VOLUME 08, 1997

VOL 08 NO 01 JANUARY 1997

Sacrococcygeal Dimples

Many newborns are referred to pediatric surgeons because a small pit, hole or sinus is identified over the skin of the sacrococcygeal region. Those located in the coccygeal area (below the intergluteal crease) are usually shallow blind ending dimples with no significance and disappear with growth of the child. Some are deep enough (1 cm) and feces may be caught in them giving the false impression that a fistulous tract is present. On few occasions the contamination may lead to a local infectious process with suppuration. If this situation arises, excision of the dimple is curative. This dimples should not be confused with the more serious situation of a sacral sinus tract (dimple above the intergluteal fold) sometimes associated with an underlying spina bifida occulta. This tract may communicate with the spinal cord and be a source of recurrent episodes of meningitis. An MRI study of the sacral area may help delineate the tract from the skin to the spinal cord structures. Excision at the time of diagnosis should be done by a physician with sound knowledge of neuroanatomy. Dermal sinuses and inclusion tumors may lead to spinal cord tethering and progressive neurologic deterioration.

1- Jones PG, Woodward AA, editors: Clinical Paediatric Surgery: Diagnosis and Management Blackwell Scientific Publications, Third Edition, 1986, Chapter 37 Pag 321-328
2- Kanev PM; Park TS:  Dermoids and dermal sinus tracts of the spine. Neurosurg Clin N Am 6(2):359-66, 1995


Sigmoid Volvulus

Sigmoid volvulus is a rare cause of mechanical intestinal obstruction in the pediatric age group. Of all colonic volvulus (cecum, transverse, and sigmoid) this is the most common (~80%). Many reports come from African children. Predisposing conditions are: irregular bowel behavior, consumption of high fiber diet, Hirschsprung's disease, chronic constipation, abnormally long sigmoid flexure, a/o absence of mesosigmoid. The condition arises after clockwise rotation of a redundant sigmoid with a fixed point. Clinically the child presents with abdominal pain of sudden onset over the left lower quadrant, vomiting and obstipation. There may be tenderness, distension, and a palpable mass. Plain abdominal films (classic omega sign) may not yield a diagnosis. Contrast study of the colon (cysto-conray enema) may be diagnostic and therapeutic. Sigmoidoscopy (with tube decompression) can achieve derotation of the bowel. Emergency surgery is needed when there's evidence of strangulation or inability to derotate the volvulus. Sigmoid resection is definitive treatment for children, but nonoperative decompression to allow for elective resection should be attempted in patients with no evidence of peritonitis. The mortality in the acute setting is significant in poor risk patients, the very young, and patients with associated anomalies.

1- Mellor MF, Drake DG: Colonic volvulus in children: value of barium enema for diagnosis and treatment in 14 children.  AJR Am J Roentgenol 162(5):1157-9, 1994
2- Ofiaeli RO: Volvulus of the sigmoid colon in paediatric patients: report of two cases. Cent Afr J Med 38(4):169-71, 1992
3- Smith SD, Golladay ES, Wagner C, Seibert JJ: Sigmoid Volvulus in Children.  South Med J 83(7):778-81, 1990
4- De Castro R, Casolari E, Caal JA, Rossi F, Federici S: Sigmoid volvulus in children: a case report. Z Kinderchir 41(2):119-21, 1986
5- Neilson IR, Youssef S: Delayed presentation of Hirschsprung's disease: acute obstruction secondary to megacolon with transverse colonic volvulus. J Pediatr Surg 25(11):1177-9, 1990
6- McCalla TH; Arensman RM; Falterman KW:  Sigmoid volvulus in children. Am Surg  51(9):514-9, 1985
7- Seger DL, Middleton D:  Childhood sigmoid volvulus. Ann Emerg Med 13(2):133-5, 1984
8- Valla JS, Louis D, Berard J, Jaubert M, de Beaujeu MJ:  Sigmoid volvulus in children. About 6 cases (author's transl)] Chir Pediatr 23(2):93-6, 1982
9-  Taneja SB, Kakar A, Ayyar RD: Sigmoidal volvulus in childhood: report of two cases. Dis Colon Rectum 20(1):62-7, 1977
10- Campbell JR, Blank E: Sigmoid volvulus in children. Pediatrics 53(5):702-5, 1974


Omphalopagus are twins joined through their abdomen and usually sharing liver (hepatic bridge), biliary tree, gastrointestinal, and genitourinary tracts. Conjoined twins occur in one of every 50,000 births. Females predominate and most (two-third) will not survive. Commonly type of conjoined twins reported are in order of frequency: thoraco-omphalopagus, thoracopagus, omphalopagus, parasitic twins and craniopagus. A high incidence of births defects not linked to conjoining (cardiac, neural tube, and orofascial cleft defects) explains the high rate of late mortality in those that survive early separation. Early prenatal diagnosis and assessment of the degree of conjoining provide the couple the option of pregnancy termination, or the physician use of cesarean section to improve survival. Separation is best delayed until the twins are relatively mature and developed (more than six months of age). Emergency separation has been needed with one twin stillborn, with gastroschisis or after development of enterocolitis. Although multiple imaging studies will be required to determine the extent of anatomical union, MRI gives information on hepato-biliary and cardiovascular structures that enable planning a safe separation. Doppler echo can also adequately document the heart status noninvasively avoiding the need for cardiac catheterization.

1- Kenigsberg K,  Harper RG: Separation of omphalopagus twins. J Pediatr Surg 17(3):255-8, 1982
2- Edmonds LD,  Layde PM: Conjoined twins in the united states, 1970-1977. Teratology 25(3):301-8, 1982
3-Lobe TE, Oldham KT, Richardson CJ: Successful separation of a conjoined biliary tract in a set of omphalopagus twins. J Pediatr Surg 24(9):930-2, 1989
4- Richardson RJ, Applebaum H, Taber P, Woolley MM, Chwals WJ, Warden MJ, Dietrich R: Use of magnetic resonance imaging in planning the separation of omphalopagus conjoined twins. J Pediatr Surg 24(7):683-4, 1989; discussion 684-5
5- Castilla EE, Lopez-Camelo JS, Orioli IM, Sanchez O, Paz JE: The epidemiology of conjoined twins in Latin America. Acta Genet Med Gemellol (Roma) 37(2):111-8, 1988
6- O'Neill JA Jr, Holcomb GW 3d, Schnaufer L, Templeton JM Jr, Bishop HC, Ross AJ 3d, Duckett JW, Norwood WI, Ziegler MM, Koop CE: Surgical experience with thirteen conjoined twins. Ann Surg 208(3):299-312, 1988
7- Rejjal AL, Nazer HM, Abu-Osba YK, Rifai A, Ahmed S: Conjoined twins: medical, surgical and ethical challenges. Aust N Z J Surg 62(4):287-91, 1992
8- Barth RA, Filly RA, Goldberg JD, Moore P, Silverman NH: Conjoined twins: prenatal diagnosis and assessment of associated malformations [published erratum appears in Radiology 1991  Jan;178(1):287] Radiology 177(1):201-7, 1990
9-Dev V, Pothineni RB, Rohatgi M, Shrivastava S: Echo-Doppler assessment of cardiac status in conjoined (thoraco-omphalopagus) twins. Pediatr Cardiol 11(2):91-2, 1990

VOL 08 NO 02 FEBRUARY 1997

Ovarian Tumors

Ovarian tumors are uncommon childhood malignancies (1%) characterized by recurrence and resistance to therapy. Aggressive surgery is limited to avoid compromising reproductive capacity and endocrine function. Low incidence and need of mulitinodal therapy encourages referral to centers dealing with effective cancer therapy.The most common histology is germ cell: dysgerminoma, teratoma, and endodermal sinus tumor. This is followed by the sex-cord stroma tumors with a low incidence of malignancy. They can cause feminization (granulosa-theca cell) and masculinization (androblastoma). Other types are: epithelial (older adolescent), lipid-cell, and gonadoblastoma. Ovarian tumors present with acute abdominal symptoms (pain) from impending rupture or torsion. They also cause painless abdominal enlargement, or hormonal changes. Preop work-up should include: human chorionic gonadotropin (HCG) and alpha-fetoprotein ( AFP) levels. Imaging studies: Ultrasound and CT-Scan. The most important prognostic factor in malignant tumors is stage of disease at time of diagnosis. Objectives of surgery are: accurate staging (inspection of peritoneal surfaces and pelvic organs, lymph node evaluation), washing and cytology of peritoneal fluid, tumor removal, and contralateral ovarian biopsy if needed. Chemotherapy consists of: bleomycin, cis-platinum, and vinblastine. Radiotherapy is generally not effective, except in dysgerminoma. Elevation of tumor markers (AFP or HCG) after therapy signals recurrence.

1- Adkins J: Malignant Germ-Cell and Ovarian Tumors, In D.M. Hays ‘Pediatric Surgical Oncology, Grune & Straton Ed, 1986, pags. 123-138
2- Ovarian Tumors in Children and Adolescents, In Huffman's ‘The Gynecology of Childhood and Adolescence. WB Saunders Inc, 2nd ed, 1981, pags. 277-349
3- Tumors of the Sexual Organs, In Altman & Schwartz's ‘Malignant Diseases of Infancy, Childhood and Adolescence'. WB Saunder Inc, 2nd ed, 1983, pags. 484-509
4- Germ Cells Tumors, In Hart Isaacs Jr's ‘Tumors of theNewborn and Infant'. Mosby Year Book Ed, 1981, pags.43-67

Barrett's Esophagus

Barrett's esophagus (BE) refers to replacement of the normal epithelium of the distal 2 to 3 cm of esophagus with metaplastic columnar epithelium containing globet cells. BE is rare (prevalence is increasing) in children. Evolves as a consequence of chronic GE reflux carrying an approximate 40-fold increase in development of malignancy in adult life. Three types of histological epithelium are described in BE: specialized columnar (intestinal metaplasia), junctionaltype (containing mucous glands), and gastric fundustype (containing chief and parietal cells) epithelium. Diagnosis of BE depends on screening endoscopic biopsies in children with reflux before and after treatment. Consequence of reflux in BE are: development of a stricture (junction between metaplastic lining and squamous epithelium), a penetrating ulcer, bleeding, dysplasia or carcinoma-in-situ. Risk factors associated to the development of carcinoma are: length of disease, male sex, smoking history, and intestinal epithelium. Cohorts of children with an increased incidence of BE: mentally retarded, on chemotherapy, cystic fibrosis, after repair of esophageal atresia, and esophageal substitution. Asymptomatic BE children should be managed with acid suppressing medical therapy (omeprazole). Fundoplication should be offered to children with BE based on complications of GE reflux, failed medical therapy, or evidence of alkaline reflux induced BE. Successful antireflux surgery is not followed by regression of the metaplastic mucosa in BE, but can arrest the cephalad progression. Long-term endoscopic surveillance is needed to detect cases of dysplasia or carcinoma before transmural infiltration occurs.

1- Haggitt RC: Barrett's esophagus, dysplasia, and adenocarcinoma. Hum Pathol 25(10):982-93, 1994
2- McDonald ML, Trastek VF, Allen MS, Deschamps C, Pairolero PC, Pairolero PC:  Barretts's esophagus: does an antireflux procedure reduce the need for endoscopic surveillance?  J Thorac Cardiovasc Surg 111(6):1135-8, 1996; discussion 1139-40
3- Bernstein IT, Kruse P, Andersen IB:  Barrett's oesophagus. Dig Dis 12(2):98-105, 1994
4- Hassall E, Israel DM, Davidson AG, Wong LT: Barrett's esophagus in children with cystic fibrosis: not a coincidental association. Am J Gastroenterol 88(11):1934-8, 1993
5- Eizaguirre I, Tovar JA, Gorostiaga L, Echeverry J, Torrado J:  [Barrett++ esophagus in children. Presentation of 12 cases] Cir Pediatr 6(2):66-8, 1993
6- Menke-Pluymers MB, Hop WC, Dees J, van Blankenstein M, Tilanus HW:  Risk factors for the development of an adenocarcinoma in columnar-lined (Barrett) esophagus. The Rotterdam Esophageal Tumor Study Group. Cancer 15;72(4):1155-8, 1993
7- Hassall E: Barrett's esophagus: new definitions and approaches in children. J Pediatr Gastroenterol Nutr 16(4):345-64, 1993
8- Hassall E: Barrett's esophagus: congenital or acquired? Am J Gastroenterol 88(6):819-24, 1993
9- Hassall E, Dimmick JE, Magee JF:  Adenocarcinoma in childhood Barrett's esophagus: case documentation and the need for surveillance in children. Am J Gastroenterol 88(2):282-8, 1993
10- Hassall E, Weinstein WM: Partial regression of childhood Barrett's esophagus after fundoplication.Am J Gastroenterol 87(10):1506-12, 1992
11- Iftikhar SY, James PD, Steele RJ, Hardcastle JD, Atkinson M:  Length of Barrett's oesophagus: an important factor in the development of dysplasia and adenocarcinoma [see comments]Gut 33(9):1155-8, 1992
12-Qualman SJ, Murray RD, McClung HJ, Lucas J:  Intestinal metaplasia is age related in Barrett's esophagus. Arch Pathol Lab Med 114(12):1236-40, 1990
13- Snyder JD, Goldman H:  Barrett's esophagus in children and young adults. Frequent association with mental retardation [see comments] Dig Dis Sci 35(10):1185-9, 1990
14- Cooper JE, Spitz L, Wilkins BM:  Barrett's esophagus in children: a histologic and histochemical study of 11 cases.  J Pediatr Surg 22(3):191-6, 1987
15- Hassall E, Weinstein WM, Ament ME:  Barrett's esophagus in childhood. Gastroenterology 89(6):1331-7, 1985
16- Cheu H, Grosfeld JL, Heofetz SA, et al: Persistent of Barrett's Esophagus in Children After Antireflux Surgery: Influence on Follow-up Care.  J Pediatr Surg 27(2): 260-266, 1992
17-Biehmann Othersen B, Ocampo RJ, Parker EF, et al: Barrett's Esophagus in Children: Diagnosis and Management. Ann Surg 217(6): 676-681, 1993
18- Bar-Maor JA, He YR, Li d: Barret's Epithelium with complete Stricture of the Esophagus: Hypothesis of its Origin. J Pediatr Surg 30(6): 893-895, 1995

Juvenile Polyps

Childhood polyps are usually juvenile (80%). Histology features a cluster of mucoid lobes surrounded by flattened mucussecreting glandular cells (mucous retention polyp), no malignant potential. Commonly seen in children age 310 with a peak at age 56. As a rule only one polyp is present, but occasionally there are two or three almost always confined to the rectal area (within the reach of the finger). Most common complaint is bright painless rectal bleeding. Occasionally the polyp may prolapse through the rectum. Diagnosis is by barium enema, rectal exam, or endoscopy. Removal by endoscopy is the treatment of choice. Rarely colotomy and excision are required.

1- Lehmann CU;  Elitsur Y: Juvenile polyps and their distribution in pediatric patients with gastrointestinal bleeding. W V Med J 92(3):133-5, 1996
2- Mestre JR:  The changing pattern of juvenile polyps. Am J Gastroenterol 81(5):312-4, 1986
3- Scott-Conner CE, Hausmann M, Hall TJ, Skelton DS, Anglin BL, Subramony C: Familial juvenile polyposis: patterns of recurrence and implications for surgical management. J Am Coll Surg 181(5):407-13, 1995
4- Ko FY, Wu TC, Hwang B: Intestinal polyps in children and adolescents--a review of 103 cases. Acta Paediatr Sin 36(3):197-202, 1995
5- Desai DC, Neale KF, Talbot IC, Hodgson SV, Phillips RK:  Juvenile polyposis. Br J Surg 82(1):14-7, 1995

Vol 08 No 03 MARCH 1997

Multiple Endocrine Neoplasia

Multiple endocrine neoplasia (MEN) refers to a familial (autosomic dominant) disorders involving several endocrine glands with hyperplasia or tumor. Two patterns are recognized: MEN 1 which comprises tumors of the pituitary, parathyroid, and pancretic islets and MEN 2 which occurs in two forms: MEN 2A includes medullary carcinoma of the thyroid (MCT), pheochromocytoma and hyperparathyroidism, and MEN 2B with MCT, pheochromocytoma, a marfanoid habitus and neuroma phenotype. The most constant (100%) and life threatening feature of MEN 2 is MCT (usually multicentric and bilateral). Calcitonin is secreted by C cells in abnormally increase amount both in C-cell hyperplasia (precursor of MCT) and MCT. The diagnosis of MCT relies on chemical elevation of basal or stimulated (pentagastrin) calcitonin levels. The MEN 2B gene mutation (exon 16) has been located to a region of chromosome 10 that contains the ret proto-oncogene (same region for the genes for MEN 2A and familial MCT). Direct DNA testing has established the place of prophylactic surgical therapy in this familial cancer syndrome before the development of biochemical or clinical disease. Management for MCT should include total thyroidectomy, and excision of suspicious lymph nodes in the central and lateral compartments of the neck. Thyroidectomy done for elevated chemical marker has a higher rate of curability than when the diagnosis is made clinically (palpable node). After thyroidectomy the child should be yearly followed monitoring plasma levels of calcitonin and carcinoembryonic antigen to detect tumor recurrence, and cathecolamines assays for pheochromocytoma.

1- Telander RL, Zimmerman D, van Heerden JA, et al: Results of Early Thyroidectomy for Medullary Thyroid Carcinoma with Multiple Endocrine Neoplasia Type 2. J Pediatr Surg 21 (12): 1190-1194, 1986
2- Girvan DP, Holliday RL: Pediatric Implications of Multiple Endocrine Neoplasia. J Pediatr Surg  22(9): 806-808, 1987
3- Mahaffey SM, Martin LW, McAdams J, et al: Multiple Endocrine Neoplasia Type IIB with Symptoms suggesting Hirschsrpung's Disease: A case report. J Pediatri Surg 25(1): 101-103, 1990
4- Decker RA, Toyama WM, O'Neill LW, et al: Evaluation of Children with Multiple Endocrine Neoplasia Type IIB Following Thyroidectomy. J Pediatr Surg 25(9): 939-943, 1990
5- O'Riordain DS, O'Brien T, Grant CS, et al: Surgical management of primary hyperparathyroidism in multiple endocrine neoplasia types 1 and 2. Surgery 114(6): 1031-1039, 1993
6- Telander R, Moir CR: Medullary Thyroid Carcinoma in Children. J Pediatr Surg 29(9): 188-193, 1994
7- O'Riordain DS, O'Brien T, Crotty TB, et al: Multiple endocrine neoplasia type 2B: More than an endocrine disorder. Surgery 118(6): 936-943, 1995
8- Pacini FP, Romei C, Miccoli P, et al: Early treatment of hereditary medullary thyroid carcinoma after attribution of multiple endocrine neoplasia type 2 gene carrier status by screening for ret gene mutations. Surgery 118(6): 1031-1036, 1995
9- Frilling A, Dralle H, Eng C, et al: Presymptomatic DNA screening in familias with multiple endocrine neoplasia type 2 and familial medullary thyroid carcinoma. Surgery 118(6): 1099-1104, 1995
9A- Wells S, Chi DD, Toshima K, et al: Predictive DNA Testing and Prophylactic Thyroidectomy in Patients at Risk for Multiple Endocrine Neoplasia Type 2A. Ann Surg 220(3): 237-250, 1994
10- Skinner MA, DeBenedetti MK, Moley JF, et al: Medullary Thyroid Carcinoma in Children with Multiple Endocrine Neoplasia Types 2A and 2B. J Pediatr Surg 31(1): 177-182, 1996
11- Shimotake T, Iwai N, Inoue K, et al: 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): 779-781, 1996


Neonatal Testicular Torsion

Neonatal testicular torsion (NTT) is a rare condition occurring most commonly during the neonatal period or before birth. The baby presents with a red, swollen scrotum the product of an extra-vaginal torsion of the spermatic cord. The diagnosis (and exclusion of other pathological conditions) is done using color Doppler ultrasound examination revealing lack of intra testicular blood flow on the affected side and normal flow within the contralateral testis (unless the condition arises bilaterally). It has been suggested that the cause of testicular regression syndrome is antenatal torsion of the testis, others belief in vascular injury secondary to birth trauma as the most likely cause. Treatment requires surgical exploration. Controversy exists concerning the timing of exploration as well as the need for contralateral orchiopexy, since some reports suggest that the contralateral testicle is not a risk for torsion. Therapy of the ipsilateral testicle is determined by operative findings. While testicular salvage is nil (subsequent atrophy is the rule), surgical intervention is necessary for any hope of testicular preservation.

1- Stone KT, Kass EJ, Cacciarelli AA, Gibson DP: Management of suspected antenatal torsion: what is the best strategy? J Urol 153(3 Pt 1):782-4, 1995
2- Sutcliffe JR, Wilson-Storey D, Smith NM:  Ante-natal testicular torsion: only one cause of the testicular regression syndrome? J R Coll Surg Edinb 41(2):99-101, 1996
3- Giannakopoulos X, Zikopoulos C, Ntourntoufi A, Andronikou S:  Intrauterine unilateral torsion of the spermatic cord. Minerva Urol Nefrol 47(2):95-6, 1995
4- Penson DF, Aronson WJ:  Segmental testicular infarction in the neonate: a case report. J Urol 153(6):1992-3, 1995
5- Cartwright PC, Snow BW, Reid BS, Shultz PK: Color Doppler ultrasound in newborn testis torsion. Urology 45(4):667-70, 1995
6- Stone KT, Kass EJ, Cacciarelli AA, Gibson DP: Management of suspected antenatal torsion: what is the best strategy?  J Urol 153(3 Pt 1):782-4, 1995
7- Tryfonas G, Violaki A, Tsikopoulos G, Avtzoglou P, Zioutis J, Limas C, Gregoriadis G, Badouraki M:  Late postoperative results in males treated for testicular torsion during childhood. J Pediatr Surg 29(4):553-6, 1994
8- Cilento BG, Najjar SS, Atala A: Cryptorchidism and testicular torsion. Pediatr Clin North Am 40(6):1133-49, 1993
9- Brandt MT, Sheldon CA, Wacksman J, Matthews P: Prenatal testicular torsion: principles of management. J Urol 147(3):670-2, 1992
10- Weingarten JL, Garofalo FA, Cromie WJ: Bilateral synchronous neonatal torsion of spermatic cord. Urology 35(2):135-6, 1990
11- LaQuaglia MP, Bauer SB, Eraklis A, Feins N, Mandell J: Bilateral neonatal torsion.  J Urol 138(4 Pt 2):1051-4, 1987
12- Burge DM: Neonatal testicular torsion and infarction: aetiology and management. Br J Urol 59(1):70-3, 1987

Intestinal Leiomyosarcomas

Intestinal leiomyosarcomas are very rare gastrointestinal tract (smooth muscle cells) sarcomas in children. Most cases have been diagnosed during the first decade of life, almost 50% in newborns, with a slight female predominance. Microscopic appearance consists of spindle cells with blunt-ended oval nuclei, mitotic figures, anaplasia and bizarre cell forms. Clinically they present with symptoms of intestinal obstruction (intussusception) and/or perforation, other times with abdominal pain or lower GI bleeding They are relatively small, confined and evenly distributed along jejunum, ileum and colon. Therapy consist of radical surgical resection with effort placed on removal of an adequate margin of normal tissue, even if adjacent organs are in consideration. Adjuvant chemotherapy should be considered for lesions with incomplete or questionable margins of resection. Metastasis occurs via the bloodstream and mainly to the lungs. Poor prognosis is associated to high-grade differentiation and inadequate resection, not cytometric DNA ploidy.

1- McGrath PC, Neifeld JP, Salzberg AM: Principles in the Management of Pediatric Intestinal Leiomyosarcomas. J Pediatr Surg 23 (10): 939-941, 1988
2- "The Soft Tissue Sarcomas" In Altman & Schwartz Malignant Disease of Infancy, Childhood and Adolescence. Second Ed, WB Saunders Co, 1983, pag 441
3- Chou FF, Eng HL, Sheen-Chen SM: Smooth muscle tumors of the gastrointestinal tract: Analysis of prognostic factors. Surgery 119(2); 171-177, 1996
4- Gupta AK, Berry M, Mitra DK: Gastrointestinal smooth muscle tumors in children: report of three cases. Pediatr Radiol, 24: 498-9, 1994
5- Furuta GT, Bross DA, Doody D, Kleinman RE: Intussusception and leiomyosarcoma of the gastrointestinal tract in a pediatric patient. Case report and review of  the literature. Dig Dis Sci, 38:  1933-7, 1993
6- Cummings SP, Lally KP, Pineiro-Carrero V, Beck DE: Colonic leiomyoma--an unusual cause of gastrointestinal hemorrhage in childhood. Report of a case. Dis Colon Rectum, 33: 511-4, 1990
7- Delucchi MA, Latorre JJ, Guirarldes E, et al: Intestinal Leiomyosarcoma in Childhood: Report of Two Cases. J Pediatr Surg 23(4): 377-379, 1988

Vol 8 No 04 APRIL 1997

Bone Marrow Transplant - Central Venous Catheters

Bone marrow transplant (BMT) recipients are an immunocompromised group that needs multiple venous access to meet all the fluid, antibiotic and nutritional requirements during periods of intensive supportive care. All BMT children will have central venous catheters (CVC) inserted before intensive therapy is initiated. CVC are silicone-made multiple lumen catheters (Raaf, Broviac, Hickman, Leonard) inserted in the operating room under local or general anesthesia using the subclavian, external jugular, or internal jugular veins. Main reasons to remove the CVC in BMT are: end of therapy, a complication has developed, or the child dies. Complications can be divided into infectious (local or systemic), mechanical (inability to infuse or withdraw blood, accidental dislodgement, pinch-off syndrome), technical (malfunction), or thrombotic. Infection (20-40%) is the most common reason for early catheter removal in this pancytopenic population. Not all episodes of sepsis results in CVC loss since bacteremias associated with skin flora (coagulase negative staphylococci) are successfully treated with systemic antibiotics. Infections has been associated with preparation, delivery and type of solution infused via the CVC, multiple use of CVC lumens, degree of adherence to strict protocols for IV tubing and dressing changes, properties of the catheters, and host immune status of the child. Tunnel or exit site infections will need catheter removal. Occlusive episodes can be managed with urokinase administration.

1- Moosa HH, Julian TB, Rosenfeld CS, et al: Complications of Indwelling Cantral Venous Catheter in Bone Marrow Transplant Recipients. SGO 172: 275-279, 1991
2- Crawford SW, Hickman RO, Ulz L, et al: Use of Hickman-Crwaford critical care catheter in marrow transplant recipients: A pulmonary artery cathter-adaptable central venous access. Critical Care Medicine 22 (2): 347-352, 1994
3- Andris DA, Krzywda EA, Schulte W, et al: Pinch-off Syndrome: A Rare Etiology for Central Venous Catheter Occlusion. J Parent and Enter Nutrition 18(6): 531-533, 1994
4- Gallardo D, Alonso E, Riu C, et al: Bone Marrow Transplantation Through Standard Central Venous Catheters. Transplantation Proceedings 27(4): 2354, 1995
5- Richard-Smith A, Buh S: Reducing Central Line Catheter Infections in Bone Marrow Transplant Patients. Nursing Clin North Amer 30(1): 45-52, 1995
6- Brandt B, DePalma J, Irwin M, etal: Comparison of Central Venous Catheter Dressing in Bone Marrow Transplant Recipients. ONF 23(5): 829-836, 1996


In 1882 Von Recklinghausen described neurofibromatosis (NF) as a syndrome of multiple skin hyperpigmentation (café au lait spots) associated with subcutaneous tumors of neural origin (neurofibromas) and skeletal malformations. Some lesions are evident at birth, while others are observed in later childhood. Neurofibromas are the most common nerve tissue tumors in childhood, may occur in any part of the body where nerve fibers exist, specially subcutaneous tissue, and are histologically classified as solitary, plexiform and diffuse.
Pain from pressure of a nerve in an enclosed space is the most common symptom. NF is inherited as autosomal dominant with high penetrance and wide variability in expression.
The size and number of café au lait spots tend to increase throughout puberty. NF is considered in two forms: NF I - peripheral predominance (cutaneous, visceral and skeletal, lesions can be lytic to bone, space occupying and or disfiguring), or NF II - central with CNS orbito-fascial involvement (high mortality). Complications of NF are divided into: structural (disfiguration, macrocephaly, scoliosis, congenital tibial pseudarthrosis), functional (pain, seizures, speech and intellectual deficit), and malignancy (sarcomatous degeneration).
Malignant cases are more common males than females, and seen with larger symptomatic tumors. Complete excision of these lesions is often difficult and local tumor recurrence is common. Lifelong close observation is warranted in most patients.
1- Fienman NL, Yakovac WC: Neurofibromatosis in childhood. J Pediatr 76: 339-346, 1970
2- Raffensperger JG, Cohen R: Plexiform neurofibromas in childhood. J Pediatr Surg 7: 144.151, 1972
3- Shearer P, Parham D, Kovnar E, Kun L, Rao B, Lobe T, Pratt C: Neurofibromatosis type I and malignancy: review of 32 pediatric cases treated at a single institution. Med Pediatr Oncol 22(2):78-83, 1994
4- Miscellaneous Skin Lesions Chapter 92 In Marc I. Rowe Essential of Pediatric Surgery. Mosby 1995, pag. 824-827.
5- Neurofibromatosis Syndrome in Smith's Recognizable Pattern of Human Malformations. Fourth Ed. Saunders, 1988, pag 452-453

Retroperitoneal Teratomas

Retroperitoneal teratoma is infrequent (5% of all teratomas). They are classified as mature, immature or malignant. Most patients are female, younger than six months, and asymptomatic. Clinically they present with increased abdominal girth or weight loss. An abdominal mass is commonly identified, and the presence of a tooth or a definitive bony structure in abdominal films is identified. Ultrasonography and contrast enhanced CT-Scans are of benefit in locating as well as diagnosing abdominal teratoma. Elevated alpha-feto protein level is found in malignant and some immature cases. Surgical excision is curative for the majority of cases since they are loosely attached to surrounding structures. Most children will have benign mature teratoma and show no evidence of recurrence. Retroperitoneal tumors containing high-grade immature elements should be managed with adjuvant chemotherapy.

1- Ko YS, Lin LH, Chen DF: Abdominal teratomas in children.Acta Paediatr Sin 36(5):342-5, 1995
2- Billmire DF, Grosfeld JL:Teratomas in childhood: analysis of 142 cases. J Pediatr Surg 21(6):548-51, 1986
3- Harms D, Schmidt D,  Leuschner I: Abdominal, retroperitoneal and sacrococcygeal tumours of the newborn and the very young infant. Report from the Kiel Paediatric Tumour Registry. Eur J Pediatr 148(8):720-8, 1989
4- Davidson AJ, Hartman DS,Goldman SM: Mature teratoma of the retroperitoneum: radiologic, pathologic, and clinical correlation. Radiology 172(2):421-5, 1989
5- Tortey P, Diard F, Chateil JF, Castel JC, Brichaux JC: [Retroperitoneal teratoma in children. Apropos of 2 cases]  J Radiol 69(6-7):449-54, 1988


Vol 8 No 05 MAY 1997

Diaphragmatic Eventration

Diaphragmatic eventration (DE) refers to an abnormally high position of part or all of the diaphragm usually associated with a marked decrease in muscle fibers and a membranous appearance of the abnormal area. Etiologically DE is congenital (developmental anomaly characterized by muscular aplasia), or acquired (paralysis due to phrenic nerve injury). Acquired eventration may be associated to use of forceps, breech presentation, tumors, brachial plexus injury, cephalhematoma, thoracic surgery, or clavicular fractures. Anatomically DE may be complete, partial or bilateral. Most children with DE are asymptomatic when incidentally first seen a will not need therapy. Motion of the affected diaphragm may be normal, absent, diminished or paradoxical. Those with symptoms develop acute respiratory distress, difficult feeding, and recurrent pneumonitis the result of decreased pulmonary parenchymal volume. Those whom will need assisted ventilation or cannot be weaned off the ventilator should be plicated. Most authors favor a transthoracic repair (plication) of the DE. Abdominal approach is used for bilateral cases. Plication must be done with sound knowledge of the anatomic distribution of the phrenic nerve. Failure to achieve extubation within a week of plication is an ominous prognostic sign. Late functional results of plication does not interfere with further development of the diaphragm.

1- Thomas TV: Congenital Eventration of the Diaphragm. Ann Thorac Surg 10 (2); 180-192, 1970
2- Wayne ER, Campbell JB, Burrington JD, Davis WS: Eventration of the Diaphragm. J Pediatr Surg 9 (5): 643-651, 1974
3- Symbas PN, Hatcher CR, Waldo W: Diaphragmatic Eventration in Infancy and Childhood. Ann Thorac Surg 24 (2): 113- 119, 1977
4- Othersen HB, Lorenzo RL: Diaphragmatic Paralysis and Eventration: Newer Approaches to Diagnosis and Operative Correcttion. J Pediatr Surg 12 (3): 309-315, 1977
5- Sarihan H,- Cay A, Akyazici R, Abes M, Imamoglu M: Congenital diaphragmatic eventration: treatment and postoperative evaluation.  J Cardiovasc Surg (Torino) 37(2):173-6, 1996
6- Campobasso P, Schieven E, Gifuini G:  [Diaphragmatic eventration in pediatric age: indications to surgery  and results] Minerva Pediatr 45(11):475-80, 1993
7- Kizilcan F, Tanyel FC, Hicsonmez A, Buyukpamukcu N: The long-term results of diaphragmatic plication.  J Pediatr Surg 28(1):42-4, 1993
8- Ribet M, Linder JL: Plication of the diaphragm for unilateral eventration or paralysis. Eur J Cardiothorac Surg 6(7):357-60, 1992
9- Jawad AJ, al-Sammarai AY, al-Rabeeah A:  Eventration of the diaphragm in children.  J R Coll Surg Edinb 36(4):222-4, 1991
10- Rodgers BM, Hawks P: Bilateral congenital eventration of the diaphragms: successful  surgical management. J Pediatr Surg 21(10):858-64, 1986
11- Smith CD, Sade RM, Crawford FA, Othersen HB: Diaphragmatic paralysis and eventration in infants. J Thorac Cardiovasc Surg 91(4):490-7, 1986

Neonatal Ovarian Cysts

With the arrival of routine prenatal sonography (US) the number of fetal pelvic-abdominal cystic lesions later confirmed as ovarian cyst has increased. The vast majority of these cysts are unilateral, benign and functional. The pathological cause of these cysts is still unknown, but suggests that there was probably an abnormal stimulation by the mother's human chorionic gonadotropin or abnormal enzyme activity of the theca interna. Most are histologically follicular cysts, lined by granulosa epithelium having a diameter greater than 1 mm on microscopic section. Although mostly asymptomatic, abdominal distension a/o palpable mass is the major clinical feature. Management of ovarian cysts in newborns is dictated by size and ultrasound characteristics (simple or complex). Most small (< 4 cm) simple cysts will involute with time and should be observed with serial ultrasounds to avoid unnecessary operations. Those greater than 5 cm increase their potential for torsion, hemorrhage, or rupture. They can be percutaneously aspirated guided by US or laparoscopy. Recurrent, large, or echo-complex cystic masses may need open surgical removal. If the ovarian tissue is viable, it should be preserved as much as possible after trimming away most of the membrane of the cyst. Infarcted ovarian cysts (chocolate cysts) may need oophorectomy.

1- Alrabeeah A, Galliani CA, Giacomantonio M, Heifetz SA, Lau H: Neonatal ovarian torsion: report of three cases and review of the literature. Pediatr Pathol 8(2):143-9, 1988
2- Lindeque BG, du Toit JP, Muller LM, Deale CJ: Ultrasonographic criteria for the conservative management of antenatally diagnosed fetal ovarian cysts.  J Reprod Med 33(2):196-8, 1988
3- Vaillant F, Ganichaud P, Denis A, Duverne C, Coupris L, Grosieux P:  [Neonatal ovarian cysts. Apropos of 4 cases]  J Gynecol Obstet Biol Reprod (Paris) 13(6):663-9, 1984
4- deSa DJ:  Follicular ovarian cysts in stillbirths and neonates. Arch Dis Child 50(1):45-50, 1975
5- Ikeda K, Suita S, Nakano H: Management of ovarian cyst detected antenatally. J Pediatr Surg 23(5):432-5, 1988
6- Watson WJ: Fetal ovarian torsion appearing as a solid abdominal mass. J Perinatol 16(4):302-4, 1996
7- Chen CC, Huang SC, Huang SC, Chuang JH:  Spontaneous resolution of neonatal ovarian cyst: report of one case. Acta Paediatr Sin 37(4):292-4, 1996
8- Sapin E, Bargy F, Lewin F, Baron JM, Adamsbaum C, Barbet JP, Helardot PG: Management of ovarian cyst detected by prenatal ultrasounds. Eur J Pediatr Surg 4(3):137-40, 1994
9- von Schweinitz D, Habenicht R, Hoyer PF: [Spontaneous regression of neonatal ovarian cysts. A prospective study]. Monatsschr Kinderheilkd 141(1):48-52, 1993
10- Muller-Leisse C, Bick U, Paulussen K, Troger J, Zachariou Z, Holzgreve W, Schuhmacher R, Horvitz A: Ovarian cysts in the fetus and neonate--changes in sonographic pattern in the follow-up and their management. Pediatr Radiol 22(6):395-400, 1992
11- Sakala EP, Leon ZA, Rouse GA: Management of antenatally diagnosed fetal ovarian cysts. Obstet Gynecol Surg 46(7):407-14, 1991
12- Matute Cardenas JA, Gomez Fraile A, Cano Novillo I, Miralles Molina M, Vilarino Mosquera A, Cuadros Garcia J, Berchi Garcia FJ: [Conservative treatment of neonatal ovarian cysts]An Esp Pediatr 33(6):549-53, 1990
13- Widdowson DJ, Pilling DW, Cook RC: Neonatal ovarian cysts: therapeutic dilemma. Arch Dis Child 63(7 Spec No):737-42, 1988
14- Nussbaum AR, Sanders RC,Hartman DS, Dudgeon DL, Parmley TH: Neonatal ovarian cysts: sonographic-pathologic correlation. Radiology 168(3):817-21, 1988
15- Ahmed S: Neonatal and childhood ovarian cysts. J Pediatr Surg 6(6):702-8, 1971

Esophageal Polyps

Polyps found in the esophagus of a child are extremely rare events. Most are identified in the esophago-gastric junction or distal third of the esophagus during routine esophagogram for gastroesophageal reflux. Polyps associated to reflux and esophagitis are of inflammatory nature and can be managed with either antireflux medication or surgically (fundoplication). Biopsy of the lesion will demonstrate squamous and gastric mucosa with inflammatory infiltrates. Other lesions with a similar radiographic appearance include: varices, foreign bodies, thickening due to esophagitis and true neoplasms. Esophageal squamous papilloma has been reported in children causing intermittent bleeding and vomiting. The pathogenesis of esophageal squamous papilloma is not known, but chronic mucosal irritation and infection with human papilloma virus are most probable mechanisms. Management is either endoscopic or surgical resection.

1- Croyle P, Nikaidoh H, Currarino G: Inflammatory esophagogastric junction polyp. J Gastroenterol 76(5):438-40, 1981
2- Jones TB, Heller RM, Kirchner SG,  Greene HL: Inflammatory esophagogastric polyp in children. AJR Am J Roentgenol 133(2):314-6, 1979
3- Tam PK, Saing H: Pediatric upper gastrointestinal endoscopy: a 13-year experience. J Pediatr Surg 24(5):443-7, 1989
4- Zitsman JL, Schullinger JN, Berdon WE: Inflammatory esophagogastric polyps: resolution following antireflux surgery. J Pediatr Surg 23(11):1016-7, 1988
5-  Odze R, Antonioli D, Shocket D, Noble-Topham S, Goldman H, Upton M: Esophageal squamous papillomas. A clinicopathologic study of 38 lesions and analysis for human papilloma virus by the polymerase chain reaction [see comments] Am J Surg Pathol 17(8):803-12, 1993
6- Arima T, Ikeda K, Satoh T, Hayashida Y, Matsuo S, Ueda K:  Squamous cell papilloma of the esophagus in a child. Int Surg 70(2):177-8, 1985

Vol 8 No 06 JUNE 1997

Total Colonic Aganglionosis

Total colonic aganglionosis (TCA) is a variety of what is commonly known as long segment Hirschsprung's disease plagued with delay in diagnosis, higher morbidity/mortality and controversial management. The colon is aganglionic sometimes involving a variable segment of distal ileum. Clinically, infants presents with small bowel obstruction, persistent obstipation, distention, or poor weight gain. Early diagnosis depends on clinical awareness of the condition in neonates with intestinal obstruction, diarrhea, or both. Barium enema changes may easily be passed as unremarkable. Radiographic findings of a shortened colon of normal caliber or the presence of "jejunalization" of the colon suggest TCA in patients with a suggestive history. However, free ileal reflux during the examination with a transition point in the ileum and retention of barium in the entire colon after the examination may be diagnostic. Diagnosis is confirmed by rectal biopsy, and the extent of aganglionosis documented after multiple intestinal biopsies. Management consists of initial enterostomy in the proximally ganglionic bowel (a source of fluid/electrolytes losses, prolonged hospitalization, and nutritional deficiency), followed by a pull-through (Soave or Duhamel) using a  side-to-side anastomosis between a segment of the aganglionic colon to the ganglionated small bowel. The procedure provides a greater surface area for absorption and fecal storage. Diarrhea and distension are temporary after the pull-through, but most patients ultimately tolerate normal feedings.

1- Cremin BJ, Golding RL: Congenital aganglionosis of the entire colon in neonates. Br J Radiol 49(577):27-33, 1976
2- Burrington JD, Wayne ER: Modified Duhamel procedure for treatment of total aganglionic colon in childhood. J Pediatr Surg 11(3):391-8, 1976
3- Louw JH: Total colonic aganglionosis. Can J Surg 21(5):397-404, 409, 1978
4-  Careskey JM, Weber TR, Grosfeld JL: Total colonic aganglionosis. Analysis of 16 cases. Am J Surg 143(1):160-8, 1982
5- Martin LW: Total colonic aganglionosis preservation and utilization of entire  colon. J Pediatr Surg 17(5):635-7, 1982
6-  Shandling B: Total colon aganglionosis--a new operation. J Pediatr Surg 19(5):503-5, 1984
7- N-Fekete C, Ricour C, Martelli H, Jacob SL, Pellerin D: Total colonic aganglionosis (with or without ileal involvement): a review of 27 cases. J Pediatr Surg 21(3):251-4, 1986
8- Ikeda K, Goto S: Total colonic aganglionosis with or without small bowel involvement:an analysis of 137 patients. J Pediatr Surg 21(4):319-22, 1986
9- Applebaum H, Richardson RJ, Wilkinson GA, Warden MJ: Alternative operative procedure for total colonic aganglionosis. J Pediatr Surg 23(1 Pt 2):49-51, 1988
10- Endo M, Watanabe K, Fuchimoto Y, Ikawa H, Yokoyama J: Long-term results of surgical treatment in infants with total colonic aganglionosis [see comments] J Pediatr Surg 29(10):1310-4, 1994
11- Hengster P, Pernthaler H, Gassner I, Menardi G: Twenty-three years of follow-up in patients with total colonic aganglionosis. Klin Padiatr 208(1):3-7, 1996

Fibrolamellar Carcinoma

Fibrolamellar carcinoma (FLC) is a rare histologic subtype of hepatocellular carcinoma associated with long-term survival and encapsulation (few mm to 1 cm thick capsule). Occurs almost exclusively in young adolescent women in the absence of cirrhosis, and the serum alpha-fetoprotein is usually normal. Some thinks that it arises in areas of  focal nodular hyperplasia with variable malignant potential. Distinctive histologic features include deeply eosinophilic polygonal hepatocytes and abundant fibrous stroma. Aromatase level are high causing gynecomastia and failure to enter puberty. A defect in chromosome one has been recently identified. Computed tomography remains the most accurate  technique for diagnosis and staging  (large, low attenuation, well-defined edges, some contain areas of calcification or necrosis). The treatment of choice remains radical operation a goal that can be achieved by including partial and total hepatectomy depending on the stage of the tumor. Statistically significant better five year survival rates are observed in patients with solitary tumors and  absent regional lymph node metastases. Cause of death generally is due to a lack of control of the primary tumor. Successful treatment appears to relate to the ability to do a total excision of the primary hepatic tumor.

1- Hany MA, Betts DR, Schmugge M, Schonle E, Niggli FK, Zachmann M, Pluss HJ:A childhood fibrolamellar hepatocellular carcinoma with increased aromatase activity and a near triploid karyotype. Med Pediatr Oncol 28(2):136-8, 1997
2- Bedi DG, Kumar R, Morettin LB, Gourley K: Fibrolamellar carcinoma of the liver: CT, ultrasound and angiography.Case report. Eur J Radiol 8(2):109-12, 1988
3-Ringe B, Wittekind C, Weimann A, Tusch G, Pichlmayr R: Results of hepatic resection and transplantation for fibrolamellar carcinoma. Surg Gynecol Obstet 175(4):299-305, 1992
4- Starzl TE,Iwatsuki S, Shaw BW Jr, Nalesnik MA, Farhi DC, Van Thiel DH: Treatment of fibrolamellar hepatoma with partial or total hepatectomy and transplantation of the liver. Surg Gynecol Obstet 162(2):145-8, 1986
5- Teitelbaum DH, Tuttle S, Carey LC, Clausen KP: Fibrolamellar carcinoma of the liver. Review of three cases and the presentation of a characteristic set of tumor markers defining this tumor. Ann Surg 202(1):36-41, 1985
6- Kearney D, Donlon JB, Mendelson RM: A case of fibrolamellar hepatocellular carcinoma. Australas Radiol 35(1):88-91, 1991
7- Soyer P, Roche A,Levesque M, Legmann P: CT of fibrolamellar hepatocellular carcinoma. J Comput Assist Tomogr 15(4):533-8, 1991
8- Adam A, Gibson RN, Soreide O, Hemingway AP, Carr DH, Blumgart LH, Allison DJ: The radiology of fibrolamellar hepatoma. Clin Radiol 37(4):355-8, 1986
9- Friedman AC, Lichtenstein JE, Goodman Z, Fishman EK, Siegelman SS, Dachman AH: Fibrolamellar hepatocellular carcinoma. Radiology 157(3):583-7, 1985
10- Craig JR, Peters RL, Edmondson HA, Omata M:Fibrolamellar carcinoma of the liver: a tumor of adolescents and young adults with distinctive clinico-pathologic features. Cancer 46(2):372-9, 1980

Byler's Disease

Byler's disease is a progressive familial intrahepatic cholestasis liver condition (autosomal recessive) initially seen in infants less than six months of age. Clinically includes jaundice, itching, failure to thrive, and death from liver cirrhosis. Laboratory findings are: elevated levels of bilirubin and aminotransferases, with normal to low levels of GGT and cholesterol. Liver biopsy is non-specific: cholestasis, liver cell plates and ballooning of hepatocytes in central zones. Presumably, the condition could arises from a transport defect that causes retention of bile salts resulting in secondary toxic hepatocyte injury. Some patients respond to medical therapy (UCDA); in other children liver transplant remains standard therapy. Recently, the use of partial bile drainage using cholecystostomy with an interposed jejunal segment to the skin is of advantage in reducing the enterohepatic circulation of bile acids interrupting the natural history of the disease, reducing its complications and preventing the need for early transplantation in many patients.

1- Edmond JC, Whitington PF: Selective Surgical Management of Progressive Familial Intrahepatic Cholestasis (Byler's Disease). J Pediatr Surg 30(12): 1635-1641, 1995
2- Muller G, Veyckemans F, Calier M, Van Obbergh LJ, De Kock M, Sokal EM, Otte JB: Anaesthetic considerations in progressive familial intrahepatic  cholestasis (Byler's disease). Can J Anaesth 42(12):1126-33, 1995
3- Jacquemin E, Dumont M, Bernard O, Erlinger S, Hadchouel M: Evidence for defective primary bile acid secretion in children with progressive familial intrahepatic cholestasis (Byler disease). Eur J Pediatr 153(6):424-8, 1994
4- Maggiore G, Bernard O, Hadchouel M, Lemonnier A, Alagille D:  Diagnostic value of serum gamma-glutamyl transpeptidase activity in  liver diseases in children. J Pediatr Gastroenterol Nutr 12(1):21-6, 1991
5- Soubrane O, Gauthier F, DeVictor D, Bernard O, Valayer J, Houssin D, Chapuis Y:  Orthotopic liver transplantation for Byler disease. Transplantation 50(5):804-6, 1990
6- Jacquemin, Heremans, Myara, Habes, Debray, Hadchouel, Sokal, Bernard: Ursodeoxycholic Acid Therapy in Paediatric Patient with Progressive Familial Intrahepatic Cholestasis. Hepatology 25: 519, 1997

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