VOLUME 09, 1997

VOL 09 NO 01 JULY 1997

Adreno Cortical Carcinoma

Adreno-cortical carcinoma (ACC) is a rare potentially fatal tumor during childhood. It occurs with significant frequency in Southern Brazil. Virilization alone (pubic and/or body hair, clitoris or penis enlargement, and adult voice), or associated to Cushing's syndrome from corticosteroid hypersecretion is the most prominent feature. Feminizing tumors are uncommon and nonfunctional tumors rare. Most children are females with a median age of four years. Other signs include: abdominal mass, hypertension, seizures, and weight loss. Features associated with malignancy included high levels of 17-keto steroids, tumor weight greater than 100 g, tumor size greater than 200 cm3, and histologic evidence of diffuse growth pattern, vascular invasion, and tumor cell necrosis. CT Scan is the single most important modality in assessing primary and metastatic disease at diagnosis, and plays an important role in defining the extent of the primary lesion preoperatively. Early diagnosis and aggressive surgical extirpation may lead to an increased survival of children with ACC. An anterior thoracoabdominal approach is favored for suspected malignancies with en bloc resection. There is no evidence that adjuvant therapy provides any additional benefit. The prognosis is age related and dependent on the resectability of the tumor. Children with small adrenocortical tumors have an excellent prognosis with surgery as sole therapy.

1- Daneman A, Chan HS,  Martin J: Adrenal carcinoma and adenoma in children: a review of 17 patients. Pediatr Radiol 13(1):11-8, 1983
2- Kay R, Schumacher OP, Tank ES: Adrenocortical carcinoma in children. J Urol 130(6):1130-2, 1983
3- Neblett WW, Frexes-Steed M,  Scott HW Jr: Experience with adrenocortical neoplasms in childhood. Am Surg 53(3):117-25, 1987
4- Chudler RM, Kay R: Adrenocortical carcinoma in children. Urol Clin North Am 16(3):469-79, 1989
5- Ribeiro RC, Sandrini Neto RS, Schell MJ, Lacerda L, Sambaio GA, Cat I: Adrenocortical carcinoma in children: a study of 40 cases.  J Clin Oncol 8(1):67-74, 1990
6- Arico M, Bossi G, Livieri C, Raiteri E, Severi F: Partial response after intensive chemotherapy for adrenal cortical carcinoma in a child. Med Pediatr Oncol 20(3):246-8, 1992
7- Sabbaga CC, Avilla SG, Schulz C. Garbers JC, Blucher D: Adrenocortical carcinoma in children: clinical aspects and prognosis. J Pediatr Surg 28(6):841-3, 1993
8- al-Salem AH, Abu-Srair HA: Recurrent adrenocortical carcinoma in a 4 year old girl. Aust N Z J Surg 64(10):723-5, 1994
9- Teinturier C, Brugieres L, Lemerle J, Chaussain JL, Bougneres PF: [Adrenocortical carcinoma in children: retrospective study of 54 cases]. Arch Pediatr 3(3):235-40, 1996
10- Michalkiewicz EL, Sandrini R, Bugg MF, Cristofani L, Caran E, Cardoso AM, de Lacerda L, Ribeiro RC: Clinical characteristics of small functioning adrenocortical tumors in children. Med Pediatr Oncol 28(3):175-8, 1997


Pheochromocytoma is a rare APUD (amine precursor uptake decarboxylate) tumor in children. A well differentiated, functionally mature neoplasm that originates mainly from the adrenal medulla, other times from the sympathetic chain, the paraganglia or organ of Zuckerkandl. Most patients are males, with an average age of 9-11 years showing sustained hypertension (80%); 24% of these tumors are bilateral, 15% multiple and 15% extra-adrenal in location. Other signs are: headache, sweating, blurred vision, fatigue, palpitations, and anorexia. An increase familial incidence as a single Mendelian dominance is also noted in the pediatric age group. Affected patients should be screened for MEN 2. The most accurate method of diagnosis is cathecolamines determination in blood and urine (VMA). CT-Scan will localize most tumors, and the yield can be increased with the complementary use of MIBG (meta-iodo-benzylguanidine), an adrenergic tissue localizing agent, particularly in extra-adrenal or metastatic lesions. Management consists of preoperative control of hypertension with alpha adrenergic blocking agents followed by transabdominal surgical resection under strict intra-operative monitoring. The high percentage of children with bilateral, extra-adrenal, and multiple tumors warrants the need for long-term follow-up. Metastatic well-differentiated tumors are better managed with radioactive MIBG. In the malignant variety chemotherapy should be used for residual disease after surgery and for metastatic disease.

1- Lugo-Vicente HL, Marquez-Grau  E: Diagnostic and Therapeutic Problems arising from a Celiac Axis Pheochromocytoma in a Child: A Case Report. Boletin Asoc Med PR 79:325-328, 1987
2- Ein SH, Weitzman S, Thorner P, Seagram CG, Filler RM: Pediatric malignant pheochromocytoma.  J Pediatr Surg 29(9):1197-201, 1994
3- Gelfand MJ: Meta-iodo-benzylguanidine in children. Semin Nucl Med 23(3):231-42, 1993
4- Aguilo F,  Tamayo N, Vazquez-Quintana E, Rabell V, Haddock L, Allende M, Pagan H, Gonzalez A: Pheochromocytoma: a twenty year experience at the University Hospital [published erratum appears in P R Health Sci J 11(1):6] P R Health Sci J 10(3):135-42, 1991
5- Daneman A: Adrenal neoplasms in children. Semin Roentgenol 23(3):205-15, 1988
6- Revillon Y, Daher P, Jan D, Buisson C, Bonnerot V, Martelli H, Nihoul-Fekete C: Pheochromocytoma in children: 15 cases. J Pediatr Surg 27(7):910-1, 1992
7- Khafagi FA, Shapiro B, Fischer M, Sisson JC, Hutchinson R, Beierwaltes WH: Pheochromocytoma and functioning paraganglioma in childhood and adolescence: role of iodine 131 metaiodobenzylguanidine. Eur J Nucl Med 18(3):191-8, 1991
8- Fonkalsrud EW:  Pheochromocytoma in childhood. Prog Pediatr Surg 26:103-11, 1991
9- Ein SH, Shandling B, Wesson D, Filler Rm: Recurrent pheochromocytomas in children.  J Pediatr Surg 25(10):1063-5, 1990


Pilomatrixoma, also known as calcifying epithelioma of Malherbe, is a benign tumor of the skin originating from hair sheath cells. Most cases occur in children less than ten-years of age with a 2:1 female to male ratio. Presents clinically as asymptomatic superficial slow-growing dermal or subcutaneous mass most commonly located in the head and neck or upper extremity. Histology shows two characteristic epithelial cells: the basophilic and ghost cells, and calcification is usually present (these features can help the diagnosis when using FNA cytology). Pilomatrixoma can be confused with an epidermal inclusion cyst, lymph node, or calcified hematoma. The "faceted stone sign" (palpation of a tumor with a faceted rocky consistency) is evident. Management consists of local excision, and recurrences are very rare. Prognosis after removal is excellent. Multiple familial pilomatricomas could be considered a cutaneous marker of Gardner syndrome.

1- Hawkins DB, Chen WT: Pilomatrixoma of the head and neck in children. Int J Pediatr Otorhinolaryngol 8(3):215-23, 1985
2- Ganz H: [Is Malherbe calcifying epithelioma in the ENT area of a rare tumor?] HNO 34(7):301-4, 1986
3- Solanki P, Ramzy I, Durr N, Henkes D: Pilomatrixoma. Cytologic features with differential diagnostic considerations. Arch Pathol Lab Med 111(3):294-7, 1987
4- Nield DV, Saad MN, Ali MH: Aggressive pilomatrixoma in a child: a case report. Br J Plast Surg 39(1):139-41, 1986
5- Grinspan Bozza NO: [Pilomatrixoma. A series of 18 surgically treated cases. A sign for its diagnosis] Med Cutan Ibero Lat Am 17(6):387-90, 1989
6- Unger P, Watson C, Phelps RG, Danque P, Bernard P: Fine needle aspiration cytology of pilomatrixoma (calcifying epithelioma of Malherbe). Report of a case. Acta Cytol 34(6):847-50, 1990
7- Marrogi AJ, Wick MR, Dehner LP: Pilomatrical neoplasms in children and young adults. Am J Dermatopathol 14(2):87-94, 1992
8- Wells NJ, Blair GK, Magee JF, Whiteman DM: Pilomatrixoma: a common, benign childhood skin tumor. Can J Surg 37(6):483-6, 1994
9- Pujol RM, Casanova JM, Egido R, Pujol J, de Moragas JM: Multiple familial pilomatricomas: a cutaneous marker for Gardner syndrome? Pediatr Dermatol 12(4):331-5, 1995
10- Kumar N, Verma K, Fine needle aspiration (FNA) cytology of pilomatrixoma. Cytopathology 7(2):125-31, 1996

Vol 09 No 02 AUGUST 1997


Hereditary spherocytosis is  a clinically heterogenous autosomal dominant red blood cell membrane disorder that causes anemia. The genetic defect results in deficiency of spectrin, the largest and most abundant structural protein of the erythrocyte membrane skeleton. The affected RBC loses its biconcave shape, strength and flexibility to the stress of circulation, becomes round and is trapped and destructs early in the spleen. Initial symptoms are those of pallor, jaundice and chronic anemia, followed by splenomegaly. Hemolytic crises are triggered by intercurrent infections. Pigment gallstones are common after the first decade of life. Labs' findings are those of many spherocytes in the peripheral smear, 8-10 mg% hemoglobin, elevated reticulocyte count, increase erythropoeisis in the bone marrow, and negative Coomb's test. Erythrocytes' shows increased osmotic fragility with autohemolysis in hypotonic solutions. Definitive therapy consists of splenectomy. This condition is the most common indication for elective splenectomy in children. The risk of overwhelming postsplenectomy sepsis makes it advisable to delay splenectomy until after six years of age unless the child becomes transfusion dependent. At the time of splenectomy, it is important to identify and remove accessory spleens. If gallstones are present, cholecystectomy should be done. A low content of spectrin and high percentage of microcyte has been used as determinants of early splenectomy as judge by the clinical severity of the disease process. Howell-Jolly bodies in erythrocyte are identified after total splenectomy.
1- Burke BE,Shotton DM: Erythrocyte membrane skeleton abnormalities in hereditary spherocytosis. Br J Haematol 54(2):173-87, 1983
2- Schroter W, Kahsnitz E: Diagnosis of hereditary spherocytosis in newborn infants. J Pediatr 103(3):460-3, 1983
3- Lux SE: A genetic defect in the binding of protein 4.1 to spectrin in a  kindred with hereditary spherocytosis. N Engl J Med 25;307(22):1367-74, 1982
4- Orringer EP, Sheldon GF: Hereditary spherocytosis. Recent experience and current concepts of pathophysiology. Ann Surg 203(1):34-9, 1986
5- Eber SW, Armbrust R, Schroter W: Variable clinical severity of hereditary spherocytosis: relation to erythrocytic spectrin concentration, osmotic fragility, and autohemolysis. J Pediatr 117(3):409-16, 1990
6- Koh MT, Ng SC: Hereditary spherocytosis: a study of 16 patients from University Hospital, Kuala Lumpur. Singapore Med J 32(1):67-9, 1991
7- Gigot JF, Legrand M, Cadiere GB, Delvaux G, de Ville de Goyet J, de Neve de Roden A, Van Vyve E, Hourlay P, Etienne J, Njinou B, et al: Is laparoscopic splenectomy a justified approach in hematologic disorders? Preliminary results of a prospective multicenter study. Belgian Group for Endoscopic Surgery. Int Surg 80(4):299-303, 1995
8- Hicks BA, Thompson WR, Rogers ZR, Guzzetta PC: Laparoscopic splenectomy in childhood hematologic disorders. J Laparoendosc Surg 6 Suppl 1:S31-4, 1996

Cystosarcoma Phylloides

First described by Muller in 1838, cystosarcoma phylloides (CSP) is a rare breast neoplasm of fibroepithelial origin rarely seen in adolescent females. Phylloides tumors have been histologically classified as benign, borderline, or malignant. Classification of malignancy is based on stromal findings of cellular atypia, anaplasia, and degree of mitotic activity. Malignancy is similar to sarcomas of the breast. Presentation of CSP is that of a relative large, movable, painless mass sought to be a fibroadenoma. Lack complete encapsulation and extends into surrounding tissue in multiple projections of different sizes. Young patients elicited a short history with rapid growth of the lesion. FNA cytology is often diagnostic of CSP. Management depends on histopathology. Benign lesions can be managed with local excision (enucleation) using a cosmetic incision. Borderline or malignant CSP should be treated with wide local excision (simple mastectomy) or re-excision to negative margins. Failure of complete excision results in local recurrence. Metastasis are bloodborn involving most frequently the lung and bones. Axillary dissection is recommended only if nodes are palpable. Adjuvant radiation is necessary, if wide local spread of the tumor is present and a resection of the lesion with a 2-cm tumorfree zone is not possible. Close followup is mandatory.
1- Mollitt DL, Golladay ES, Gloster ES, Jimenez JF: Cystosarcoma Phylloides in the Adolescent Female. J Pediatr Surg 22(10): 907-910, 1987
2- Hines JR, Murad TM, Beal JM:  Prognostic indicators in cystosarcoma phylloides. Am J Surg 153(3):276-80, 1987
3- Blanckaert D, Lecourt O, Loeuille GA, Six J, Laurent JC: [Phyllodes tumor of the breast in an 11-year-old child] Pediatrie 43(5):405-8, 1988
4- Sheen-Chen SM, Chou FF, Chen WJ: Cystosarcoma phylloides of the breast: a review of clinical, pathological and therapeutic option in 18 cases. Int Surg 76(2):101-4, 1991
5- Vesely F, Baco E, Hudcova D: [Cystosarcoma phylloides in adolescent women] Rozhl Chir 71(9):456-63, 1992
6- Mallebre B, Ebert A, Perez-Canto A, Hopp H, Opril F, Weitzel H: [Cystosarcoma phylloides of the breast. A retrospective analysis of 12 cases] Geburtshilfe Frauenheilkd 56(1):35-40, 1996
7- Briggs R, Walters M. Rosenthal D: Cystosarcoma phylloides in adolescent female patients. Am J Surg 146: 712-714, 1983
8- Moore MP, Kinne DW: Breast Sarcoma. Surg Clin NA 76(2): 383-392, 1996

Vaginal Agenesis

It is estimated that vaginal atresia (or agenesis) occurs in every one to 5-10,000 birth, the result of the Mayer-Rokitansky syndrome (partial or complete absence of the uterus with an absent or hypoplastic vagina), or an intersexual condition. In either case the vagina may be represented by a solid epithelial cord. Usually there is concomitant aplasia of the upper part of the paramesonephric ducts so that the uterus and tubes are rudimentary.  The ovaries are usually normal. Associated anomalies of the urinary tract are frequent. Those cases not diagnosed in early infancy will eventually present during adolescence with primary amenorrhea and cyclic pelvic abdominal pain. US, CT, and recently MRI is extremely useful in delineating the anatomic malformation. Many ingenious procedures have been devised for creation of a neovagina in children: skin-flaps and bowel (colon) replacement vaginoplasty. They are accomplished during puberty.
1- Martinez-Mora J, Castellvi IA, Lopez-Ortiz P: Neovagina in Vaginal Agenesis: Surgical Methods and Long-Term Results. J Pediatr Surg 27(1): 10-14, 1992
2- Strubbe EH,  Willemsen WN, Lemmens JA, Thijn CJ, Rolland R: Mayer-Rokitansky-Kuster-Hauser syndrome: distinction between two forms based on excretory urographic, sonographic, and laparoscopic findings. AJR Am J Roentgenol 160(2):331-4, 1993
3- Orozco-Sanchez J, Neri-Vela R, Flores-Mendez MS, Sandoval-Sevilla SF, Leon-Cordova K: [Congenital atresia of the vagina] Bol Med Hosp Infant Mex 48(9):648-55, 1991
4- Hitchcock RJ, Malone PS: Colovaginoplasty in infants and children. Br J Urol 73(2):196-9, 1994
5- Salvatore CA, Lodovicci O: Vaginal agenesis: an analysis of ninety cases. Acta Obstet Gynecol Scand 57(1):89-94, 1978

Vol 09 No 03 SEPTEMBER 1997

Idiopathic Thrombocytopenic Purpura

Idiopathic thrombocytopenic purpura (ITP) is probably the most frequent cause of persistent thrombocytopenia in children. Antedated by a viral (usually respiratory) infection, ITP is characterized by acute hemorraghic manifestations such as petechia, purpura, and mucous membrane bleeding (epistaxis). Platelet number and survival time are reduced significantly causing prolonged bleeding time. This is caused by rapid destruction of the reticuloendothelial system (spleen, bone marrow, and liver) due to IgG binding to platelets (autoinmune). Acute ITP regresses spontaneously within 3 to 6 months in 80% of cases. Initial management may consist of short steroid course and/or immunoglobulin. Chronic ITP sets itself if thrombocytopenia persists longer than six months. When this occurs, most cases are asymptomatic except during intercurrent infectious process. Platelet counts below 50,000/mm3, failure of medical therapy, long-term child restriction, or persistent bleeding may need splenectomy. Complete clinical cure is obtained in 70-80% of instances following splenectomy with a low operative risk.  Recurrent symptoms may be caused by a missed accessory spleen. Laparoscopic splenectomy is a safe, cost-effective procedure with reduced hospital stay, faster recovery, better cosmetic results and least trauma. Isolated splenic thrombocytolysis and hyperplasia of megakaryocytopoiesis and of splenic follicles after splenectomy correlated with better stable remission and platelet counts. The response to splenectomy has been found to correlate directly with the response to immunoglobulin therapy. Intermittent immunoglobulin therapy with observation is a reasonable alternative until spontaneous remission occurs.
1- E. Thomas Boles: The Spleen. In Medad Schiller ‘Pediatric Surgery of the Liver, Pancreas and Spleen', W.B. Saunders, 1991, pag 210-212
2- Facon T, Caulier MT, Fenaux P, Plantier I, Marchandise X, Ribet M, Jouet JP, Bauters F: Accessory spleen in recurrent chronic immune thrombocytopenic purpura. Am J Hematol 41(3):184-9, 1992
3- Reid MM: Chronic idiopathic thrombocytopenic purpura: incidence, treatment, and outcome. Arch Dis Child 72(2):125-8, 1995
4- Yoshida K, Yamazaki Y, Mizuno R, Yamadera H, Hara A, Yoshizawa J, Kanai M: Laparoscopic splenectomy in children. Preliminary results and comparison with the open technique. Surg Endosc 9(12):1279-82, 1995
5- Winde G, Schmid KW, Lugering N, Fischer R, Brandt B, Berns T, Bunte H: Results and prognostic factors of splenectomy in idiopathic thrombocytopenic purpura. J Am Coll Surg 183(6):565-74, 1996
6- Law C, Marcaccio M, Tam P, Heddle N, Kelton JG: High-dose intravenous immune globulin and the response to splenectomy in patients with idiopathic thrombocytopenic purpura. N Engl J Med 22;336(21):1494-8, 1997

Malone Stoma

This safe and highly effective novel technique was introduced by PS Malone in 1990. Consist of building a continent appendicocecostomy through which the cecum is intermittently catheterized for administration of an antegrade enema to manage fecal soiling, incontinence, and even intractable constipation if nonsurgical management has failed. This includes children with anorectal malformations, neuropathic etiology (spina bifida, spinal cord injury), and sphincteric trauma. Originally described as reversing the appendix and reimplanting it into the cecum with a submucous tunnel to diminish reflux, the procedure has undergone several modifications such as orthotopic cecal imbrication, the fixation of the ileocecal region at the inner side of the abdominal wall after creation of an appendicocutaneous catheterizable stoma,  laparoscopic approach, tubularized cecal or ileal flap in those without an appendix, and the use of a button. The child will catheterize once or twice a day  washing his distal colon. Most families are satisfied since the child spends more time in normal activities and school. A level of commitment by the child and family is needed. Children with colonic motility disorders and incontinence may not beneficiate from this procedure. The stoma might break, stenosed, closed, or bleed in a few cases. If the child is socially continent with a regimen of enemas, suppositories or washouts, he should continue that way.
1- Malone PS, Ransley PG, Kiely EM: Preliminary report: The antegrade continent enema. Lancet 336: 1217-1218, 1990
2- Squire R, Kiely EM, Carr B, Ransley PG, Duffy PG: The Clinical Application of the Malone Antegrade Colonic Enema. J Pediatr Surg 28(8): 1012-1015, 1993
3- Griffiths DM, Malone PS: The Malone Antegrade Continence Enema. J Pediatr Surg 30(1): 68-71, 1995
4- Goepel M, Sperling H, Stohrer M, Otto T, Rubben H: Management of neurogenic fecal incontinence in myelodysplastic children by a modified continent appendiceal stoma and antegrade colonic enema. Urology 49(5):758-61, 1997
5-  Yamamoto T, Kubo H, Honzumi M: Fecal incontinence successfully managed by antegrade continence enema in children: a report of two cases. Surg Today 26(12):1024-8, 1996
6- Kiely EM, Ade-Ajayi N, Wheeler R: Antegrade continence enemas in the management of intractable faecal incontinence. J R Soc Med 88(2):103P-104P, 1995

RET Oncogene

The RET proto-oncogene is a protein tyrosine kinase gene (Ret protein) expressed in the cells derived from the neural crest. Germline mutations in the RET gene have been associated with neuroblastoma, pheochromocytoma, multiple endocrine neoplasia (MEN) 2, familial medullary thyroid carcinoma (MTC), radiation-induced thyroid papillary carcinoma, and recently Hirschsprung's disease. The Ret protein might have a critical role in the embryogenesis of the enteric nervous system. RET analysis is a suitable method to detect asymptomatic children with MEN at risk to develop MTC allowing us to consider thyroidectomy at a very early stage of neoplasm development (C-cell hyperplasia) or prophylactically.
1- Lyonnet S,  Edery P, Mulligan LM, Pelet A, Dow E, Abel L, Holder S, Nihoul-Fekete C, Ponder BA, Munnich A: [Mutations of RET proto-oncogene in Hirschsprung disease] C R Acad Sci III 317(4):358-62, 1994
2- Rossel M, Schuffenecker I, Schlumberger M, Bonnardel C, Modigliani E, Gardet P, Navarro J, Luo Y, Romeo G, Lenoir G, et al: Detection of a germline mutation at codon 918 of the RET proto-oncogene in French MEN 2B families. Hum Genet 95(4):403-6, 1995
3- Martucciello G, Favre A, Takahashi M, Jasonni V: Immunohistochemical localization of RET protein in Hirschsprung's disease. J Pediatr Surg 30(3):433-6, 1995
4- Fugazzola L, Pilotti S, Pinchera A, Vorontsova TV, Mondellini P, Bongarzone I, Greco A, Astakhova L, Butti MG, Demidchik EP, et al: Oncogenic rearrangements of the RET proto-oncogene in papillary thyroid carcinomas from children exposed to the Chernobyl nuclear accident. Cancer Res 55(23):5617-20, 1995
5- Komminoth P, Kunz EK, Matias-Guiu X, Hiort O, Christiansen G, Colomer A, Roth J, Heitz PU: Analysis of RET protooncogene point mutations distinguishes heritable from nonheritable medullary thyroid carcinomas. Cancer 76(3):479-89, 1995
6- Shimotake T, Iwai N, Inoue K, Inazawa J, Nishisho I: Germline mutations of the RET proto-oncogene in pedigree with MEN type 2A: DNA analysis and its implications for pediatric surgery.  J Pediatr Surg 31(6):779-81, 1996

Vol 09 No 4 OCTOBER 1997

Perforated Appendicitis

Perforated appendicitis continues to occur at a similar rate than twenty years ago, although there are scattered reports that children with symptoms of appendicitis evaluated in managed care systems have shown a tendency to lower perforation rates. Morbidity, mortality and longer hospital stays takes its toll when this vestigial organ inflames and ruptures. Each stage in appendicitis has a unique form of management. The standard goal of therapy for children with perforated appendicitis is: aggressive hydration, immediate appendectomy, systemic antibiotic therapy, copious peritoneal saline irrigation, wound irrigation, and primary closure in the best hands. Open peritoneal lavage has been found superior to  intraperitoneal tube drainage or closed postoperative peritoneal lavage in the management of perforated appendicitis in children. Some authors have found that the technique of peritoneal lavage rather than the disease process itself  may be responsible for some postoperative complications such as small bowel adhesions and obstruction. Leaving the wound open brings painful local care, augment absence from school,  increases the hospital stay, and is not necessary in most children. Subcuticular incisional closure results in minimal wound care and excellent cosmetic results.  Lavage of the surgical wound with physiologic serum is an effective, safe and inexpensive method to prevent infection of the wound following appendectomy.

1- Lund DP, Murphy EU: Management of perforated appendicitis in children: a decade of aggressive treatment. J Pediatr Surg 29(8):1130-3; discussion 1133-4, 1994
2- Samelson SL, Reyes HM: Management of perforated appendicitis in children--revisited. Arch Surg 122(6):691-6, 1987
3-Toki A, Ogura K, Horimi T, Tokuoka H, Todani T, Watanabe Y, Uemura S, Urushihara N, Noda T, Sato Y, et al: Peritoneal lavage versus drainage for perforated appendicitis in children. Surg Today 25(3):207-10, 1995
4-Buanes TA, Andersen GP, Jacobsen U, Nygaard K: Perforated appendicitis with generalized peritonitis. Prospective, randomized evaluation of closed postoperative peritoneal lavage. Eur J Surg 157(4):277-9, 1991
5- Hallerback B, Andersson C, Englund N, Glise H, Nihlberg A, Solhaug J, Wahlstrom B: A prospective randomized study of continuous peritoneal lavage postoperatively in the treatment of purulent peritonitis. Surg Gynecol Obstet 163(5):433-6, 1986
6- Putnam TC, Gagliano N, Emmens RW: Appendicitis in children. Surg Gynecol Obstet 170(6):527-32, 1990
7- Karp MP, Caldarola VA, Cooney DR, Allen JE, Jewett TC Jr: The avoidable excesses in the management of perforated appendicitis in children. J Pediatr Surg 21(6):506-10, 1986
8- Santini I;  Lugo-Vicente HL;  Pacheco R: [Perforated appendicitis in children: evaluation of delayed diagnosis]. P R Health Sci J 14(4):263-7, 1995

Nephrectomy in Neuroblastoma

Removal of a kidney involved with neuroblastoma (NB) should be done for radiographic and intraoperative evidence of gross kidney involvement. A recent study group from Japan for advanced NB (Stage III and IV)  evaluated the preservation of the ipsilateral kidney at surgery on subsequent length of survival showing a better survival in the group of children whose kidney was preserved, although they confess bias in the selection of patients since the decision (for either nephrectomy or kidney preservation) was not randomized.  Kidney invasion in NB is by direct penetration through the capsule or by extension along blood vessels. For curability, stage III needs pathological (histological) evidence of gross tumor removal to be effective therapy.  When the tumor is adhere to vital structures you should not risk injury to nerves, major blood vessels or organs (this includes the kidney) by performing radical excision since there is no improvement in survival. Furthermore unilateral nephrectomy could bring a higher risk of chemotherapyinduced renal impairment. The main reasons to avoid nephrectomy in cases of advanced NB are that survival is not improved, and morbidity/mortality is increased.

1- 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
2- Albregts AE, Cohen MD, Galliani CA: Neuroblastoma invading the kidney. J Pediatr Surg 29(7):930-3, 1994
3- 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
4- 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):937-40; discussion 940-1, 1995
5- Schell M, Cochat P, Hadj-Aissa A, Bouffet E, Dubourg L, Brunat-Mentigny M: Renal function following unilateral nephrectomy for neuroblastoma and Wilms' tumour. Pediatr Nephrol 9(5):579-82, 1995

Preauricular Sinus

Preauricular sinus is a common congenital condition that does not always cause symptoms. It arises from the anterior aspect of the helix, not branchial cleft remnant related. The sinus passes through the skin anteriorly to end in a racemose group of pre-auricular cysts. Its origin can be traced to imperfect fusion of the six tubercles that form the pinna. Symptoms occur after an episode of infection. Recurrent infection may lead to development of a pre-auricular ulcer. Complete excision of the sinus tract and its associated cyst down to the temporalis fascia effect a cure. Surgery should be avoided during incipient infection. Excision under general anesthesia with the use of an extended incision with removal of all epithelial component down to the temporalis fascia will be usually needed. Massaging the sinus may avoid longterm occlusion of its lumen with its attendant possibility of infection.

1- Hornibrook J, Robertson MS: The management of preauricular sinus. N Z Med J 11;97(747):18-9, 1984
2- Chami RG, Apesos J: Treatment of asymptomatic preauricular sinuses: challenging conventional wisdom. Ann Plast Surg 23(5):406-11, 1989
3- Joseph VT, Jacobsen AS: Single stage excision of preauricular sinus. Aust N Z J Surg 65(4):254-6, 1995
4- Currie AR, King WW, Vlantis AC, Li AK: Pitfalls in the management of preauricular sinuses. Br J Surg 83(12):1722-4, 1996


June 1997 marked the long awaited creation of the Puerto Rico Association of Pediatric Surgeons (PRAPS). ‘Pediatric Surgery Update' becomes the official publication of PRAPS.

Vol 09 No 5 NOVEMBER 1997

Primary Hyperparathyroidism

Primary Hyperparathyroidism (HPT) considered once a rare disease has an incidence of 0.01% to 0.02% since the availability of immunocytochemical screening tests. HPT is more prevalent in females, with a ratio of 3:2. Children are rarely afflicted by this condition. Dehydration and respiratory distress result in higher mortality rates in neonatal and infantile HPT. Hyperplastic parathyroid tissue is the most prevalent histopathological finding during this developmental stage. After the age of one, adenomas, a collection of chief cells surrounded by a rim of normal tissue at the outer perimeter of the gland, are more commonly found. Nonspecific and diffuse symptoms such as fatigue, nausea, vomiting, constipation, weakness, dizziness, weight loss, failure to thrive, and personality changes are frequently found on initial presentation of the illness. Skeletal lesions are often instrumental in the pediatric setting, with an incidence of 44% in children. Other complications less common in children are renal calculi, pancreatitis, neuromuscular dysfunction, pseudogout and anemia. A diagnosis should be suspected by elevated calcium levels and diminished phosphorus levels in blood chemistry studies. Other studies such as ultrasonography and isotope scans are helpful tools in diagnosing the condition. Management is surgical. Indications for surgery are the presence of complications (such as pancreatitis, renal calculi, etc.), life-threatening hypercalcemia, hypercalciuria,  sex, and a young patient.
1 - Cope, Oliver: The story of primary hyperparathyroidism at the Massachusetts General Hospital. New England Journal of Medicine  66: 1174-82, 1966
2 - Bilezikien, John P: Primary Hyperparathyroidism. Primer on the Metabolic Bone Disease and Disorders of Mineral Metabolism 2nd edition. Lippincott-Raven pag. 155-58.
3 - Bjernulf A, Hall K, Sjogren I, et al: Primary hyperparathyroidism in children. Acta Paediatr Scand 59:249-58, 1970.
4 - Allo M, Thompson NW, Harness JK, et al: Primary hyperparathyroidism in children, adolescents and young adults. World Journal of Surgery. 6 :771-5, 1982
5 - Huang, et al :Primary Hyperparathyroidism in children : Report of a case and a brief review of the literature. Journal of the Formosan Medical Association 92:1095-8, 1993

Ewing's Sarcoma

Ewing's sarcoma is the second most common primary malignant bone tumor of children. Comprise 25% of primary bone tumors. This small round blue cell neoplasm most commonly arises from the diaphysis of long bones (femur, humerus, tibia and fibula). Current thinking links Ewing's sarcoma and primitive neuroectodermal tumor by a shared chromosomal translocation  (11;22)(q24;q12)  and by evidence of neuroectodermal origin. Other less common sites of development are pelvis, ribs, jaw and vertebra. Soft tissue extension is common and extensive. Extraosseous origin has been reported infrequently. The tumor is clinically characterized by pain, tenderness and motion limitless. Pathologic fractures may be present. Constitutional symptoms such as weight loss, malaise, fever and chills may also be present. Ewing's is a destructive lesion of bone associated with periosteal reaction. Findings in simple films include onion-skin formation, endosteal buttressing and formation of Codman's triangle. Only a high quality biopsy can determine histological diagnosis. Multiple agent chemotherapy is essential for long-term survival. Multi agent protocols are superior to singleagent therapies. Radiation therapy and surgery are employed for local control of primary tumors. Disease-free survival rate is now between 60 and 70%. Children with metastatic disease and pelvic primary have a worse prognosis.
1 - Jurgens HF:  Ewing's sarcoma and peripheral primitive neuroectodermal tumor. Curr Opin Oncol 6(4):391-6, 1994
2 - Vlasak R, Sim FH: Ewing's sarcoma. Orthop Clin North Am 27(3):591-603, 1996
3 - Christie DR, Bilous AM, Carr PJ: Diagnostic difficulties in extraosseous Ewing's sarcoma: a proposal for diagnostic criteria. Australas Radiol 41(1):22-8, 1997
4 - Meyers PA: Malignant bone tumors in children: Ewing's sarcoma. Hematol Oncol Clin North Am 1(4):667-73, 1987
5 - Horowitz ME: Ewing's sarcoma: current status of diagnosis and treatment. Oncology (Huntingt) 3(3):101-6; discussion 106-9, 1989
6 - Hays DM: Pediatric Surgical Oncology. Grune & Stratton Pub. 1986 pag 245-248.
7 - Granowetter L: Ewing's sarcoma. Curr Opin Oncol 3(4):684-8, 1991
8 - Gentet JC,  Panuel M,  Sheiner C,  Coze C,  Capodano AM,  Bollini G,  Bernard JL, Raybaud C: [Ewing's tumor: current knowledge and --ignorance]. Pediatrie (Bucur) 47(12):799-807, 1992


This  argentaffin cell tumor causes interest because of its diverse presentation, hormonal secretion, and malignant potential. The carcinoid is the most common neoplasm of the GI tract in childhood and may occur at any site along the alimentary tract. Above the diaphragm is commonly identified in the bronchus, and below the diaphragm in the appendix. Female predominates, the tumor is seldom life-threatening, and children rarely develop hormonal hypersecretion of 5-hydroxy indole acetic acid (Carcinoid syndrome). Carcinoids are usually discovered as an incidental finding during surgery done for other reasons. The appendix tumor arise from subepithelial endocrine cells with exclusive growth in the lamina propria beneath the epithelial crypts. Most tumors are found in the tip of the appendix. Simple appendectomy is curative in most cases. Tumors larger than 2 cm invading neighboring structures may need right hemicolectomy. Long term follow-up is imperative.
1 - Moartel CL, Weiland LH, Telander RL: Carcinoid Tumor of the Appendix in the First Two Decades of Life. J Pediatr Surg 25 (10): 1073-1075, 1990
2 - Olney JR, Urdaneta LF, Al-Jurf AS, Jochimsen PR, Shirazi SS: Carcinoid tumors of the gastrointestinal tract. Am Surg 51(1):37-41, 1985
3 - Moertel CG, Weiland LH, Nagorney DM, Dockerty MB: Carcinoid tumor of the appendix: treatment and prognosis. N Engl J Med 31;317(27):1699-701, 1987
4 - Jonsson T, Johannsson JH, Hallgrimsson JG: Carcinoid tumors of the appendix in children younger than 16 years. A retrospective clinical and pathologic study. Acta Chir Scand 155(2):113-6, 1989
5 - Parkes SE, Muir KR, al Sheyyab M, Cameron AH, Pincott JR, Raafat F, Mann JR: Carcinoid tumours of the appendix in children 1957-1986: incidence, treatment and outcome [see comments] Br J Surg 80(4):502-4, 1993
6 - Corpron CA, Black CT, Herzog CE,  Sellin RV, Lally KP,  Andrassy RJ: A half century of experience with carcinoid tumors in children. Am J Surg 170(6):606-8, 1995

Vol 09 No 6 DECEMBER 1997

Gastric Volvulus

Gastric volvulus (GV) is a rare cause of regurgitation, retching or vomiting in children produced by an abnormal rotation of one part of the stomach around another. The volvulus can be either  organoaxial if it develops along a line joining the hiatus and pylorus, mesenteroaxial if it occurs along the greater and lesser curvature axis (most common form seen in children), or a combination of both. GV may be idiopathic or secondary to other disorder and may be acute or chronic in nature. Most GV are secondary to deficient fixation of the gastric ligaments,  acute in nature and associated to another underlying disorder, most commonly a diaphragmatic defect (eventration or hiatal hernias). Other associations include asplenia, pre-duodenal portal vein and previous Nissen fundoplication. The main symptoms of secondary GV are vomiting and respiratory difficulty whereas those of idiopathic GV are abdominal distension, pain and weight loss with or without failure to thrive. Borchardt's triad (unproductive vomiting, epigastric distension, and inability to pass NG tube) is not always present in the pediatric patients. The diagnosis is suspected with plain abdominal films (large air-fluid level) and confirmed with upper contrast GI studies. A nasogastric tube will often relieve the acute situation. Surgical therapy is mandatory for the simultaneous correction of both the anatomical defects of gastric fixation and malposition and the diaphragmatic problem. Through an abdominal approach the stomach is decompressed, the volvulus is untwisted, the associated anomaly and cruras are repaired, and gastric fixation (gastropexy) is done. Anterior gastropexy in at least two places will prevent recurrent volvulus;  gastrostomy alone may be followed by recurrence. Laparoscopy can accomplish this task.

1- Youssef SA,  Di Lorenzo M,  Yazbeck S,  Ducharme JC: [Gastric volvulus in children] Chir Pediatr 28(1):39-42, 1987
2- Cameron AE, Howard ER: Gastric volvulus in childhood.  J Pediatr Surg 22(10):944-7, 1987
3- Honna T, Kamii Y, Tsuchida Y: Idiopathic gastric volvulus in infancy and childhood.  J Pediatr Surg 25(7):707-10, 1990
4- Fung KP, Rubin S, Scott RB: Gastric volvulus complicating Nissen fundoplication. J Pediatr Surg 25(12):1242-3, 1990
5- Alawadhi A, Chou S, Soucy P: Gastric volvulus--a late complication of gastrostomy. Can J Surg 34(5):485-6, 1991
6-  Miller DL, Pasquale MD, Seneca RP, Hodin E: Gastric volvulus in the pediatric population. Arch Surg 126(9):1146-9, 1991
7- Cameron BH, Blair GK: Laparoscopic-guided gastropexy for intermittent gastric volvulus. J Pediatr Surg 28(12):1628-9, 1993
8- McIntyre RC Jr, Bensard DD, Karrer FM, Hall RJ, Lilly JR: The pediatric diaphragm in acute gastric volvulus.  J Am Coll Surg 178(3):234-8, 1994
9- Andiran F, Tanyel FC, Balkanci F, Hicsonmez A: Acute abdomen due to gastric volvulus: diagnostic value of a single plain radiograph. Pediatr Radiol 25 Suppl 1:S240, 1995
10- Georgacopulo P, Pavanello P, Shweiki F, Guerra D: [Gastric volvulus in childhood] Minerva Pediatr 48(6):275-8, 1996
11- Leitao B, Mota CR, Enes C, Ferreira P, Vieira P, Requeijo D: Acute gastric volvulus and congenital posterolateral diaphragmatic hernia. Eur J Pediatr Surg 7(2):106-8, 1997
12- Other Conditions of the Upper Gastrointestinal Tract, In Marc Rowe ‘Essentials of Pediatric Surgery', Mosby Publisher 1995, pag. 504-505
13- Cameron BH, Vajarvandi V, Blair GK et al.  The intermittent and variable features of gastric volvulus in childhood.  Pediatr Surg Int 10:26-29, 1995


Giant Cystic Meconium Peritonitis

Giant Cystic Meconium Peritonitis (GCMP) is an unusual form of intrauterine bowel perforation. The bowel perforation persists after birth and the peritoneal cavity becomes transformed into a huge meconium-filled giant pseudocyst with a thick membrane lining. The correct diagnosis can be reached either prenatally or soon after birth with the help of Ultrasonography or simple abdominal films respectively. Characteristically, a large cyst with dense calcifications or eggshell appearance is diagnostic. Intrabdominal calcifications once thought pathognomonic of meconium peritonitis can also be seen in other conditions such as hamartomas, adrenal hemorrhage or hemangiomas. Clinically the baby shows severe abdominal distension, bilious vomiting and failure to pass meconium. Delay in diagnosis may colonize and infect the pseudocyst creating a septic atmosphere. Associated cystic fibrosis should always be thought. The etiology of GCMP might be an intrauterine bowel atresia perforation, volvulus, intussusception or vascular accident. Remaining bowel is short. Management consists of decortication or removal of the cyst membrane with re-anastomosis of the GI tract if the surrounding inflammation permits. Other times temporary enterostomy may be necessary. The prognosis once dismal has improved in the last twenty years.

1- Kolawole TM, Bankole MA, Olurin EO, Familusi JB: Meconium peritonitis presenting as giant cysts in neonates. Br J Radiol 46(551):964-7, 1973
2- Careskey JM, Grosfeld JL,  Weber TR, Malangoni MA: Giant cystic meconium peritonitis (GCMP): improved management based on clinical and laboratory observations.  J Pediatr Surg 17(5):482-9, 1982
3- Marchildon MB: Meconium Peritonitis and Spontaneous Gastric Perforation. Clinics in Perinatology. 5(1): 79, 1976
4- Chitnis MR,  Natarajan VM, Phadke DM, Golhar KB: Giant cystic meconium peritonitis. Indian J Pediatr 58(4):554-5, 1991
5- Neuhauser EBD: The roentgen diagnosis of fetal meconium peritonitis. AJR 51: 421-424, 1944


Congenital Mesoblastic Nephroma

Congenital Mesoblastic Nephroma (CMN), a fetal renal hamartoma, is the most common renal tumor in neonates. Usually presenting as an asymptomatic abdominal mass occupying most of the involved kidney, CMN is characterized by benign clinical behavior, gross appearance similar to uterine fibroids, and excellent prognosis after removal. Prenatally diagnosed CMN may be associated with polyhydramnios, low birth weight and premature labor. Large tumors might benefit from cesarean section. CT Scan shows a solid neoplasm with intrarenal distortion of the collecting system. Microscopically, typical CMN is composed of sheets of spindleshaped cells. Atypical CMN (cellular variant) is a potentially more aggressive tumor composed mainly of primitive mesenchymal cells, but also usually contains varying numbers of differentiating fibroblasts and myofibroblasts. Management of CMN consists of simple nephrectomy with lymph node sampling to detect rare cases simulating malignancy.

1-  Snyder HM 3d, Lack EE, Chetty-Baktavizian A, Bauer SB, Colodny AH, Retik AB: Congenital mesoblastic nephroma: relationship to other renal tumors of infancy.  J Urol 126(4):513-6, 1981
2- Gerber A, Gold JH, Bustamante S, Lorch V, Mirza M: Congenital mesoblastic nephroma. J Pediatr Surg 16(5):758-9, 1981
3- Chan HS, Cheng MY, Mancer K, Payton D, Weitzman SS, Kotecha P, Daneman A:  Congenital mesoblastic nephroma: a clinicoradiologic study of 17 cases  representing the pathologic spectrum of the disease.  J Pediatr 111(1):64-70, 1987
4- Matsumura M, Nishi T, Sasaki Y, Yamada R, Yamamoto H, Ohhama Y, Tanaka Y, Kurosu F, Amano K: Prenatal diagnosis and treatment strategy for congenital mesoblastic nephroma. J Pediatr Surg 28(12):1607-9, 1993

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