VOLUME 17, 2001

Volume 17 No 01 JULY 2001

Hidradenitis Suppurativa

Hidradenitis suppurativa refers to a chronic relapsing cutaneous inflammation of the apocrine glands of the body. Chronic hidradenitis suppurativa can affect, in order of preference, the axilla, perineum, scrotum, inguinal, scalp, palms of the hand, soles of the feet and the submammary region. Hidradenitis suppurativa usually manifests itself after puberty (androgen-dependent disorder). Females are more commonly affected than males. The inflammatory process begins as a local occluding spongiform infundibulo-folliculitis of a sweat gland with subsequent rupture and secondary bacterial infection. Symptoms include pain, swelling, purulent discharge, and pruritus of the affected region. Associated medical conditions include diabetes and obesity. Initial management consists of general hygienic measures with antibiotics, antiandrogens and estrogens. Surgery is needed when the condition is at an advanced stage with cellulitis and scarring. Nonoperative treatment is disappointing. Total excision of all apocrine-bearing axillary tissue with primary closure is the treatment of choice. Operative treatment can be safely accomplished even when draining sinuses are present. Recurrence results from inadequate excision or an unusually wide distribution of apocrine glands, but physical factors such as obesity, local pressure, and skin maceration play a role. Radical surgery gives good symptomatic control of severe hidradenitis suppurativa of the axilla, inguinoperineal, and perianal regions but is less satisfactory for submammary disease.

1- Harrison BJ, Mudge M, Hughes LE: Recurrence after surgical treatment of hidradenitis suppurativa. Br Med J 294(6570):487-9, 1987
2- Lewis F, Messenger AG, Wales JK: Hidradenitis suppurativa as a presenting feature of premature adrenarche. Br J Dermatol 129(4):447-8, 1993
3- Boer J, Weltevreden EF: Hidradenitis suppurativa or acne inversa. A clinicopathological study of early lesions. Br J Dermatol 135(5):721-5, 1996
4- Wenzel FG, Horn TD: Nonneoplastic disorders of the eccrine glands. J Am Acad Dermatol 38(1):1-17, 1998
5- Mengesha YM, Holcombe TC, Hansen RC: Prepubertal hidradenitis suppurativa: two case reports and review of the literature. Pediatr Dermatol 16(4):292-6, 1999
6- Naimer SA, Zvulunov A, Ben-Amitai D, Landau M: Plantar hidradenitis in children induced by exposure to wet footwear. Pediatr Emerg Care 16(3):182-3, 2000

Desmoid Tumors

Desmoid fibromatosis (DF) is a benign, locally aggressive tumor, with a strong propensity for infiltrative growth and local recurrence.  More than 50% of the tumors develop within the first five years of life as an asymptomatic, firm, solid mass. DF can be found on the head and neck, upper or lower extremities, the abdomen or the trunk. The lower extremities are the most frequent sites of manifestation. The most frequent types are the infantile fibromatosis (head, neck, shoulder, upper arm or thigh), extra-abdominal fibromatosis (chest wall, back and thigh)  and fibromatosis colli (neck). Superficial lesions tend to be slow growing, small and rarely involve deep structures. Deep-seated DF tends to be faster growing, larger and involves deeper structures (extra-abdominal). Except fibromatosis colli that tends to regress spontaneously, infantile and extra-abdominal DF is best managed by gross total resection achieving negative margins unless tumor excision is either particularly dangerous or likely to result in significant physical handicap. Radiation or chemotherapy is most often used with recurrent disease or as an alternative to mutilating surgery. Adjuvant radiation therapy improves local control. DF have no tendency to metastasize. It is believed they should be treated as low-grade malignancies with documentation of histologic margins and close clinical follow-up.

1- Ayala AG, Ro JY, Goepfert H, Cangir A, Khorsand J, Flake G: Desmoid fibromatosis: a clinicopathologic study of 25 children. Semin Diagn Pathol 3(2):138-50, 1986
2- Lopez R, Kemalyan N, Moseley HS, Dennis D, Vetto RM: Problems in diagnosis and management of desmoid tumors. Am J Surg 159(5):450-3, 1990
3- Humar A, Chou S, Carpenter B: Fibromatosis in infancy and childhood: the spectrum. J Pediatr Surg 28(11):1446-50, 1993
4- Faulkner LB, Hajdu SI, Kher U, La Quaglia M, Exelby PR, Heller G, Wollner N: Pediatric desmoid tumor: retrospective analysis of 63 cases. J Clin Oncol 13(11):2813-8, 1995
5- Lewis JJ, Boland PJ, Leung DH, Woodruff JM, Brennan MF: The enigma of desmoid tumors. Ann Surg 229(6):866-72, 1999
6- Merchant TE, Nguyen D, Walter AW, Pappo AS, Kun LE, Rao BN: Long-term results with radiation therapy for pediatric desmoid tumors. Int J Radiat Oncol Biol Phys 47(5):1267-71, 2000

Dieulafoy Lesion

First described by a French surgeon in 1897, Dieulafoy's lesion (DL) also known as cirsoid aneurysm, is a rare cause of massive upper gastrointestinal hemorrhage. Most DL occurs due to an abnormal submucosa artery in the stomach particularly along the lesser curvature in the region supplied by the left gastric artery and within six centimeters of the gastroesophageal junction. They have also been reported to occur in the jejunum and rectum. The problem with this lesion is that bleeding is intermittent and massive. With fiberoptic endoscopy the source of upper and lower gastrointestinal bleeding can be identified in 80 to 85% of children. The endoscopic  appearance of DL may range from a pinpoint dot (two to 5 mm), clot, or tortuous vessel, to blood oozing or spurting from normal mucosa. There is no surrounding mucosal ulceration. Other times diagnosis is made at operation, as endoscopy and arteriography fail to identify the lesion. Repeated endoscopies might be needed. Management consists of endoscopic sclerotherapy injection and laser photocoagulation. If this fails, surgery follows. Simple ligation of the bleeding dot is all that is required to control the hemorraghe.

1- McClave SA, Goldschmid S, Cunningham JT, Boyd WP: Dieulafoy's cirsoid aneurysm of the duodenum. Dig Dis Sci 33(7):801-5, 1988
2- Tooson JD, Marsano LS, Gates LK: Pediatric rectal Dieulafoy's lesion. Am J Gastroenterol 90(12):2232-3, 1995
3- Driver CP, Bruce J: An unusual cause of massive gastric bleeding in a child. J Pediatr Surg 32(12):1749-50, 1997
4- Skok P: Endoscopic hemostasis in exulceratio simplex-Dieulafoy's disease hemorrhage: a review of 25 cases. Endoscopy 30(7):590-4, 1998
5-Stockwell JA, Werner HA, Marsano LS: Dieulafoy's lesion in an infant: a rare cause of massive gastrointestinal bleeding. J Pediatr Gastroenterol Nutr 31(1):68-70, 2000

Volume 17 No 02 AUGUST 2001

Jugular Phlebectasia

A mass that appears in the neck upon straining (Valsalva maneuver), coughing, sneezing or crying may be the result of a laryngocele, jugular phlebectasia or superior mediastinal tumor. Jugular phlebectasia (also known as venous congenital cyst, venous aneurysm, venous ectasia or essential venous dilatation) refers to an isolated abnormal fusiform or saccular dilatation of the internal jugular vein and it usually present with a swelling in the right posterior triangle of the neck. Most  patients are children, boys being more twice as often affected as girls. Phlebectasia may affect any vein in the neck, especially in this sequence: internal jugular, external jugular, anterior jugular and the superficial communicans. Jugular phlebectasia is an asymptomatic benign condition whose etiology is unknown. Absence of a wide mediastinum or air in the mass on simple chest films eliminates a mediastinal tumor or laryngocele respectively. Non-invasive diagnosis of jugular phlebectasia can be achieved using ultrasonography combined with Doppler flow imaging and spiral computerized tomography scan with contrast. No treatment is indicated for this benign self-limiting condition, except for the few patients who complain of symptoms (feeling of constriction, choking, bluish discoloration, thrombosis, discomfort during physical activity or tongue pain) and require surgical removal of the affected vein. Surgical removal for cosmetic purposes alone consists of a unilateral excision of the internal or external jugular vein, a procedure that produces no gross side-effects.

1- Bowdler DA, Singh SD: Internal jugular phlebectasia. Int J Pediatr Otorhinolaryngol 12(2):165-71, 1986
2- Nwako FA, Agugua NE, Udeh CA, Osuorji RI: Jugular phlebectasia. J Pediatr Surg 24(3):303-5, 1989
3- Yokomori K, Kubo K, Kanamori Y, Takemura T, Yamamoto T: Internal jugular phlebectasia in two siblings: manometric and histopathologic studies of the pathogenesis. J Pediatr Surg 25(7):762-5, 1990
4-Balik E, Erdener A, Taneli C, Mevsim A, Sayan A, Yuce G: Jugular phlebectasia in children. Eur J Pediatr Surg 3(1):46-7, 1993
5- Pul N, Pul M: External jugular phlebectasia in children. Eur J Pediatr 154(4):275-6, 1995
6- Sander S, Elicevik M, Unal M, Vural O: Jugular phlebectasia in children: is it rare or ignored? J Pediatr Surg 34(12):1829-32, 1999

Ganglion Cysts

Ganglion cysts are synovial cysts that appear in the wrist or foot after minor trauma or stress. Most occur in the dorsal wrist area. Presence of a colorless to pale-yellow gelatinous material in the aspirate is pathognomonic of ganglion cysts. FNA smears are monotonous showing abundant mucoid material, single cells resembling histiocytes, a few tight clusters of cells, some collagen fibers, and some red blood cells with altered shapes. Serial microscopic studies have shown evidence of a one way valvelike system between the affected joint and the ganglion. Diagnosis is physical, though ultrasound findings (cysts with a mean diameter of 1.4 cm and projection into the joint or tendon) are of help. Management consists of excision. Recurrence rates are high ranging between 10 and 35%. Ganglion cysts in the volar aspect of the wrist have a higher incidence of postoperative complications (nerve and radial artery damage). Intralesional injection of hyaluronidase has been found a safe, fast, well accepted and cost-effective alternative to surgical excision.

1- Oertel YC, Beckner ME, Engler WF: Cytologic diagnosis and ultrastructure of fine-needle aspirates of ganglion cysts. Arch Pathol Lab Med 110(10):938-42, 1986
2- Paivansalo M, Jalovaara P: Ultrasound findings of ganglions of the wrist. Eur J Radiol 13(3):178-80, 1991
3- Otu AA: Wrist and hand ganglion treatment with hyaluronidase injection and fine needle aspiration: a tropical African perspective. J R Coll Surg Edinb 37(6):405-7, 1992
4- Gundes H, Cirpici Y, Sarlak A, Muezzinoglu S: Prognosis of wrist ganglion operations. Acta Orthop Belg 66(4):363-7, 2000

Anal Warts

Warts in the perianal region of prepubertal infant or child most commonly are the result of human papilloma virus (HPV) infestation. Main clinical manifestations of anal warts are cauliflower-like Condylomata Acuminata that usually involves moist surfaces, keratotic and smooth papular warts usually on dry surfaces, and subclinical flat warts that can be found on any mucosal or cutaneous surface. Mode of transmission can occur from an infected maternal birth canal (perinatally), by autoinoculation or heteroinoculation from common hand warts, through sexual abuse and possibly indirect transmission via fomites. Often, the mode of transmission is unknown. HPV-DNA typing is a useful technique that helps identify the genital types involved (6 or 11, 16 or 18) alerting the physician to proceed with a careful assessment for sexual abuse. Predisposing factors for anal warts include social problems, lack of hygiene, promiscuity, diabetes and ammoniacal erythema. The appearance of the papilloma is diagnostic. Management includes cytotoxic agents (podophyllin, podophyllotoxin and fluorouracil), destructive procedures (scissor excision, cryotherapy, electrocautery, and laser photocoagulation) and recently topical interferon hydrogel. In case of transmission by sexual abuse child protection is warranted.

1- Armstrong DK, Handley JM: Anogenital warts in prepubertal children: pathogenesis, HPV typing and management. Int J STD AIDS 8(2):78-81, 1997
2- Derksen DJ: Children with condylomata acuminata. J Fam Pract 34(4):419-23, 1992
3- Martinon Sanchez F, Martinon Sanchez ML, Farina Guerrero P, Michelena del Riego M, Mato Prada J: Condyloma acuminatum in children. An Esp Pediatr 28(1):15-8, 1988
4- Handley JM, Maw RD, Bingham EA, Horner T, Bharucha H, Swann A, Lawther H, Dinsmore WW: Anogenital warts in children. Clin Exp Dermatol 18(3):241-7, 1993
5- Obalek S, Jablonska S, Favre M, Walczak L, Orth G: Condylomata acuminata in children: frequent association with human papillomaviruses responsible for cutaneous warts. J Am Acad Dermatol 23(2 Pt 1):205-13, 1990
6- Boyd AS: Condylomata acuminata in the pediatric population. Am J Dis Child 144(7):817-24, 1990

Volume 17 No 03 SEPTEMBER 2001

Duodenal Duplications

Bowel duplications are congenital anomalies that occur anywhere in the gastrointestinal tract from mouth to anus. Duplications are spherical or tubular in shape, located in or adjacent to the wall of part of the gastrointestinal tract (dorsal or mesenteric side of the native bowel), have smooth muscle in their walls, and are lined by alimentary tract mucosa. Duodenal duplications (DD) comprise 5 to 10% of all bowel duplications. DD usually present with a palpable abdominal mass, obstructive symptoms (vomiting), bleeding (from heterotopic gastric mucosa), perforation or pancreatitis. Most develop symptoms during the first two years of life. DD are usually attached by a common muscularis posteromedial to the duodenum, partly embedded in the pancreas with no communication with the lumen. Diagnosis can be established by the finding of "gut signature" on ultrasound - the hyperechoic inner layer produced by the mucosa surrounded by a hypoechoic outer layer caused by smooth muscle. CT, ERCP and MR-Cholangiography can identify location, size, associated structures and bile ductal anatomy. Management of duodenal duplications depends on the association of this lesion to the native duodenum, pancreas and biliary system. Preop or intraoperative cholangiography should be done whenever the biliary system is involved. Preferred management is complete excision whenever possible, but mucosal stripping or marsupialization are acceptable alternatives.

1- Macpherson RI: Gastrointestinal tract duplications: clinical, pathologic, etiologic, and radiologic considerations. Radiographics 13(5):1063-80, 1993
2- Stern L, Warner BW: Gastrointestinal Duplications. Seminars Pediatr Surg 9(3): 135-140, 2000
3- Lyer CP, Mahour GH: Duplications of the Alimentary Tract in Infants and Children. J Pediatr Surg 30(9): 1267-1270, 1995
4- Siddiqui AM, Shamberger RC, Filler RM, Perez-Atayde AR, Lillehei CW: Enteric Duplications of the Pancreatic Head: Definite Management by Local Resection. J Pediatr Surg 33(7): 1117-1121, 1998
5- Lad RJ, Fitzgerald P, Jacobson K: An unusual cause of recurrent pancreatitis: duodenal duplication cyst. Can J Gastroenterol 14(4):341-5, 2000
6- Stringer MD, Spitz L, Abel R, Kiely E, Drake DP, Agrawal M, Stark Y, Brereton RJ: Management of alimentary tract duplication in children. Br J Surg 82(1):74-8, 1995


Gynecomastia refers to abnormal breast enlargement in males. In children, gynecomastia can be classified as simple pubertal, pathological, general obesity and pectoral muscle hypertrophy. Most cases of gynecomastia are simple pubertal associated to a transient or permanent disturbance in steroid hormone physiology occurring when the male breast is exposed to a decreased ratio of androgen to estrogen. Pubertal gynecomastia can be managed non-operatively since breast enlargement start one year after the onset of puberty and subside two years later. Pathological gynecomastia is associated with drug use (steroids, digitalis, spironolactone, marijuana), chronic liver disease or malignancy (Leydig cell tumor of the testis). General obesity is associated with fat deposition surrounding breast tissue that lends itself to weight reduction. Diagnosis is by history and physical exam. Routine endocrine work-up is not cost effective. Persistent pain, uncertain diagnosis and cosmetic reasons (embarrassment or distress) are the major reasons for operation. Subcutaneous mastectomy through a periareolar incision gives the best cosmetic results. Common late postoperative sequelae are inverted areolae, hypopigmentation and hypoesthesia of the areolar region.

1- Mahoney CP: Adolescent gynecomastia. Differential diagnosis and management. Pediatr Clin North Am 37(6):1389-404, 1990
2- West KW, Rescorla FJ, Scherer LR 3rd, Grosfeld JL: Diagnosis and treatment of symptomatic breast masses in the pediatric population. J Pediatr Surg 30(2):182-6, 1995
3- Park AJ, Lamberty BG: Gynaecomastia: have Webster's lessons been ignored?. J R Coll Surg Edinb 43(2):89-92, 1998
4- Bowers SP, Pearlman NW, McIntyre RC Jr, Finlayson CA, Huerd S: Cost-effective management of gynecomastia. Am J Surg 176(6):638-41, 1998
5- Mellor SG, McCutchan JD: Gynaecomastia and occult Leydig cell tumour of the testis. Br J Urol 63(4):420-2, 1989
6- Persichetti P, Berloco M, Casadei RM, Marangi GF, Di Lella F, Nobili AM: Gynecomastia and the complete circumareolar approach in the surgical management of skin redundancy. Plast Reconstr Surg 107(4):948-54, 2001

Idiopathic Bowel Perforation

Bowel perforation causing pneumoperitoneum during the neonatal period is usually associated with necrotizing enterocolitis (leading cause), atresias, meconium ileus, and Hirschsprung's disease. Other times they are iatrogenic (misplaced tubes, vigorous resuscitation and drug-related). There is a group of newborns with spontaneous isolated bowel perforation and no evidence of the above disease disorder termed idiopathic bowel perforation (IBP). IBP can occur in the stomach, small, large bowel, and appendix. The perforation is single, small, on the antimesenteric wall, measuring less than one cm with almost minimal surrounding bowel necrosis. A suggested etiologic factor consists of ischemic necrosis secondary to a very localized vascular accident. Diagnosis is made after finding pneumoperitoneum is found in simple abdominal films. After resuscitation, hydration and antibiotherapy, management is surgical. Gastric perforations are sutured and in proximal bowel perforations resection and anastomosis can be done. In distal bowel perforations ganglionic segment should be exteriorized. Hirschsprung's disease should be excluded by multiple seromuscular frozen section and rectal biopsy. IBP carries a good prognosis with a survival rate above 80%.

1- Bax NM, Pearse RG, Dommering N, Molenaar JC: Perforation of the appendix in the neonatal period. J Pediatr Surg 15(2):200-2, 1980
2- Zamir O, Shapira SC, Udassin R, Peleg O, Arad I, Nissan S: Gastrointestinal perforations in the neonatal period. Am J Perinatol 5(2):131-3, 1988
3- Zamir O, Goldberg M, Udassin R, Peleg O, Nissan S, Eyal F: Idiopathic gastrointestinal perforation in the neonate. J Pediatr Surg 23(4):335-7, 1988
4- Weinberg G, Kleinhaus S, Boley SJ: Idiopathic intestinal perforations in the newborn: an increasingly common entity. J Pediatr Surg 24(10):1007-8, 1989
5-  St-Vil D, LeBouthillier G, Luks FI, Bensoussan AL, Blanchard H, Youssef S: Neonatal gastrointestinal perforations. J Pediatr Surg 27(10):1340-2, 1992
6- Harms K, Ludtke FE, Lepsien G, Speer CP: Idiopathic intestinal perforations in premature infants without evidence of necrotizing enterocolitis. Eur J Pediatr Surg 5(1):30-3, 1995

Volume 17 No 04 OCTOBER 2001

Congenital Esophageal Stenosis

Esophageal stenosis in children can be of congenital (5%) or most commonly acquired nature (95%). Acquired stenosis is the result of repaired esophageal atresia, caustic injury, penetrating injury or reflux esophagitis. Congenital esophageal stenosis (CES) can be the result of a membranous diaphragm, segmental hypertrophy of the muscularis and submucosal layer (submucosal fibrosis), or presence of ectopic tracheobronchial rest. CES most commonly affect the middle and distal third of the esophagus and rarely cause symptoms in the neonatal period. Symptoms can be vomiting of undigested food, regurgitation, food impaction, difficulty swallowing solid and failure to thrive. CES affecting the upper third of the esophagus is very rare and usually produce respiratory symptoms such as stridor and repeated respiratory infections. Esophageal atresia is associated with one-third of cases of CES. To establish a diagnosis investigation has to include esophagogram (relatively long, smooth circumferential narrowing), esophagoscopy with biopsy, pH monitoring and in selected cases manometry. Recognition of the correct etiologic factor that caused the stricture will pave the way for adequate management. CES is managed with forceful dilatation or hydrostatic balloon dilatation, while resection with anastomosis will be needed for intractable (fibromuscular hypertrophy) cases and those harboring tracheobronchial rests. Most intractable cases are due to the presence of tracheobronchial rest.

1- Dominguez R, Zarabi M, Oh KS, Bender TM, Girdany BR: Congenital oesophageal stenosis. Clin Radiol 36(3):263-6, 1985
2- Shoshany G, Bar-Maor JA: Congenital stenosis of the esophagus due to tracheobronchial remnants: a missed diagnosis. J Pediatr Gastroenterol Nutr 5(6):977-9, 1986
3- Kawahara H, Imura K, Yagi M, Kubota A: Clinical characteristics of congenital esophageal stenosis distal to associated esophageal atresia. Surgery 129(1):29-38, 2001
4- Shorter NA, Mooney DP, Vaccaro TJ, Sargent SK: Hydrostatic balloon dilation of congenital esophageal stenoses associated with esophageal atresia. J Pediatr Surg 35(12):1742-5, 2000
5- Diab N, Daher P, Ghorayeb Z, Korkmaz G: Congenital esophageal stenosis. Eur J Pediatr Surg 9(3):177-81, 1999
6- Dohil R, Hassall E: Esophageal stenosis in children. Gastrointest Endosc Clin N Am 8(2):369-90, 1998
7- Olguner M, Ozdemir T, Akgur FM, Aktug T: Congenital esophageal stenosis owing to tracheobronchial remnants: a case report. J Pediatr Surg 32(10):1485-7, 1997
8- Sarihan H, Abes M: Congenital esophageal stenosis. J Cardiovasc Surg 38(4):421-3, 1997
9- Newman B, Bender TM: Esophageal atresia/tracheoesophageal fistula and associated congenital esophageal stenosis. Pediatr Radiol 27(6):530-4, 1997
10- Murphy SG, Yazbeck S, Russo P: Isolated congenital esophageal stenosis. J Pediatr Surg 30(8):1238-41, 1995

Limb Body Wall Complex

Limb-body wall complex (LBWC) is a rare, lethal, sporadic congenital anomaly pattern in body-wall defects. Diagnosis of this entity is based on finding at least two of the three following characteristics: (1) neural tube defect (exencephaly/encephalocele and facial clefts), (2) body-wall disruption (thoraco- and/or abdominoschisis, diaphragmatic hernia, eventration, cloacal exstrophy, absent external genitalia or abnormal internal genitalia) and, (3) limb abnormalities (ectrodactyly, phocomelia). Three pathogenic mechanisms have been proposed: early amnion rupture, vascular disruption and embryonic dysgenesis. The internal defects are secondarily to vascular disruption. Cocaine abuse has been associated with LBWC by impairing uteroplacental fetal blood flow during critical periods of development. The limb defects are due to mechanical rupture through the amnion in the presence of a persistent extraembryonic coelom or from adhesions of the amnion to necrotic embryonic tissue. Diagnosis of LBWC is established with prenatal sonography. LBWC is usually incompatible with life.

1- Patten RM, Van Allen M, Mack LA, Wilson D, Nyberg D, Hirsch J, Viamont T: Limb-body wall complex: in utero sonographic diagnosis of a complicated fetal malformation. AJR Am J Roentgenol 146(5):1019-24, 1986
2- Van Allen MI, Curry C, Gallagher L: Limb body wall complex: I. Pathogenesis. Am J Med Genet 28(3):529-48, 1987
3- Colpaert C, Bogers J, Hertveldt K, Loquet P, Dumon J, Willems P: Limb-body wall complex: 4 new cases illustrating the importance of examining placenta and umbilical cord. Pathol Res Pract 196(11):783-90, 2000
4- Chen CP: Prenatal diagnosis of limb-body wall complex with craniofacial defects, amniotic bands, adhesions and upper limb deficiency. Prenat Diagn 21(5):418-9, 2001

Ingrown Toe Nails

Onychocryptosis is the medical term use to describe ingrown toe nails (ITN). Congenital hyperplasia of the nail bed lead to this condition in infants and children. Adolescent and athletes are  susceptible to develop ITN. Other conditions associate with ingrown nails are hallux valgus, claw toes, gout, diabetes, arthritis, or fungus infections of the nails. Initial management should be conservative inserting cotton wool pledgets moistened with an antiseptic under the ingrowing nail edge along with oral antibiotics if there are signs of infection. Insertion of the pledget under anesthesia improves the results and chronicity does not adversely affect the outcome though the procedure is time consuming. Other alternatives are segmental total nail avulsion, nail edge excision, nail matrix phenol cauterization or wedge resections. Total nail avulsion and nail edge excision carries the highest recurrence rate. Nail matrix phenolization is an effective short procedure that can be done under the presence of an infection with a low recurrence rate. Radical wedge resection is simple, has a low recurrence rate, leaves patients with an intact, pain-free, cosmetically acceptable nail-bearing toe, and permits the wearing of normal shoes within a short period. In children conservative treatment is more promising than in adults. If unsuccessful the next reliable surgical approach is wedge resection. Attentions to hygiene and always cutting nails transversely are important preventing factors.

1- Herold HZ, Baruchin AM, Shmueli G, Daniel D, Naoum A: Radical wedge resection for ingrown toenail: long-term results. J Dermatol Surg Oncol 11(5):513-7, 1985
2- Senapati A: Conservative outpatient management of ingrowing toenails. J R Soc Med 79(6):339-40, 1986
3- Connolly B, Fitzgerald RJ: Pledgets in ingrowing toenails. Arch Dis Child 63(1):71-2, 1988
4- Grieg JD, Anderson JH, Ireland AJ, Anderson JR: The surgical treatment of ingrowing toenails. J Bone Joint Surg Br 73(1):131-3, 1991
5- Kimata Y, Uetake M, Tsukada S, Harii K: Follow-up study of patients treated for ingrown nails with the nail matrix phenolization method. Plast Reconstr Surg 95(4):719-24, 1995
6- Lazar L, Erez I, Katz S: A conservative treatment for ingrown toenails in children. Pediatr Surg Int 15(2):121-2, 1999

Volume 17 No 5 NOVEMBER 2001

Fibrosing Pancreatitis

Chronic pancreatitis does occur in young children and is most commonly caused by hereditary pancreatitis or idiopathic fibrosing pancreatitis. Fibrosing pancreatitis is a chronic process of unknown etiology characterized by extensive infiltration of the pancreatic parenchyma by fibrous tissue. Most reported cases are females. Fibrosing pancreatitis is a rare cause of intermittent colicky abdominal pain, recurrent vomiting, weight loss, steatorrhea, pancreatic exocrine and endocrine insufficiency and obstructive jaundice that can be seen in early childhood. In cases of obstructive jaundice the sonographic demonstration of a dilated biliary tree and common bile duct compressed by an enlarged pancreas may be the first suggestion of this disease process. Diagnosis is suggested by imaging studies (US, CT-Scan, MRCP or ERCP) revealing diffuse enlargement of the pancreas, predominantly the head. Open pancreatic biopsy showing acinar cell atrophy and extensive fibrosis is diagnostic. Cystic fibrosis should be excluded by appropriate testing. Management should be directed toward complications. Children with obstructive jaundice should undergo sphincterotomy to drain the common bile duct obstruction or bilio-enteric bypass.

1- Meneely RL, O'Neill J, Ghishan FK: Fibrosing pancreatitis--an obscure causes of painless obstructive jaundice: a case report and review of the literature. Pediatrics 67(1):136-9, 1981
2- Atkinson GO Jr, Wyly JB, Gay BB Jr, Ball TI, Winn KJ: Idiopathic fibrosing pancreatitis: a cause of obstructive jaundice in childhood. Pediatr Radiol 18(1):28-31, 1988
3- Buchta RM, Bell L: Chronic fibrosing pancreatitis in a 12-year-old female. J Adolesc Health 12(5):395-7, 1991
4- Amerson JL, Ricketts RR: Idiopathic fibrosing pancreatitis: a rare cause of obstructive jaundice in children. Am Surg 62(4):295-9, 1996
5- Lewin-Smith MR, Dipalma JS, Hoy GR, Colon AR, Garvin DF: Chronic fibrosing pancreatitis in childhood: report of a case and literature review. Pediatr Pathol Lab Med 16(4):681-90, 1996
6- Sylvester FA, Shuckett B, Cutz E, Durie PR, Marcon MA: Management of fibrosing pancreatitis in children presenting with obstructive jaundice. Gut 43(5):715-20, 1998

Foreign Body Ingestion

Accidental foreign body (FB) ingestion such as - coins, fish bones, toys plastic parts, jewels, batteries, safety pin, needles, etc. - is a common problem in children, specially infants and toddlers. Infants usually swallow button batteries while coins are the most frequently swallowed objects in children over the age of three years. No child ingests more than one FB. Management of esophageal FB differs from the rest of the gastrointestinal tract. Diagnosis is made by either chest-x-ray, barium swallow or esophagoscopy. They should be suspected when the child develops excessive salivation, vomiting, respiratory distress, recent-onset asthma, dysphagia and hematemesis. FB in the esophagus should be removed urgently to avoid erosion and perforation, specially those lodge in the upper-third of the esophagus. They can be removed using flexible fiberoptic endoscopy, balloon catheter or bougienage. Beyond the stomach, foreign body should be managed conservatively. This means follow-up visits until the FB spontaneously appears in the feces. In cases of coins ingestion serious complications are extremely rare. There is no need to x-ray monitor coins or any other metallic FB. More than 85% of all ingested FB passes spontaneously through the rectum despite nature or length. Surgery will be needed in less than 2% of all ingested FB. Patients with previous abdominal surgery are at increased risk. Development of abdominal pain, distension or profuse bleeding is an indication to remove the FB surgically.

1- Suita S, Ohgami H, Nagasaki A, Yakabe S: Management of pediatric patients who have swallowed foreign objects. Am Surg 55(9):585-90, 1989
2- Gilchrist BF, Valerie EP, Nguyen M, Coren C, Klotz D, Ramenofsky ML: Pearls and perils in the management of prolonged, peculiar, penetrating esophageal foreign bodies in children. J Pediatr Surg 32(10):1429-31, 1997
3- Hachimi-Idrissi S, Corne L, Vandenplas Y: Management of ingested foreign bodies in childhood: our experience and review of the literature. Eur J Emerg Med 5(3):319-23, 1998
4- Calkins CM, Christians KK, Sell LL: Cost analysis in the management of esophageal coins: endoscopy versus bougienage. J Pediatr Surg 34(3):412-4, 1999
5- Cheng W, Tam PK: Foreign-body ingestion in children: experience with 1,265 cases. J Pediatr Surg 34(10):1472-6, 1999
6- Soprano JV, Mandl KD: Four strategies for the management of esophageal coins in children. Pediatrics 105(1):e5, 2000

Inflammatory Pseudotumor

Inflammatory pseudotumor, better known as inflammatory myofibroblastic tumor (IMT) is a rare benign solid tumor mimicking a malignant neoplasm that grows to a large size. Though most commonly encountered in the lung and mediastinum, IMT has also been identified in the abdomen (liver, pancreas, stomach, omentum), retroperitoneum, pelvis (bladder), and extremity of children. Imaging studies suggest a well-circumscribed mass of soft tissue density producing displacement or invasion of surrounding structures. Needle biopsy is not considered a reliable diagnostic method. Diagnosis must be established by histology after surgical excision. Pathologically there is proliferation of plasma cells, lymphocytes and histiocytes in a benign looking spindle-shaped stroma (myofibroblasts). Etiology is unknown. Symptoms at presentation depend on site of development. Total excision of the lesion is sufficient for accurate treatment in most cases. Recurrence is common and should also be managed with surgery. Adjuvant therapy has limited application.

1- Brown G, Shaw DG: Inflammatory pseudotumors in children: CT and ultrasound appearances with histopathological correlation. Clin Radiol  50(11):782-6, 1995
2- Bonnet JP, Basset T, Dijoux D: Abdominal inflammatory myofibroblastic tumors in children: report of an appendiceal case and review of the literature. J Pediatr Surg  31(9):1311-4, 1996
3- Ciftci AO, Akcoren Z, Tanyel FC, Senocak ME, Caglar M, Hicsonmez A: Inflammatory pseudotumor causing intestinal obstruction: diagnostic and therapeutic aspects. J Pediatr Surg  33(12):1843-5, 1998
4- Cerfolio RJ, Allen MS, Nascimento AG, Deschamps C, Trastek VF, Miller DL, Pairolero PC: Inflammatory pseudotumors of the lung. Ann Thorac Surg  67(4):933-6, 1999
5- Sanders BM, West KW, Gingalewski C, Engum S, Davis M, Grosfeld JL: Inflammatory pseudotumor of the alimentary tract: clinical and surgical experience. J Pediatr Surg  36(1):169-73, 2001
6- Karnak I, Senocak ME, Ciftci AO, Caglar M, Bingol-Kologlu M, Tanyel FC, Buyukpamukcu N: Inflammatory myofibroblastic tumor in children: diagnosis and treatment. J Pediatr Surg  36(6):908-12, 2001

Volume 17 No 6 DECEMBER 2001


Gastrinomas, the cause of the Zollinger-Ellison syndrome, are gastrin producing tumors that result in fulminant peptic ulceration and diarrhea. In children, half the cases are associated with malignancy, metastasis and multicentricity. Epidemiologically 75% gastrinomas are sporadic and 25% associated with MEN type 1. Diagnosis of gastrinoma is confirmed after documenting fasting serum hypergastrinemia (> 200 pg/ml), markedly elevated gastric output, a positive secretin-stimulated gastrin analysis or histologic confirmation of a neuroendocrine tumor. Most tumors are found in the head, body and tail of the pancreas, followed by duodenum, liver and lymph nodes. Localization studies include CT-scan, MRCP, octreotide scan (specially indium-labeled pentetreotide), endoscopic US, and Imamura test (selective arterial secretin injection test). Radical resection of the gastrinoma to achieve biochemical cure even when tumor is extrapancreatic and in lymph nodes is the cornerstone of therapy. Unresectable or metastatic cases are managed with pharmacologic therapy using proton pump inhibitors and octreotide. Total gastrectomy is reserved for absolute failure of both medical and surgical management. Gastrinomas in children are slow growing, indolent, and compatible with long life. Prognostic factors correlating with long-term survival are small tumor size, absence of metastatic disease and non-pancreatic location of primary tumors. Factors that do not affect survival include age at diagnosis, sex, presence of lymph node metastases, associated multiple endocrine neoplasia, and method of ulcer treatment.

1- Bonfils S, Landor JH, Mignon M, Hervoir P: Results of surgical management in 92 consecutive patients with Zollinger-Ellison syndrome. Ann Surg  194(6):692-7, 1981
2- Wilson SD: The role of surgery in children with the Zollinger-Ellison syndrome. Surgery  92(4):682-92, 1982
3- Imamura M, Takahashi K, Isobe Y, Hattori Y, Satomura K, Tobe T: Curative resection of multiple gastrinomas aided by selective arterial secretin injection test and intraoperative secretin test. Ann Surg  210(6):710-8, 1989
4- Wilson SD: Zollinger-Ellison syndrome in children: a 25-year follow-up. Surgery  110(4):696-702, 1991
5- Farley DR, van Heerden JA, Grant CS, Miller LJ, Ilstrup DM: The Zollinger-Ellison syndrome. A collective surgical experience. Ann Surg  215(6):561-9, 1992
6- Zimmer T, Stolzel U, Bader M, Koppenhagen K, Hamm B, Buhr H, Riecken EO, Wiedenmann B: Endoscopic ultrasonography and somatostatin receptor scintigraphy in the preoperative localization of insulinomas and gastrinomas. Gut  39(4):562-8, 1996

Epigastric Hernias

Congenital epigastric defects occur anywhere in the linea alba from the navel to the xiphoid process. They represent almost 5% of all hernias defect that presents in children. Most epigastric hernias occur in the midline, are small (15-25 mm), asymptomatic and reducible. Multiple fascial defects can also be present in 20% of all cases. The defect might arise congenitally from an abnormally wide orifice of a blood vessel during development of the linea alba. The bump is the result of a piece of preperitoneal fat stuck through the fascial defect. Tenderness is an unusual symptom while growth of the defect occurs with time. Most surgeons recommend repair of the defect at the time of presentation. Repair is an outpatient procedure done under general anesthesia with low morbidity and risk of recurrence. Voluminous epigastric hernia (5-10 cm) with a sac that contains epiploic appendages or viscera (ileum loops, stomach) has also been rarely reported in infants.

1- Coats RD, Helikson MA, Burd RS: Presentation and management of epigastric hernias in children. J Pediatr Surg  35(12):1754-6, 2000
2- Askar OM: Aponeurotic Hernias: Recent observations upon paraumbilical and epigastric hernias. Surg Clin North Am 64: 315-333, 1984
3- Corsale I, Palladino E: Diagnosis and treatment of epigastric hernia. Analysis of our experience. Minerva Chir  55(9):607-10, 2000
4- Ameh EA: Giant congenital epigastric hernia. West Afr J Med  18(2):151-2, 1999

Necrotizing Fasciitis

Necrotizing fasciitis (NF) is a rare, severe, rapidly progressive soft tissue infection involving the skin, subcutaneous tissue and superficial fascia. Most cases occur during the newborn period. In newborns NF is most frequently associated with omphalitis, necrotizing enterocolitis, balanitis, urachal anomalies and fetal monitoring. In older children NF can be seen as a postoperative complication (appendectomy, gastrostomy), in neutropenia after chemotherapy and secondarily to varicella infestation. Most frequent sites of initial involvement are the abdominal wall, followed by thorax, back, scalp, and extremity. Infection is usually polymicrobial containing both gram positive (beta-hemolytic Streptococcus and Staphylococcus aureus) and gram negative bacteria (E. Coli and Pseudomonas). Important clinical findings in infants with NF are tachycardia, abnormal white blood cell count, systemic toxicity, severe edema, and, in older children pain out of proportion to the apparent degree of infection. The child can also develop wound crepitation and radiographic evidence of subcutaneous gas. An ominous sign indicative of the need for immediate radical debridement is the appearance of a patch of dusky or gangrenous skin. Initial management consists of antibiotics, intravenously afluids, blood transfusions, calcium replacement (due to saponification) and general patient support. Neutropenic patients benefit from granulocyte transfusions. Surgical procedures include extensive debridement as soon as possible and as needed for continued necrosis, secondary closure and skin grafting. Mortality ranges from 10 to 60%. Improved survival requires early diagnosis followed by prompt aggressive surgical debridement.

1- Kosloske AM, Cushing AH, Borden TA, Woodside JR, Klein MD, Kulasinghe HP, Bailey WC: Cellulitis and necrotizing fasciitis of the abdominal wall in pediatric patients. J Pediatr Surg  16(3):246-51, 1981
2- Farrell LD, Karl SR, Davis PK, Bellinger MF, Ballantine TV: Postoperative necrotizing fasciitis in children. Pediatrics  82(6):874-9, 1988
3- Samuel M, Freeman NV, Vaishnav A, Sajwany MJ, Nayar MP: Necrotizing fasciitis: a serious complication of omphalitis in neonates. J Pediatr Surg  29(11):1414-6, 1994
4- Murphy JJ, Granger R, Blair GK, Miller GG, Fraser GC, Magee JF: Necrotizing fasciitis in childhood. J Pediatr Surg  30(8):1131-4, 1995
5- Moss RL, Musemeche CA, Kosloske AM: Necrotizing fasciitis in children: prompt recognition and aggressive therapy improve survival. J Pediatr Surg  31(8):1142-6, 1996
6- Hsieh WS, Yang PH, Chao HC, Lai JY: Neonatal necrotizing fasciitis: a report of three cases and review of the literature. Pediatrics  103(4):e53, 1999
7- Abbott RE, Marcus JR, Few JW, Farkas AM, Jona J: Necrotizing fasciitis in infancy: an uncommon setting and a prognostic disadvantage. J Pediatr Surg  34(9):1432-4, 1999

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