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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 progress

past twenty ye ars in diagnosis and management of bilateral Wilms tumor with marked improvement in prognosis. Diagnosis of bilaterality can be confirmed either

imaging or rarely while performing exploratory laparotomy. Some of the bette r prognostic factors associated with bilateral Wilms tumor are a patient age less tha

lower stage of the most advanced lesions, favorable histology and negative nodal involvement. Most children (96%) with stage V tumors have favorable histolog

bilateral Wilms tumor entails initial biopsy of both tumor masses, staging of lymph node metastas is followed by preoperative chemotherapy. Cytoreductive chemo

rese ction reduces tumor burden and permits more renal preservation procedures . Jointly, renal salvage procedures (partial nephrectomy and enucleation) have b

conserve renal parenchyma. This has facilitated the use of parenchymal-sparing operations, with the potential advantage of decreasing the incidence of end-stage

inoperable tumors and extensive intravascular tumor extension can also benefit from this approach. Long-term follow-up reve als a group of children who deve lop

when 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, 19872- 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

19923- Ritchey ML, Coppes MJ: The management of sy nchron ous bilateral Wilms tumor. Hematol Oncol Clin North Am 9(6):1303-15, 19954- 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 rep

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 as pects . Eur J Ped iatr 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 affec

maturation in children. Incidence is growing and etiology is undete rmined. Diarrhea, abdominal pain, failure to thrive and weight loss are the most frequent clinicaes tablished by colonoscopy or imaging studies (CT-Scan). Initial management is medical and consists of azulfidine or 5-amino salicylic acid preparations, local an

metronidazole, immunosuppressives, and enteral and/or parenteral nutrition. Indication for surgery is limited to complications of the dise ase proces s and includes

perforation, absce ss , seve re malabsorption and growth retardation, persistent bowel obstruction, fistulas (entero-enteric and entero-urinary) and strictures . Surge

limited resection and anastomosis or stricturoplasty. Best long-term results after surgery occurs in children with disease confine to the small bowel and ileocecal r

involvement (Panenteritis), preoperative use of 6-MP, and colonic involvement is associated with early relapse. Early relapse after surgery is also seen after fail

independent of disease location as the sole indication for surgery and in children undergoing resection within one year of the onset of symptoms.

References:

1- Post uma R, Moroz SP: Pediatric Crohn's diseas e. J Ped iatr 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, Wes son DE, Shandling B, Corey M, Sherman PM: Factors influencing pos toperat ive recurrence of Crohn's diseas e in childhood. Gut 32(5):491-5, 1991

4-Telander RL: Surgical management of Crohn's diseas e in children. Curr Opin Pediatr 7(3):328-34, 1995

5- Patel HI, Leichtner A M, Colodny AH, Shamberger RC: Surgery for Crohn's diseas e in infants and children. J Pediatr Surg 32(7):1063-7, 1997

6- Beattie RM: Therapy of Crohn's d isease in childhoo d. Paediatr Drugs 2(3):193-203, 20007- 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 patien

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 an

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 i

fingers, whereas in the foot, amputations of the hallux are most often noted. The congenital (intrauterine) band, a product of rupture amnion, produces compress io

affected limb. Multiple anomalies are associated with most ABS cases such as orbital defects, lid anomalies, lacrimal outflow obstruction, ocular malformations, w

fascial cleft, cleft palate and lips. Follow-up ultrasound exams have afforded the opportunity of observing the in utero process of limb strangulation and subseque

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 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, Whitn ey TM, Alman B, Garmel S, Connelly RJ: Amniotic band syn drome in fetal lambs. I: Fet oscopic release and morphometric outco me. J Pediatr Surg 30(7):974-8, 1995

4- Bahadoran P, Lacou r JP, Terrisse A, Ortonn e JP: Congenital con striction b and o f the t runk. Pediatr Dermatol 14(6):470-2, 1997

5- Bodamer OA, Popek EJ, Bacino C: Atyp ical present ation of amniotic band s equen ce. Am J Med Genet 22;100(2):100-2, 20016- Muras kas JK, McDonnell JF, Chudik RJ, Salyer KE, Glynn L: Amniotic band s yndrome with significant orofacial clefts and dis ruptions and dis tortions of craniofacial structures . J Pediatr Surg 38(4):63

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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 deve lopment. In some children the terminal ileum is affe cted. Cause of UC remains unknown. Peak incidence is bet

11 years). UC originates as an acute inflammation of the crypts (cryptitis) developing tissue reaction of chronicity. More than 90% children with UC have modera

Clinically, the child with UC develops bloody diarrhea, abdominal cramps, anemia, fever, tachycardia, hypoalbuminemia and weight loss. Colonoscopy is diagnosti

includes restriction of milk protein from diet, parenteral nutrition, ste roids, sulfasalazine, metronidazole, 6-mercaptopurine, cyclosporine and tracolimus. UC can b

of the colon. Indications for surgery in UC include inability to attain growth and development under medical therapy, fulminant disease refractory to medical thera

perforation and toxic megacolon. Surgical management consists of total proctocolectomy and ileal pouch anal anastomosis. The J-pouch is the s implest to construabove the dentate line after surgery produces recurrent inflammatory disease and high risk of developing carcinoma. A low risk of bladder dysfunction and impot

nerves is associated with proctocolectomy. Postop complications are associated with duration of the disease and length/dosage of medication (steroids). Long-ter

good 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 Su rg 3(1): 28-32, 1994

2- Fonkalsrud EW: Surgical management of ulcerative colitis in childhood. Semin Ped iatr Surg 3(1):33-8, 1994

3- Nicholls S, Vieira MC, Majrowski WH, Shan d W S, Savage MO, Walker-Smith JA : Linear g rowth after colectomy for ulcerative co litis 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

bette r than s traight pull-throug h? J Pediatr Surg 31(4):530-3, 1996

5- Hyams JS, Davis P, Grancher K, Lerer T, Jus tinich CJ, Markowitz J: Clinical out come of u lcerative colitis in ch ildren. 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 Gas

7- Dolgin SE, Shlasko E, Gorfine S, Benkov K, Leleiko N: Restorat ive procto colectomy in children with ulcerative colitis utilizing rectal mucosectomy with or withou t diverting ileostomy. J Pediatr Su rg 34

8- Fonkalsrud EW, Thakur A, Beanes S: Ileoanal po uch p rocedures in children. Pediatr Surg 36(11):1689-92, 20019- Rintala RJ, Lindahl HG: Proctoco lectomy and J-pouch ileo-anal anas tomosis in children. J Ped iatr Surg 37(1):66-70, 200210- 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

pos toperat ive 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 i

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 a

body tumors (CBT) are extra-adrenal paragangliomas diagnosed early in life which can have familial inheritance. Familial case s are of autosomic dominant, bilate

multicentric. Diagnostic work-up includes angiography, CT and MRI. Surgical excision is the treatment of choice for CBT and glomus tumors. Almost three-fourt

paragangliomas are adherent to or surround adjacent arteries and cranial nerves. Their resection can res ult in neurovascular injury, stroke and exces sive blood l

References:1- Parry DM, Li FP, Strong LC, Carney JA , Schot tenfeld D, Reimer RR, Grufferman S: Carotid body tumors in humans : genet ics an d ep idemiology. J Natl Cancer Ins t 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- Bish op GB Jr, Urist MM, el Gammal T, Peters GE, Madd ox WA: Paragan gliomas of th e neck. Arch Surg 127(12):1441-5, 1992

4- Varudkar AS, Kokandkar HR, Gumaste GG, Bhople KS, Kumbhakarna NR: Carotid bo dy p aragang lioma with coexistent pheochromocytoma in childhood. Indian J Cancer 30(3):109-12, 1993

5- Plukker JT, Brongers EP, Vermey A, Krikke A, v an den Dungen JJ: Outcome of s urgical treatment for ca rotid bod y parag anglioma. 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-thro

disease and to a lesser extent following repair of imperforate anus. The main factors associated with this medical problem are the muscular pseudo incontinence a

frequent bowel movements, postoperative diarrhea and the alkali milieu in contact with the perianal skin. The rash can include mild redness, skin excoriation, pse

nodules depending on length of time of skin irritative contact. Histology s hows benign epidermal hyperplasia, reactive acanthosis or psoriasiform spongiotic derm

condition 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 p

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

closure 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, 1

2- Bourrat E, Vaquin C, Prigent F, Rybo jad M: Perianal pap ulonod ular dermatitis in Hirschs prung diseas e. An n Dermatol Venereol 123(9):549-51, 1996

3- Goldberg NS, Esterly NB, Rothman KF, Fallon JD, Cropley TG, Szaniawski W, Glassman M: Perianal pseu do v errucous papu les and nodu les in ch ildren. 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 patho phys iology of d iaper dermatitis. Adv Dermatol 3:75-98, 1988

VOLUME 21 No 03 SEPTEMBER 2003

Ectopic Thymus

Finding ectopic thymus in a ce rvical mass is a rare diagnosis found sporadically in infants and children. Cervical thymic les ions can either be symptomless or caus

dysphagia, especially in the young infant. Aberrant migration of thymic tiss ue 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

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Occasionally, parathyroid glands have been associated with the thymic remnants. Most cases remain asymptomatic. The thymus is a paired organ which deve lops

pharyngeal pouch and descends into the superior mediastinum between the 6th and 12th weeks of fetal development. Failure of the unilateral gland to descend ex

ectopic cervical thymic tissue. Another pathogenetic mechanism includes sequestration of accessory cervical foci of thymic tissue along the normal cervical pathw

chest-x-ray without evidence of absent thymic shadows suggests this mechanism of se questration. The most common cervical location is along the anterior borde

muscle lateral to the thyroid gland and near the carotid sheath. Malignant transformation of ectopic thymus tissue has been documented. Diagnosis is rarely don

Management 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, Pat hology, 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, Wes sel H, Finke R, Dralle H: Ectopic intrat hyroidal thy mus diagn osed as a so lid thyroid lesion: cas e report and rev iew of the literature. J Pediatr Surg 32(8):1241-3, 1997

4- Krysta MM, Gorecki WJ, Miezynski WH: Thymic tiss ue manifesting as a pos terior mediastinal mass in two children. J Ped iatr Surg 33(4):632-4, 1998

5- Terzakis G, Louve rdis D, Vlachou S, Anas tasopou los G, Dokianakis G, Tsikou-Papafragou A: Ectopic thy mic cys t in th e neck. J Laryng ol Otol 114(4):318-20, 2000

6- Bernig T, Weigel S, Mukodzi S, Beck JF, Wiersbitzky H, von Suchodo letz H, Warzok R: Ectopic cervical thymus in a 12-year-old boy: a case report. Ped iatr Hematol Oncol 17(8):713-7, 2000

7- Saggese D, Ceroni Compadretti G, Cartaroni C: Cervical ectop ic thymus : a case report and review of the literature. In t J Ped iatr 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. Mesente

associated 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 strep

Salmonella species. The diagnosis of mesenteric adenitis is principally one of exclusion. CT-Scan can help decide whether the child has mesenteric adenitis when

can be clearly seen. Otherwise, since it can be very difficult to distinguish appendicitis from mesenteric adenitis the diagnosis is establish at surgery. Laparoscop

differentiate 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: Suppu rative mesent eric lymphad enitis, a forg otten clinical entity: report of two case s. J Pediatr Surg 10(6):969-70, 1975

2- Acho ng DM, Oates E, Harris B: Mes enteric lymphaden itis depicted by indium 111-labeled white blood cell imaging. J Ped iatr 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 Roen

4- Arda IS, Ergin F, Varan B, Demirhan B, As lan H, Ozyaylali I: Acute abd omen caus ed b y Salmonella typhimurium infection in children. J Pediatr Su rg 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 cond itions. A prosp ective s tudy . 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 primar

esophageal atresia includes stumps at least three ve rtebral bodies apart (approximately 3 cm in length) or longer. Many techniques have been deve loped to deal

proximal esophageal stump dilatation, waiting for the stump to grow spontaneously with time, use of myotomies, multistage e xtrathoracic esophageal elongation, e

curvature, replacement of esophagus with stomach, jejunum or colon to mention a few. Consensus between pediatric surgeons worldwide is that there is no bette r

child than the native esophagus. Other authors have found that infants with an exclusive intraabdominal pouch will not reach sufficient elongation and should be c

candidate for esophageal replacement. A recent innovative technique described by Foker using external traction sutures in the esophageal ends have demonstrat

lengthening 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 Su

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.

4- Boyle EM Jr, Irwin ED, Foker JE: Primary repair of ultra-long-gap esophageal atres ia: results without a lengthen ing proced ure. Ann Thorac Su rg 57(3):576-9, 1994

5- Foker JE, Linden BC, Boyle EM Jr, Marquardt C: Development o f a true primary repair for t he full spectrum of es ophag eal atresia. A nn Su rg 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, 20037- 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 a

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

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-n

establish a diagnosis. Unfortunately, needle aspiration has yield false-negative results in patients with cystic papillary carcinomas. The cysts in patients with canc

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 th

feeling are side effects of ethanol sclerotherapy. Fearfully, you could be also sclerosing a hidden papillary carcinoma. The most definitive management of thyroid

Thyroid lobectomy harboring the cyst should be performed to children demonstrating probable or proven cytologic malignant changes and those with recurrence o

aspiration and suppressive therapy. Other factors such as s ize (greater than 3 cm in diameter), history of neck irradiation or family thyroid cancer, and cervical l

given weight in favor of surgical resection.

References:

1- Lugo-Vicente H, Ortiz VN, Irizarry H, Camps JI, Pag an V: Pediatric thyroid nodu les: management in the era of fine need le asp iration. J Pediatr Surg 33(8):1302-5, 1998

2- Desjardins JG, Khan AH, Mo ntup et P, Collin PP, Leboeu f G, Polychron akos C, Simard P, Boisvert J, Dube LJ: Management of t hyroid n odules in children: a 20-year experience. J Ped iatr Surg 22(8):736-

3- Muller N, Cooperb erg PL, Suen KC, Thors on SC: Needle asp iration biopsy in cy stic p apillary carcinoma of t he th yroid. AJR Am J Roent genol 144(2):251-3, 1985

4- Hammer M, Worts man J, Folse R: Cancer in cys tic lesions of the thyroid. A rch 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

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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- Yoskov itch A, Laberge JM, Rodd C, Sinsky A, Gaskin D: Cyst ic thyroid les ions 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 fro

branchial arches. Cervical clefts are almost always midline in location. This rare developmental anomaly represents failure of the branchial arches to fuse in the

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 fema

covered by an exudative thin desquamating epithelium which toughens and dries during the following wee ks creating scarring and contracture. The covering epith

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 m

The mucoid discharge is the product of e ctopic salivary glands. Be neath the cleft there is a firm submucosal fibrous cord. Differential diagnosis includes branchial

duct cysts (or fistula) and ectopic bronchogenic cysts. Occasionally, associated heart lesions have been described. Unlike thyroglossal duct cysts, midline cervical

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

Management consists of complete excis ion of all pathologic tissue along with the underlying cord. The wound can be closed primarily using a z-plasty technique. E

contracture and deformity of the mandible.

References:

1- Maschka DA, Clemons JE, Jan is JF: Congen ital midline cervical cleft. Case report and rev iew. Ann Oto l Rhinol Laryngol 104(10 Pt 1):808-11, 1995

2- Hirokawa S, Uotani H, Okami H, Tsukada K, Fut atani T, Hashimoto I: A cas e of co ngenital midline cerv ical cleft with co ngen ital 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, Fest en C: The midline cervical cleft. J Ped iatr Surg 26(12):1391-3, 1991

5- Eastlack JP, Howard RM, Frieden IJ: Congenital midline cervical cleft: cas e repo rt and review of th e English lang uage literature. Pediatr Dermatol 17(2):118-22, 2000

6- Bergevin MA, Sheft S, Myer C 3rd, McAdams AJ: Congenital midline cervical cleft. Pediatr Path ol 9(6):731-9, 1989

7- Gargan TJ, McKinnon M, Mulliken JB: Midline cervical cleft. Plas t Recon str 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 estim

lesions in children are eos inophilic granulomas. Eosinophilic granuloma arises from an abnormal proliferation of histiocytes. Children develop the lesions during t

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 depe

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

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: Eosino philic granuloma in the temporal bo ne an d s kull. Otolaryngol Head Neck Surg 91(4):358-65, 1983

2- Ruge JR, Tomita T, Naidich TP, Hah n YS, McLone DG: Scalp and ca lvarial mass es of infants and children. Neurosurgery 22(6 Pt 1):1037-42, 1988

3- Greis PE, Hankin FM: Eosinoph ilic granuloma. The management of solitary les ions of bo ne. 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, 19915- Plassch aert F, Craig C, Bell R, Cole WG, Wund er JS, Alman BA: Eosinophilic granuloma. A different b ehaviour in children than in adults . J Bone Jo int Surg Br 84(6):870-2, 2002

Volume 21 No 05 NOVEMBER 2003

Lymphangiomas - Fibrin Seal

Lymphangiomas are congenital cystic tumors deve loping in the neck, axilla, chest and trunk. Characteristically they are composed of multiple sacs or loculation o

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 red

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 lymph

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 afte

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 fo

sets into an elastic coagulum. Thrombin transforms fibrinogen into fibrin. The inhibitor prevents premature degradation of fibrin. Fibrin glue is adhesive, it is local

regenerates tissue that favor the synthesis of collagen. The adhesive can effectively seal tissues surfaces and eliminate potential dead spaces. The technique for

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 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 t he trea tment of pe rsistent lymphatic drainag e. J Pediatr Su rg 23(12):1188-9, 1988

2- Castanon Garcia-Alix M, Marg arit Mallol J, Martin Hortiguela ME, Salarich d e Arbell J: [Fibrin adh esive: a n ew therapeutic alternative in the t reatment of cyst ic lymphangioma]. An Esp Ped iatr 38(4):30

3- Gutierrez San Roman C, Barrios J, Lluna J, Menor F, Poque t J, Ruiz S: Treatment of cervical lymphangioma us ing 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 anato

abdomen of adults and small children, the cavity of the pediatric patient resembles a horizontally oriented ellipsoid. Be ing barrel-shaped a transverse incision pro

four quadrants in young children. The younger the child, the larger proportionately is the cos to-iliac space, allowing for easier lateral extension of the incision. In

transverse incision is ideal to explore all four quadrants and solve almost every surgical congenital abdominal condition. Another advantage of transverse incisio

the low incidence of fascial dehiscence, hernia formation, and evisceration of transverse incisions. Whether to use mass fascial closure or layer closure no signific

of complications has bee n identified in abdominal incisions in children. Finally, transverse closure carries better cosmetic results than vertical incision. In older ch

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still the incision of choice in conditions that require rapid intra-abdominal entry such as trauma.

References:

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: Ab dominal incisions--vertical or trans verse? Pos tgrad 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- Grantcharo v TP, Rosenberg J: Vertical compared with trans verse incisions in abdominal surg ery. Eur J Surg 167(4):260-7, 2001

Myoblastoma

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

trunk and extremity during the life of a child. Other sites described includes the oral cavity (tongue, gingiva, trachea, larynx), esophagus, breast, perineum and pa

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 loc

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 wit

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 t

References:

1- Apisarnt hanarax P: Granular cell tumor. An an alysis o f 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 gran ular cell tumor of so ft tiss ue: diagno stic criteria and clinicopat hologic correlation. A m J Surg Patho l 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.

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 in

is higher in premature babies (3-5%). Almost all inguinal hernias in children are the indirect type (99%). The fe w direct hernias in children are the res ult of previo

disruption. Management of inguinal hernias in infants and children is s traightforward: outpatient surgery after diagnosis for most case s. The procedure consists o

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

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 edemat

Infection of the wound after hernia repair predisposing to tissue breakdown and a higher recurrence rate. 4) Connective tissue disorders (Hurler, Ehlers-Danlos,

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 ris

Conditions causing increase intra-abdominal pressure (VP shunts, posterior urethral valves, bladder exs trophy repair, weight lifting and respiratory conditions) ar

of recurrence in children. Re pair of the recurrent hernia is done through the inguinal scar or using laparoscopy. The sac is mobilized and ligated. Laparoscopic rep

hernia has the advantage of pass ing through a virgin field reducing damage to vas or vesse ls and allowing inspection of the area with direct purse string repair of

References:

1- Grosfeld JL, Minnick K, Shedd F, Wes t KW, Rescorla FJ, Vane DW: Inguinal hernia in ch ildren: factors affecting recurren ce in 62 case s. J Pediatr Surg 26(3):283-7, 1991

2- Steinau G, Treutne r KH, Feeken G, Schumpelick V: Recurrent ingu inal hernias in infants and children. Wo rld 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 ingu inal and abdominal wall hernias. Semin Ped iatr 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 p

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 i

age group. Strangulation is the ischemic effect caused on the trapped viscera by the incarcerated defect. Incarceration occurs in almost one-third of inguinal hernichildren 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

hernia sac, and these changes make torsion more likely. This risk warrants treating the asymptomatic irreducible ovary as any other incarcerated hernia with urg

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 r

repair within the next five to seven days to avoid re-incarceration.

References:

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 infant s: the fate of the t estis following incarce ration. J Pediatr Su rg 19(1):44-6, 1984

3- Boley SJ, Cahn D, Lauer T, Weinb erg G, Kleinhaus S: The irreducible ov ary: a t rue emergency . J Pediatr Surg 26(9):1035-8, 1991

4- Stephens BJ, Rice WT, Koucky CJ, Gruenb erg JC: Optimal timing of elective indirect inguinal hernia repa ir in healthy ch ildren: clinical cons iderations for improved out come. World J Su rg 16(5):952-6, 1

5- Stylianos S, Jacir NN, Harris BH: Incarceration of inguinal hernia in infants prior to elective repair. J Ped iatr Surg 28(4):582-3, 1993

6- Gahukamble DB, Khamage AS: Early versu s d elayed repa ir of reduced incarcerated inguinal hernias in the pediatric pop ulation. J Pediatr Su rg 31(9):1218-20, 1996

7- Niedzielski J, Kr l R, Gawlowska A: Could incarcerat ion of inguinal hernia in children be preven ted? 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). AC

procedures in children with SCD. Clinically ACS includes feve r, cough, chest pain, leukocytosis and a new infiltrate in chest films. The most common etiologic fact

infection, pulmonary fat embolism (infarcted bone marrow) and hypoventilation. History of pulmonary disease is a predictive factor for the ACS. The risk of ACS

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age with the highest incidence in small children. Abdominal surgery is a high risk situation to develop postoperative ACS with characteristic basilar atelectasis aft

laparoscopic surgery. Preoperative transfusion reduces pulmonary complications and is beneficial. Management of ACS encompasse s antibiotics, hydration, oxyg

analgesia, bronchodilators, s upportive respiratory therapy and antiinflammatory agents.

References:

1- Ware RE, Filston HC: Surgical management of ch ildren with hemoglobinop athies. Surg Clin North Am 72(6):1223-36, 19922- 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

1994

3- Dreyer ZE: Ches t infections and s yndromes in sickle cell disease of childhood . Semin Resp ir Infect 11(3):163-72, 19964- Vichinsky EP, Neumayr LD, Earles AN, Williams R, Lennette ET, Dean D, Nickerson B, Orringer E, McKie V, Bellevue R, Daeschn er C, Manci EA: Causes and outco mes of th e acut e ches t syndrome in

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 s ickle cell disea se. Post grad Med J 79(933):384-90, 2003

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