VOLUME 21, 2003

Volume 21 No 01 JULY 2003

Bilateral Wilms Tumor

Synchronous bilateral Wilms tumor, also termed Stage V Wilms disease, occurs in approximately 5% of all cases of Wilms tumor in children. Definitive progress has been made during the past twenty years in diagnosis and management of bilateral Wilms tumor with marked improvement in prognosis. Diagnosis of bilaterality can be confirmed either most commonly during initial imaging or rarely while performing exploratory laparotomy. Some of the better prognostic factors associated with bilateral Wilms tumor are a patient age less than three years at diagnosis, lower stage of the most advanced lesions, favorable histology and negative nodal involvement. Most children (96%) with stage V tumors have favorable histology. Currently, management of bilateral Wilms tumor entails initial biopsy of both tumor masses, staging of lymph node metastasis followed by preoperative chemotherapy. Cytoreductive chemotherapy before surgical resection reduces tumor burden and permits more renal preservation procedures. Jointly, renal salvage procedures (partial nephrectomy and enucleation) have been recommended to conserve renal parenchyma. This has facilitated the use of parenchymal-sparing operations, with the potential advantage of decreasing the incidence of end-stage renal disease. Patients with inoperable tumors and extensive intravascular tumor extension can also benefit from this approach.  Long-term follow-up reveals a group of children who develops insidious renal compromise when more than 70% parenchyma is compromise. Bilateral Wilms tumor with unfavorable histology is associated with poor prognosis.

1- Blute ML, Kelalis PP, Offord KP, Breslow N, Beckwith JB, D'Angio GJ: Bilateral Wilms tumor. J Urol 138(4 Pt 2):968-73, 1987
2- Shaul DB, Srikanth MM, Ortega JA, Mahour GH: Treatment of bilateral Wilms' tumor: comparison of initial biopsy and chemotherapy to initial surgical resection in the preservation of renal mass and function. J Pediatr Surg 27(8):1009-14, 1992
3- Ritchey ML, Coppes MJ: The management of synchronous bilateral Wilms tumor. Hematol Oncol Clin North Am 9(6):1303-15, 1995
4- Horwitz JR, Ritchey ML, Moksness J, Breslow NE, Smith GR, Thomas PR, Haase G, Shamberger RC, Beckwith JB: Renal salvage procedures in patients with synchronous bilateral Wilms' tumors: a report from the National Wilms' Tumor Study Group. J Pediatr Surg 31(8):1020-5, 1996
5- Ritchey ML: The role of preoperative chemotherapy for Wilms' tumor: the NWTSG perspective.
National Wilms' Tumor Study Group. Semin Urol Oncol 17(1):21-7, 1999
6- Tomlinson GS, Cole CH, Smith NM: Bilateral Wilms' tumor: a clinicopathologic review. Pathology 31(1):12-6, 1999
7- Fuchs J, Wunsch L, Flemming P, Weinel P, Mildenberger H: Nephron-sparing surgery in synchronous bilateral Wilms' tumors. J Pediatr Surg 34(10):1505-9, 1999
8- LinniPaya K, Horcher E, Lawrenz K, Rebhandl W, Zoubek A: Bilateral Wilms' tumor--surgical aspects. Eur J Pediatr Surg 11(2):99-104, 2001

Crohn's Disease

Crohn's (terminal ileitis) is a chronic, transmural inflammatory bowel disease most frequently involving the terminal ileum and proximal colon that adversely affect growth and sexual maturation in children. Incidence is growing and etiology is undetermined. Diarrhea, abdominal pain, failure to thrive and weight loss are the most frequent clinical feature. Diagnosis is established by colonoscopy or imaging studies (CT-Scan). Initial management is medical and consists of azulfidine or 5-amino salicylic acid preparations, local and systemic steroids, metronidazole, immunosuppressives, and enteral and/or parenteral nutrition. Indication for surgery is limited to complications of the disease process and includes failure of medical therapy, perforation, abscess, severe malabsorption and growth retardation, persistent bowel obstruction, fistulas (entero-enteric and entero-urinary) and strictures. Surgery can be accomplished using limited resection and anastomosis or stricturoplasty. Best long-term results after surgery occurs in children with disease confine to the small bowel and ileocecal region. Diffuse ileocolonic involvement (Panenteritis), preoperative use of 6-MP, and colonic involvement is associated with early relapse. Early relapse after surgery is also seen after failure of medical therapy independent of disease location as the sole indication for surgery and in children undergoing resection within one year of the onset of symptoms.

1- Postuma R, Moroz SP: Pediatric Crohn's disease. J Pediatr Surg 20(5):478-82, 1985
2- Davies G, Evans CM, Shand WS, Walker-Smith JA: Surgery for Crohn's disease in childhood: influence of site of disease and operative procedure on outcome. Br J Surg 77(8):891-4, 1990
3- Griffiths AM, Wesson DE, Shandling B, Corey M, Sherman PM: Factors influencing postoperative recurrence of Crohn's disease in childhood. Gut  32(5):491-5, 1991
4-Telander RL: Surgical management of Crohn's disease in children. Curr Opin Pediatr 7(3):328-34, 1995
5- Patel HI, Leichtner AM, Colodny AH, Shamberger RC: Surgery for Crohn's disease in infants and children. J Pediatr Surg 32(7):1063-7, 1997
6- Beattie RM: Therapy of Crohn's disease in childhood. Paediatr Drugs 2(3):193-203, 2000
7- Baldassano RN, Han PD, Jeshion WC, Berlin JA, Piccoli DA, Lautenbach E, Mick R, Lichtenstein GR: Pediatric Crohn's disease: risk factors for postoperative recurrence. Am J Gastroenterol 96(7):2169-76, 2001
8- Dokucu AI, Sarnacki S, Michel JL, Jan D, Goulet O, Ricour C, Nihoul-Fekete C: Indications and results of surgery in patients with Crohn's disease with onset under 10 years of age: a series of 18 patients. Eur J Pediatr Surg 12(3):180-5, 2002

Amniotic Band Syndrome

Amniotic or constricting band syndrome (ABS) refers to progressive intrauterine  amputation of fingers or limbs associated with a wide spectrum of congenital anomalies involving the trunk and craniofacial region. Incidence is one in 15,000 livebirths. Most amputations occur in the upper limb. In the hand, digital amputations are most common in the index, middle, and ring fingers, whereas in the foot, amputations of the hallux are most often noted. The congenital (intrauterine) band, a product of rupture amnion, produces compression and chronic ischemia of the affected limb. Multiple anomalies are associated with most ABS cases such as orbital defects, lid anomalies, lacrimal outflow obstruction, ocular malformations, waist constriction, clubfoot, fascial cleft, cleft palate and lips. Follow-up ultrasound exams have afforded the opportunity of observing the in utero process of limb strangulation and subsequent spontaneous lysis of an amniotic band in a few cases. Management depends on clinical findings at birth. Prognosis depends on the severity of the abnormalities and the involvement of internal organs.

1- Light TR, Ogden JA: Congenital constriction band syndrome. Pathophysiology and treatment. Yale J Biol Med 66(3):143-55, 1993
2- Froster UG, Baird PA: Amniotic band sequence and limb defects: data from a population-based study. Am J Med Genet 46(5):497-500, 1993
3- Crombleholme TM, Dirkes K, Whitney TM, Alman B, Garmel S, Connelly RJ: Amniotic band syndrome in fetal lambs. I: Fetoscopic release and morphometric outcome. J Pediatr Surg 30(7):974-8, 1995
4- Bahadoran P, Lacour JP, Terrisse A, Ortonne JP: Congenital constriction band of the trunk. Pediatr Dermatol 14(6):470-2, 1997
5- Bodamer OA, Popek EJ, Bacino C: Atypical presentation of amniotic band sequence. Am J Med Genet 22;100(2):100-2, 2001
6-  Muraskas JK, McDonnell JF, Chudik RJ, Salyer KE, Glynn L: Amniotic band syndrome with significant orofacial clefts and disruptions and distortions of craniofacial structures. J Pediatr Surg 38(4):635-638, 2003

Volume 21 No 02 AUGUST 2003

Ulcerative Colitis

Ulcerative colitis (UC) is a chronic debilitating inflammatory disease of the bowel affecting primarily the mucosa and to a lesser extent to the adjacent submucosa. UC affects mainly the rectum and colon causing delayed growth and development. In some children the terminal ileum is affected. Cause of UC remains unknown. Peak incidence is between 1.5 and 17 years (mean 11 years). UC originates as an acute inflammation of the crypts (cryptitis) developing tissue reaction of chronicity. More than 90% children with UC have moderate to severe disease. Clinically, the child with UC develops bloody diarrhea, abdominal cramps, anemia, fever, tachycardia, hypoalbuminemia and weight loss. Colonoscopy is diagnostic. Medical management includes restriction of milk protein from diet, parenteral nutrition, steroids, sulfasalazine, metronidazole, 6-mercaptopurine, cyclosporine and tracolimus. UC can be cured by surgical resection of the colon. Indications for surgery in UC include inability to attain growth and development under medical therapy, fulminant disease refractory to medical therapy, extensive rectal bleeding, perforation and toxic megacolon. Surgical management consists of total proctocolectomy and ileal pouch anal anastomosis. The J-pouch is the simplest to construct. Retention of mucosa above the dentate line after surgery produces recurrent inflammatory disease and high risk of developing carcinoma. A low risk of bladder dysfunction and impotence due to damage to pelvic nerves is associated with proctocolectomy. Postop complications are associated with duration of the disease and length/dosage of medication (steroids). Long-term function after surgery is good in more than 90% of children with high patient satisfaction.

1- Ament M, Vargas JH: Medical Therapy for Ulcerative Colitis in Childhood. Semin Pediatr Surg 3(1): 28-32, 1994
2- Fonkalsrud EW: Surgical management of ulcerative colitis in childhood. Semin Pediatr Surg 3(1):33-8, 1994
3- Nicholls S, Vieira MC, Majrowski WH, Shand WS, Savage MO, Walker-Smith JA: Linear growth after colectomy for ulcerative colitis in childhood. J Pediatr Gastroenterol Nutr 21(1):82-6, 1995
4- Rintala RJ, Lindahl H: Restorative proctocolectomy for ulcerative colitis in children--is the J-pouch
better than straight pull-through? J Pediatr Surg 31(4):530-3, 1996
5- Hyams JS, Davis P, Grancher K, Lerer T, Justinich CJ, Markowitz J: Clinical outcome of ulcerative colitis in children. J Pediatr 129(1):81-8, 1996
6- Durno C, Sherman P, Harris K, Smith C, Dupuis A, Shandling B, Wesson D, Filler R, Superina R, Griffiths A: Outcome after ileoanal anastomosis in pediatric patients with ulcerative colitis. J Pediatr Gastroenterol Nutr 27(5):501-7,1998
7- Dolgin SE, Shlasko E, Gorfine S, Benkov K, Leleiko N: Restorative proctocolectomy in children with ulcerative colitis utilizing rectal mucosectomy with or without diverting ileostomy. J Pediatr Surg 34(5):837-9, 1999
8- Fonkalsrud EW, Thakur A, Beanes S: Ileoanal pouch procedures in children.  Pediatr Surg 36(11):1689-92, 2001
9- Rintala RJ, Lindahl HG: Proctocolectomy and J-pouch ileo-anal anastomosis in children. J Pediatr Surg 37(1):66-70, 2002
10- Mahadevan U, Loftus EV Jr, Tremaine WJ, Pemberton JH, Harmsen WS, Schleck CD, Zinsmeister AR, Sandborn WJ: Azathioprine or 6-mercaptopurine before colectomy for ulcerative colitis is not associated with increased postoperative complications. Inflamm Bowel Dis 8(5):311-6, 2002

Carotid Body Tumor

Chemodectomas (tumors of chemoreceptors cells origin) are called carotid body tumors when they occur in the carotid artery, and glomus tumors if they appear in the jugular vein. Sex distribution for the two major types is equal for males and females. A few cases have been reported in the pediatric age. They are associated with neck swelling and hypertension. Carotid body tumors (CBT) are extra-adrenal paragangliomas diagnosed early in life which can have familial inheritance. Familial cases are of autosomic dominant, bilateral in location and multicentric. Diagnostic work-up includes angiography, CT and MRI. Surgical excision is the treatment of choice for CBT and glomus tumors. Almost three-fourth of CBT and cervical paragangliomas are adherent to or surround adjacent arteries and cranial nerves. Their resection can result in neurovascular injury, stroke and excessive blood loss.

1- Parry DM, Li FP, Strong LC, Carney JA, Schottenfeld D, Reimer RR, Grufferman S: Carotid body tumors in humans: genetics and epidemiology. J Natl Cancer Inst 68(4):573-8, 1982
2- Dickinson PH, Griffin SM, Guy AJ, McNeill IF: Carotid body tumour: 30 years experience. Br J Surg 73(1):14-6, 1986
3- Bishop GB Jr, Urist MM, el Gammal T, Peters GE, Maddox WA: Paragangliomas of the neck.  Arch Surg  127(12):1441-5, 1992
4- Varudkar AS, Kokandkar HR, Gumaste GG, Bhople KS, Kumbhakarna NR: Carotid body paraganglioma with coexistent pheochromocytoma in childhood. Indian J Cancer 30(3):109-12, 1993
5- Plukker JT, Brongers EP, Vermey A, Krikke A, van den Dungen JJ: Outcome of surgical treatment for carotid body paraganglioma. Br J Surg 88(10):1382-6, 2001

Perianal Dermatitis

Diaper dermatitis is a group of skin disorders resulting from attack of the skin by physical, chemical, enzymatic, and microbial factors in the diaper environment. Perineal or diaper dermatitis after colo-anal surgical procedures can be a troublesome condition in children. The two most common procedures associated with perianal dermatitis are pull-through for Hirschsprung's disease and to a lesser extent following repair of imperforate anus. The main factors associated with this medical problem are the muscular pseudo incontinence associated with the procedure, frequent bowel movements, postoperative diarrhea and the alkali milieu in contact with the perianal skin. The rash can include mild redness, skin excoriation, pseudo verrucous papules and nodules depending on length of time of skin irritative contact. Histology shows benign epidermal hyperplasia, reactive acanthosis or psoriasiform spongiotic dermatitis. Managing this condition can be very challenging. This includes water barrier agents, local therapy (A&D, nystatin, zinc oxide), binding agents (cholestyramine) and anti-diarrhea medication. The lesions regress when the irritating factor is removed. A novel approach to reduce the perianal diaper rash associated after closure of colostomy in infants is to paint the perianal skin with the liquid effluent of the colostomy at least two weeks prior to the intended procedure. This permits the perianal skin and proprioception reflex mechanism  to adjust to the external skin milieu after closure of a colostomy.

1- Rodriguez Cano L, Garcia-Patos Briones V, Pedragosa Jove R, Castells Rodellas A: Perianal pseudo verrucous papules and nodules after surgery for Hirschsprung disease. J Pediatr 125(6 Pt 1):914-6, 1994
2- Bourrat E, Vaquin C, Prigent F, Rybojad M: Perianal papulonodular dermatitis in Hirschsprung disease. Ann Dermatol Venereol 123(9):549-51, 1996
3- Goldberg NS, Esterly NB, Rothman KF, Fallon JD, Cropley TG, Szaniawski W, Glassman M: Perianal pseudo verrucous papules and nodules in children. Arch Dermatol 128(2):240-2, 1992
4- Hanlon M, Cofone E: Patient with frequent liquid stools resulting in a chemical dermatitis and a perianal ulcer. J Wound Ostomy Continence Nurs 23(3):174-7, 1996
5- Berg RW: Etiology and pathophysiology of diaper dermatitis. Adv Dermatol 3:75-98, 1988


Ectopic Thymus

Finding ectopic thymus in a cervical mass is a rare diagnosis found sporadically in infants and children. Cervical thymic lesions can either be symptomless or cause severe dyspnea and dysphagia, especially in the young infant. Aberrant migration of thymic tissue occurs with ectopic thymus in the superior & posterior mediastinum, bases of the skull, tracheal bifurcation, and cervical region. Aberrant ectopic thymic tissue can present as either a solid or cystic mass in the neck of the child. Cervical location (85%) and cystic nature (70%) is found more commonly. Occasionally, parathyroid glands have been associated with the thymic remnants. Most cases remain asymptomatic. The thymus is a paired organ which develops from the 3rd and 4th pharyngeal pouch and descends into the superior mediastinum between the 6th and 12th weeks of fetal development. Failure of the unilateral gland to descend explains the finding of the ectopic cervical thymic tissue. Another pathogenetic mechanism includes sequestration of accessory cervical foci of thymic tissue along the normal cervical pathway of descend. A normal chest-x-ray without evidence of absent thymic shadows suggests this mechanism of sequestration.  The most common cervical location is along the anterior border of the sternocleidomastoid muscle lateral to the thyroid gland and near the carotid sheath.  Malignant transformation of ectopic thymus tissue has been documented. Diagnosis is rarely done preoperatively. Management consists of complete surgical excision. Symptoms due to pressure on neighboring structures are promptly eliminated after excision. Prognosis is excellent.

1- Tovi F, Mares AJ: The aberrant cervical thymus. Embryology, Pathology, and clinical implications. Am J Surg 136(5):631-7, 1978
2- Spigland N, Bensoussan AL, Blanchard H, Russo P: Aberrant cervical thymus in children: three case reports and review of the literature. J Pediatr Surg 25(11):1196-9, 1990
3- Gimm O, Krause U, Wessel H, Finke R, Dralle H: Ectopic intrathyroidal thymus diagnosed as a solid thyroid lesion: case report and review of the literature. J Pediatr Surg 32(8):1241-3, 1997
4- Krysta MM, Gorecki WJ, Miezynski WH: Thymic tissue manifesting as a posterior mediastinal mass in two children. J Pediatr Surg 33(4):632-4, 1998
5- Terzakis G, Louverdis D, Vlachou S, Anastasopoulos G, Dokianakis G, Tsikou-Papafragou A: Ectopic thymic cyst in the neck. J Laryngol Otol 114(4):318-20, 2000
6- Bernig T, Weigel S, Mukodzi S, Beck JF, Wiersbitzky H, von Suchodoletz H, Warzok R: Ectopic cervical thymus in a 12-year-old boy: a case report. Pediatr Hematol Oncol 17(8):713-7, 2000
7- Saggese D, Ceroni Compadretti G, Cartaroni C: Cervical ectopic thymus: a case report and review of the literature. Int J Pediatr Otorhinolaryngol 66(1):77-80, 2002

Mesenteric Adenitis

Mesenteric lymphadenitis is the condition most commonly  mimicking acute appendicitis resulting in a high rate of negative appendectomies in children. Mesenteric adenitis is frequently associated with an upper respiratory infection. Clinical presentation includes fever, leukocytosis and low abdominal pain. Mesenteric adenitis can be the result of a viral or bacterial infection. Viruses implicated includes Epstein-Barr, Adenovirus type 3, influenza B and Coxsackie B. Bacteria associated with mesenteric adenitis includes hemolytic streptococci, Yersinia and Salmonella species. The diagnosis of mesenteric adenitis is principally one of exclusion. CT-Scan can help decide whether the child has mesenteric adenitis when the lymph nodes aggregates can be clearly seen. Otherwise, since it can be very difficult to distinguish appendicitis from mesenteric adenitis the diagnosis is establish at surgery. Laparoscopy can also be useful to differentiate appendicitis from mesenteric adenitis. After surgery the postoperative course of children with mesenteric adenitis is usually uneventful and recovery is rapid.

1- Alvear DT, Kain TM 3rd: Suppurative mesenteric lymphadenitis, a forgotten clinical entity: report of two cases. J Pediatr Surg 10(6):969-70, 1975
2-  Achong DM, Oates E, Harris B: Mesenteric lymphadenitis depicted by indium 111-labeled white blood cell imaging. J Pediatr Surg 28(12):1550-2, 1993
3- Macari M, Hines J, Balthazar E, Megibow A: Mesenteric adenitis: CT diagnosis of primary versus secondary causes, incidence, and clinical significance in pediatric and adult patients. AJR Am J Roentgenol 178(4):853-8, 2002
4- Arda IS, Ergin F, Varan B, Demirhan B, Aslan H, Ozyaylali I: Acute abdomen caused by Salmonella typhimurium infection in children.  J Pediatr Surg 36(12):1849-52, 2001
5- Gilmore OJ, Browett JP, Griffin PH, Ross IK, Brodribb AJ, Cooke TJ, Higgs MJ, Williamson RC: Appendicitis and mimicking conditions. A prospective study. Lancet 2(7932):421-4, 1975

Esophageal Elongation

One unresolved surgical problem in pediatrics deals with babies born with esophageal atresia and a long segment between the esophageal stumps enabling primary anastomosis. Long gap esophageal atresia includes stumps at least three vertebral bodies apart  (approximately 3 cm in length) or longer. Many techniques have been developed to deal with this problem such as proximal esophageal stump dilatation, waiting for the stump to grow spontaneously with time, use of myotomies, multistage extrathoracic esophageal elongation, elongation of the lesser curvature, replacement of esophagus with stomach, jejunum or colon to mention a few. Consensus between pediatric surgeons worldwide  is that there is no better esophageal substitute in a child than the native esophagus. Other authors have found that infants with an exclusive intraabdominal pouch will not reach sufficient elongation and should be considered early as a candidate for esophageal replacement. A recent innovative technique described by Foker using external traction sutures in the esophageal ends have demonstrated to produce sufficient lengthening within 6-10 days for a true primary anastomosis to be accomplished.

1- Kimura K, Soper RT: Multistaged extrathoracic esophageal elongation for long gap esophageal atresia. J Pediatr Surg 29(4):566-8, 1994
2- Fernandez MS, Gutierrez C, Ibanez V, Lluna J, Barrios JE, Vila JJ, Garcia-Sala C: Long-gap esophageal atresia: reconstruction preserving all portions of the esophagus by Scharli's technique. Pediatr Surg Int 14(1-2):17-20, 1998
3- Maksoud-Filho JG, Goncalves ME, Tannuri U, Maksoud JG: An exclusively intraabdominal distal esophageal segment prevents primary delayed anastomosis in children with pure esophageal atresia.  J Pediatr Surg 37(11):1521-5, 2002
4-  Boyle EM Jr, Irwin ED, Foker JE: Primary repair of ultra-long-gap esophageal atresia: results without a lengthening procedure. Ann Thorac Surg 57(3):576-9, 1994
5- Foker JE, Linden BC, Boyle EM Jr, Marquardt C: Development of a true primary repair for the full spectrum of esophageal atresia. Ann Surg 226(4):533-41, 1997
6- Gaglione G, Tramontano A, Capobianco A, Mazzei S: Foker's technique in oesophageal atresia with double fistula: a case report. Eur J Pediatr Surg 13(1):50-3, 2003
7- Al-Qahtani AR, Yazbeck S, Rosen NG, Youssef S, Mayer SK: Lengthening technique for long gap esophageal atresia and early anastomosis. J Pediatr Surg 38(5):737-9, 2003

VOLUME 21 No 04 OCTOBER 2003

Thyroid Cysts

Pediatric thyroid nodules are a source of concern for physicians as they can harbor a malignancy. Initial work-up should include neck ultrasonography to define anatomic location and determine whether we are dealing with a cystic, solid or mixed lesion. Cystic and mixed solid-cystic thyroid masses in children are most commonly benign lesions thought to arise from necrosis and degeneration of thyroid nodules. In a few cases (8%) a malignancy can present as a cystic lesion. Next step in management of a cystic thyroid lesion is fine-needle aspiration cytology to establish a diagnosis. Unfortunately, needle aspiration has yield false-negative results in patients with cystic papillary carcinomas. The cysts in patients with cancer appear to originate from necrosis of tumors measuring between two and 4 cm in diameter. Ethanol or tetracycline sclerotherapy has been found safe and effective in the management of thyroid cysts. Pain and drunken feeling are side effects of ethanol sclerotherapy. Fearfully, you could be also sclerosing a hidden papillary carcinoma. The most definitive management of thyroid cysts is surgical excision. Thyroid lobectomy harboring the cyst should be performed to children demonstrating probable or proven cytologic malignant changes and those with recurrence of the cyst after serial aspiration and suppressive therapy.  Other factors such as size (greater than 3 cm in diameter), history of neck irradiation or family thyroid cancer, and cervical lymphadenopathy should be given weight in favor of surgical resection.

1- Lugo-Vicente H, Ortiz VN, Irizarry H, Camps JI, Pagan V: Pediatric thyroid nodules: management in the era of fine needle aspiration. J Pediatr Surg 33(8):1302-5, 1998
2- Desjardins JG, Khan AH, Montupet P, Collin PP, Leboeuf G, Polychronakos C, Simard P, Boisvert J, Dube LJ: Management of thyroid nodules in children: a 20-year experience. J Pediatr Surg 22(8):736-9, 1987
3- Muller N, Cooperberg PL, Suen KC, Thorson SC: Needle aspiration biopsy in cystic papillary carcinoma of the thyroid. AJR Am J Roentgenol 144(2):251-3, 1985
4- Hammer M, Wortsman J, Folse R: Cancer in cystic lesions of the thyroid. Arch Surg 117(8):1020-3, 1982
5- Rosen IB, Provias JP, Walfish PG: Pathologic nature of cystic thyroid nodules selected for surgery by needle aspiration biopsy. Surgery 100(4):606-13, 1986
6- Sarda AK, Bal S, Dutta Gupta S, Kapur MM: Diagnosis and treatment of cystic disease of the thyroid by aspiration. Surgery 103(5):593-6, 1988
7- Yoskovitch A, Laberge JM, Rodd C, Sinsky A, Gaskin D: Cystic thyroid lesions in children. J Pediatr Surg 33(6):866-70, 1998

Cervical Clefts

Congenital clefts can rarely occur in the face or the neck of a child. Fascial cleft, also known as congenital macrostomia, is a transverse deformity developing from the first and second branchial arches. Cervical clefts are almost always midline in location. This rare developmental anomaly represents failure of the branchial arches to fuse in the midline and presents at birth with a ventral midline defect of the skin of the neck extending for a variable distance from the chin to the suprasternal notch. Most cases reported are white females. Inially the cleft is covered by an exudative thin desquamating epithelium which toughens and dries during the following weeks creating scarring and contracture. The covering epithelium lacks sweat glands, sebaceous glands or hair follicles. The cranial end of the cleft has a nipple-like protuberance while the caudal end presents as an opening to a sinus tract where mucoid secretions can be seen. The mucoid discharge is the product of ectopic salivary glands. Beneath the cleft there is a firm submucosal fibrous cord. Differential diagnosis includes branchial cleft anomaly, thyroglossal duct cysts (or fistula) and ectopic bronchogenic cysts. Occasionally, associated heart lesions have been described. Unlike thyroglossal duct cysts, midline cervical cleft has no anatomical association with the hyoid bone. In a few cases a bony prominence of the mandible is palpable and seen as a spur in x-ray films. The spur is due to traction of the fibrous cord on the bone. Management consists of complete excision of all pathologic tissue along with the underlying cord. The wound can be closed primarily using a z-plasty technique. Early surgery avoids neck contracture and deformity of the mandible.

1- Maschka DA, Clemons JE, Janis JF: Congenital midline cervical cleft. Case report and review. Ann Otol Rhinol Laryngol 104(10 Pt 1):808-11, 1995
2- Hirokawa S, Uotani H, Okami H, Tsukada K, Futatani T, Hashimoto I: A case of congenital midline cervical cleft with congenital heart disease. J Pediatr Surg 38(7):1099-101, 2003
3- Ayache D, Ducroz V, Roger G, Garabedian EN: Midline cervical cleft. Int J Pediatr Otorhinolaryngol 20;40(2-3):189-93, 1997
4- van der Staak FH, Pruszczynski M, Severijnen RS, van de Kaa CA, Festen C: The midline cervical cleft. J Pediatr Surg 26(12):1391-3, 1991
5- Eastlack JP, Howard RM, Frieden IJ: Congenital midline cervical cleft: case report and review of the English language literature. Pediatr Dermatol 17(2):118-22, 2000
6- Bergevin MA, Sheft S, Myer C 3rd, McAdams AJ: Congenital midline cervical cleft. Pediatr Pathol 9(6):731-9, 1989
7- Gargan TJ, McKinnon M, Mulliken JB: Midline cervical cleft. Plast Reconstr Surg 76(2):225-9, 1985

Eosinophilic Granuloma

Langerhans cell histiocytosis, also known as eosinophilic granuloma, is a localized benign tumor seen in bones, skull, ribs, spine, pelvis and scalp area. It is estimated that 7% of all scalp lesions in children are eosinophilic granulomas. Eosinophilic granuloma arises from an abnormal proliferation of histiocytes. Children develop the lesions during the first decade of life manifesting pain, tenderness and swelling of the affected areas. Males are affected twice as much as females. The clinical course for most patients is benign depending on the location of the lesion. Simple X-ray of the lesion will show a lytic, well-defined "punched-out" lesion in bone with marginal reactive sclerosis. CT-Scan will describe the extent of the disease process. Biopsy is imperative to establish a histologic diagnosis. Management consists of observation alone, curettage, low-dose radiation therapy or intralesional injection of steroids.

1- Appling D, Jenkins HA, Patton GA: Eosinophilic granuloma in the temporal bone and skull. Otolaryngol Head Neck Surg 91(4):358-65, 1983
2- Ruge JR, Tomita T, Naidich TP, Hahn YS, McLone DG: Scalp and calvarial masses of infants and children. Neurosurgery 22(6 Pt 1):1037-42, 1988
3- Greis PE, Hankin FM: Eosinophilic granuloma. The management of solitary lesions of bone. Clin Orthop (257):204-11, 1990
4- Martinez-Lage JF, Poza M, Cartagena J, Vicente JP, Biec F, de las Heras M: Solitary eosinophilic granuloma of the pediatric skull and spine. The role of surgery. Childs Nerv Syst 7(8):448-51, 1991
5- Plasschaert F, Craig C, Bell R, Cole WG, Wunder JS, Alman BA: Eosinophilic granuloma. A different behaviour in children than in adults. J Bone Joint Surg Br 84(6):870-2, 2002

Volume 21 No 05 NOVEMBER 2003

Lymphangiomas - Fibrin Seal

Lymphangiomas are congenital cystic tumors developing in the neck, axilla, chest and trunk. Characteristically they are composed of multiple sacs or loculation of lymphatic fluid. Cysts can vary in size from microcysts to large cystlike dilatations. The tumor can compress vital structure. The close relation of this benign tumor with vital structures reduces the chances of complete surgical excision in many cases. This has brought forth the need for alternative therapy such as sclerotherapy. Two such agents in use today for sclerosing lymphangiomas are OK-432 and fibrin glue sealant. OK-432 has not yet been approved by FDA. In 1988 fibrin glue successfully sealed a postoperative persistent lymphatic drainage in a chid after persistent drainage. Fibrin glue or sealant, an FDA approved product, consists of a mixture of fibrinogen, thrombin, a fibrinolysis inhibitor and calcium in separate vials. When mixed they form a viscous solution that sets into an elastic coagulum. Thrombin transforms fibrinogen into fibrin. The inhibitor prevents premature degradation of fibrin. Fibrin glue is adhesive, it is locally hemostatic and it regenerates tissue that favor the synthesis of collagen. The adhesive can effectively seal tissues surfaces and eliminate potential dead spaces. The technique for fibrin seal of lymphangiomas consists in percutaneous puncture of the cyst and aspiration of the whole content followed by introduction of fibrin sealant into the cavity. The amount injected consist of 10 to 15% of the suctioned volume. Treatment can be repeated. Results are very encouraging with few minor side effects such as erythema and cellulitis.

1- Giberson WG, McCarthy PM, Kaufman BH: Fibrin glue for the treatment of persistent lymphatic drainage. J Pediatr Surg 23(12):1188-9, 1988
2- Castanon Garcia-Alix M, Margarit Mallol J, Martin Hortiguela ME, Salarich de Arbell J: [Fibrin adhesive: a new therapeutic alternative in the treatment of cystic lymphangioma]. An Esp Pediatr 38(4):304-6, 1993
3- Gutierrez San Roman C, Barrios J, Lluna J, Menor F, Poquet J, Ruiz S: Treatment of cervical lymphangioma using fibrin adhesive. Eur J Pediatr Surg 3(6):356-8, 1993
4- Castanon Garcia-Alix M, Margarit Mallol J, Garcia Baglietto A, Martin Hortiguera ME, Morales Fochs L: [Cystic lymphangioma: treatment with adhesive fibrin tissue. Follow-up study]. Cir Pediatr Jan;9(1):36-9, 1996
5- Castanon M, Margarit J, Carrasco R, Vancells M, Albert A, Morales L: Long-term follow-up of nineteen cystic lymphangiomas treated with fibrin sealant. J Pediatr Surg 34(8):1276-9, 1999
6- Honig JF, Merten HA: Surgical removal of intra- and extraoral cavernous lymphangiomas using intraoperative-assisted intralesional fibrin glue injections. J Craniofac Surg 11(1):42-5, 2000

Abdominal Incisions

Pediatric surgeons utilize several types of abdominal incision to approach different surgical problems in newborns, infants and children. In most children and during the first five years of life transverse incisions are preferred. It has been demonstrated that the younger the child, the relatively larger the abdominal cavity and wall. Because of the anatomical differences between the abdomen of adults and small children, the cavity of the pediatric patient resembles a horizontally oriented ellipsoid. Being barrel-shaped a transverse incision provides better exposure to all four quadrants in young children. The younger the child, the larger proportionately is the costo-iliac space, allowing for easier lateral extension of the incision. In babies a supraumbilical transverse incision is ideal to explore all four quadrants and solve almost every surgical congenital abdominal condition. Another advantage of transverse incision over longitudinal incision is the low incidence of fascial dehiscence, hernia formation, and evisceration of transverse incisions. Whether to use mass fascial closure or layer closure no significant statistic difference in rate of complications has been identified in abdominal incisions in children. Finally, transverse closure carries better cosmetic results than vertical incision. In older children a midline incision is still the incision of choice in conditions that require rapid intra-abdominal entry such as trauma.

1- Gauderer MW: A rationale for routine use of transverse abdominal incisions in infants and children. J Pediatr Surg 16(4 Suppl 1):583-6, 1981
2- Ellis H, Coleridge-Smith PD, Joyce AD: Abdominal incisions--vertical or transverse? Postgrad Med J 60(704):407-10, 1984
3- Chana RS, Saxena VC, Agarwal A: A prospective study of closure techniques of abdominal incisions in infants and children. J Indian Med Assoc 91(3):59-61, 1993
4- Waldhausen JH, Davies L: Pediatric postoperative abdominal wound dehiscence: transverse versus vertical incisions. J Am Coll Surg 190(6):688-91, 2000
5- Grantcharov TP, Rosenberg J: Vertical compared with transverse incisions in abdominal surgery. Eur J Surg 167(4):260-7, 2001


Granular cell tumor (GCT), also known as myoblastoma, is a lesion of unknown etiology and histogenesis rarely found in children. GTC most commonly appear in the skin or soft tissue of the trunk and extremity during the life of a child. Other sites described includes the oral cavity (tongue, gingiva, trachea, larynx), esophagus, breast, perineum and parotid gland. A preference for the Negro race has been identified in several series. Most GCT are benign single lesions. A few patients develop multiple benign lesions. GCT tends to recur locally. The cytopathologic features of GCT are distinctive enough to allow a correct diagnosis using FNA cytology. The malignant variant of granular cell tumor is a high-grade sarcoma with a high rate of metastases and a short survival. Management of benign GCT consists of local excision leaving margins free of tumor. Very rarely GCT has been reported to metastasize to the lung.

1- Apisarnthanarax P: Granular cell tumor. An analysis of 16 cases and review of the literature. J Am Acad Dermatol 5(2):171-82, 1981
2- Morrison JG, Gray GF Jr, Dao AH, Adkins RB Jr: Granular cell tumors. Am Surg 53(3):156-60, 1987
3- Fanburg-Smith JC, Meis-Kindblom JM, Fante R, Kindblom LG: Malignant granular cell tumor of soft tissue: diagnostic criteria and clinicopathologic correlation. Am J Surg Pathol 22(7):779-94, 1998
4- Billeret Lebranchu V: [Granular cell tumor. Epidemiology of 263 cases]. Arch Anat Cytol Pathol 47(1):26-30, 1999
5- Mallik MK, Das DK, Francis IM, al-Abdulghani R, Pathan SK, Sheikh ZA, Luthra UK: Fine needle aspiration cytology diagnosis of a cutaneous granular cell tumor in a 7-year-old child. A case report. Acta Cytol 45(2):263-6, 2001

Volume 21 No 06 DECEMBER 2003

Recurrent Inguinal Hernias

Inguinal hernias continue to be the most common congenital pathology in children needing surgical repair early in life. Approximately 1-3% of children have an inguinal hernia. The incidence is higher in premature babies (3-5%). Almost all inguinal hernias in children are the indirect type (99%). The few direct hernias in children are the result of previous surgery or inguinal floor disruption. Management of inguinal hernias in infants and children is straightforward: outpatient surgery after diagnosis for most cases. The procedure consists of high ligation of the hernial sac. Incidence of developing a recurrent inguinal hernia is around 0.8%. Most recurrences occur two years after the initial surgery. Several factors play a role in increasing hernia recurrence. These are: 1) Missed sac or inadequate ligation of the indirect sac. 2) Children operated for incarcerated inguinal hernias since tissue is more friable and edematous at the time of surgery. 3) Infection of the wound after hernia repair predisposing to tissue breakdown and a higher recurrence rate. 4) Connective tissue disorders (Hurler, Ehlers-Danlos, etc.). 5) Growth failure and poor nutrition. 6) Prematurity has been identified as a co-morbid factor in hernia recurrence. 7) Children hernia repair done by non-pediatric surgeons is also a risk factor for recurrence. 8) Conditions causing increase intra-abdominal pressure (VP shunts, posterior urethral valves, bladder exstrophy repair, weight lifting and respiratory conditions) are also related to higher rate of recurrence in children. Repair of the recurrent hernia is done through the inguinal scar or using laparoscopy. The sac is mobilized and ligated. Laparoscopic repair of recurrent inguinal hernia has the advantage of passing through a virgin field reducing damage to vas or vessels and allowing inspection of the area with direct purse string repair of the defect.

1- Grosfeld JL, Minnick K, Shedd F, West KW, Rescorla FJ, Vane DW: Inguinal hernia in children: factors affecting recurrence in 62 cases. J Pediatr Surg 26(3):283-7, 1991
2- Steinau G, Treutner KH, Feeken G, Schumpelick V: Recurrent inguinal hernias in infants and children. World J Surg 19(2):303-6, 1995
3- Esposito C, Montupet P: Laparoscopic treatment of recurrent inguinal hernia in children. Pediatr Surg Int 14(3):182-4, 1998
4- Perlstein J, Du Bois JJ: The role of laparoscopy in the management of suspected recurrent pediatric hernias. J Pediatr Surg 35(8):1205-8, 2000
5- Meier AH, Ricketts RR: Surgical complications of inguinal and abdominal wall hernias. Semin Pediatr Surg 12(2):83-88, 2003

Incarcerated Inguinal Hernias

Incarceration and strangulation are the most dreaded complications of inguinal hernias in children. Incarceration refers to viscera (bowel, ovaries, bladder) that protrudes through the inguinal defect and cannot return back to its anatomical position without manipulation or surgery. Bowel incarceration in infants with inguinal hernia is a notable cause of intestinal obstruction in this age group. Strangulation is the ischemic effect caused on the trapped viscera by the incarcerated defect. Incarceration occurs in almost one-third of inguinal hernias. It is more common in children less than one year of age and males. With prolonged incarceration there can also occur testicular infarction. In infant girls the normal anatomy is altered when an ovary is trapped in a hernia sac, and these changes make torsion more likely. This risk warrants treating the asymptomatic irreducible ovary as any other incarcerated hernia with urgency. Incarceration increases the rate of complications, is seen in a younger population of children and increases hospital stay. Children with incarcerated hernia should have a trial of manual reduction followed by prompt repair within the next five to seven days to avoid re-incarceration.

1- Palmer BV: Incarcerated inguinal hernia in children. Ann R Coll Surg Engl 60(2):121-4, 1978
2- Puri P, Guiney EJ, O'Donnell B: Inguinal hernia in infants: the fate of the testis following incarceration. J Pediatr Surg 19(1):44-6, 1984
3- Boley SJ, Cahn D, Lauer T, Weinberg G, Kleinhaus S: The irreducible ovary: a true emergency. J Pediatr Surg 26(9):1035-8, 1991
4- Stephens BJ, Rice WT, Koucky CJ, Gruenberg JC: Optimal timing of elective indirect inguinal hernia repair in healthy children: clinical considerations for improved outcome. World J Surg 16(5):952-6, 1992
5- Stylianos S, Jacir NN, Harris BH: Incarceration of inguinal hernia in infants prior to elective repair. J Pediatr Surg 28(4):582-3, 1993
6- Gahukamble DB, Khamage AS: Early versus delayed repair of reduced incarcerated inguinal hernias in the pediatric population. J Pediatr Surg 31(9):1218-20, 1996
7- Niedzielski J, Kr l R, Gawlowska A: Could incarceration of inguinal hernia in children be prevented? Med Sci Monit 9(1):CR16-8, 2003

Acute Chest Syndrome

Acute chest syndrome (ACS) is a pneumonia like illness, the most frequent cause of hospitalization and morbidity in children with Sickle Cell Disease (SCD). ACS can also occur after surgical procedures in children with SCD. Clinically ACS includes fever, cough, chest pain, leukocytosis and a new infiltrate in chest films. The most common etiologic factors for ACS include infection, pulmonary fat embolism (infarcted bone marrow) and hypoventilation. History of pulmonary disease is a predictive factor for the ACS. The risk of ACS is inversely proportional to age with the highest incidence in small children. Abdominal surgery is a high risk situation to develop postoperative ACS with characteristic basilar atelectasis after either open or laparoscopic surgery. Preoperative transfusion reduces pulmonary complications and is beneficial. Management of ACS encompasses antibiotics, hydration, oxygenation, transfusion, analgesia, bronchodilators, supportive respiratory therapy and antiinflammatory agents.

1- Ware RE, Filston HC: Surgical management of children with hemoglobinopathies. Surg Clin North Am 72(6):1223-36, 1992
2- Castro O, Brambilla DJ, Thorington B, Reindorf CA, Scott RB, Gillette P, Vera JC, Levy PS: The acute chest syndrome in sickle cell disease: incidence and risk factors. The Cooperative Study of Sickle Cell Disease. Blood 15;84(2):643-9, 1994
3- Dreyer ZE: Chest infections and syndromes in sickle cell disease of childhood. Semin Respir Infect 11(3):163-72, 1996
4- Vichinsky EP, Neumayr LD, Earles AN, Williams R, Lennette ET, Dean D, Nickerson B, Orringer E, McKie V, Bellevue R, Daeschner C, Manci EA: Causes and outcomes of the acute chest syndrome in sickle cell disease. National Acute Chest Syndrome Study Group. N Engl J Med 342(25):1855-65, 2000
5- Wales PW, Carver E, Crawford MW, Kim PC: Acute chest syndrome after abdominal surgery in children with sickle cell disease: Is a laparoscopic approach better? J Pediatr Surg 36(5):718-21, 2001
6- Siddiqui AK, Ahmed S: Pulmonary manifestations of sickle cell disease. Postgrad Med J 79(933):384-90, 2003

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