safe alternative to open surgery in adolescent patients with Crohn disease. Lap bowel resection has offered a faster recovery of pulmonary function, fewer complications, and shorter length of stay compared with conventional surgery for selected patients undergoing ileocolic resection for Crohn's disease.

1- Wexner SD, Johansen OB: Laparoscopic bowel resection: advantages and limitations. Ann Med  24(2):105-10, 1992
2- Meijerink WJ, Eijsbouts QA, Cuesta MA, van Hogezand RA, Ringers J, Meuwissen SG, Griffioen G, Bemelman WA: Laparoscopically assisted bowel surgery for inflammatory bowel disease. The
combined experiences of two academic centers. Surg Endosc  13(9):882-6, 1999
3- Hamel CT, Hildebrandt U, Weiss EG, Feifelz G, Wexner SD: Laparoscopic surgery for inflammatory bowel disease. Surg Endosc  15(7):642-5, 2001
4- Diamond IR, Langer JC: Laparoscopic-assisted versus open ileocolic resection for adolescent Crohn disease. J Pediatr Gastroenterol Nutr  33(5):543-7, 2001
5- Milsom JW, Hammerhofer KA, Bohm B, Marcello P, Elson P, Fazio VW: Prospective, randomized trial comparing laparoscopic vs. conventional surgery for refractory ileocolic Crohn's disease. Dis Colon Rectum  44(1):1-8, 2001
6- Rothenberg SS: Laparoscopic Segmental Intestinal Resection. Semin Pediatr Surg 11(4): 211-216, 2002

Volume 20 No 02 FEBRUARY 2003

Ondine's Curse

Congenital Central hypoventilation syndrome (CCHS), also known as Ondine curse, is a rare disorder of ventilatory control characterized by a lack of response to normal respiratory stimulants, especially hypercapnia. The child develops prolonged apnea, cyanosis and hypoventilation in the absence of cardiac, pulmonary or neuromuscular disease. Low respiratory rates during sleep are characteristic of Ondine's curse. Initial mechanical ventilation dependency is followed by ability to sustain respiration during awake periods. Ondine's curse is believed to be caused by aberrant development of neural crest tissue (neurocristopathy), and as such is associate with Hirschsprung's disease mostly of the long segment variety. It can also be associated with multifocal congenital neuroblastoma, ganglioneuroma, neuroma and hypothalamic dysfunction. Abnormalities of the eye and autonomic nervous system are also common. CCHS can potentially be managed with long-term assisted mechanical ventilation, or electrodes surgically implanted on the phrenic nerve to pace the diaphragm. Electrodes can be placed thoracoscopically. Diaphragmatic pacing can result in successful weaning from mechanical ventilation, appears to be effective in reducing pulmonary vascular resistance and pulmonary hypertension, and improve the quality of life in children with CCHS. Diaphragmatic fatigue and loss of phrenic nerve conductivity seem to be causes of failure of electrical pacing. The prognosis for patients with CCHS is poor with death resulting from pulmonary infections or cardiac failure due to pulmonary hypertension from hypoxemia.

1- Oren J, Kelly DH, Shannon DC: Long-term follow-up of children with congenital central hypoventilation syndrome. Pediatrics  80(3):375-80, 1987
2- El-Halaby E, Coran AG: Hirschsprung's Disease Associated with Ondine's Curse: Report of Three Cases and Review of the Literature. J Pediatr Surg 29(4): 530-535, 1994
3- Flageole H, Adolph VR, Davis GM, Laberge JM, Nguyen LT, Guttman FM: Diaphragmatic pacing in children with congenital central alveolar hypoventilation syndrome. Surgery 118(1):25-8, 1995
4- Stovroff M, Dykes F, Teague WG: The complete spectrum of neurocristopathy in an infant with congenital hypoventilation, Hirschsprung's disease, and neuroblastoma. J Pediatr Surg  30(8):1218-21, 1995
5- Shaul DB, Danielson PD, McComb JG, Keens TG: Thoracoscopic placement of phrenic nerve electrodes for diaphragmatic pacing in children. J Pediatr Surg  37(7):974-8, 2002

Splenic Autotransplantation

Splenic autotransplantation (SAT) as a means of preserving some immunologic function after emergent (trauma), or elective (Schistosomiasis and Gaucher's disease) splenectomy in children has been reported in several studies. Thinly slice splenic segments (around 20 to 30 gm tissue) are deposited into a greater omentum pouch. Studies have shown that these implants grow with time, taking around five years. More than 30% of normal splenic tissue is needed for adequate filtration and immunologic clearance. After implantation Technetium scanning is use to show viability of the graft. Fc-receptor scintigram with IgG coated and Tc-labeled RBC are use to verify mononuclear phagocyte function. Filtration capacity is follow-up with disappearance of Howell-Jolly bodies and platelet numbers. Phagocytosis and Immunologic capacity is done measuring tuftsin and IgM respectively, and NBT test for evaluating phagocytic function of granulocytes. Complications associated with SAT are adhesive small bowel obstruction, torsion and aseptic necrosis of the transplant. A better antibiotic response to pneumococcal vaccine is found in patients after splenectomy and autotransplantation. SAT is simple and appears to preserve splenic function.

1- Patel J, Williams JS, Shmigel B, Hinshaw JR: Preservation of splenic function by autotransplantation of traumatized spleen in man. Surgery  90(4):683-8, 1981
2-  Tzoracoleftherakis E, Alivizatos V, Kalfarentzos F, Androulakis J: Complications of splenic tissue reimplantation. Ann R Coll Surg Engl  73(2):83-6, 1991
3- Miyano T, Yamataka A, Ohshiro K, Yamashiro Y: Heterotopic splenic autotransplantation for splenomegaly secondary to Gaucher's disease--a case of siblings. J Pediatr Surg  29(12):1572-4, 1994
4- Yamataka A, Fujiwara T, Tsuchioka T, Kurosu Y, Sunagawa M: Heterotopic splenic autotransplantation in a neonate with splenic rupture, leading to normal splenic function. J Pediatr Surg  31(2):239-40, 1996
5-  Weber T, Hanisch E, Baum RP, Seufert RM: Late results of heterotopic autotransplantation of splenic tissue into the greater omentum. World J Surg  22(8):883-9, 1998
6- Leemans R, Manson W, Snijder JA, Smit JW, Klasen HJ, The TH, Timens W: Immune response capacity after human splenic autotransplantation: restoration of response to individual pneumococcal vaccine subtypes. Ann Surg 229(2):279-85, 1999

Laparoscopic Varicocelectomy

Varicoceles develop during early adolescence. Varicocelectomy represents the treatment of choice for scrotal varicocele associated with scrotal pain or discomfort, testicular atrophy or infertility (low sperm count). Prepubertal varicocelectomy is controversial. Traditionally, the surgical procedure entailed an inguinal or high retroperitoneal open approach to ligate the internal spermatic veins associated significant postop morbidity (recurrence and postop hydrocele formation) and prolonged return to normal activity. During the past ten years the technique has take advantage of the laparoscopic approach including minimal surgical trauma, lower morbidity, cost and time sparing, faster recovery, better microscopic dissection with preservation of the spermatic artery along amenable bilateral ligation without a second incision. The Palomo mass high retroperitoneal ligation of the internal spermatic vessels results in a significant decrease in failure rate as compared with artery-sparing procedures. The Palomo approach is also safe after previous inguinal hernia repair. The procedure entails mass clipping and division of the spermatic veins. Collateral veins should be cauterized. Scrotal subdermal injection of methylene blue help contrast delineates and preserve lymphatics vessels to reduce the incidence of postop hydrocele formation.

1- Hagood PG, Mehan DJ, Worischeck JH, Andrus CH, Parra RO: Laparoscopic varicocelectomy: preliminary report of a new technique. J Urol  147(1):73-6, 1992
2- Belloli G, Musi L, D'Agostino S: Laparoscopic surgery for adolescent varicocele: preliminary report on 80 patients. J Pediatr Surg  31(11):1488-90, 1996
3-  Bebars GA, Zaki A, Dawood AR, El-Gohary MA: Laparoscopic versus open high ligation of the testicular veins for the treatment of varicocele. JSLS  4(3):209-13, 2000
4- Cohen RC: Laparoscopic varicocelectomy with preservation of the testicular artery in adolescents. J Pediatr Surg  36(2):394-6, 2001
5- Esposito C, Monguzzi G, Gonzalez-Sabin MA, Rubino R, Montinaro L, Papparella A, Esposito G, Settimi A, Mastroianni L, Zamparelli M, Sacco R, Amici G, Damiano R, Innaro N: Results and complications of laparoscopic surgery for pediatric varicocele. J Pediatr Surg  36(5):767-9, 2001
6-  Richter F, Stock JA, LaSalle M, Sadeghi-Nejad H, Hanna MK: Management of prepubertal varicoceles-results of a questionnaire study among pediatric urologists and urologists with infertility  training. Urology  58(1):98-102, 2001
7- Barqawi A, Furness P 3rd, Koyle M: Laparoscopic Palomo varicocelectomy in the adolescent is safe after previous ipsilateral inguinal surgery. BJU Int  89(3):269-72, 2002
8- Podkamenev VV, Stalmakhovich VN, Urkov PS, Solovjev AA, Iljin VP: Laparoscopic surgery for pediatric varicoceles: Randomized controlled trial. J Pediatr Surg  37(5):727-9, 2002

Volume 20 No 03 MARCH 2003

Ostomy Prolapse

Though colostomies ileostomies or jejunostomies play a major role in the management of gastrointestinal disorder in children they can be associated with complications. These include skin excoriation, retraction, prolapse and stricture. Most stomal creation is required during the neonatal period. Stomal prolapse is the most common complications encounter in clinical practice (20%) since very dilated bowel must be brought through a thin abdominal wall. Colostomies sited in the transverse colon have the highest incidence of prolapse. Whenever possible the stoma should be constructed away from the surgical wound. End enterostomies rarely prolapse. Prolapses are more common with loop enterostomies and usually involves the distal non-functioning limb. Mild prolapse is usually inconsequential and requires no revision. All prolapses (proximal or distal) should be manage as an emergency trying initially to reduce it promptly before swelling, mechanical obstruction or ischemia ensues. For reduction to be effective consciously sedate the child, lubricate the bowel with jelly to avoid mucosal injury and start from distal to proximal squeezing gently the intestinal wall. If the prolapse becomes severe, recurrent, or occurs in the proximal limb causing mechanical obstruction then surgical revision becomes necessary. Some temporary measures suggested by other authors include purse string suture based on the Thiersch principle or anchoring the bowel loop to the skin away from the stoma. Whenever possible early closure of the stoma should be done.

1- Gauderer MW, Izant RJ Jr.: A technique for temporary control of colostomy prolapse in children. J Pediatr Surg  20(6):653-5, 1985
2- Golladay ES, Bernay F, Wagner CW: Prevention of prolapse in pediatric enterostomas with purse string technique. J Pediatr Surg  25(9):990-1, 1990
3- Millar AJ, Lakhoo K, Rode H, Ferreira MW, Brown RA, Cywes S: Bowel stomas in infants and children. A 5-year audit of 203 patients. S Afr J Surg  31(3):110-3, 1993
4- Cheung MT: Complications of an abdominal stoma: an analysis of 322 stomas. Aust N Z J Surg  65(11):808-11, 1995
5- Nour S, Beck J, Stringer MD: Colostomy complications in infants and children. Ann R Coll Surg Engl  78(6):526-30, 1996
6- Steinau G, Ruhl KM, Hornchen H, Schumpelick V: Enterostomy complications in infancy and childhood. Langenbecks Arch Surg  386(5):346-9, 2001
7- Duchesne JC, Wang YZ, Weintraub SL, Boyle M, Hunt JP: Stoma complications: a multivariate analysis. Am Surg  68(11):961-6, 2002

Pancreatitis in Choledochal Cysts

Choledochal cysts (CC) can produce abdominal pain, obstructive jaundice, or pancreatitis. The recurrent abdominal pain associated to hyperamylasemia has been called "pseudopancreatitis" since true inflammation of the pancreas is absent. Acute edematous or necrotizing pancreatitis associated with CC is very rare. It is believed that when the bile duct pressure increases due to obstructive cholangitis pancreatic enzymes in bile may regurgitate into the blood stream and produce hyperamylasemia. Cholangiovenous reflux of amylase might cause hyperamylasemia. Other clinical studies have found that amylase in the biliary tract have access to the blood stream probably through a sinusoidal pathway by cholangiovenous reflux, and a lymphatic pathway, via the Disse's space and denuded cyst wall with bile duct pressure increases. Common denominator is an increase in ductal pressure of a partially closed common channel system. Infants with CC do not show hyperamylasemia due to physiologically low level of pancreatic amylase. The abdominal complaint and hyperamylasemia can easily subside in a short period of time with conservative treatment resulting in no demonstrable pancreatic dysfunction. Once this occurs appropriate surgical management of the choledochal cyst which includes cyst excision and Roux-en-Y hepatico-jejunostomy reconstruction should be done.

1- Todani T, Urushihara N, Watanabe Y, Toki A, Uemura S, Sato Y, Morotomi Y: Pseudopancreatitis in choledochal cyst in children: intraoperative study of amylase levels in the serum. J Pediatr Surg  25(3):303-6, 1990
2- Davenport M, Stringer MD, Howard ER: Biliary amylase and congenital choledochal dilatation. J Pediatr Surg  30(3):474-7, 1995
3- Urushihara N, Todani T, Watanabe Y, Uemura S, Morotomi Y, Wang ZQ: Does hyperamylasemia in choledochal cyst indicate true pancreatitis? An experimental study. Eur J Pediatr Surg  5(3):139-42, 1995
4- Okada A, Higaki J, Nakamura T, Fukui Y, Kamata S: Pancreatitis associated with choledochal cyst and other anomalies in childhood. Br J Surg  82(6):829-32, 1995
5- Komuro H, Makino SI, Yasuda Y, Ishibashi T, Tahara K, Nagai H: Pancreatic complications in choledochal cyst and their surgical outcomes. World J Surg  25(12):1519-23, 2001


Acute parotitis is a self-limiting disease most commonly associated with mumps (epidemic parotitis) in children. Other times the parotitis is associated with bacterial infection progressing to frank suppuration. Recurrent parotitis, also known as juvenile recurrent parotitis, is characterized by a cyclic swelling of the parotid glands associated with discomfort and/or pain in the absence of external inflammatory changes during a period of several years. The condition mainly affects children between the ages of three and six, males being more commonly affected. The symptoms peak in the first year of school and usually begin to subside after puberty. Retrograde infection induced by the mumps virus and upper respiratory infection play a major role in the etiology of recurrent parotitis. Sialography demonstrates sialectasia. Children with recurrent parotid swelling needs to be screened for underlying systemic immune disorders such as Sjogren's syndrome. With time the recurrent episodes reduce salivary flow, while increasing the chloride, sodium, copper, albumin, IgA and lactoferrin concentration. Etiology of juvenile recurrent parotitis is a combination of congenital malformation of portions of the salivary ducts and a set-in infection. Treatment is conservative.

1- Park JW: Recurrent parotitis in childhood. Clin Pediatr (Phial)  31(4):254-5, 1992
2- Mandel L, Kaynar A: Recurrent parotitis in children. N Y State Dent J  61(2):22-5, 1995
3- Giglio MS, Landaeta M, Pinto ME: Microbiology of recurrent parotitis. Pediatr Infect Dis J  16(4):386-90, 1997
4- Chitre VV, Premchandra DJ: Recurrent parotitis. Arch Dis Child  77(4):359-63, 1997

Volume 20 No 04 APRIL 2003

Partial Splenectomy

Partial splenectomy (PS) is a safe and effective alternative for several hemolytic disorders and hemoglobinopathies in children including hereditary spherocytosis, sickle cell disease and b-thalassemia. It is the operation of choice for all benign splenic conditions. The main objective of partial splenectomy is preserve splenic contribution to host defense and reduce the incidence of post splenectomy sepsis (4% risk; mortality rate 1.5%), while reducing symptoms of hemolysis and sequestration from the systemic disorder. The risk of septicemia is even higher in very young children (< four years old). Technically, the goal of the procedure is to remove 80% of splenic tissue by preserving either the upper or lower pole. The preserved remnant has not proved to reduce the possibility of post splenectomy sepsis due to the prohibited large clinical trial needed, but has been found to preserve partial phagocytic function and normal immunoglobulin level, reduce Howell-Jolly bodies with confirmed visualization on scintigraphy. In hereditary spherocytosis partial splenectomy has achieved two aims: decrease red cell destruction and preserve the phagocytic and immunologic functions of the spleen. Because of chronic hemolysis the size of the spleen remnant has a tendency to increase with time, specially rapid the younger the child. Follow-up is closely needed in these children to determine if they need conversion to total splenectomy. For sickle cell anemia partial splenectomy has greatly reduced the acute sequestration crisis, need for hospitalization and subsequent transfusions but does not always preserve splenic function. This is probably cause by progressive auto-infarction of the splenic remnant. Since sickle cell patients autoinfarct between 36 and 60 months of life the procedure would benefit more children with less than 48 months of age.

1- Nouri A, de Montalembert M, Revillon Y, Girot R: Partial splenectomy in sickle cell syndromes. Arch Dis Child  66(9):1070-2, 1991
2- Tchernia G, Gauthier F, Mielot F, Dommergues JP, Yvart J, Chasis JA, Mohandas N: Initial assessment of the beneficial effect of partial splenectomy in hereditary spherocytosis. Blood  81(8):2014-20, 1993
3- Svarch E, Vilorio P, Nordet I, Chesney A, Batista JF, Torres L, Gonzalez A, de la Torre E: Partial splenectomy in children with sickle cell disease and repeated episodes of splenic sequestration. Hemoglobin  20(4):393-400, 1996
4- Kimber C, Spitz L, Drake D, Kiely E, Westaby S, Cozzi F, Pierro A: Elective partial splenectomy in childhood. J Pediatr Surg  33(6):826-9, 1998
5- Idowu O, Hayes-Jordan A: Partial splenectomy in children under 4 years of age with hemoglobinopathy. J Pediatr Surg  33(8):1251-3, 1998
6- al-Salem AH, al-Dabbous I, Bhamidibati P: The role of partial splenectomy in children with thalassemia. Eur J Pediatr Surg  8(6):334-8, 1998
7- de Buys Roessingh AS, de Lagausie P, Rohrlich P, Berrebi D, Aigrain Y: Follow-up of partial splenectomy in children with hereditary spherocytosis. J Pediatr Surg  37(10):1459-63, 2002
8- Rice HE, Oldham KT, Hillery CA, Skinner MA, O'Hara SM, Ware RE: Clinical and hematologic benefits of partial splenectomy for congenital hemolytic anemias in children. Ann Surg  237(2):281-8, 2003

Congenital Rectal Stenosis

Congenital rectal stenosis (CRS) is a rare anorectal malformation characterized by a tubular defect in which an stenotic distal rectum partially communicates with the distal anal canal. The stenosis is located at the natural limit of rectum and anal canal (immediately above the pectinate line), runs for one to two centimeters in distance while the child is born with a normal-looking external anus. The defect has all the necessary elements responsible for bowel control including excellent muscle component, an anal canal located within the limits of the external sphincters with all the nerve ending that afford normal sensation and a normal sacrum. CRS is usually detected in the newborn during the initial physical examination. Rectal stimulation can cause propulsive watery output. Local ischemia occurring late during fetal life may be the mechanism responsible for the creation of rectal stenosis of the middle and/or upper rectum. CRS is the hallmark finding in the Currarino's triad (sacral bony abnormality, presacral mass and rectal stenosis). Management of CRS consists of serial daily dilatations increasing the size of the dilator progressively or continuous balloon distension. Failure of conservative therapy (dilatation) should alert the physician to the presence of an associated pathologic condition in the presacral space. Chronic constipation is a common post-dilatation feature in these patients.

1- Malangoni MA, Grosfeld JL, Ballantine TV, Kleiman M: Congenital rectal stenosis: a sign of a presacral pathologic condition. Pediatrics  62(4):584-7, 1978
2- Stone HH, Wilkinson AW: Experimental production of rectal stenosis and atresia in the rabbit. J Pediatr Surg  18(1):89-90, 1983
3- O'Riordain DS, O'Connell PR, Kirwan WO: Hereditary sacral agenesis with presacral mass and anorectal stenosis: the Currarino triad. Br J Surg  78(5):536-8, 1991
4- Zia-w-Miraj Ahmad M, Brereton RJ, Huskisson L: Rectal atresia and stenosis. J Pediatr Surg  30(11):1546-50, 1995

Omental Infarction

Omental infarction is a very rare painful condition that can mimic appendicitis, pyelitis or cholecystitis in children. This condition is different from omental torsion which is usually associated with intraabdominal pathology such as omental cysts, hernias, tumors or adhesions. Etiology of omental infarction is unknown. Torsion occurs with omental long axis rotation resulting in venous  obstruction followed by arterial obstruction, infarction and gangrene. The omentum usually rotates around the distal right gastro-epiploic artery causing right lower abdominal pain. Obesity is a well-known predisposing factor. All patients present with acute onset of right lower quadrant pain. Clinically, the child develops local tenderness with peritoneal signs but without gastrointestinal symptoms. CT scan (defined area of fat interspersed with hyper attenuating streaks) is diagnostic. Laparoscopy will confirm the diagnosis. Surgical resection of the infarcted omentum results in immediate resolution of pain with minimal morbidity. Others believe that with preoperative diagnosis resection should depend on symptoms.

1- Puylaert JB: Right-sided segmental infarction of the omentum: clinical, US, and CT findings. Radiology  185(1):169-72, 1992
2- Helmrath MA, Dorfman SR, Minifee PK, Bloss RS, Brandt ML, DeBakey ME: Right lower quadrant pain in children caused by omental infarction. Am J Surg  182(6):729-32, 2001
3- Cervellione RM, Camoglio FS, Bianchi S, Balducci T, Dipaola G, Giacomello L, Chironi C, Erculiani E, Ottolenghi A: Secondary omental torsion in children: report of two cases and review of the literature. Pediatr Surg Int  18(2-3):184-6, 2002
4- Grattan-Smith JD, Blews DE, Brand T: Omental infarction in pediatric patients: sonographic and CT findings. AJR Am J Roentgenol 178(6):1537-9, 2002
5- Varjavandi V, Lessin M, Kooros K, Fusunyan R, McCauley R, Gilchrist B: Omental Infarction: Risk Factors in Children. J Pediatr Surg 38(2): 233-235, 2003

Volume 20 N0 05 MAY 2003


Appendicitis is still the most common condition requiring emergent abdominal surgery in childhood caused by obstruction of the appendiceal lumen, most commonly a fecalith. The obstruction distends the lumen leading to arterial occlusion and infarction. Initially visceral periumbilical pain occurs carried by afferent sympathetic fibers to T10 dermatome. With progression of inflammation the pain shifts to the right lower quadrant. Anorexia, nausea and vomiting follow. Point tenderness in the right lower quadrant (or the persistence of right lower quadrant pain) is the most reliable physical finding. Fever is usually present. Laboratory findings are an elevated white blood cell count in most instances. Very high WBC's > 18,000 may indicate perforation. Radiographic findings may include ileus, appendicolith (pathognomonic finding), splinting and abdominal wall edema. Ultrasound and CT-scan have improved the diagnostic accuracy in children with suspected appendicitis. Appendicitis is managed with appendectomy, open ( right lower either quadrant horizontal muscle splitting incision) or laparoscopic. Preoperative antibiotics and hydration are mandatory in all cases of suspected appendicitis. All wounds are closed primarily without drains. Complicated appendicitis (gangrenous and perforated) receives postoperative antibiotics until the child clinical condition improves, fever subsides, ileus are gone and the WBC count normalizes. Postoperative persistent fever, ileus or leukocytosis mandates an imaging search (usually CT-Scan) for intraabdominal collections. These collections can be managed with percutaneous drainage and continued antibiotherapy.

1- Santini I, Pacheco R, Lugo-Vicente HL: Perforated Appendicitis in Children: Evaluation of a delayed diagnosis (Spanish). PR Health Science J 14(4):263-267, 1995
2- Dilley A, Wesson D, Munden M, Hicks J, Brandt M, Minifee P, Nuchtern J: The impact of ultrasound examinations on the management of children with suspected appendicitis: a 3-year analysis. J Pediatr Surg  36(2):303-8, 2001
3- Gwynn LK: The diagnosis of acute appendicitis: clinical assessment versus computed tomography evaluation. J Emerg Med  21(2):119-23, 2001
4- Meguerditchian AN, Prasil P, Cloutier R, Leclerc S, Peloquin J, Roy G: Laparoscopic appendectomy in children: A favorable alternative in simple and complicated appendicitis. J Pediatr Surg  37(5):695-8, 2002
5- Chen C, Botelho C, Cooper A, Hibberd P, Parsons SK: Current practice patterns in the treatment of perforated appendicitis in children. J Am Coll Surg  196(2):212-21, 2003
6- Emil S, Laberge JM, Mikhail P, Baican L, Flageole H, Nguyen L, Shaw K: Appendicitis in children: A ten-year update of therapeutic recommendations. J Pediatr Surg  38(2):236-42, 2003
7- Newman K, Ponsky T, Kittle K, Dyk L, Throop C, Gieseker K, Sills M, Gilbert J: Appendicitis 2000: Variability in practice, outcomes, and resource utilization at thirty pediatric hospitals. J Pediatr Surg  38(3):372-9, 2003

Acute Cholecystitis

Acute cholecystitis in children is a rare disease entity associated with hemolytic diseases (sickle-cell disease, hereditary spherocytosis and thalassemia), severe intercurrent illness and congenital anomalies. Children presents with fever, nausea, vomiting, acute abdominal right upper quadrant pain (positive Murphy' sign from a distended, tender gallbladder) and leukocytosis. In a few patient a mass may be present in the right upper quadrant. With jaundice the suspicion of common bile ducts stones should be raised. Chronic cholecystitis with cholelithiasis is a more common presentation in children than acute cholecystitis. Diagnosis can be establish with ultrasound (thickened gallbladder wall)  and use of HIDA bilioenteric studies to asses patency of the cystic duct (procedure of choice). Initial management consist of hydration and antibiotics to cool down the inflammatory process. This is followed by early laparoscopic cholecystectomy except in patients presenting with a gallbladder phlegmon later than seven days after the onset of the attack. Laparoscopic cholecystectomy for acute cholecystitis in patients with sickle cell disease has also been found to be safe and recommended in experienced hands with adequate preoperative preparation of the child.

1- Ziv Y, Feigenberg Z, Dintsman M: Acute inflammation and distension of the gall bladder in infancy. Aust Paediatr J  23(1):53-4, 1987
2- Coughlin JR, Mann DA: Detection of acute cholecystitis in children. Can Assoc Radiol J  41(4):213-6, 1990
3- Reiss R, Nudelman I, Gutman C, Deutsch AA: Changing trends in surgery for acute cholecystitis. World J Surg  14(5):567-70, 1990
4- Rescorla FJ, Grosfeld JL: Cholecystitis and cholelithiasis in children. Semin Pediatr Surg 1(2):98-106, 1992
5- Lugo-Vicente HL: Trends in Management of Gallbladder Disorders in Children. Pediatr Surg Internat 12(5-6):348-352, 1997
6- Holcomb GW 3rd, Morgan WM 3rd, Neblett WW 3rd, Pietsch JB, O'Neill JA Jr, Shyr Y: Laparoscopic cholecystectomy in children: lessons learned from the first 100 patients. J Pediatr Surg  34(8):1236-40, 1999
7- Avrutis O, Friedman SJ, Meshoulm J, Haskel L, Adler S: Safety and success of early laparoscopic cholecystectomy for acute cholecystitis. Surg Laparosc Endosc Percutan Tech  10(4):200-7, 2000
8-  Sinha R, Sharma N: Acute cholecystitis and laparoscopic cholecystectomy. JSLS 6(1):65-8, 2002

Surgical Ethics

Surgical ethics is based on recognition of the rights of patients who require care by a surgeon. The four basic principles of ethics that we will consider in a series of future reviews are beneficence, no-maleficence, respect for autonomy and justice in what pertains the practice of surgery and medicine. We must recognize that patients have seven basic rights: 1- the right not to be killed intentionally or negligently, 2- not to be harmed by intent or negligence and 3- not to be deceived by the surgeon. Patients also have the right to: 4- be adequately informed about the risks and benefit of surgery, 5- be treated by a knowledgeable competent practitioner, 6- to have his or her health and well-being more highly valued than the surgeon's own economic interest, and to 7- decide whether to accept treatment under the conditions described. Surgeons must act as moral fiduciary of the patient always avoiding them to come into self-interest conflicts. Honesty is the general fiduciary commitment to protect and promote the interest of the patient if surgical ethics is to guide the clinical judgement and practice of surgeons in a comprehensive way.

1- Mc Cullough LB, Jones JW, Brody BA: Surgical Ethics. 1st ed. New York: Oxford University Press, 1998
2- H. Tristram Engelhardt, Jr.: The Foundation of Bioethics, 2nd ed. New York: Oxford University  Press, 1996

Volume 20 No 06 JUNE 2003

Cricopharyngeal Achalasia

Congenital cricopharyngeal achalasia (CCA) is an important but relatively seldom diagnosed cause of dysphagia in children caused by failure of the cricopharyngeal muscle to relax at the appropriate time during the third period of swallowing mechanisms in the absence of other motor abnormalities. Age of initial presentation ranges from birth to six months. Symptoms include failure to thrive, regurgitation of food, choking, cyanosis, nasal reflux, coughing and recurrent aspiration pneumonia. Cine-esophagogram with fluoroscopic observation of the swallowing mechanism will establish the diagnosis in most children. The characteristic sign is a round and regular posterior narrowing on the posterior wall of the esophagus at the level of C4-C5 with enlargement of the hypopharynx. Esophageal motility studies will quantify changes and also evaluate lower esophageal dysfunction not easily identified in esophagograms. Though CCA can rarely be found as an isolated condition, it is usually associated with neurologic deficit as seen in myelomeningocele and Arnold Chiari malformations. Management of CCA includes positioning, nasogastric lavage feedings, balloon dilatation of the upper esophagus or surgical myotomy of the cricopharyngeal muscle. If all other measures fails, cricopharyngeal myotomy is a safe and effective operation with excellent results. Symptomatic relief is immediate and complete with no long-term recurrence documented.

1- Lernau OZ, Sherzer E, Mogle P, Nissan S: Congenital cricopharyngeal achalasia treatment by dilatations. J Pediatr Surg  19(2):202-3, 1984
2- Skinner MA, Shorter NA: Primary neonatal cricopharyngeal achalasia: a case report and review of the literature. J Pediatr Surg  27(12):1509-11, 1992
3- Raboei E, Luoma R: Neonatal cricopharyngeal achalasia--a case report. Eur J Pediatr Surg  10(2):130-2, 2000
4- Brooks A, Millar AJ, Rode H: The surgical management of cricopharyngeal achalasia in children. Int J Pediatr Otorhinolaryngol  56(1):1-7, 2000
5- Muraji T, Takamizawa S, Satoh S, Nishijima E, Tsugawa C, Tamura A, Shimizu N: Congenital cricopharyngeal achalasia: Diagnosis and surgical management. J Pediatr Surg  37(5):E12, 2002


Ganglioneuroma is a rare and benign neural mature crest tumor of the sympathetic nervous system. They arise wherever sympathetic tissue exists and may be seen in the neck, posterior mediastinum, adrenal gland, retroperitoneum, and pelvis. The clinical presentation of most patients is that of an asymptomatic slow growing solid mass in an older child. On many occasions the tumor is found incidentally after simple chest films or abdominal imaging studies. Imaging studies cannot differentiate a ganglioneuroma from its malignant counterpart neuroblastoma. Rarely the child develops respiratory problems, painless spinal deformity or neurologic deficit before the mass is found. Most of these tumors are hormone silent. Histologically, ganglioneuroma is completely differentiated and composed of mature ganglion cells, Schwann's cells and neuropils. Ganglioneuromas frequently produce somatostatin and vasoactive intestinal peptide (VIP). Management consists of surgical excision whenever possible. Transperitoneal laparoscopic adrenalectomy has been safely performed when this tumor arises from the adrenal gland.

1- Young DG: Thoracic neuroblastoma/ganglioneuroma. J Pediatr Surg  18(1):37-41, 1983
2- Girgert R, Schweizer P, Schwable J: Neuroblastoma: induction of differentiation (Part I). Basical science in pediatric surgery.  Eur J Pediatr Surg  10(2):79-82, 2000
3- Miller KA, Albanese C, Harrison M, Farmer D, Ostlie DJ, Gittes G, Holcomb GW 3rd: Experience with laparoscopic adrenalectomy in pediatric patients. J Pediatr Surg  37(7):979-82, 2002
4- Lonergan GJ, Schwab CM, Suarez ES, Carlson CL: Neuroblastoma, ganglioneuroblastoma, and ganglioneuroma: radiologic-pathologic correlation. Radiographics  22(4):911-34, 2002
5- C. Thomas Blank: Neuroblastoma, in Andrassy "Pediatric Surgical Pathology", WB Saunders Co, Philadelphia, 1998, pages 177-180.


As we enter the 21st century, minimal invasive surgical procedure will continue to demonstrate its superiority over conventional open surgery in terms of pain control, convalescence, hospital stay, cosmesis and achieving its purpose. Thoracoscopy using video-endoscopic technique is reliantly replacing open thoracotomy in many chest conditions in children. As diagnostic aid, thoracoscopy can be use for biopsy of lung, pleural, foregut and mediastinal benign and malignant masses. Therapeutically, thoracoscopically has been utilized for excision of esophageal duplication cysts, closure of patent ductus arteriosus, pleurodesis, resection of lung (bullectomy, lobectomy or segmentectomy), thymectomy, anterior spinal fusion procedures for scoliosis, management of empyema, and resection of mediastinal cysts and tumors. Almost the entire anterior and posterior mediastinum is visible by thoracoscopy. Advantages of thoracoscopic procedure consist in less pain, less compromise of pulmonary reserve, shorter hospital stay and costs. Contraindications for thoracoscopy consist of inability to develop a pleural window with the scope and patients in high pressure mechanical ventilation in need of lung biopsy due to the high incidence of postop air leak. Once the thoracoscope is in you should always perform an evaluation of the anatomy before committing the child to an open thoracotomy.

1- Rothenberg SS: Thoracoscopy in infants and children. Semin Pediatr Surg 7(4):194-201, 1998
2- Michel JL, Revillon Y, Montupet P, Sauvat F, Sarnacki S, Sayegh N, N-Fekete C: Thoracoscopic treatment of mediastinal cysts in children. J Pediatr Surg  33(12):1745-8, 1998
3- Rothenberg SS: Thoracoscopic lung resection in children. J Pediatr Surg  35(2):271-4, 2000
4- Kogut KA, Bufo AJ, Rothenberg SS, Lobe TE: Thoracoscopic thymectomy for myasthenia gravis in children. J Pediatr Surg  35(11):1576-7, 2000
5- Subramaniam R, Joseph VT, Tan GM, Goh A, Chay OM: Experience with video-assisted thoracoscopic surgery in the management of complicated pneumonia in children. J Pediatr Surg  36(2):316-9, 2001
6- Partrick DA, Rothenberg SS: Thoracoscopic resection of mediastinal masses in infants and children: an evaluation of technique and results. J Pediatr Surg  36(8):1165-7, 2001
7- Partrick DA, Bensard DD, Teitelbaum DH, Geiger JD, Strouse P, Harned RK: Successful thoracoscopic lung biopsy in children utilizing preoperative CT-guided localization. J Pediatr Surg  37(7):970-3, 2002
8- Smith TJ, Rothenberg SS, Brooks M, Bealer J, Chang J, Cook BA, Cullen JW: Thoracoscopic surgery in childhood cancer. J Pediatr Hematol Oncol  24(6):429-35, 2002
9- Warmann S, Fuchs J, Jesch NK, Schrappe M, Ure BM: A prospective study of minimally invasive techniques in pediatric surgical oncology: preliminary report. Med Pediatr Oncol  40(3):155-7, 2003
10- Rodgers BM: The Role of Thoracoscopy in Pediatric Surgical Practice. Semin Pediatr Surg 12(1): 62-70, 2003

Journal Club