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PEDIATRIC SURGERY UPDATE VOLUME 19, 2002

Volume 19 No 01 JULY 2002
Laparoscopic Inguinal Herniography

Besides using the laparoscopic technique for identifying a contralateral patent processus vaginalis using a trans-inguinal approach or repair a recurrent inguinal hernia, the approach can also be use for repair of inguinal hernias in children. The technique consists in closing the neck of the hernial sac with a purse string suture. Advantages of th include the ability to diagnose and repair concomitantly a contralateral hernia defect due to excellent visual effect, the ability to diagnose a direct inguinal hernia, better co less surgical dissection. This last issue is called "access trauma" or repair of all the damage caused in gaining the initial access to ligate a hernial sac. Another benefit is t dissection of the cord structures so that the risk of superior displacement of the testis, testicular atrophy, injury to vas deferens and stitch granuloma is less likely. Disadv longer procedure, higher recurrent rates, development of residual hydrocele in males along with the complications attendant in violating the abdominal cavity (vascular and caused by Veress needle and trocar insertion). Most surgeons still prefer to do an open subcutaneous procedure with high ligation of hernia sacs as standard repair of ingu infants and children.

References: 1- Schier F, Montupet P, Esposito C: Laparoscopic Inguinal Herniography in Children: A Three-Center Experience with 933 repairs. J Pediatr Surg 37(3): 395-397, 2002 2- Geisler DP, Jegathesan S, Parmley MC, McGee JM, Nolen MG, Broughan TA: Laparoscopic exploration for the clinically undetected hernia in infancy and childhood. Am J Surg 182(6):693-6, 2001 3- Schafer M, Lauper M, Krahenbuhl L: Trocar and Veress needle injuries during laparoscopy.Surg Endosc 15(3):275-80, 2001 4- Schier F: Direct inguinal hernias in children: laparoscopic aspects. Pediatr Surg Int 16(8):562-4, 2000 5- Schier F: Laparoscopic surgery of inguinal hernias in children--initial experience. J Pediatr Surg 35(9):1331-5, 2000 6- Montupet P, Esposito C: Laparoscopic Treatment of Congenital Inguinal Hernia in Children. J Pediatr Surg 34(3): 420-423, 1999

Congenital Diaphragmatic Hernia Update

Congenital Diaphragmatic Hernia (CDH) continues to carry a high mortality and morbidity. The mortality is the result of lethal pulmonary hypoplasia, the presence of a le anomaly (usually a cardiac defect), central nervous system bleeding/injury from aggressive medical management (i.e., ECMO), or iatrogenic barotrauma. Lethal pulmonar probably rest its solution in fetal intervention as more prenatal techniques are used to identify the fetus with such a problem (progressive ipsilateral pulmonary artery hypo fetal echo-doppler and fetal lung volume measurement by magnetic resonance imaging correlates with progressive fetal lung hypoplasia and worse postnatal outcomes). T trends in management of babies suffering from CDH rest on three principles that have been found to reduce mortality and morbidity: 1) use of soft mechanical ventilation mechanical ventilation and minimal inspiratory pressure settings) with permissive hypercapnia, 2) delay surgical repair until hemodynamic, ventilatory and acid-base stabil ipsilateral chest tube placement since they cause negative pressure gradient reducing lung deflation leading to lung injury and barotrauma.

References:

1- Boloker J, Bateman DA, Wung JT, Stolar CJ: Congenital Diaphragmatic Hernia in 120 Infants Treated Consecutively with Permissive Hypercapnia/Spontaneous Respiration/Elective Repair. J Pediatr Surg 37(3): 3 2- Sokol J, Bohn D, Lacro RV, Ryan G, Stephens D, Rabinovitch M, Smallhorn J, Hornberger LK: Fetal pulmonary artery diameters and their association with lung hypoplasia and postnatal outcome in congenital di Obstet Gynecol 186(5 Pt 1):1085-1090, 2002 3- Mahieu-Caputo D, Sonigo P, Dommergues M, Fournet JC, Thalabard JC, Abarca C, Benachi A, Brunelle F, Dumez Y: Fetal lung volume measurement by magnetic resonance imaging in congenital diaphragmatic h 2001 4- Kays DW, Langham MR, Ledbetter DJ, et al: Detrimental Effects of Standard Medical Therapy in Congenital Diaphragmatic Hernia. Ann Surg 230: 340-351, 1999 5- Serrano P, Reyes G, Lugo-Vicente HL: Congenital Diaphragmatic Hernia: Mortality Determinants in a Hispanic Population. PR Health Science J 17(4): 317-321, 1998

Fecal Incontinence

Fecal incontinence (FI) in children is usually the result of a congenital (imperforate anus, Hirschsprung's disease and myelomeningocele) or acquired (trauma, pelvic tumor spinal cord injury) condition. FI causes a psychological, developmental and social stress of great magnitude. Assessment of the problems includes the use of anal tonometr and pudendal nerve terminal motor latencies. Rectal emptying and angulation can be evaluated with defecograms, MRI or scintigraphic proctography. The management o HD biopsychosocial treatment consisting of explanation, extinction of fear and avoidance behavior, learning new defecation behavior, learning an adequate straining techn generalization toward daily life have been effective. Biofeedback is a harmless and inexpensive treatment coordinating pelvic floor muscle contraction with the sensation o has brought partial success in children with FI. Other less effective forms of treatment are dynamic graciloplasty and sacral nerve stimulation. Functional constipation asso incontinence and the presence of segmental dilatation of the sigmoid or rectum responds poorly to medical management. The megabowel lacks an adequate anorectal refle propulsive function causing a functional obstruction. Segmental resection of the dilated segment has brought successful relief of symptoms. Antegrade enemas by way of a appendicocecostomy or cecostomy tube can achieve total colonic evacuation and socially controlled continence in children with anorectal malformations and caudal agenes percentage of cases.

References: 1- Darakhsan AA, Williams NS: Recent Innovations in the Management of Fecal Incontinence. Sem Pediatr Surg 11(2): 83-90, 2002 3- Heymen S, Jones KR, Ringel Y, Scarlett Y, Whitehead WE: Biofeedback treatment of fecal incontinence: a critical review. Dis Colon Rectum 44(5):728-36, 2001 5- Whitehead WE, Wald A, Norton NJ: Treatment options for fecal incontinence. Dis Colon Rectum 44(1):131-42, 2001 6- Lee SL, Rowell S, Greenholz SK: Therapeutic cecostomy tubes in infants with imperforate anus and caudal agenesis. J Pediatr Surg 37(3):345-7, 2002 7- Rintala RJ: Fecal incontinence in anorectal malformations, neuropathy, and miscellaneous conditions. Semin Pediatr Surg 11(2):75-82, 2002

2- van Kuyk EM, Brugman-Boezeman AT, Wissink-Essink M, Severijnen RS, Festen C, Bleijenberg G: Defecation problems in children with Hirschsprung's disease: a biopsychosocial approach. Pediatr Surg Int 16

4- Lee SL, DuBois JJ, Montes-Garces RG, Inglis K, Biediger W: Surgical management of chronic unremitting constipation and fecal incontinence associated with megarectum: A preliminary report. J Pediatr Surg 37

Volume 19 No 02 AUGUST 2002

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Ovarian Hernia

Inguinal hernias are the most common surgical pathology seen in infants and children. A lump in the inguinal canal area of a newborn or infant female is most probably an o inguinal hernia. On very rare occasion the lump is a testis in a child with testicular feminization syndrome. Clinically the irreducible ovarian lump is usually asymptomatic, tender mass within the labia majora. Ultrasound can determine the nature of the gonad present. The main problem with an ovarian hernia in infants is the incidence of ovar associated before repair. Ovaries trapped within inguinal hernias undergo torsion far more commonly than ovaries and tubes in the normal pelvic position increasing the ch Torsion can occur at any time after diagnosis of the hernia. The incarcerated ovarian pedicle is narrowed and lengthened within the defect and the internal ring serves as a which a twist can occur. The risk of torsion and infarction creates the view that ovarian hernias should be repaired at the earliest elective opportunity if they can be reduce Children with edema, tenderness or skin discoloration in the inguinal area should be repaired immediately. Early recognition and management of this condition reduce the infarction. During repair surgeons must be aware that in 20% of girls with inguinal hernia, the fallopian tubes occasionally with the ovary or uterus comprise the wall of the component).

References: 1- Goske MJ, Emmens RW, Rabinowitz R: Inguinal ovaries in children demonstrated by high resolution real-time ultrasound. Radiology 151(3):635-6, 1984 2- Boley SJ, Cahn D, Lauer T, Weinberg G, Kleinhaus S: The irreducible ovary: a true emergency. J Pediatr Surg 26(9):1035-8, 1991 3- Ozbey H, Ratschek M, Schimpl G, Hollwarth ME: Ovary in hernia sac: prolapsed or a descended gonad? J Pediatr Surg 34(6):977-80, 1999 4- Merriman TE, Auldist AW: Ovarian torsion in inguinal hernias. Pediatr Surg Int 16(5-6):383-5, 2000

Diversion Colitis

Diversion colitis is an inflammatory state resulting from a nutritional deficiency in the lumen of the colonic epithelium. The colitis develops in segments of the colon and re diversion of the fecal stream persisting indefinitely unless the excluded segment is reanastomosed. Diversion colitis is characterized by rectal discomfort, pain, discharge, bleeding. Symptoms occur three months or more after bowel diversion. Diagnosis is established by colonic or rectal biopsy. Histologic abnormalities included aphthous ulce atrophy and abscesses, a villous colonic surface, and a mixed acute and chronic inflammatory infiltrate with patchy lymphoid hyperplasia. This condition is caused by the a short-chain fatty acids, the preferred metabolic substrates of colonic epithelium. In children diversion colitis can be seen in bowel derived Hirschsprung's disease, imperfor colitis and Crohn's disease. It has also been reported to also occurs after sigmoid neovagina reconstruction. Adjunctive management includes the use of a topical mixture o acids (propionate, acetate, butyrate) and/or 5-Aminosalicylic acid (5-ASA) to control symptoms. Definitive treatment consists of excision of rectum or stomal closure.

References: 1- Harig JM, Soergel KH, Komorowski RA, Wood CM: Treatment of diversion colitis with short-chain-fatty acid irrigation. N Engl J Med 320(1):23-8, 1989 2- Komorowski RA: Histologic spectrum of diversion colitis. Am J Surg Pathol 14(6):548-54, 1990 3- Toolenaar TA, Freundt I, Huikeshoven FJ, Drogendijk AC, Jeekel H, Chadha-Ajwani S: The occurrence of diversion colitis in patients with a sigmoid neovagina. Hum Pathol 24(8):846-9, 1993 4- Grant NJ, Van Kruiningen HJ, Haque S, West AB: Mucosal inflammation in pediatric diversion colitis: a quantitative analysis. J Pediatr Gastroenterol Nutr 25(3):273-80, 1997 5- Kiely EM, Ajayi NA, Wheeler RA, Malone M: Diversion Procto-Colitis: Response to Treatment with Short-Chain Fatty Acids. J Pediatr Surg 36(10): 1514-1517, 2001

Duodenal Atresia

Duodenal atresia (DA) is the most common congenital anomaly associated with the duodenum and the most common atresia found in the GI tract. The double-bubble appe stomach and duodenal bulb seen prenatally (US) as two anechoic cysts or after birth (simple abdominal films) is characteristically diagnostic. DA is associated one-third of Down's syndrome followed by cardiac malformations (20%). Prenatal karyotyping and fetal echocardiogram of suspected DA cases will establish the association. Clinically presents with bilious vomiting and epigastric distension (dilated stomach) depending whether the atresia occurs proximally (10%) or distally (90%) to the papilla of Vater. from DA needs urgent differentiation from malrotation which carries the risk of midgut volvulus. If in doubt a small upper GI series using a water soluble contrast is recom correction of electrolytes imbalances, management of DA consists of diamond-shaped duodeno-duodenostomy. If the proximal duodenum is massively dilated a tapering du (imbrication or stapler resection) will help reduce the possibilities of a functional anastomotic obstruction. Late complications include motility disorders, megaduodenum, ga reflux, duodenal-gastric reflux, gastritis, peptic ulcer disease, blind loop syndrome and biliary-pancreatic conditions which may be observed months to years after surgical

References: 1- Ein SH, Kim PC, Miller HA: The late nonfunctioning duodenal atresia repair--a second look. J Pediatr Surg 35(5):690-1, 2000

2- Murshed R, Nicholls G, Spitz L: Intrinsic duodenal obstruction: trends in management and outcome over 45 years (1951-1995) with relevance to prenatal counseling. Br J Obstet Gynaecol 106(11):1197-9, 1999 3- Takahashi A, Tomomasa T, Suzuki N, Kuroiwa M, Ikeda H, Morikawa A, Matsuyama S, Tsuchida Y: The relationship between disturbed transit and dilated bowel, and manometric findings of dilated bowel in pat atresia and stenosis. J Pediatr Surg 32(8):1157-60, 1997 4- Upadhyay V, Sakalkale R, Parashar K, Mitra SK, Buick RG, Gornall P, Corkery JJ: Duodenal atresia: a comparison of three modes of treatment. Eur J Pediatr Surg 6(2):75-7, 1996 5- Grosfeld JL, Rescorla FJ: Duodenal atresia and stenosis: reassessment of treatment and outcome based on antenatal diagnosis, pathologic variance, and long-term follow-up. World J Surg 17(3):301-9, 1993 6- Akhtar J, Guiney EJ: Congenital duodenal obstruction. Br J Surg 79(2):133-5, 1992 7- Kimura K, Mukohara N, Nishijima E, Muraji T, Tsugawa C, Matsumoto Y: Diamond-shaped anastomosis for duodenal atresia: an experience with 44 patients over 15 years. J Pediatr Surg 25(9):977-9, 1990

Volume 19 No 03 SEPTEMBER 2002

Parotid Mass

A parotid mass in a child creates great concern and should be managed promptly since a high percentage of cases will harbor a malignant tumor. The more common benign children includes hemangioma, pleomorphic adenoma and lymphangiomas. Infants with a rapidly enlarging violaceous or soft tumor in the parotid region harbors a hemang Hemangioendothelioma is the most common parotid gland tumor of childhood. They seldom need excision as spontaneous regression is the norm. An asymptomatic, slow g the most common presentation in older children. Tenderness is associated with an infectious process. Diagnosis includes the use of Doppler ultrasound, CT-Scan, MRI an aspiration (FNA) biopsy. Salivary gland carcinoma is rare in children. Most common histological type is mucoepidermoid either as a primary neoplasm or secondary malig irradiation. Management consists of superficial or total parotidectomy extent which is selected during the surgical procedure depending on deep gland or fascial nerve invo must be widely excised. Recurrence is managed with postoperative irradiation for high or intermediate grade malignancies. Rhabdomyosarcoma arising in the parotid glan locally invasive aggressive tumor that presents early with swelling and symptoms of seventh nerve deficit. This tumor needs surgical excision followed by chemotherapy an

References:

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1- Zurrida S, Alasio L, Tradati N, Bartoli C, Chiesa F, Pilotti S: Fine-needle aspiration of parotid masses. Cancer 72(8):2306-11, 1993 2- Rogers DA, Rao BN, Bowman L, Marina N, Fleming ID, Schropp KP, Lobe TE: Primary malignancy of the salivary gland in children. J Pediatr Surg 29(1):44-7, 1994 3- Khadaroo RG, Walton JM, Ramsay JA, Hicks MJ, Archibald SD: Mucoepidermoid carcinoma of the parotid gland: a rare presentation in a young child. J Pediatr Surg 33(6):893-5, 1998 4- Salomao DR, Sigman JD, Greenebaum E, Cohen MB: Rhabdomyosarcoma presenting as a parotid gland mass in pediatric patients: fine-needle aspiration biopsy findings. Cancer 84(4):245-51, 1998 5- Roebuck DJ, Ahuja AT: Hemangioendothelioma of the parotid gland in infants: sonography and correlative MR imaging. AJNR Am J Neuroradiol 21(1):219-23, 2000 6- Orvidas LJ, Kasperbauer JL, Lewis JE, Olsen KD, Lesnick TG: Pediatric parotid masses. Arch Otolaryngol Head Neck Surg 126(2):177-84, 2000

Perineal Hemangioma

Hemangiomas continue to be the most common benign tumor in infants. Perineal hemangiomas involving the scrotum, perianal region and rectum have been sporadically d the anogenital area have a strong predilection for females. The clinical presentation can start with an undiagnosed non-healing ulcer, a pale macule, erythematous papule, stain in the perineal region. Recurrent perianal blood loss can be caused by a rectal hemangioma. Local factors such as abrasion or maceration plays a potentiating role in MRI helps determine the extension of the hemangioma toward the pelvis and surrounding tissues. Though most hemangiomas spontaneously involute over a period of yea the perineal and ano-rectal region are troublesome lesions causing repeated ulcerations and subsequent pain. The subcutaneous perineal hemangioma can be managed con systemic steroids or using alpha interferon therapy depending on the symptomatology of the child. Diffuse cavernous hemangioma of the colon, rectum and anus can be ma temporary colostomy followed by modified endorectal pull-through.

References: 1- Liang MG, Fireden IJ: Perineal and Lip Ulceration as the Presenting Manifestation of hemangioma of Infancy. Pediatrics 99(2): 256-259, 1997 2- Bouchard S, Yazbeck S, Lallier M: Perineal hemangioma, anorectal malformation, and genital anomaly: a new association? J Pediatr Surg 34(7):1133-5, 1999 3- Pohlen U, Kroesen AJ, Berger G, Buhr HJ: Diagnostics and surgical treatment strategy for rectal cavernous hemangiomas based on three case examples. Int J Colorectal Dis 14(6):300-3, 1999 4- Takamatsu H, Akiyama H, Noguchi H, Tahara H, Kajiya H: Endorectal pull-through operation for diffuse cavernous hemangiomatosis of the sigmoid colon, rectum and anus. Eur J Pediatr Surg 2(4):245-7, 1992 5- Achauer BM, Vander Kam VM: Ulcerated anogenital hemangioma of infancy. Plast Reconstr Surg 87(5):861-6, 1991

Fetus-in-Fetu

Fetus-in-fetu is a very rare condition in which a malformed parasitic twin is found inside the body of its partner as an abdominal fetiform calcified mass. They are typically retroperitoneum. Symptoms relate to the mass effect and include abdominal distension, feeding difficulty, vomiting and dyspnea. It represents an abortive attempt of ident one fetus (the parasite) is drawn into the abdominal cavity of the host fetus (the autosite) in early intrauterine life and is attached retroperitoneally, with the blood supply to supplied by the host superior mesenteric vessels. There exist much controversy in the literature whether a fetus-in-fetu is a well-formed teratoma (tumor composed of the layers) or not. To be coined fetus-in-fetu the mass must demonstrate true organogenesis and an axial skeleton separating them from teratomas, which never undergo orga Nonvisualization of the vertebral axis on radiography or on CT Scan does not exclude the diagnosis of fetus-in-fetu as it can be seen by the pathologist. Chromosomal anal shows normal chromosomes identical to the host. Management consists of complete surgical resection in as much as malignant recurrence has been reported.

References: 1- Eng HL, Chuang JH, Lee TY, Chen WJ: Fetus in fetu: a case report and review of the literature. J Pediatr Surg 24(3):296-9, 1989 2- Hopkins KL, Dickson PK, Ball TI, Ricketts RR, O'Shea PA, Abramowsky CR: Fetus-in-fetu with malignant recurrence. J Pediatr Surg 32(10):1476-9, 1997 3- Hoeffel CC, Nguyen KQ, Phan HT, Truong NH, Nguyen TS, Tran TT, Fornes P: Fetus in fetu: a case report and literature review. Pediatrics 105(6):1335-44, 2002 4- Mills P, Bornick PW, Morales WJ, Allen M, Gilbert-Barness E, Johnson PK, Quintero R: Ultrasound prenatal diagnosis of fetus in fetu. Ultrasound Obstet Gynecol 18(1):69-71, 2001 5- Federici S, Prestipino M, Domenichelli V, Antonellini C, Sciutti R, Domini R: Fetus in fetu: report of an additional, well-developed case. Pediatr Surg Int 17(5-6):483-5, 2001

Volume 19 No 04 OCTOBER 2002

Colonic Atresia

Colonic Atresia (complete absence of the lumen of the colon) is a rare cause of congenital bowel obstruction comprising almost 5% of all cases of bowel atresia in newborn (CA) can be associated with distal skeletal deformities, imperforate anus, Hirschsprung's and abdominal wall defects. Complicated cases are associated with partial or tota and severe deformities of the genitourinary tract (bladder and cloacal exstrophy). Colonic Atresia is the result of an intrauterine mesenteric vascular accident. Clinically th distension, bilious vomiting and obstipation. Simple abdominal films shows hugely distended distal bowel loops and air-fluid levels. Barium enema will show a microcolon. O intervention is urgent since a competent ileo-cecal valve makes the obstruction a close loop. During surgery patency of the gastrointestinal tract must be confirmed distall avoid missing a second associated atresia. For atresias located in the right colon, resection and end to oblique primary anastomosis is recommended. Atresias located in th critically ill children or complex combinations of absent hindgut should be initially managed with a diverting colostomy. Suspect the coexistence of Hirschsprung's disease a when a functional obstruction is present after repair of the atresia. Suction rectal biopsy before closing the colostomy should solve this problem. Prognosis depends on type associated malformations.

References: 1- Coran AG, Eraklis AJ: Atresia of the colon. Surgery 65: 828-831, 1969 2- Powell RW, Raffensperger JG: Congenital Colonic Atresia. J Pediatr Surg 17(2): 166-170, 1982 3- Pohlson EC, Hatch EI Jr, Glick PL, Tapper D: Individualized management of colonic atresia. Am J Surg 155(5):690-2, 1988 4- Davenport M, Bianchi A, Doig CM, Gough DC: Colonic atresia: current results of treatment. J R Coll Surg Edinb 35(1):25-8, 1990 5- Williams MD, Burrington JD: Hirschsprung's disease complicating colon atresia. J Pediatr Surg 28(4):637-9, 1993 6- Dalla Vecchia LK, Grosfeld JL, West KW, Rescorla FJ, Scherer LR, Engum SA: Intestinal atresia and stenosis: a 25-year experience with 277 cases. Arch Surg 133(5):490-6, 1998 7- Akgur FM, Olguner M, Hakguder G, Ozer E, Aktug T: Colonic atresia associated with Hirschsprung's disease: it is not a diagnostic challenge. Eur J Pediatr Surg 8(6):378-9, 1998 8- Karnak I, Ciftci AO, Senocak ME, Tanyel FC, Buyukpamukcu N: Colonic atresia: surgical management and outcome. Pediatr Surg Int 17(8):631-5, 2001

Colonic Strictures

Colonic strictures in infants are most commonly the result of a late complication from neonatal necrotizing enterocolitis (NEC). Other times they can be congenital (stenosi children receiving high dose pancreatic enzyme supplement (cystic fibrosis) or after inflammatory bowel disease. Post-NEC strictures occur both after medical and surgica medically-treated infants symptoms of bowel obstruction usually begin six to eight weeks after resolution of NEC, while surgically managed infants develop asymptomatic an enterostomy. The clinical manifestations of colonic strictures include abdominal distention, bilious vomiting, hematochezia, diarrhea, disaccharide intolerance, chronic g
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loss and growth failure. Splenic flexure and left colon are the most common sites of NEC stricture formation. Contrast study of the distal bowel can establish the diagnosis cases. Although routine studies have no advantage over clinical follow-up, some studies propose a diagnostic protocol using an early upper GI study with non-ionic water s material followed by a contrast enema in suspicious cases as a reliable method of diagnosing strictures. Contrast enemas are essential prior to restoring bowel continuity a Balloon catheter dilatation of the stricture has been tried but recurrence closure rates are too high to be recommended as definite therapy. Once identified management co and primary anastomosis of the stricture.

References: 1- Kosloske AM, Burstein J, Bartow SA: Intestinal obstruction due to colonic stricture following neonatal necrotizing enterocolitis. Ann Surg 192(2):202-7, 1980 2- Ball WS Jr, Seigel RS, Goldthorn JF, Kosloske AM: Colonic strictures in infants following intestinal ischemia. Treatment by balloon catheter dilatation. Radiology 149(2):469-71, 1983 3- Hartman GE, Drugas GT, Shochat SJ: Post-necrotizing enterocolitis strictures presenting with sepsis or perforation: risk of clinical observation. J Pediatr Surg 23(6):562-6, 1988 4- Nanjundiah P, Lifschitz CH, Gopalakrishna GS, Cochran WJ, Klish WJ: Intestinal strictures presenting with gastrointestinal blood loss. J Pediatr Surg 24(2):174-6, 1989 5- Radhakrishnan J, Blechman G, Shrader C, Patel MK, Mangurten HH, McFadden JC: Colonic strictures following successful medical management of necrotizing enterocolitis: a prospective study evaluating early studies. J Pediatr Surg 26(9):1043-6, 1991 6- Collins MH, Azzarelli B, West KW, Chong SK, Maguiness KM, Stevens JC: Neuropathy and vasculopathy in colonic strictures from children with cystic fibrosis. J Pediatr Surg 31(7):945-50, 1996

Colonic Volvulus

Volvulus of the colon represent a rare cause of bowel obstruction in children. Colonic volvulus can occur in the sigmoid (most common site), transverse and cecal colon. Th same pathogenetic mechanism: a long freely redundant mobile colon and mesocolon, lack of fixation and short mesenteric attachment of the proximal and distal mesocolic volvulus have been associated to chronic constipation, high fiber diet, mental retardation and Hirschsprung's disease. Central abdominal pain of sudden onset followed by distension, tenderness, bloody mucoid discharge or inability to pass flatus is characteristic. Simple abdominal films will show large bowel dilated loops with air-fluid levels. showing a bird beak appearance of the colon and air-contrast mirror image in the proximal end is diagnostic, and in a few cases therapeutic. Management is operative sinc enema reduction is associated with an unacceptable high rate of recurrence. Surgery consist of resection of the involved part with end-to-end anastomosis for limited segm colopexy for extensive bowel involvement.

References: 1- Mellor MF, Drake DG: Colonic volvulus in children: value of barium enema for diagnosis and treatment in 14 children. AJR Am J Roentgenol 162(5):1157-9, 1994 2- Ismail A: Recurrent colonic volvulus in children. J Pediatr Surg 32(12):1739-42, 1997 3- Samuel M, Boddy SA, Nicholls E, Capps S: Large bowel volvulus in childhood. Aust N Z J Surg 70(4):258-62, 2000 4- Mercado-Deane MG, Burton EM, Howell CG: Transverse colon volvulus in pediatric patients. Pediatr Radiol 25(2):111-2, 1995 5- Houshian S, Sorensen JS, Jensen KE: Volvulus of the transverse colon in children. J Pediatr Surg 33(9):1399-401, 1998 6- Salas S, Angel CA, Salas N, Murillo C, Swischuk L: Sigmoid volvulus in children and adolescents. J Am Coll Surg 190(6):717-23, 2000 7- Samuel M, Boddy SA, Capps S: Volvulus of the transverse and sigmoid colon. Pediatr Surg Int 16(7):522-4, 2000

Volume 19 No 05 NOVEMBER 2002

Esophago-Gastric Dissociation

Gastroesophageal reflux is a common and distressing problem in neurologically affected children. Couple with diminished salivary clearance, pharyngo-esophageal incoord esophageal dysmotility the incidence of pulmonary aspiration and infection is increased in this pediatric population. Fundoplication and gastrostomy are effective in provid reducing aspiration and improving quality of care. Unfortunately, a large number of these kids develop complications after the fundoplication. They include: wrap herniatio or paraesophageal), disruption, gagging, retching and recurrent preop GER symptoms (vomiting and pneumonia are the most troublesome). Theories explaining this high r supine position, swallowing incoordination, esophageal dysmotility, spasticity, seizures, delayed gastric emptying, chronic constipation and scoliosis (increase intraabdomin Recurrent reflux can be managed with redo fundoplication, though postoperative complications increase significantly. A viable alternative is esophago-gastric dissociation consists of closing the esophagogastric junction and establishing bowel continuity with a Roux-en-y esophagojejunostomy and an end-to-side jejuno-jejunostomy while feed the gastrostomy along with unrestricted oral intake. The procedure allows free passage of saliva avoiding the obstructive nature of a fundoplication (tight wrap) in a child dysmotility. EGD is indicated in children with pathological reflux and severely neurological disorders, poor or none oral feeding and difficult to manage pharyngo-esophage and esophageal dysmotility.

References: 1- Pearl RH, Robie DK, Ein SH, Shandling B, Wesson DE, Superina R, Mctaggart K, Garcia VF, O'Connor, Filler RM: Complications of gastroesophageal antireflux surgery in neurologically impaired versus neurolog Pediatr Surg 25(11):1169-73, 1990 2- Bianchi A: Total Esophagogastric Dissociation: An Alternative Approach. J Pediatr Surg 32(9): 1291-1294, 1997 3- Danielson PD, Emmens RW: Esophagogastric Disconnection for Gastroesophageal Reflux in Children with Severe Neurological Impairment. J Pediatr Surg 34(1): 84-87, 1999 4- Gatti C, Federici di Abriola G, Villa M, De Angelis P, Laviani R, La Sala E, Dall'Oglio L: Esophagogastric Dissociation versus Fundoplication: Which is Best for Severely Neurologically Impaired Children. J Pediat

Testicular Torsion

Whenever an infant or child develops acute scrotal pain, swelling and/or redness of the scrotum the diagnosis of testicular torsion should be highly entertained. Testicular most common pediatric urological emergency. TT can occur extravaginally or intravaginally. Extravaginal TT is a perinatal event (in utero) which occurs when the spermat torsion at the level of the external ring causing strangulation of all the scrotal content. The infant present with a firm, hard, painless scrotal mass. In few cases exploration testis. The infarcted testis is removed and the contralateral testis pexed to avoid a future problem of this nature. Intravaginal TT occurs due to an anomalous attachment vaginalis (bell clapper deformity) causing the gonad to twist upon its vascular pedicle. Due to the disparity in size and weight of pubertal testis, torsion occurs most frequen Child develops sudden scrotal pain, edema, nausea and vomiting. Cremasteric reflex is lost. Color Doppler sonography (readily available and non-invasive) in experienced Technetium scan are both reliable in suggesting the diagnosis. Immediate scrotal exploration is the best means available of establishing the diagnosis and saving a testis in remove a dead testis or relieve the torsion the contralateral testis should also be pexed.

References: 1- Hermann D: The pediatric acute scrotum. Pediatr Ann 18(3):198-202, 1989 2- Cilento BG, Najjar SS, Atala A: Cryptorchidism and testicular torsion. Pediatr Clin North Am 40(6):1133-49, 1993 3- Kass EJ, Lundak B: The acute scrotum. Pediatr Clin North Am 44(5):1251-66, 1997 4- Duncan ND, Gabay L, Coard KC, Antoine M, McDonald A: Torsion of an intra-abdominal testicle in a neonate. Case report and review of the literature. West Indian Med J 46(4):126-7, 1997

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5- Burgher SW: Acute scrotal pain. Emerg Med Clin North Am 16(4):781-809, 1998 6- Munden MM, Trautwein LM: Scrotal pathology in pediatrics with sonographic imaging. Curr Probl Diagn Radiol 29(6):185-205, 2000

Alkaline Reflux

Alkaline (pH > 7) gastroesophageal reflux (AGER) is not so rare in children affecting 10% of all refluxers. Acid reflux is more common (50%), followed by combined acid(20%). AGER causes gastritis and esophagitis and is associated with repetitive vomiting, excessive crying and chronic respiratory disease. In an alkaline environment try deconjugated bile salts produce the greatest injury to the esophageal mucosa. Simultaneous gastroesophageal pH monitoring can help establish the diagnosis. Medication aimed to the individual pH pattern. Use of prokinetics to enhance motility might be a better solution in the child with AGER. The child with an antireflux procedure and gas dysfunction might continue with dyspeptic symptoms and gastritis needing further therapy. The aim is guide clinicians to choose efficient anti-reflux therapy based on the t

References: 1- Vandenplas Y, Loeb H: Alkaline gastroesophageal reflux in infancy. J Pediatr Gastroenterol Nutr 12(4):448-52, 1991 2- Malthaner RA, Newman KD, Parry R, Duffy LF, Randolph JG: Alkaline gastroesophageal reflux in infants and children. J Pediatr Surg 26(8):986-90, 1991 3- Tovar JA, Wang W, Eizaguirre I: Simultaneous gastroesophageal pH monitoring and the diagnosis of alkaline reflux. J Pediatr Surg 28(10):1386-91, 1993

4-Wenzl TG, Silny J, Schenke S, Peschgens T, Heimann G, Skopnik H: Gastroesophageal reflux and respiratory phenomena in infants: status of the intraluminal impedance technique. J Pediatr Gastroenterol Nutr 28 5- Wenzl TG, Moroder C, Trachterna M, Thomson M, Silny J, Heimann G, Skopnik H: Esophageal pH monitoring and impedance measurement: a comparison of two diagnostic tests for gastroesophageal reflux. J Pe 34(5):519-23, 2002

Volume 19 No 06 DECEMBER 2002

Dermatofibrosarcoma Protuberans

Dermatofibrosarcoma protuberans (DFSP) is a rare, low grade malignant soft tissue tumor of the skin and subcutaneous tissue with a high propensity for local invasion and Though mostly seen in middle age, a few cases have been described in infants and children between the ages of 14 months and 12 years. Clinically, DFSP presents as an a firm nodular lesion fixed to the skin but mobile over the deep fascia, with a pattern of slow, progressive growth. Diagnosis is established after excisional biopsy or punch b common location of this tumor is the trunk followed by the extremities. CT-scan is useful to determine the tumor extent of penetration. DFSP has two histologic variants: t grade tumor, and a high-grade rare fibrosarcomatous variant demonstrating necrosis, high mitotic rate (> 10 mitoses high-power fields) and presence of pleomorphic areas associated with a poor clinical outcome. Metastasis is rare. Lung metastasis are most common, while lymph node metastasis is exceedingly. Rare. Wide surgical excision u three centimeters with inclusion of superficial fascia is currently the standard therapy in children. Mohs micrographic surgical excision has wide acceptance among adults c radiosensitive tumor.

References: 1- McKee PH, Fletcher CD: Dermatofibrosarcoma protuberans presenting in infancy and childhood. J Cutan Pathol 18(4):241-6, 1991 2- Keshen TH, Cederna PS, Savell VH Jr, Platz CE, Chang P, Ricciardelli EJ: Clinical and pathological features of pediatric dermatofibrosarcoma protuberans. Ann Plast Surg 35(6):633-7 , 1995 3- Pappo AS, Rao BN, Cain A, Bodner S, Pratt CB: Dermatofibrosarcoma protuberans: the pediatric experience at St. Jude Children's Research Hospital. Pediatr Hematol Oncol 14(6):563-8, 1997 4- Bouyssou-Gauthier ML, Labrousse F, Longis B, Bedane C, Bernard P, Bonnetblanc JM: Dermatofibrosarcoma protuberans in childhood. Pediatr Dermatol 14(6):463-5, 1997 5- Mentzel T, Beham A, Katenkamp D, Dei Tos AP, Fletcher CD: Fibrosarcomatous ("high-grade") dermatofibrosarcoma protuberans: clinicopathologic and immunohistochemical study of a series of 41 cases with significance. Am J Surg Pathol 22(5):576-87, 1998 6- Marcus JR, Few JW, Senger C, Reynolds M: Dermatofibrosarcoma protuberans and the Bednar tumor: treatment in the pediatric population. J Pediatr Surg 33(12):1811-4, 1998 7- Checketts SR, Hamilton TK, Baughman RD: Congenital and childhood dermatofibrosarcoma protuberans: a case report and review of the literature. J Am Acad Dermatol 42(5 Pt 2):907-13, 2000 8- Bowne WB, Antonescu CR, Leung DH, Katz SC, Hawkins WG, Woodruff JM, Brennan MF, Lewis JJ: Dermatofibrosarcoma protuberans: A clinicopathologic analysis of patients treated and followed at a single institution. Cancer 88(12):2711-20, 2000

Congenital Cystic Adenomatoid Malformation

Congenital Cystic Adenomatoid Malformation (CCAM) is a rare lung bud lesion characterized by the presence of a multicystic mass of immature pulmonary tissue due to development of lobar and segmental bronchi. Embryologic origin is cessation of bronchiolar maturation with mesenchymal overgrowth occurring in the late 5th or 6th week CCAM is divided into Type 1 (large, > 2 cm, irregular widely space cysts; the most common type), Type II (smaller, < 1 cm, closer together; more numerous cysts resemb bronchioles), and Type III (large, solid appearance with small unrecognizable cyst). Due to communication between bronchiole-like structure and cyst significant air trappin acute life-threatening respiratory distress (tachypnea, cyanosis and chest wall retraction) in one-third of cases. The other clinical presentation is late-onset pulmonary infe and dyspnea). Most cases have unilobar involvement. Bad prognostic factors associated with CCAM are fetal hydrops, polyhydramnios, increase mediastinal shift, large s presence of pulmonary hypoplasia. Postnatal diagnosis is confirmed with chest CT-Scan. Symptomatic infants with CCAM should undergo immediate resection of the lung lobectomy). Limited lung resection (segmentectomy) preserves more functioning lung parenchyma and represents an alternative in bilobar disease. Since asymptomatic le spontaneous regression, a policy of watchful observation with periodic imaging followed by elective resection is justified. Children with large lesions (> 50%) of the hemith significant chance of requiring surgical intervention. Better height and weight growth has been found in patients operated upon in later childhood compared with those oper

References: 1-Mentzer SJ, Filler RM, Phillips J: Limited pulmonary resections for congenital cystic adenomatoid malformation of the lung. J Pediatr Surg 27(11):1410-3, 1992 3- Miller JA, Corteville JE, Langer JC: Congenital cystic adenomatoid malformation in the fetus: natural history and predictors of outcome. J Pediatr Surg 31(6):805-8, 1996 4- Kim WS, Lee KS, Kim IO, Suh YL, Im JG, Yeon KM, Chi JG, Han BK, Han MC: Congenital cystic adenomatoid malformation of the lung: CT-pathologic correlation. AJR Am J Roentgenol 168(1):47-53, 1997

2- Dumez Y, Mandelbrot L, Radunovic N, Revillon Y, Dommergues M, Aubry MC, Aubry JP, Narcy F, Sonigo P: Prenatal management of congenital cystic adenomatoid malformation of the lung. J Pediatr Surg 28(1

5- Sapin E, Lejeune V, Barbet JP, Carricaburu E, Lewin F, Baron JM, Barbotin-Larrieu F, Helardot PG: Congenital adenomatoid disease of the lung: prenatal diagnosis and perinatal management. Pediatr Surg Int 12( 6- van Leeuwen K, Teitelbaum DH, Hirschl RB, Austin E, Adelman SH, Polley TZ, Marshall KW, Coran AG, Nugent C: Prenatal diagnosis of congenital cystic adenomatoid malformation and its postnatal presentat and natural history. J Pediatr Surg 34(5):794-8, 1999 7- Pinter A, Kalman A, Karsza L, Verebely T, Szemledy F: Long-term outcome of congenital cystic adenomatoid malformation. Pediatr Surg Int 15(5-6):332-5, 1999 8- Keidar S, Ben-Sira L, Weinberg M, Jaffa AJ, Silbiger A, Vinograd I: The postnatal management of congenital cystic adenomatoid malformation. Isr Med Assoc J 3(4):258-61, 2001

Retching

Retching, choking and gagging are severe, debilitating oral-motor dysfunctional complex symptoms seen rarely after fundoplication and gastrostomy placement in neurolo children. Retching is not a symptom of reflux, but the first part of the ejection phase of the emetic reflex consisting of rhythmic contraction of the entire diaphragm and an
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wall muscles. The etiology of this complex symptom is still not known. It is argued that following fundoplication gastric dysrhythmia ensues due to vagal afferent damage, g injury, altered morphology or increased gastric filling. Initial management of postoperative retching includes antiemetics (dopamine or hydroxytryptamine receptors antag domperidone or ondansetron. If symptoms persist, the child can be paced in continuous gastric feedings. With no improvement jejunal feedings bypassing the stomach to m vagal afferent stimulation results in significant relief. Children who retch preoperatively or have a hypersensitive emetic reflex has three times the risk of retching after fu Retching can cause wrap migration or disruption. Development of a paraesophageal hernia from failed crural repair is a surgical correctable cause of postoperative retchin fundoplication.

References: 1- Borowitz SM, Borowitz KC: Oral dysfunction following Nissen fundoplication. Dysphagia 7(4):234-7, 1992 2- Ravelli AM, Milla PJ: Vomiting and gastroesophageal motor activity in children with disorders of the central nervous system. J Pediatr Gastroenterol Nutr 26(1):56-63, 1998 3- Kimber C, Kiely EM, Spitz L: The failure rate of surgery for gastro-oesophageal reflux. J Pediatr Surg 33(1):64-6, 1998 4- Richards CA, Andrews PL, Spitz L, Milla PJ: Nissen fundoplication may induce gastric myoelectrical disturbance in children. J Pediatr Surg 33(12):1801-5, 1998 5- Richards CA, Milla PJ, Andrews PL, Spitz L: Retching and vomiting in neurologically impaired children after fundoplication: predictive preoperative factors. J Pediatr Surg 36(9):1401-4, 2001

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