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  6/18/12 PSU Vol 21, 20031/6home.coqui.net/titolugo/PSU21.html#2141 PEDIATRIC SURGERY UPDATE © 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 progresspast twenty years in diagnosis and management of bilateral Wilms tumor with marked improvement in prognosis. Diagnosis of bilaterality can be confirmed eitherimaging or rarely while performing exploratory laparotomy. Some of the better prognostic factors associated with bilateral Wilms tumor are a patient age less thalower stage of the most advanced lesions, favorable histology and negative nodal involvement. Most children (96%) with stage V tumors have favorable histologbilateral Wilms tumor entails initial biopsy of both tumor masses, staging of lymph node metastasis followed by preoperative chemotherapy. Cytoreductive chemoresection reduces tumor burden and permits more renal preservation procedures. Jointly, renal salvage procedures (partial nephrectomy and enucleation) have bconserve renal parenchyma. This has facilitated the use of parenchymal-sparing operations, with the potential advantage of decreasing the incidence of end-stageinoperable tumors and extensive intravascular tumor extension can also benefit from this approach. Long-term follow-up reveals a group of children who developwhen more than 70% parenchyma is compromise. Bilateral Wilms tumor with unfavorable histology is associated with poor prognosis.   References:   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 f 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 repStudy 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 affecmaturation in children. Incidence is growing and etiology is undetermined. Diarrhea, abdominal pain, failure to thrive and weight loss are the most frequent clinicaestablished by colonoscopy or imaging studies (CT-Scan). Initial management is medical and consists of azulfidine or 5-amino salicylic acid preparations, local anmetronidazole, immunosuppressives, and enteral and/or parenteral nutrition. Indication for surgery is limited to complications of the disease process and includesperforation, abscess, severe malabsorption and growth retardation, persistent bowel obstruction, fistulas (entero-enteric and entero-urinary) and strictures. Surgelimited resection and anastomosis or stricturoplasty. Best long-term results after surgery occurs in children with disease confine to the small bowel and ileocecal rinvolvement (Panenteritis), preoperative use of 6-MP, and colonic involvement is associated with early relapse. Early relapse after surgery is also seen after failindependent of disease location as the sole indication for surgery and in children undergoing resection within one year of the onset of symptoms.   References:   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):2169-8- 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 patien2002   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 anand 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 ifingers, whereas in the foot, amputations of the hallux are most often noted. The congenital (intrauterine) band, a product of rupture amnion, produces compressioaffected limb. Multiple anomalies are associated with most ABS cases such as orbital defects, lid anomalies, lacrimal outflow obstruction, ocular malformations, wfascial cleft, cleft palate and lips. Follow-up ultrasound exams have afforded the opportunity of observing the in utero process of limb strangulation and subsequeamniotic band in a few cases. Management depends on clinical findings at birth. Prognosis depends on the severity of the abnormalities and the involvement of int References:   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):63  6/18/12 PSU Vol 21, 20032/6home.coqui.net/titolugo/PSU21.html#2141 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.rectum and colon causing delayed growth and development. In some children the terminal ileum is affected. Cause of UC remains unknown. Peak incidence is bet11 years). UC srcinates as an acute inflammation of the crypts (cryptitis) developing tissue reaction of chronicity. More than 90% children with UC have moderaClinically, the child with UC develops bloody diarrhea, abdominal cramps, anemia, fever, tachycardia, hypoalbuminemia and weight loss. Colonoscopy is diagnostiincludes restriction of milk protein from diet, parenteral nutrition, steroids, sulfasalazine, metronidazole, 6-mercaptopurine, cyclosporine and tracolimus. UC can bof the colon. Indications for surgery in UC include inability to attain growth and development under medical therapy, fulminant disease refractory to medical theraperforation and toxic megacolon. Surgical management consists of total proctocolectomy and ileal pouch anal anastomosis. The J-pouch is the simplest to construabove the dentate line after surgery produces recurrent inflammatory disease and high risk of developing carcinoma. A low risk of bladder dysfunction and impotnerves is associated with proctocolectomy. Postop complications are associated with duration of the disease and length/dosage of medication (steroids). Long-tergood in more than 90% of children with high patient satisfaction.   References:   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 Gas7- 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 348- 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 as postoperative complications. Inflamm Bowel Dis 8(5):311-6, 2002   Carotid Body Tumor Chemodectomas (tumors of chemoreceptors cells srcin) are called carotid body tumors when they occur in the carotid artery, and glomus tumors if they appear idistribution 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 abody tumors (CBT) are extra-adrenal paragangliomas diagnosed early in life which can have familial inheritance. Familial cases are of autosomic dominant, bilatemulticentric. Diagnostic work-up includes angiography, CT and MRI. Surgical excision is the treatment of choice for CBT and glomus tumors. Almost three-fourtparagangliomas are adherent to or surround adjacent arteries and cranial nerves. Their resection can result in neurovascular injury, stroke and excessive blood l References:   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.after colo-anal surgical procedures can be a troublesome condition in children. The two most common procedures associated with perianal dermatitis are pull-throdisease and to a lesser extent following repair of imperforate anus. The main factors associated with this medical problem are the muscular pseudo incontinence afrequent bowel movements, postoperative diarrhea and the alkali milieu in contact with the perianal skin. The rash can include mild redness, skin excoriation, psenodules depending on length of time of skin irritative contact. Histology shows benign epidermal hyperplasia, reactive acanthosis or psoriasiform spongiotic dermcondition can be very challenging. This includes water barrier agents, local therapy (A&D, nystatin, zinc oxide), binding agents (cholestyramine) and anti-diarrhearegress 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 peffluent of the colostomy at least two weeks prior to the intended procedure. This permits the perianal skin and proprioception reflex mechanism to adjust to theclosure of a colostomy.   References:   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, 12- 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   VOLUME 21 No 03 SEPTEMBER 2003 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 causdysphagia, especially in the young infant. Aberrant migration of thymic tissue occurs with ectopic thymus in the superior & posterior mediastinum, bases of the sk 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  6/18/12 PSU Vol 21, 20033/6home.coqui.net/titolugo/PSU21.html#2141 Occasionally, parathyroid glands have been associated with the thymic remnants. Most cases remain asymptomatic. The thymus is a paired organ which developspharyngeal pouch and descends into the superior mediastinum between the 6th and 12th weeks of fetal development. Failure of the unilateral gland to descend exectopic cervical thymic tissue. Another pathogenetic mechanism includes sequestration of accessory cervical foci of thymic tissue along the normal cervical pathwchest-x-ray without evidence of absent thymic shadows suggests this mechanism of sequestration. The most common cervical location is along the anterior bordemuscle lateral to the thyroid gland and near the carotid sheath. Malignant transformation of ectopic thymus tissue has been documented. Diagnosis is rarely donManagement consists of complete surgical excision. Symptoms due to pressure on neighboring structures are promptly eliminated after excision. Prognosis is exc References:   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. Mesenteassociated with an upper respiratory infection. Clinical presentation includes fever, leukocytosis and low abdominal pain. Mesenteric adenitis can be the result of Viruses implicated includes Epstein-Barr, Adenovirus type 3, influenza B and Coxsackie B. Bacteria associated with mesenteric adenitis includes hemolytic strepSalmonella species. The diagnosis of mesenteric adenitis is principally one of exclusion. CT-Scan can help decide whether the child has mesenteric adenitis whencan be clearly seen. Otherwise, since it can be very difficult to distinguish appendicitis from mesenteric adenitis the diagnosis is establish at surgery. Laparoscopdifferentiate appendicitis from mesenteric adenitis. After surgery the postoperative course of children with mesenteric adenitis is usually uneventful and recovery References:   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 Roen4- 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 primaresophageal atresia includes stumps at least three vertebral bodies apart (approximately 3 cm in length) or longer. Many techniques have been developed to dealproximal esophageal stump dilatation, waiting for the stump to grow spontaneously with time, use of myotomies, multistage extrathoracic esophageal elongation, ecurvature, replacement of esophagus with stomach, jejunum or colon to mention a few. Consensus between pediatric surgeons worldwide is that there is no betterchild than the native esophagus. Other authors have found that infants with an exclusive intraabdominal pouch will not reach sufficient elongation and should be ccandidate for esophageal replacement. A recent innovative technique described by Foker using external traction sutures in the esophageal ends have demonstratlengthening within 6-10 days for a true primary anastomosis to be accomplished.   References:   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 Su3- 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.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 adetermine whether we are dealing with a cystic, solid or mixed lesion. Cystic and mixed solid-cystic thyroid masses in children are most commonly benign lesionsand 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-nestablish a diagnosis. Unfortunately, needle aspiration has yield false-negative results in patients with cystic papillary carcinomas. The cysts in patients with cancnecrosis of tumors measuring between two and 4 cm in diameter. Ethanol or tetracycline sclerotherapy has been found safe and effective in the management of thfeeling are side effects of ethanol sclerotherapy. Fearfully, you could be also sclerosing a hidden papillary carcinoma. The most definitive management of thyroidThyroid lobectomy harboring the cyst should be performed to children demonstrating probable or proven cytologic malignant changes and those with recurrence oaspiration and suppressive therapy. Other factors such as size (greater than 3 cm in diameter), history of neck irradiation or family thyroid cancer, and cervical lgiven weight in favor of surgical resection.   References:   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):736-3- 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    6/18/12 PSU Vol 21, 20034/6home.coqui.net/titolugo/PSU21.html#2141 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 frobranchial arches. Cervical clefts are almost always midline in location. This rare developmental anomaly represents failure of the branchial arches to fuse in thewith 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 femacovered by an exudative thin desquamating epithelium which toughens and dries during the following weeks creating scarring and contracture. The covering epithsebaceous 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 mThe mucoid discharge is the product of ectopic salivary glands. Beneath the cleft there is a firm submucosal fibrous cord. Differential diagnosis includes branchialduct cysts (or fistula) and ectopic bronchogenic cysts. Occasionally, associated heart lesions have been described. Unlike thyroglossal duct cysts, midline cervicalassociation 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 theManagement consists of complete excision of all pathologic tissue along with the underlying cord. The wound can be closed primarily using a z-plasty technique. Econtracture and deformity of the mandible.   References:   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 estimlesions in children are eosinophilic granulomas. Eosinophilic granuloma arises from an abnormal proliferation of histiocytes. Children develop the lesions during tmanifesting pain, tenderness and swelling of the affected areas. Males are affected twice as much as females. The clinical course for most patients is benign depelesion. 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 is imperative to establish a histologic diagnosis. Management consists of observation alone, curettage, low-dose radiation therapy or intralesional injection of ste References:   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 ovary in size from microcysts to large cystlike dilatations. The tumor can compress vital structure. The close relation of this benign tumor with vital structures redsurgical excision in many cases. This has brought forth the need for alternative therapy such as sclerotherapy. Two such agents in use today for sclerosing lymphfibrin 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 afteglue or sealant, an FDA approved product, consists of a mixture of fibrinogen, thrombin, a fibrinolysis inhibitor and calcium in separate vials. When mixed they fosets into an elastic coagulum. Thrombin transforms fibrinogen into fibrin. The inhibitor prevents premature degradation of fibrin. Fibrin glue is adhesive, it is localregenerates tissue that favor the synthesis of collagen. The adhesive can effectively seal tissues surfaces and eliminate potential dead spaces. The technique forconsists in percutaneous puncture of the cyst and aspiration of the whole content followed by introduction of fibrin sealant into the cavity. The amount injected cosuctioned volume. Treatment can be repeated. Results are very encouraging with few minor side effects such as erythema and cellulitis.   References:   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):303- 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;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 durintransverse incisions are preferred. It has been demonstrated that the younger the child, the relatively larger the abdominal cavity and wall. Because of the anatoabdomen of adults and small children, the cavity of the pediatric patient resembles a horizontally oriented ellipsoid. Being barrel-shaped a transverse incision profour quadrants in young children. The younger the child, the larger proportionately is the costo-iliac space, allowing for easier lateral extension of the incision. Intransverse incision is ideal to explore all four quadrants and solve almost every surgical congenital abdominal condition. Another advantage of transverse incisiothe low incidence of fascial dehiscence, hernia formation, and evisceration of transverse incisions. Whether to use mass fascial closure or layer closure no significof complications has been identified in abdominal incisions in children. Finally, transverse closure carries better cosmetic results than vertical incision. In older ch
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