VOLUME 13, 1999

Volume 13 No 01 JULY 1999

Tongue Cysts

Cysts in the tongue are rare in children. The differential diagnosis includes: mucoceles (retention cyst), cystic hygroma, thyroglossal cysts, cysts of foregut origin and teratoma. They usually presents at birth interfering with mouth closure, swallowing, normal feeding or even causing respiratory problems. Complications of the cyst include infection, hemorraghe and rupture leading to increase in size and asphyxia, inability to feed and aspiration pneumonia. Mucoceles (ranula) may appear in the base of the tongue and needs excision or marsupialization. Multiple cysts can be a cystic hygroma in the tongue that may need partial glossectomy or management with sclerosing substances. Lingual thyroglossals are found in the base of the tongue and can safely be managed with marsupialization without excision or Sistrunk procedure. Cysts of foregut origin presents in the neonatal period, can be found in the anterior part of the dorsum of the tongue needing complete surgical excision through a sagittal glossal split. Cyst aspiration is inadequate definitive treatment. A teratoma likewise needs surgical excision for cure.

1- Urao M, Teitelbaum DH, Miyano T: Lingual thyroglossal duct cyst: a unique surgical approach. J Pediatr Surg 31(11):1574-6, 1996
2- Surana R, Losty P, Fitzgerald RJ: Heterotopic gastric cyst of the tongue in a newborn. Eur J Pediatr Surg 3(2):110-1, 1993
3- Wiersma R, Hadley GP, Bosenberg AT, Chrystal V: Intralingual cysts of foregut origin. J Pediatr Surg 27(11):1404-6, 1992
4- Velcek FT, Klotz DH, Hill CH, Ladogana LE: Tongue lesions in children. J Pediatr Surg 14(3):238-46, 1979

Primary Immunodeficiency

The body mounts an immune response by producing antibodies (B cells), inciting cellular division (T cells), cascading complement and producing phagocytosis. Congenital absence or deficiency of this response not associated to a secondary illness is known as primary immunodeficiency (PI). PI is very rare (400 new cases/year in the USA), and is divided into deficiency of: antibody (most common), T-cell, combined T and B-cell, phagocytic or complement activity. Most cases of PI are identified in the first year of life. Primary symptom is recurrent infections with unusual organisms (low pathogenicity) that is severe, prolonged and resistant to conventional therapy. T-cell deficiency occurs before the age of six months while antibody deficiency presents clinically after this age due to transplacental protection. Phagocytic and complement deficiency is present since birth as the baby might show a lack of pus formation or delayed umbilical cord detachment. After respiratory tract, the skin is the 2nd most common site of infection, i.e., thrush, furuncles, abscess, fistulas and cellulitis followed by the GI tract. The child usually appears chronically ill with growth failure. Extensive lab testing will establish the diagnosis. Live vaccine should be avoided. The child with PI who will undergo a surgical procedure needs: protective isolation, antibiotic prophylaxis, nutritional backup, physiologic monitoring and selective replacement of defective substance (immunoglobulins) a/o immunologic enhancing agents use. Bone marrow transplant can sometimes correct a PI.

1- Peerles AG, Stiehm ER: The Immunocompromised Child, In Fonkalsrud and Krummel "Infections and Immunologic Disorders in Pediatric Surgery", WB Saunders Co, 1993, pag 77-90
2- Stiehm ER: Human intravenous immunoglobulin in primary and secondary antibody deficiencies. Pediatr Infect Dis J 16(7):696-707, 1997
3- Pachman LM, Lynch PA, Silver RK, Ozog DL, Poznanski AK: Primary immunodeficiency disease in children: an update. Curr Probl Pediatr 19(1):1-64, 1989
4- Primary immunodeficiency diseases. Report prepared for the WHO by a scientific group on immunodeficiency. Clin Immunol Immunopathol 28(3):450-75, 1983
5- Pappas BE: Primary immunodeficiency disorders in infancy. Neonatal Netw 18(1):13-22, 1999


The most common forms of lymphedema in children affect the lower extremity. Lymphedema in children can be classified into: congenital, precox and tarda. The underlying pathology is absence (10%) or hypoplasia (90%) of the lymphatic channels of the lower extremity. Diagnosis of lymphedema is made clinically. Congenital lymphedema appears early in life, involves more than one extremity, rarely extends above the knee and enlarges at a slower rate than body growth. The swelling becomes less pronounced with age, and no specific therapy is required in two thirds of cases. Lymphedema precox appears during adolescence, occur mostly in females, extends to the groin and is associated with yellow nails (inadequate lymphatic drainage). Lymphedema tarda occurs spontaneously in middle age people. In unilateral cases a retroperitoneal tumor or venous obstruction should be rule out. Complications of lymphedema include swelling, brawny edema, cellulitis and lymphangitis. Management consists of extremity care, compressive support stocking and leg elevation. In severe cases the option is excision of excess skin and subcutaneous lymphatics (modified Kondoleon procedure).

1- Fonkalsrud EW: Surgical management of congenital lymphedema in infants and children. Arch Surg 114(10):1133-6, 1979
2- Hilliard RI, McKendry JBJ, Phillips MJ: Congenital abnormalities of the lymphatic system: a new clinical classification. Pediatrics 86:988, 1990
3- Levine C: Primary disorders of the lymphatic vessels: a unified concept. J Pediatr Surg 24:233, 1989
4- Fonkalsrud EW: Congenital Malformations of the Lymphatic System. Semm Pediatr Surg 3(2): 62-69, 1994

Volume 13 No 02 AUGUST 1999

Segmental Volvulus

Volvulus, an acute surgical emergency, refer to clockwise rotation of the bowel causing lymphatic, venous or arterial occlusion. In malrotation the volvulus include the midgut (from the second portion of the duodenum to mid-transverse colon). On rare occasions volvulus involve only the small bowel in a segmental fashion affecting a portion of jejunum, ileum or both. Segmental volvulus can be the result of a predisposing anatomical defects such as: congenital bands (a persistent omphalomesenteric band or Meckel diverticulum fixed to the anterior abdominal wall), acquired adhesive bands after a surgical procedure, abnormal foreshortened mesenteric defect (segmental or basilar), hanging tumors (mesenteric cyst), intraluminal lesions of the small bowel (meconium ileus), worms (Ascariasis) and even VP shunts. Clinically, the child will present with sudden intestinal obstruction (bilious vomiting, abdominal distension, signs of peritonitis) associated with variable changes of ischemic bowel (shock, metabolic acidosis and dehydration). Recognition of volvulus before infarction occurs is imperative for bowel survival. Management must be prompt and consist of counterclockwise detorsion of the affected segment, removing the anatomic cause, and depending on the viability of the affected segment, resection with anastomosis. In broad-base mesenteric root defects (basilar) pexing of the bowel may be needed.  Due to the segmental nature loss of massive bowel is fortunately rarely seen.

1- Maung M, Saing H: Intestinal volvulus: an experience in a developing country. J Pediatr Surg 30(5):679-81, 1995
2- Black PR, Mueller D, Crow J, Morris RC, Husain AN: Mesenteric defects as a cause of intestinal volvulus without malrotation and as the possible primary etiology of intestinal atresia. J Pediatr Surg 29(10):1339-43, 1994
3- Wilkinson TS, Stone HH: Intestinal volvulus without malrotation in a four-month-old infant. Am Surg 33(5):3665-6, 1967
4- Rescorla FJ, Shedd FJ, Grosfeld JL, Vane DW, West KW: Anomalies of intestinal rotation in childhood: analysis of 447 cases. Surgery 108(4):710-5, 1990
5- Abrahamson RH: Acute small intestinal volvulus. Int Surg 52(3):210-4, 1969
6- Medina V, Diaz H, Lorenzo A, Carrillo A, Gonzalez F: Jejuno-ileal volvulus, a rare cause of intestinal obstruction. Rev Esp Enferm Dig 87(12):883-4, 1995

Macklin effect

The most common cause of pneumoperitoneum in neonates is perforated necrotizing enterocolitis followed by spontaneous gastric perforation. In rare occasions pneumoperitoneum can arise from ruptured pulmonic blebs (minute rupture in alveoli subjected to the stress of mechanical ventilation) dissecting retroperitoneally into the abdomen rather than outwardly into the pleura. This is known as the Macklin effect published originally in 1943. Precise diagnosis of this type of "medical" pneumoperitoneum will reduce a needless laparotomy in seriously ill infants. Some suggestions pointing toward this etiology are the presence of interstitial emphysema, retrocardiac pneumomediastinum, pneumothorax, dissection of air into the soft tissues of the neck, the absence of fluid or meconium in the peritoneum and the presence of air in the stomach in a child with severe pulmonary disease (Hyaline membrane disease) on mechanical ventilation. Previous X-ray may note the absence of dilated bowel loops, edema or pneumatosis. Contrast bowel studies (with water soluble material) and peritoneal paracentesis is needed to distinguish bowel perforation from an intrathoracic origin of the air. Evacuation of the air and supportive management is all that is needed.

1- Wintermark M, et al: Blunt traumatic pneumomediastinum: using CT to reveal the Macklin effect.. Am J Roentgenol. 172(1):129-30, 1999
2- Brotman S, et al: The Macklin effect: a cause of pneumoperitoneum. Md State Med J. 30(9):30, 1981
3- Rosenfeld DL, Cordell CE, Jadeja N: Retrocardiac pneumomediastinum: radiographic finding and clinical implications. Pediatrics 85(1):92-7, 1990
4- Cohen MD, Schreiner R, Lemons J: Neonatal pneumoperitoneum without significant adventitious pulmonary air: Use of metrizamide to rule out perforation of the bowel. Pediatrics 69(5):587-9, 1982
5- Zerella JT, McCullough JY: Pneumoperitoneum in infants without gastrointestinal perforation. Surgery 89(2):163-7, 1981
6- Summers B: Pneumoperitoneum associated with artificial ventilation. Br Med J 1:1528-30, 1979
7- Steves M, Ricketts RR: Pneumoperitoneum in the newborn infant. Am Surg 53(4):226-30, 1987
8- Campbell RE, Boggs TR Jr, Kirkpatrick JA Jr: Early neonatal pneumoperitoneum from progressive massive tension pneumomediastinum. Radiology 114(1):121-6, 1975

Mesocolic Hernias

Mesocolic hernias (MH) are rare congenital malformations accounting for one-third of all internal hernias and arising from an error of rotation of the midgut with entrapment of the small intestine beneath the developing colon. Right (Waldeyer's hernia) and left MH are distinct entities varying in embryological origin. Failure of rotation of the pre-arterial midgut segment associated to normal post-arterial segment rotation results in a right MH with the small bowel trapped behind the mesentery of the right and transverse colon. A left MH occurs when the unsupported area of descending mesocolon between the inferior mesenteric vein and posterior parietal attachment is ballooned by the small bowel as it migrates to the left abdominal cavity. Both MH can presents with chronic or acute abdominal obstruction, gangrene and bowel perforation. UGIS is diagnostic. Management consists of reduction, repositioning of the bowel and sparing the inferior mesenteric vessels during the repair of left MH and re-positioning the right colon to the  left side of the abdomen for repair of right MH.

1- Khan MA, Lo AY, Vande Maele DM: Paraduodenal hernia. Am Surg 64(12):1218-22, 1998
2- Dengler WC, Reddy PP: Right paraduodenal hernia in childhood: a case report. J Pediatr Surg 24(11):1153-4, 1989
3- Brigham RA, Fallon WF, Saunders JR, Harmon JW: Paraduodenal hernia: diagnosis and surgical management. Surgery 96(3):498-502, 1984
4- Gagic NM: Right paraduodenal hernia. Can J Surg 25(1):71-2, 1982
5- Azouz EM, Doyon M: Waldeyer's hernia. J Can Assoc Radiol 27(2):108-10, 1976

Volumen 13 No 3 SEPTEMBER 1999

Laparoscopic Adrenalectomy

Adrenalectomy is another procedure that has yielded to the advantages of the laparoscopic approach in very specific situations. In the pediatric age the indications for adrenalectomy are mostly tumor related: neuroblastoma (the most common adrenal mass in a child), pheochromocytoma, adenoma, ganglioneuroma, neurofibroma, fibromas, incidentalomas  and lipomas. Benign, well encapsulated, fibrous, and non-infiltrative lesions within the adrenal gland lend themselves to laparoscopic removal. Malignant (due to risk of  port metastasis), large, infiltrative or lesions that need lymph node sampling, have bilateral involvement will need the conventional open approach. In children the lateral intra-abdominal approach provides a greater working place instead of the retroperitoneal approach used in adults. Right adrenal gland removal needs liver mobilization and secure closure of the short adrenal vein. Left adrenal removal is easier and can be accomplished after mobilization of spleno-colonic ligaments. Although the lap approach takes longer, the overall advantages are less pain, less morbidity, shorter hospital stay and better cosmetic results.

1- Lopoo JB, Albanese CT, Jennings RW, Tyrell D, Harrison MR, Duh QY: Laparoscopic Adrenalectomy in Children. Pediatr Endosurgery & Innovat Tech 2(3): 107- 110, 1998
2- Yoshimura K, Yoshioka T, Miyake O, Matsumiya K: Comparison of clinical outcomes of laparoscopic and conventional open adrenalectomy. J Endourol 12(6):555-9, 1998
3- Linos DA, Stylopoulos N, Boukis M, Souvatzoglou A: Anterior, posterior, or laparoscopic approach for the management of adrenal diseases? Am J Surg 173(2):120-5, 1997
4- Fernandez-Cruz L, Benarroch G, Torres E, Astudillo E, Saenz A; Taura P: Laparoscopic approach to the adrenal tumors. J Laparoendosc Surg 3(6):541-6, 1993

Laparoscopic Ladd's Procedure

Malrotation of the bowel can be associated with midgut volvulus defined as clockwise rotation of the small bowel around the superior mesenteric vessels axis causing ischemia. The diagnosis is done with the help of both an upper contrast study and barium enema. Asymptomatic malrotation with near-point fixation of the duodenojejunal and ileocolic segment should undergo prophylactic Ladd's. Ladd's procedure consists of release of congenital duodeno-colic bands, separation of the duodeno-jejunal from the ileo-colic segment in a transverse fashion with removal of the appendix for cases of bowel malrotation associated with or without midgut volvulus. Asymptomatic malrotation referred for Ladds procedure can be done laparoscopically using a three port technique. Ladd's bands and duodenocolic adhesions are divided exposing the SMA that lies between the duodenum and ascending colon. The base of the mesentery root is widened using sharp dissection leaving the small bowel on the right abdomen and the colon on the left to prevent volvulus. The appendix is removed to avoid future diagnostic problems. Advantages of laparoscopy are: less pain, less ileus, brief hospital stay and better cosmesis. In cases of midgut volvulus laparoscopy is debatable since it may be difficult and dangerous to deal with dilated a/o ischemic bowel.

1- Lessin MS, Luks FI: Laparoscopic appendectomy and duodenocolonic dissociation (LADD) procedure for malrotation. Pediatr Surg Int 13(2-3):184-5, 1998
2- Gross E, Chen MK, Lobe TE: Laparoscopic evaluation and treatment of intestinal malrotation in infants. Surg Endosc 10(9):936-7, 1996
3- Bass KD, Rothenberg SS, Chang JH: Laparoscopic Ladd's procedure in infants with malrotation. J Pediatr Surg 33(2):279-81, 1998
4- Waldhausen JH, Sawin RS: Laparoscopic Ladd's procedure and assessment of malrotation. J Laparoendosc Surg 6 Suppl 1:S103-5, 1996
5- Mazziotti MV, Strasberg SM, Langer JC: Intestinal rotation abnormalities without volvulus: the role of laparoscopy. J Am Coll Surg 185(2):172-6, 1997


Myofibromatosis refers to a rare benign soft tissue tumor seen mostly in newborns and young infants. The child develops firm, discrete, flesh-colored to purple nodules in skin, muscle, bone a/o subcutaneous tissue with particular predilection for the head and neck region and trunk. Etiology is unknown. Microscopically, they are well-circumscribed nodules consisting of short bundles of plump, spindle-shaped cells displaying characteristics intermediate between fibroblasts and smooth muscle cells with central necrosis and a prominent vascular pattern. Clinically two types are described: solitary and multicentric (generalized) form. Most infants (74%) have a solitary lesions that respond to conservative surgical excision. The multicentric variant with a poorer prognosis may involve muscle, bone and viscera. Visceral lesions are associated with significant morbidity and mortality generally within the first few months of life secondary to obstruction of a vital organ (bowel and lung), failure to thrive, or infection. Spontaneous regression has been identified in one-third of the patients after a two-year period suggesting that multiple lesions not affecting vital function, resulting in growth anomalies, or demonstrating rapid aggressive growth can be managed conservatively.

1- Schrodt BJ, Callen JP: A case of congenital multiple myofibromatosis developing in an infant. Pediatrics 104(1 Pt 1):113-5 , 1999
2- Beck JC, Devaney KO, Weatherly RA, Koopmann CF Jr, Lesperance MM: Pediatric myofibromatosis of the head and neck. Arch Otolaryngol Head Neck Surg 125(1):39-44, 1999
3- Coffin CM, Neilson KA, Ingels S, Frank-Gerszberg R, Dehner LP: Congenital generalized myofibromatosis: a disseminated angiocentric myofibromatosis. Pediatr Pathol Lab Med 15(4):571-87, 1995
4- Salamah MM, Hammoudi SM, Sadi AR: Infantile myofibromatosis. J Pediatr Surg 23(10):975-7, 1988
5- Chung EB, Enzinger FM: Infantile myofibromatosis. Cancer 15;48(8):1807-18, 1981

Volume 13 No 4 OCTOBER 1999

Mixed Gonadal Dysgenesis

Mixed gonadal dysgenesis (MGD) is an intersexual genetic abnormality caused by a defect in the sex chromosomes (gonosomes) associated with dysgenetic gonads and retained Müllerian structures. The most common gonosomal aberration in MGD is 45 X0/46 XY mosaic karyotype. The external genitalia could be normal looking female and these children will present later in life with primary amenorrhea. Otherwise, it could be ambiguous: clitoromegaly and urogenital sinus to a sizable phallus with hypospadia. A uterus and one or both fallopian tubes may also be present. MGD is characterized by a streak gonad and a contralateral testis (that is typically cryptorchid) or bilateral streak testes. The testis might show prepubertal tubules lined by a few spermatogonia and immature Sertoli cells. Female gender assignment is usually preferred, but male assignment is an alternative in instances of extreme virilization. Dysgenetic gonads with the presence of a Y chromosome or a translocated fragment have a significant risk of developing malignant gonadoblastoma (though seminoma and dysgerminoma can occur). Routine early bilateral gonadectomy is advice in MGD. The child to be raised as a female will need clitoral recession and vaginoplasty in early infancy. If it is to be raised as male, then various types of hypospadias repair can be done, gonads can be replaced with prostheses, the prepenile scrotum reconstructed and Müllerian structures removed.

1- Hendren WH: Surgical approach to intersex problems. Semin Pediatr Surg 7(1):8-18, 1998
2- Calabrese F, Valente M: Mixed gonadal dysgenesis: histological and ultrastructural findings in two cases. Int J Gynecol Pathol 15(3):270-5, 1996
3- Federman DD, Donahoe PK: Ambiguous genitalia--etiology, diagnosis, and therapy. Adv Endocrinol Metab 6:91-116, 1995
4- Borer JG, Nitti VW, Glassberg KI: Mixed gonadal dysgenesis and dysgenetic male pseudohermaphroditism. J Urol 153(4):1267-73, 1995
5- Krasna IH, Lee ML, Smilow P, Sciorra L, Eierman L: Risk of malignancy in bilateral streak gonads: the role of the Y chromosome. J Pediatr Surg 27(11):1376-80, 1992
6- Coran AG, Polley TZ Jr: Surgical management of ambiguous genitalia in the infant and child. J Pediatr Surg 26(7):812-20, 1991

Recurrent Intussusception

Intussusception, the most common cause of bowel obstruction in infants, occurs mostly in the ileo-cecal area (distal ileum invaginates inside the colon) and it is idiopathic (or caused by hypertrophied Peyer patches after Adenovirus III infestation). Initial management consists of hydrostatic or air enema reduction. Recurrent intussusception (RI) occurs in up to 10% of cases after hydrostatic reduction and to a lesser degree after surgical reduction (3%). Patients with RI have fewer symptoms with a shorter duration. Most RI occurs within six months of the initial episode. With multiple recurring episodes a search for a pathological lead point (Meckel's diverticulum, polyp, ectopic gastric mucosa, duplication or lymphoid hyperplasia) is imperative. RI even in the face of previous surgery is not a contraindication to try radiological reduction since the success rate is still high and the morbidity low. The child with perforation, signs of peritonitis, irreducible intussusception or a diagnosed lead point should undergo immediate surgical reduction.

1- Daneman A, Alton DJ, Lobo E, Gravett J, Kim P: Patterns of recurrence of intussusception in children: a 17-year review. Pediatr Radiol 28(12):913-9, 1998
2- Hasegawa T, Ueda S, Tazuke Y, Monta O, Sakurai T, Takahara N, Tanaka T, Habukawa C: Colonoscopic diagnosis of lymphoid hyperplasia causing recurrent intussusception: report of a case. Surg Today 28(3):301-4, 1998
3- Fecteau A, Flageole H, Nguyen LT, Laberge JM, Shaw KS, Guttman FM: Recurrent intussusception: safe use of hydrostatic enema. J Pediatr Surg 31(6):859-61, 1996
4- Champoux AN, Del Beccaro MA, Nazar-Stewart V: Recurrent intussusception. Risks and features. Arch Pediatr Adolesc Med 148(5):474-8, 1994
5- Pierro A, Donnell SC, Paraskevopoulou C, Carty H: Indications for laparotomy after hydrostatic reduction for intussusception. J Pediatr Surg 28(9):1154-7, 1993
6- Ein SH: Recurrent intussusception in children. J Pediatr Surg 10(5):751-5, 1975

Fibrous Hamartoma

Establishing a diagnosis on clinical or radiological grounds of a subcutaneous mass in an infant or child could be a challenging dilemma, not to mention poorly cost-effective work-up. Fibrous hamartoma is an uncommon benign, soft tissue, painless nodule of rapid growth usually seen during the first two years of life. Initially described in 1956, this lesion is most frequently found in boys, solitary, identified in the subcutis or lower dermis of the axillary region, upper arm, upper trunk, inguinal region, scalp, scrotum and perineum. The lesion varies in size, is firm, fixed to the underlying tissue and not encapsulated. A few cases show overlying skin changes (pigmentation, hair). Pathologically they show well-defined bundles of dense, uniform, fibrous connective tissue projecting into fat, primitive mesenchyme and mature adipose tissue. Diagnosis has been established by fine-needle aspiration technique rarely. Standard care consists of local excision to establish a diagnosis. Observation is not a good option given the uncertainty of missing a malignant lesion. Incomplete removal might result in recurrence and need of wider local excision. Mutilating surgery is unnecessary. The prognosis is excellent.

1- Lee JT, Girvan DP, Armstrong RF: Fibrous Hamartoma of Infancy. J Pediatr Surg 23(8): 759-761, 1988
2-  Dickey GE, Sotelo-Avila C: Fibrous hamartoma of infancy: current review. Pediatr Dev Pathol 2(3):236-43, 1999
3- Popek EJ, Montgomery EA, Fourcroy JL: Fibrous hamartoma of infancy in the genital region: findings in 15 cases. J Urol 152(3):990-3, 1994
4- Albukerk J, Wexler H, Dana M, Silverman J: A case of fibrous hamartoma of infancy. J Pediatr Surg 14(1):80-2, 1979
5- Jadusingh IH: Fine needle aspiration cytology of fibrous hamartoma of infancy. Acta Cytol 41(4 Suppl):1391-3, 1997

Volume 13 No 5 NOVEMBER 1999

Urachal Remnants

Urachal remnants are uncommon anomalies that present symptoms in infancy or early childhood. The urachus is formed in early embryonic life as a tubular connection between the dome of the bladder and the allantoic stalk. This fistulous tract obliterates into a median ligament. Rare persistence of this remnant after birth can lead to a completely patent urachus, a sinus (opening to the navel), a diverticulum (opening to the bladder),  a cyst (both end close but central portion remains open) or alternating sinus (cyst-like structure can drain to either the bladder or umbilicus). Neonatal patent urachus can undergo spontaneous involution. Otherwise, the transitional epithelium is replaced by granulation tissue and the main symptom of a patent urachus is urine discharge from the umbilicus associated with cellulitis, pain and swelling. A cyst or diverticulum can present as a midline, localized, painful abdominal mass associated with fever and leucocytosis. The diagnosis can be done with ultrasound (cystic process is shown), cystography (the ventral position of urachal diverticulum and fistula is seen, and a bladder-neck obstruction diagnosed) or fistulography (a connection with the bladder is demonstrated). If infected initial management consists of antibiotics, otherwise cure is accomplished with excision of the urachal remnant with a cuff of bladder (to avoid recurrent infection, stone formation and later development of carcinoma), double layer closure and drainage.

1- Zieger B, Sokol B, Rohrschneider WK, Darge K, Troger J: Sonomorphology and involution of the normal urachus in asymptomatic newborns. Pediatr Radiol 28(3):156-61, 1998
2- Suita S, Nagasaki A: Urachal remnants. Semin Pediatr Surg 5(2):107-15, 1996
3- Nagasaki A, Handa N; Kawanami T: Diagnosis of urachal anomalies in infancy and childhood by contrast fistulography, ultrasound and CT. Pediatr Radiol 21(5):321-3, 1991
4- Goldman IL, Caldamone AA, Gauderer M, Hampel N, Wesselhoeft CW, Elder JS: Infected urachal cysts: a review of 10 cases. J Urol 140(2):375-8, 1988
5- Newman BM; Karp MP; Jewett TC; Cooney DR: Advances in the management of infected urachal cysts. J Pediatr Surg 21(12):1051-4, 1986

Medullary Thyroid Carcinoma

Medullary thyroid carcinoma (MTC) is a rare, solid, thyroid neoplasm with amyloid stroma that arise from the parafollicular C-cells, is inherited as autosomal dominant, and may develop in childhood sporadically or associated with a multiple endocrine neoplasia (MEN) or Familial syndrome. In sporadic cases the presentation occurs in adolescence as a thyroid nodule. Production of thyrocalcitonin by MTC and its precursor (C-cell hyperplasia) permits diagnosis and follow-up, though a significant number of these children are not cured by surgery due to extensive disease at diagnosis. DNA testing has found that the RET proto-oncogene mutation is associated with MCT development in kindreds of sporadic cases, MEN or familial MTC syndromes. This has permitted early (prophylactic) gland removal in infancy (MEN IIB)  or early childhood (MEN IIA) before biochemical or clinical MCT develops. MCT metastasizes to local lymph nodes followed by lungs, bone and liver. Management consists of total thyroidectomy and central lymph node sampling with general sampling if enlarged.

1- Telander RL, Moir CR: Medullary thyroid carcinoma in children. Semin Pediatr Surg 3(3):188-93, 1994
2- Wells SA Jr, Chi DD, Toshima K, Dehner LP, Coffin CM, Dowton SB, Ivanovich JL, DeBenedetti MK, Dilley WG, Moley JF, et al: Predictive DNA testing and prophylactic thyroidectomy in patients at risk for multiple endocrine neoplasia type 2A. Ann Surg 220(3):237-47, 1994
3- Skinner MA, DeBenedetti MK, Moley JF, Norton JA, Wells SA Jr: Medullary thyroid carcinoma in children with multiple endocrine neoplasia types 2A and 2B. J Pediatr Surg 31(1):177-81, 1996
4- La Quaglia MP, Telander RL:  Differentiated and medullary thyroid cancer in childhood and adolescence. Semin Pediatr Surg 6(1):42-9, 1997
5- Skinner MA, Wells SA Jr: Medullary carcinoma of the thyroid gland and the MEN 2 syndromes. Semin Pediatr Surg 6(3):134-40, 1997
6- Lallier M, St-Vil D, Giroux M, Huot C, Gaboury L: Prophylactic thyroidectomy for medullary thyroid carcinoma in gene carriers of MEN2 syndrome. J Pediatr Surg 33(6):846-8, 1998
7- van Heurn LW, Schaap C, Sie G, Haagen AA, Gerver WJ, Freling G, van Amstel HK, Heineman E:  Predictive DNA testing for multiple endocrine neoplasia 2: a therapeutic challenge of prophylactic thyroidectomy in very young children. J Pediatr Surg 34(4):568-71, 1999

Gastric Perforation

Gastric perforation (GP) is a rare abdominal catastrophe seen chiefly in premature infants. GP occurs primarily (spontaneously) after selective ischemia with blood shunting during periods of neonatal asphyxia. Secondary GP is caused by mechanical disruption (excessive distension or instrumentation) as observed in situations such as: aggressive mask resuscitation, duodenal atresia, esophageal atresia with TE fistula, volvulus of the stomach, esophageal intubation, mechanical ventilation and nasogastric tubes (iatrogenic). Boys are more commonly affected than girls. Sudden onset of abdominal distension, feeding intolerance, respiratory distress, metabolic acidosis, shock and hypoactivity within the first week of life is characteristic. Pneumoperitoneum is seen in plain abdominal films. Most GP occurs along the greater curvature of the stomach between the smooth muscle layers. With severely sick infants, temporary peritoneal drainage with lavage removes gas and acid, decompresses the abdomen improving ventilation (abdominal compartment syndrome), and grants time to stabilized the sick infant (improve acidosis, shock and coagulopathy) in preparation for surgical closure of the perforation. GP carries a high mortality rate.

1- St-Vil D, LeBouthillier G, Luks FI, Bensoussan AL, Blanchard H, Youssef S: Neonatal gastrointestinal perforations. J Pediatr Surg 27(10):1340-2, 1992
2- Shashikumar VL, Bassuk A, Pilling GP IV, Cresson SL: Spontaneous gastric rupture in the newborn: a clinical review of nineteen cases. Ann Surg 182(1):22-5, 1975
3- Rosser SB, Clark CH, Elechi EN: Spontaneous neonatal gastric perforation. J Pediatr Surg 17(4):390-4, 1982
4- Tan CE, Kiely EM, Agrawal M, Brereton RJ, Spitz L: Neonatal gastrointestinal perforation. J Pediatr Surg 24(9):888-92, 1989
5- Houck WS Jr, Griffin JA 3d: Spontaneous linear tears of the stomach in the newborn infant. Ann Surg 193(6):763-8, 1981
6- Aviles T, Lugo-Vicente H, Ocasio MT, Guiven A, Pagán V, Lliteras O, Vázquez H: Perforated NEC: The role of percutaneous peritoneal drainage (submitted for publication).

Volume 13 No 6 DECEMBER 1999

Bowel Management

Fecal incontinence (FI) could be the outcome after surgery for anorectal malformations (imperforate anus) or Hirschsprung's disease. FI can be further subdivided by history and contrast studies into those with a tendency to constipation (megasigmoid) or diarrhea (a non-dilatated colon running straight from the splenic flexure to the anus). Bowel management aims to improve the personal and social burden inherent to this problem promoting independence in the affected child. The most effective regimen consists of regular and complete emptying of the colon limiting the episodes of fecal soilage. This is accomplished with the use of a daily enema program, dietary manipulation, laxatives and drugs. The enema is administered while the child sits in the toilet at a rate of 10-20 cc/kg of weight. To avoid spillage the silastic tube must have a balloon to seal the distal rectum. After enema administration the balloon is deflated and the child allowed to evacuate the colonic content. Leaving the balloon partially inflated encourages the child to expel it as a biofeedback mechanism allowing some patients to realize they have some minimal control to be exploited. If the enema program is effective in a 3 to 6 month period, the child can become a candidate for a Malone procedure (appendicostomy). Children with constipation and megasigmoid needs large volume enemas. Likewise, the large megasigmoid can cause overflow pseudo incontinence that is only helped with sigmoid resection. Those with diarrhea may need constipating diet and anti-motility drugs. A few children that continue with incontinence in spite adequate therapy might benefit from a permanent colostomy.

1- Shandling B, Gilmour RF: The Enema Continence Catheter in Spina Bifida: Successful Bowel Management. J Pediatr Surg 22(3): 271-273, 1987
2- Blair GK, Djonlic K, Fraser GC, Arnold WD, Murohy JJ, Irwin B: The Bowel Management Tube: An Effective Means for Controlling Fecal Incontinence. J Pediatr Surg 27(10): 1269-1272, 1992
3- Peña A, Guardino K, Tovilla JM, Levitt MA, Rodriguez G, Torres R: Bowel Management for Fecal Incontinence in Patients with Anorectal malformations. J Pediatr Surg 33(1): 133-137, 1998
4- Levitt MA, Soffer SZ, Pena A: Continent appendicostomy in the bowel management of fecally incontinent children. J Pediatr Surg 32(11):1630-3, 1997
5- Paidas CN: Fecal incontinence in children with anorectal malformations. Semin Pediatr Surg 6(4):228-34, 1997
6- Gleeson RM: Bowel continence for the child with a neurogenic bowel. Rehabil Nurs 15(6):319-21, 1990

Bladder Rhabdomyosarcoma

Bladder Rhabdomyosarcoma (B-RMS) is the most common tumor of the lower genitourinary tract in children  (mean appearance at five years of age). Most B-RMS arise from the submucous tissue of the bladder base, trigone and neck infiltrating the prostatic urethra and surrounding pelvic fascia. Only 20% arise from the bladder dome. Urinary or fecal retention, hematuria, UTI or palpable mass are initial presentation. Embryonal (75%) and botryoid (25%) histological variant predominates. MRI is recommended as the key method of diagnosis and follow-up of pelvic RMS. Unless staging demonstrates a small or dome lesion amenable to surgical resection, initial management should consist of high dose multiagent chemotherapy (VAC) and low-dose irradiation (40 Gy). Response could be: 1- complete, needing follow-up imaging and cystoscopy, 2- partial (< 50%) or none, needing total cystectomy or anterior exenteration. Survival is hampered by metastasis, local invasion and prostatic origin of the tumor. These children need aggressive treatment modality including total cystectomy. Bladder function preservation is difficult to achieve due to location of the lesion, radiation cystitis and hemorrhagic cystitis (cyclophosphamide).

1- Ragab AH, Heyn R, Tefft M, Hays DN, Newton WA Jr, Beltangady M: Infants younger than 1 year of age with rhabdomyosarcoma. Cancer 58(12):2606-10, 1986
2- Loughlin KR, Retik AB, Weinstein HJ, Colodny AH, Shamberger RC, Delorey M, Tarbell N, Cassady JR, Hendren WH: Genitourinary rhabdomyosarcoma in children. Cancer 15;63(8):1600-6, 1989
3-Hays DM, Lawrence W Jr, Crist WM, Wiener E, Raney RB Jr, Ragab A, Tefft M, Webber B, Johnston J, Maurer HM: Partial cystectomy in the management of rhabdomyosarcoma of the bladder: a report from the Intergroup Rhabdomyosarcoma Study.  J Pediatr Surg 25(7):719-23, 1990
4- La Quaglia MP, Ghavimi F, Herr H, Mandell L, Pennenberg D, Hajdu S, Exelby PR: Prognostic factors in bladder and bladder-prostate rhabdomyosarcoma. J Pediatr Surg 25(10):1066-72, 1990
5- Hicks BA, Hensle TW, Burbige KA, Altman RP: Bladder management in children with genitourinary sarcoma. J Pediatr Surg 28(8):1019-22, 1993
6- Hays DM, Raney RB, Wharam MD, Wiener E, Lobe TE: Children with vesical rhabdomyosarcoma (RMS) treated by partial cystectomy with neoadjuvant or adjuvant chemotherapy, with or without radiotherapy. A report from the Intergroup Rhabdomyosarcoma Study (IRS) Committee. J Pediatr Hematol Oncol 17(1):46-52, 1995
7- Regine WF, Fontanesi J, Kumar P, Ayers D, Bowman LC, Pappo AS, Coffey DH, Avery L, Rao BN, Kun LE: Local tumor control in rhabdomyosarcoma following low-dose irradiation: comparison of group II and select group III patients. Int J Radiat Oncol Biol Phys 31(3):485-91, 1995
8- Heyn R, Newton WA, Raney RB, Hamoudi A, Bagwell C, Vietti T, Wharam M, Gehan E, Maurer HM: Preservation of the bladder in patients with rhabdomyosarcoma. J Clin Oncol 15(1):69-75, 1997
9- Merguerian PA, Agarwal S, Greenberg M, Bagli DJ, Khoury AE, McLorie GA: Outcome analysis of rhabdomyosarcoma of the lower urinary tract. J Urol 160(3 Pt 2):1191-4, 1998

Fournier's Gangrene

Necrotizing fascitis of the perineum, genitalia (scrotum and penis) and the abdominal wall is commonly known as Fournier's Gangrene (FG).  FG is an emergency condition that is very rarely seen in the pediatric age. Thrombosis of small arteries in the genital area results in ischemic injury. In children, FG is seen after circumcision, insect bites, anorectal trauma, burns, diaper rash, perianal skin abscesses, and bone marrow transplant. Most affected children are infants. Patients develop abrupt, rapidly progressive gangrenous infection with fever, perineal pain, swelling and blistering of the genital area with systemic signs of toxicity.  Infecting organisms comprise both aerobic and anaerobic organisms. Management consists of broad spectrum antimicrobial therapy, aggressive and frequent surgical debridement, and if necessary, urinary and colonic diversions to control the infection. Fortunately pediatric cases can be successfully managed with a more conservative surgical approach and have a significantly lower mortality rate than adult cases.

1- Adams JR Jr, Mata JA, Venable DD, Culkin DJ, Bocchini JA Jr, Fournier's gangrene in children. Urology 35(5):439-41, 1990
2- Martinelli G, Alessandrino EP, Bernasconi P, Caldera D, Colombo A, Malcovati L, Gaviglio MR, Vignoli GP, Borroni G, Bernasconi C: Fournier's gangrene: a clinical presentation of necrotizing fasciitis after bone marrow transplantation. Bone Marrow Transplant 22(10):1023-6, 1998
3- Efem SE: The features and aetiology of Fournier's gangrene. Postgrad Med J 70(826):568-71, 1994
4- Muelder K: Fournier's gangrene. World J Surg. 7(5):677, 1983
5- Adeyokunnu AA: Fournier's syndrome in infants. A review of cases from Ibadan, Nigeria. Clin Pediatr (Phila) 22(2):101-3, 1983

Journal Club