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PEDIATRIC SURGERY UPDATE VOLUME 28, 2007

PSU Volume 28 No 01 JANUARY 2007
Idiopathic Scrotal Edema
This is a very uncommon cause of acute scrotal swelling, but considered the commonest cause of the 'acute scrotum' in prepubertal boys. Idiopathic scrotal swelling is characterized by edema and erythema of the scrotal wall, is usually bilateral and can sometimes involve the shaft of the penis. The swelling and erythema can extend into the abdominal wall and perineum. Children affected with acute scrotal swelling are four to six years in age with symptoms present for less than 24 hours at the time of initial medical evaluation. The cause of the swelling is usually not identified but can be associated to reaction to an allergen, bug bite, contact dermatitis or angioneurotic edema. Leukocytosis is absent, urinalysis is usually normal and urine culture is sterile. Peripheral eosinophilia is present in some patients. A connection with trauma, periurethral disease, or streptococcal disease appears unlikely. The differential diagnosis includes torsion of the testis or one of the testicular appendages, hydrocele, varicocele, trauma, tumor, idiopathic scrotal edema, and Henoch-Schnlein purpura. Color Duplex ultrasound of the scrotum will show increase testicular blood flow and thickening of skin and muscle of the scrotum. Exploration is required when a normal testis cannot absolutely be identified. Swelling usually resolves within two to five days. Management consists of bed rest, reassurance, and oral histamine.

References: 1- Kaplan GW: Acute idiopathic scrotal edema. J Pediatr Surg. 12(5):647-9, 1977 2- Najmaldin A, Burge DM: Acute idiopathic scrotal oedema: incidence, manifestations and aetiology. Br J Surg. 74(7):634-5, 1987 3- Rabinowitz R, Hulbert WC Jr: Acute scrotal swelling. Urol Clin North Am. 22(1):101-5, 1995 4- van Langen AM, Gal S, Hulsmann AR, De Nef JJ: Acute idiopathic scrotal oedema: four cases and a short review. Eur J Pediatr. 160(7):455-6, 2001 5- Klin B, Lotan G, Efrati Y, Zlotkevich L, Strauss S: Acute idiopathic scrotal edema in children--revisited. J Pediatr Surg. 37(8):1200-2, 2002 6- Abul F, Al-Sayer H, Arun N: The acute scrotum: a review of 40 cases. Med Princ Pract. 14(3):177-81, 2005

Epididymitis
Acute inflammation of the epididymis is an infectious process which usually occurs during adolescent years, very rarely during prepubertal ages. The infectious process is caused by a distal urethral obstruction, ectopic ureter entering the seminal vesicles or epididymis, or after instrumentation. Bacterial or viral organisms are involved in the infectious process. Epididymitis seems to be more common than acute testicular torsion. Early clinical manifestations of epididymitis include scrotal edema, pain, erythema, tenderness with an associated reactive hydrocele. The epididymis turns elongated and exquisitely tender to palpation. The differential
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diagnosis includes testicular torsion, torsion of the testicular appendage or idiopathic scrotal edema. The urinalysis will demonstrate pyuria with bacteriuria. Leukocytosis is also identified. Color Doppler ultrasound or testicular scans can determine rapidly if we are dealing with torsion due to reduced or absent testicular blood flow in need of urgent surgery. In the event of doubt or absence of imaging studies the diagnosis of an acute scrotum requires scrotal exploration. Management of epididymitis includes intravenous antibiotics, pain medication, scrotal support and bed rest. Further renal ultrasound and excretory urography are needed after the episode subsides to determine a congenital urologic anomaly.

References: 1- Weber DM, Rosslein R, Fliegel C: Color Doppler sonography in the diagnosis of acute scrotum in boys. Eur J Pediatr Surg. 10(4):235-41, 2000 2- McAndrew HF, Pemberton R, Kikiros CS, Gollow I: The incidence and investigation of acute scrotal problems in children. Pediatr Surg Int. 18(5-6):435-7, 2002 3- Haecker FM, Hauri-Hohl A, von Schweinitz D: Acute epididymitis in children: a 4-year retrospective study. Eur J Pediatr Surg. 15(3):180-6, 2005 4- Nickel JC, Teichman JM, Gregoire M, Clark J, Downey J: Prevalence, diagnosis, characterization, and treatment of prostatitis, interstitial cystitis, and epididymitis in outpatient urological practice: the Canadian PIE Study. Urology. 66(5):935-40, 2005 5- Karmazyn B, Steinberg R, Livne P, Kornreich L, Grozovski S, Schwarz M, Ziv N, Freud E: Duplex sonographic findings in children with torsion of the testicular appendages: overlap with epididymitis and epididymoorchitis. J Pediatr Surg. 41(3):500-4, 2006 6- Schalamon J, Ainoedhofer H, Schleef J, Singer G, Haxhija EQ, Hollwarth ME: Management of acute scrotum in children-the impact of Doppler ultrasound. J Pediatr Surg. 41(8):1377-80, 2006

Gastrostomy
Facilitating feeding directly to the stomach through a gastrostomy tube can be a life saving procedure for children. The most common indications for gastrostomy placement are the permanent or temporary need for enteral feeding access, the need for gastric decompression, and an access route to the esophagus for dilatations. The gastrostomy can be done open, laparoscopically or percutaneously depending on the general health of the child and associated medical conditions. When a child is referred for gastrostomy a reflux work-up should be done if the child has clinical history or sign of reflux. Work-up includes esophagogram and pH analysis. Should the work-up demonstrate reflux an antireflux procedure is recommended. Complications associated with a gastrotomy include those associated with the procedure such as bleeding, leakage with peritonitis, injury to the colon, wound infection, tube malfunction or migration leading to distal bowel obstruction. With time and use of the gastrotomy the child can develop a gastrostomy prolapse, granuloma formation, persistent gastrocutaneous fistula after tube removal, gastrocolic fistulas, volvulus around a malposition tube and erosion of the gastrotomy tube through adjacent organs. Once tube feeding is established there is a positive impact on the lives of the child and family.

References: 1- Gauderer MW Gastrostomy techniques and devices. Surg Clin North Am. 72(6):1285-98, 1992 2- Davies BW, Watson AR, Coleman JE, Rance CH: Do gastrostomies close spontaneously? A review of the fate of gastrostomies following successful renal transplantation in children. Pediatr Surg Int. 17(4):326-8, 2001 3- Sleigh G, Brocklehurst P: Gastrostomy feeding in cerebral palsy: a systematic review. Arch Dis Child. 89(6):534-9, 2004
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4- Janik TA, Hendrickson RJ, Janik JS, Landholm AE: Analysis of factors affecting the spontaneous closure of a gastrocutaneous fistula. J Pediatr Surg. 39(8):1197-9, 2004 5- Conlon SJ, Janik TA, Janik JS, Hendrickson RJ, Landholm AE: Gastrostomy revision: incidence and indications. J Pediatr Surg. 39(9):1390-5, 2004 6- Hazel R: The psychosocial impact on parents of tube feeding their child. Paediatr Nurs. 18(4):19-22, 2006

PSU Volume 28 No 02 FEBRUARY 2007

Gonadoblastoma
Gonadoblastoma is a sex cord gonadal tumor that contains both germ cell and sex cord stromal elements. It occurs almost exclusively in sexually abnormally individuals with gonadal dysgenesis and Y-containing cells, while other cases occur in children with mixed gonadal dysgenesis (mosaic 45XO/46XY). The combination of the Y chromosome with a dysgenetic gonad is all that is needed for a gonadoblastoma or dysgerminoma to develop. The tumor is usually quite small and calcifications are common. Almost 40% of all gonadoblastomas are bilateral. The germ cell component may outgrow the stromal elements and result in the formation of a dysgerminoma. Most cases will appear in young female adults with history of primary amenorrhea during teenage years and virilization. Management of gonadoblastoma consists of removal of both dysgenetic gonads irrespective of the bilaterality of the lesion. Because these tumors occur in up to 50% of patients with gonadal dysgenesis early bilateral prophylactic gonadectomy should be performed. Gonadoblastomas can exhibit either benign or malignant features, though most cases are benign tumors that have a good prognosis after excision. Gonadectomy can either be done open or laparoscopically. With the presence of malignant germ cell elements, chemotherapy will be needed. Other children at risk to develop gonadoblastoma later in life include those with Turners and androgen insensitivity syndrome.

References: 1- Olsen MM, Caldamone AA, Jackson CL, Zinn A: Gonadoblastoma in infancy: indications for early gonadectomy in 46XY gonadal dysgenesis. J Pediatr Surg. 23(3):270-1, 1988 2- Gibbons B, Tan SY, Yu CC, Cheah E, Tan HL: Risk of gonadoblastoma in female patients with Y chromosome abnormalities and dysgenetic gonads. J Paediatr Child Health. 35(2):210-3, 1999 3- Gravholt CH, Fedder J, Naeraa RW, Muller J: Occurrence of gonadoblastoma in females with Turner syndrome and Y chromosome material: a population study. J Clin Endocrinol Metab. 85(9):3199-202, 2000 4- Uno T, Kazui T, Muhammad BA: Laparoscopic surgery for gonadal dysgenesis in children. Surg Laparosc Endosc Percutan Tech. 9(2):151-5, 1999 5- Mazzanti L, Cicognani A, Baldazzi L, Bergamaschi R, Scarano E, Strocchi S, Nicoletti A, Mencarelli F, Pittalis M, Forabosco A, Cacciari E: Gonadoblastoma in Turner syndrome and Y-chromosome-derived material. Am J Med Genet A. 135(2):150-4, 2005 6- Templeman CL, Fallat ME: Bening Ovarian Masses. Semm Pediatr Surg. 14(2): 93-99, 2005 7- Bianco B, Lipay MV, Melaragno MI, Guedes AD, Verreschi IT: Detection of hidden Y mosaicism in Turner's syndrome: importance in the prevention of gonadoblastoma. J Pediatr Endocrinol Metab. 19(9):1113-7, 2006

Encopresis
Encopresis refers to the involuntary loss of formed, semiformed, or liquid stools into the child's underwear in the presence of constipation Solid fecal material accumulated in the distal rectum
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unable to be discharged appropriately produces seepage of more proximally fecal fluid which escapes unconsciously into the cloths of the child. It's a very difficult social and physical problem to manage satisfactorily in the child. Encopresis is a complex abnormal motility disorder, requiring a multidisciplinary approach. The most common causes associated with encopresis consist of slow transit functional constipation, Hirschsprung's disease and anorectal malformations. Severely constipated children with encopresis in whom outpatient management has failed frequently require several days of hospitalization, as well as conventional treatments involving cathartics and enemas. A balanced electrolyte solution of the nonabsorbable polymer polyethylene glycol (GoLytely) offers a safe and efficient method for clearing the intestine in such cases. Children with encopresis have normal functioning internal sphincter and can acquire normal bowel control using biofeedback therapy to correct the abnormal defecation dynamics. A continent appendicostomy (Malone procedure) is a promising treatment that completely cleanses the colon, increases the child's autonomy, and decreases the chance of soiling in intractable cases of encopresis with pseudo-incontinence.

References: 1- Ingebo KB, Heyman MB: Polyethylene glycol-electrolyte solution for intestinal clearance in children with refractory encopresis. A safe and effective therapeutic program. Am J Dis Child. 142(3):340-2, 1988 2- Bulut M, Tekant G: Encopretic children: experience with fifty cases. Turk J Pediatr. 33(3):167-72, 1991 3- Loening-Baucke V: Encopresis and soiling. Pediatr Clin North Am. 43(1):279-98, 1996 4- Iwai N, Iwata G, Kimura O, Yanagihara J: Is a new biofeedback therapy effective for fecal incontinence in patients who have anorectal malformations? J Pediatr Surg. 32(11):1626-9, 1997 5- Hutson JM, McNamara J, Gibb S, Shin YM: Slow transit constipation in children. J Paediatr Child Health. 37(5):426-30, 2001 6- Di Lorenzo C, Benninga MA: Pathophysiology of pediatric fecal incontinence. Gastroenterology. 126(1 Suppl 1):S33-40, 2004

Torsion Fallopian Tubes

Torsion of a fallopian tube is a very rare event presenting in premenarchal girls and postmenarchal teenagers. Factors associated with torsion include abnormally long tube and mesosalpinx, adnexal venous congestion and abnormal peristalsis. Other times extrinsic factors such as pelvic masses or trauma are the principal cause of torsion. Torsion is more common in the right fallopian tube. Diagnosis is difficult due to nonspecific symptoms. Primordial symptoms consist of sudden abdominal pain, nausea, and vomiting. Other times the child will develop abdominal tenderness with peritonitis. Pelvic ultrasound with color Doppler can identify an elongated cystic mass with variable septs and scattered internal echoes. CT-Scan can demonstrate thickened of the affected tube with hemorrhage. The gold standard of diagnosis and management consists of operative laparoscopy. With isolated tubal torsion the tube can be untwisted. If torsion occurs due to a pelvic mass, the mass should be excised. If no mass or accompanied ovarian torsion is present, the isolated tube should be fixed to the peritoneum or cul-de-sac. Unless a high index of suspicion is maintained for torsion of the fallopian tube in adolescent females, this disorder may not be detected until after tubal destruction.

References: 1- Evans JP: Torsion of the normal uterine adnexa in premenarchal girls. J Pediatr Surg. 13(2):195-6, 1978 2- Hockberger RF, Sternbach G: Torsion of the fallopian tube. JACEP. 7(8):315-7, 1978
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3- Ghossain MA, Buy JN, Bazot M, Haddad S, Guinet C, Malbec L, Hugol D, Truc JB, Poitout P, Vadrot D: CT in adnexal torsion with emphasis on tubal findings: correlation with US. J Comput Assist Tomogr. 18(4):619-25, 1994 4- Rizk DE, Lakshminarasimha B, Joshi S: Torsion of the fallopian tube in an adolescent female: a case report. J Pediatr Adolesc Gynecol. 15(3):159-61, 2002 5- Perlman S, Hertweck P, Fallat ME: Paratubal and tubal abnormalities. Semm Pediatr Surg 14(2): 124-134, 2005 6- Pinkert M, Klein Z, Tepper R, Beyth Y: Hydrosalpinx with adnexal torsion in an adolescent virgin patient--A diagnostic dilemma: case report and review of the literature. J Pediatr Adolesc Gynecol. 19(4):297-9, 2006

PSU Volume 28 No 03 MARCH 2007

Budd-Chiari Syndrome
Obstruction to the hepatic venous outflow tract is commonly known as Budd-Chiari Syndrome. The Budd-Chiari syndrome (BCS) in children can be the result of a congenital or acquired web in the inferior vena cava, a thrombotic, inflammatory, neoplastic process or an hypercoagulable state (antithrombin 3 deficiency). Hepatic venous outflow obstruction produces hepatic dysfunction producing abdominal pain, ascites, jaundice, hepatosplenomegaly, portal hypertension and cirrhosis. The factors that influence management of the BCS include the state of hepatic dysfunction, type of presentation (acute or chronic), how much venous occlusion is present and the presence of collateral circulation. Pulsed Doppler ultrasound, venography and liver biopsy are very helpful in diagnosis. Management of BCS in children has included use of anticoagulation, thrombolytic therapy, angioplasty with or without stenting, transjugular intrahepatic portosystemic shunts and surgical portosystemic shunts. This last choice has fewer options in the face of liver transplantation and does not improve survival. The combination of thrombolytic therapy and balloon angioplasty is the best option in the acute setting of BCS or during the first four weeks after development of the syndrome. Late or chronic presentation with established hepatic cirrhosis and portal hypertension sequelae is best managed with liver transplantation. Early diagnosis offers the best possible chance of cure.

References: 1- Singh V, Sinha SK, Nain CK, Bambery P, Kaur U, Verma S, Chawla YK, Singh K: Budd-Chiari syndrome: our experience of 71 patients. J Gastroenterol Hepatol. 15(5):550-4, 2000 2- Perello A, Garcia-Pagan JC, Gilabert R, Suarez Y, Moitinho E, Cervantes F, Reverter JC, Escorsell A, Bosch J, Rodes J: TIPS is a useful long-term derivative therapy for patients with Budd-Chiari syndrome uncontrolled by medical therapy. Hepatology. 35(1):132-9, 2002 3- Benesch M, Urban C, Deutschmann H, Hausegger KA, Hollwarth M: Management of Budd-Chiari syndrome by hepatic vein stenting after extended right hepatectomy. J Pediatr Surg. 37(11):1640-2, 2002 4- Rossle M, Olschewski M, Siegerstetter V, Berger E, Kurz K, Grandt D: The Budd-Chiari syndrome: outcome after treatment with the transjugular intrahepatic portosystemic shunt. Surgery. 135(4):394-403, 2004 5- Yamada T, Tanaka K, Ogura Y, Ko S, Nakajima Y, Takada Y, Uemoto S: Surgical techniques and long-term outcomes of living donor liver transplantation for Budd-Chiari syndrome. Am J Transplant. 6(10):2463-9, 2006 6- Cauchi JA, Oliff S, Baumann U, Mirza D, Kelly DA, Hewitson J, Rode H, McCulloch M, Spearman W, Millar AJ: The Budd-Chiari syndrome in children: the spectrum of management. J Pediatr Surg. 41(11):1919-23, 2006

Pyoderma Gangrenosum
Pyoderma gangrenosum is a rare, poorly understood, ulcerative skin disorder that occurs in all age groups. A systemic disorder such as inflammatory bowel disease (e.g., ulcerative colitis, Crohn's
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disease), malignancy or juvenile rheumatoid arthritis is usually associated in almost three-fourth of cases seen in children. Pyoderma Gangrenosum (PG) often begins as a small pustule, but results in localized skin destruction and ulceration which is characterized by an expanding ulceration with undermined violaceous borders. PG characteristically involves ulceration in the buttocks, thighs and perianal area while sparing the legs. Lower legs are the most commonly affected sites in adults. Infants appear to have an unusual distribution of perianal and genital lesions not often described in other age groups. The distribution of lesions in children is similar, often involving the lower extremities, but pyoderma gangrenosum of the head and face appears to be more common in children. Infants may have ulcers in genital and perianal areas. An altered immune response could be the origin of PG. Diagnosis is established by biopsy. Histologically, lymphocytic and/or leukocytoclastic vasculitis is present in most of the biopsy specimens obtained from the borders of the lesions. The most frequently prescribed treatment for children is systemic corticosteroids, which generally are very effective.

References: 1- Graham JA, Hansen KK, Rabinowitz LG, Esterly NB: Pyoderma gangrenosum in infants and children. Pediatr Dermatol. 11(1):10-7, 1994 2- Dourmishev AL, Miteva I, Schwartz RA: Pyoderma gangrenosum in childhood. Cutis. 58(4):257-62, 1996 3- von den Driesch P: Pyoderma gangrenosum: a report of 44 cases with follow-up. Br J Dermatol. 137(6):1000-5, 1997 4- Mlika RB, Riahi I, Fenniche S, Mokni M, Dhaoui MR, Dess N, Dhahri AB, Mokhtar I: Pyoderma gangrenosum: a report of 21 cases. Int J Dermatol. 41(2):65-8, 2002 5- Dinulos JG, Darmstadt GL, Len MK, Rutledge JC, Murray KF: Infantile Crohn disease presenting with diarrhea and pyoderma gangrenosum. Pediatr Dermatol. 23(1):43-8, 2006 6- Koturoglu G, Vardar F, Ozkinay F, Kurugol Z, Akalin T, Ozkinay C: Pyoderma gangrenosum in a six-month-old boy. Turk J Pediatr. 48(2):159-61, 2006

Sertoli-Leydig Ovarian Tumors

Sertoli-Leydig cell ovarian tumors are rare androgen producing tumors causing masculinization in most girls. A few are nonfunctional tumors. Sertoli-Leydig cell tumors used to be called arrhenoblastoma or androblastomas. One-third of all Sertoli-Leydig cell tumors (SLCT) occurs in children. Most SLCT are unilateral. Histologic diagnosis depends on the presence of heterologous endodermal and mesenchymal components. The androgenic effect of the tumor causes accelerated somatic growth and amenorrhea in prepubertal girls. Postpubertal girls develops irregular menstrual cycles, hirsutism and masculinization. Most affected children usually present with a pelvic mass. Testosterone and alpha-fetoprotein produced by the tumor are used as genetic tumor markers. Diagnosis is usually done by ultrasound or CT-Scan in association with the masculinizing clinical picture. Management consists of unilateral salpingo-oophorectomy. Poorly differentiated tumors might need adjuvant chemotherapy and radiotherapy. Prognosis correlates most meaningfully with the stage and degree of differentiation of the tumor. High-stage tumors are all clinically malignant.

References: 1- Young RH, Scully RE: Ovarian Sertoli-Leydig cell tumors. A clinicopathological analysis of 207 cases. Am J Surg Pathol. 9(8):543-69, 1985 2- Talerman A: Ovarian Sertoli-Leydig cell tumor (androblastoma) with retiform pattern. A clinicopathologic study. Cancer. 60(12):3056-64, 1987
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3- Larsen WG, Felmar EA, Wallace ME, Frieder R: Sertoli-Leydig cell tumor of the ovary: a rare cause of amenorrhea. Obstet Gynecol. 79(5 ( Pt 2)):831-3, 1992 4- Lantzsch T, Stoerer S, Lawrenz K, Buchmann J, Strauss HG, Koelbl H: Sertoli-Leydig cell tumor. Arch Gynecol Obstet. 264(4):206-8, 2001 5- Chen FY, Sheu BC, Lin MC, Chow SN, Lin HH: Sertoli-Leydig cell tumor of the ovary. J Formos Med Assoc. 103(5):38891, 2004 6- Schneider DT, Calaminus G, Harms D, Gobel U; German Maligne Keimzelltumoren Study Group: Ovarian sex cord-stromal tumors in children and adolescents. J Reprod Med. 50(6):439-46, 2005

PSU Volume 28 No 04 APRIL 2007

Gallstone Ileus
Gallstone ileus is a very rare disorder characterized by mechanical obstruction of the gastrointestinal tract due to incipient impacted gallstones that passes through a bilio-enteric fistula. Impaction followed by obstruction can occur at the ileum, duodenum or stomach (Bouveret's syndrome). Diagnosis is usually delayed due to lack of specific signs of biliary disease. The classic triad of Rigler (small bowel obstruction, ectopic gallstones and air in the biliary tree) is visualized on abdominal plain films in only one-thirds of cases. Age ranges from 13 to 87 years with most cases seen in older patients. Most bilio-enteric fistulas are cholecystoduodenal type, with a few choledochoduodenal. Work-up includes ultrasound, upper gastrointestinal series with water soluble contrast medium and contrast enhanced computed tomography (CT). Preoperative diagnosis of gallstone ileus significantly increases by combining plain film and US findings. Management consists initially of simple enterotomy (enterolithotomy) which can be done laparoscopically assisted. This is followed by takedown of the bilioenteric fistula and cholecystectomy in a later stage procedure if the medical condition of the patient permits and he continues symptomatic. Some reports encourage enterolithotomy, repair of the fistula and cholecystectomy in one procedure. Other workers report that enterolithotomy alone is adequate treatment in the elderly, and subsequent cholecystectomy is not mandatory. Early diagnosis and treatment improve the outcome.

References: 1- Kasahara Y, Umemura H, Shiraha S, Kuyama T, Sakata K, Kubota H: Gallstone ileus. Review of 112 patients in the Japanese literature. Am J Surg. 140(3):437-40, 1980 2- Lobo DN, Jobling JC, Balfour TW: Gallstone ileus: diagnostic pitfalls and therapeutic successes. J Clin Gastroenterol. 30(1):72-6, 2000 3- Ripolles T, Miguel-Dasit A, Errando J, Morote V, Gomez-Abril SA, Richart J: Gallstone ileus: increased diagnostic sensitivity by combining plain film and ultrasound. Abdom Imaging. 26(4):401-5, 2001 4- Agresta F, Bedin N: Gallstone ileus as a complication of acute cholecystitis. Laparoscopic diagnosis and treatment. Surg Endosc. 16(11):1637, 2002 5- Doko M, Zovak M, Kopljar M, Glavan E, Ljubicic N, Hochstadter H: Comparison of surgical treatments of gallstone ileus: preliminary report. World J Surg. 27(4):400-4, 2003 6- Masannat Y, Masannat Y, Shatnawei A: Gallstone ileus: a review. Mt Sinai J Med. 73(8):1132-4, 2006 7- Nichol PF, Adzick NS: Gallstone ileus 20 years after a Kasai procedure using a stapled antireflux valve. J Pediatr Surg. 42(1):264-6, 2007

Laparoscopic Gastrostomy
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During the past 20 years the number of children without associated surgical pathology needing a feeding gastrostomy has increased considerably. The most common indications to construct a gastrostomy are a permanent or temporal need for enteral feeding access, need for gastric decompression or an access route to the esophagus for controlled dilatations. The gastrostomy can be constructed using an open, percutaneous or laparoscopic technique. In addition when the child has previous gastric surgery or surgery in the upper abdomen, is obese, or an anatomic distortion of the body such as kyphoscoliosis precluding percutaneous placement of a gastrostomy the procedure can be done laparoscopically completely or laparoscopic-assisted percutaneously endoscopic gastrostomy. The lap procedure uses a two-trocars technique, stomach is insufflated and fasteners (T-anchors) or sutures are passed through the abdominal wall to fix the stomach. Using seldinger technique a needle is passed to the stomach followed by a guide wire and progressive dilatations until finally a properly sized gastrostomy tube is passed and the balloon inflated. Alternatively, the child can undergo a percutaneous placement of the gastrostomy under laparoscopic vision to avoid complications such as colon perforation in unique anatomic distorted children.

References: 1- Kellnar ST, Till H, Bohn R: Laparoscopically assisted performance of gastrostomy--a simple, safe and minimal invasive technique. Eur J Pediatr Surg. 9(5):297-8, 1999 2- Tomicic JT, Luks FI, Shalon L, Tracy TF: Laparoscopic gastrostomy in infants and children. Eur J Pediatr Surg. 12(2):10710, 2002 3- Zamakhshary M, Jamal M, Blair GK, Murphy JJ, Webber EM, Skarsgard ED: Laparoscopic vs percutaneous endoscopic gastrostomy tube insertion: a new pediatric gold standard? J Pediatr Surg. 40(5):859-62, 2005 4- Yu SC, Petty JK, Bensard DD, Partrick DA, Bruny JL, Hendrickson RJ: Laparoscopic-assisted percutaneous endoscopic gastrostomy in children and adolescents. JSLS. 9(3):302-4, 2005 5- Backman T, Arnbjornsson E, Berglund Y, Larsson LT: Video-assisted gastrostomy in infants less than 1 year. Pediatr Surg Int. 22(3):243-6, 2006 6- Kawahara H, Kubota A, Okuyama H, Shimizu Y, Watanabe T, Tani G, Hiroaki Y, Okada A: One-trocar laparoscopy-aided gastrostomy in handicapped children. J Pediatr Surg. 41(12):2076-80, 2006

Feeding Jejunostomy
Neurologically impaired children benefit from receiving alimentation directly into the stomach. On occasion due to gastric emptying dysfunction, severe retching, or unmanageable recurrent gastroesophageal reflux after failed fundoplication the need for a feeding alternative arises. Such alternative could be feeding directly into the jejunum while venting the stomach. Feeding directly to the jejunum can be done with transpyloric gastrojejunal tube placement, catheter or needle jejunostomy, transgastric jejunostomy through a preexisting gastrostomy, or creating an open rouxen-y tube feeding jejunostomy. The most significant complications are prolapse, leakage and perforation of the stoma. Feeding does not have to be elemental diet only. Children with unmanageable seizure activity in need of multiple drug therapy might not benefit of jejunostomy feeding. The roux-en-y jejunostomy can be performed laparoendoscopically. The use of gastrojejunostomy tubes can be hampered by frequent need of tube manipulation, tube brokage, blockaded or dislodgement.

References: 1- DeCou JM, Shorter NA, Karl SR: Feeding Roux-en-Y jejunostomy in the management of severely neurologically impaired
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children. J Pediatr Surg. 28(10):1276-9, 1993 2- Yoshida NR, Webber EM, Gillis DA, Giacomantonio JM: Roux-en-Y jejunostomy in the pediatric population. J Pediatr Surg. 31(6):791-3, 1996 3- Gilchrist BF, Luks FI, DeLuca FG, Wesselhoeft CW Jr: A modified feeding Roux-en-Y jejunostomy in the neurologically damaged child. J Pediatr Surg. 32(4):588-9, 1997 4- Langer JC, Mazziotti MV, Winthrop AL: Roux-en-Y jejunostomy button in infants. Pediatr Surg Int. 16(1-2):40-2, 2000 5- Neuman HB, Phillips JD: Laparoscopic Roux-en-Y feeding jejunostomy: a new minimally invasive surgical procedure for permanent feeding access in children with gastric dysfunction. J Laparoendosc Adv Surg Tech A. 15(1):71-4, 2005 6- Raval MV, Phillips JD: Optimal enteral feeding in children with gastric dysfunction: surgical jejunostomy vs image-guided gastrojejunal tube placement. J Pediatr Surg. 41(10):1679-82, 2006

PSU Volume 28 No 05 MAY 2007

Portal Hypertension
Portal hypertension (PH) in children is caused by increased portal venous flow from such conditions as hemangiomas or hepatic arterioportal fistulas, or by increase resistance to flow from conditions such as portal vein thrombosis, liver cirrhosis, congenital fibrosis, biliary atresia, neonatal hepatitis or hepatic vein thrombosis. In children, extrahepatic obstruction due to portal vein thrombosis is the most common cause. Most common presentation of PH is upper gastrointestinal bleeding from esophageal or gastric varices, followed by splenomegaly with hypersplenism. Diagnostic studies include liver function tests, upper endoscopy, color Doppler US, splenoportography and MRI. Initial management of PH can entail the use of vasoactive beta blockers such as propanolol or somatostatin. Bleeding varices can be managed with banding or sclerotherapy. Children with favorable liver function, but unfavorable anatomy and continuous variceal bleeding can benefit from a devascularization procedure. Those with unfavorable liver function and bleeding can benefit from transjugular intrahepatic portosystemic shunt (TIPS), though shunt thrombosis is a problem the smaller the kid. Children with favorable anatomy can benefit from a distal splenorenal (or splenoadrenal) shunt, or a makeshift shunt such as the Rex shunt between the inferior mesenteric vein and a branch of the portal vein high in the hepatic hilum using autologous vein graft. Liver transplantation is the treatment of choice for children with PH complicating end-stage liver cirrhosis.

References: 1- Karrer FM: Portal hypertension. Semin Pediatr Surg. 1(2):134-44, 1992 2- Maksoud JG, Goncalves ME: Treatment of portal hypertension in children. World J Surg. 18(2):251-8, 1994 3- Karrer FM, Narkewicz MR: Esophageal varices: current management in children. Semin Pediatr Surg. 8(4):193-201, 1999 4- Ryckman FC, Alonso MH: Causes and management of portal hypertension in the pediatric population. Clin Liver Dis. 5(3):789-818, 2001 5- Ling SC: Should children with esophageal varices receive beta-blockers for the primary prevention of variceal hemorrhage? Can J Gastroenterol. 19(11):661-6, 2005 6- Schettino GC, Fagundes ED, Roquete ML, Ferreira AR, Penna FJ: Portal vein thrombosis in children and adolescents. J Pediatr (Rio J). 82(3):171-8, 2006

Breast Papilloma
Breast juvenile papilloma in children is a rare benign lesion featuring atypical papillary duct
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hyperplasia and numerous cysts. They manifest clinically as a localized, multinodular mass that is usually interpreted as a juvenile fibroadenoma. Most cases occur in females, though some cases in males have been reported. Mean age of diagnosis occurs during the late adolescent years. Left breast is affected slightly more often than the right. Patterns of menarche, marital history, parity, and use of birth control pills are not exceptional for women in this age group. No instance is found of maternal use of estrogens during pregnancy. Family history of breast carcinoma is seen in onethirds of all cases of papillomatosis. Juvenile secretory carcinoma can be associated with papillomatosis. Breast ultrasonography will show an ill-defined, inhomogeneous mass with numerous small, hypoechoic areas, but cannot differentiate a fibroadenoma from papilloma. Excisional biopsy through a periareolar incision will establish the diagnosis. Should a secretory carcinoma be found wide local excision is warranted. Due to the precancerous nature of papillomatosis, long-term yearly follow-up is recommended.

References: 1- Rosen PP, Holmes G, Lesser ML, Kinne DW, Beattie EJ: Juvenile papillomatosis and breast carcinoma. Cancer. 55(6):134552, 1985 2- Ferguson TB Jr, McCarty KS Jr, Filston HC: Juvenile secretory carcinoma and juvenile papillomatosis: diagnosis and treatment. J Pediatr Surg. 22(7):637-9, 1987 3- Batchelor JS, Farah G, Fisher C: Multiple breast papillomas in adolescence. J Surg Oncol. 54(1):64-6, 1993 4- Rice HE, Acosta A, Brown RL, Gutierrez C, Alashari M, Mintequi D, Rodriguez A, Chavarrfa O, Azizkhan RG: Juvenile papillomatosis of the breast in male infants: two case reports. Pediatr Surg Int. 16(1-2):104-6, 2000 5- Ohlinger R, Schwesinger G, Schimming A, Kohler G, Frese H: Juvenile papillomatosis (JP) of the female breast (Swiss Cheese Disease) -- role of breast ultrasonography. Ultraschall Med. 26(1):42-5, 2005 6- Sonmez K, Turkyilmaz Z, Karabulut R, Demirogullari B, Ozen IO, Moralioglu S, Basaklar AC, Kale N: Surgical breast lesions in adolescent patients and a review of the literature. Acta Chir Belg. 106(4):400-4, 2006

Pelvic Inflammatory Disease

Acute pelvic inflammatory disease (PID) is a major gynecologic health problem in the USA, afflicting more than 1 million women each year. PID continues to be a common diagnosis among adolescent girls presenting with low abdominal pain. Adolescents have a higher rate of diagnosis of PID than any other age group. PID is an ascending polymicrobial infection affecting the upper genital tract. Risk factors associated to PID include young age, age at first intercourse, multiple sex partners, the presence of bacterial vaginosis, vaginal douching, the use of an intrauterine contraceptive device, and a history of a sexually transmitted disease. Classic symptoms of pain, fever, and a history of high-risk sexual behavior, is easily diagnosed with a high degree of specificity in PID. Unfortunate, most females with PID demonstrate atypical symptoms which sometimes mimic appendicitis discovering the disease during the appendectomy. Abnormal vaginal discharge full of neutrophils is an indicator of PID, along with a positive vaginal culture for Chlamydia or Gonorrhea. Management of PID entails the use of broad-spectrum antibiotics, which represent the cornerstone of therapy and must adequately cover the polymicrobial spectrum of pathogens implicated in this infection, which includes Neisseria gonorrhoeae, Chlamydia trachomatis, and specific cervicovaginal anaerobic and aerobic bacteria. Sequelae associated with PID includes infertility, ectopic pregnancy, and chronic pelvic pain syndromes. The sexual partner of the affected patient should also be treated.

References:
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1- Soper DE: Surgical considerations in the diagnosis and treatment of pelvic inflammatory disease. Surg Clin North Am. 71(5):947-62, 1991 2- Quan M: Pelvic inflammatory disease: diagnosis and management. J Am Board Fam Pract. 7(2):110-23, 1994 3- Blythe MJ: Pelvic inflammatory disease in the adolescent population. Semin Pediatr Surg. 7(1):43-51, 1998 4- Patel DR: Management of pelvic inflammatory disease in adolescents. Indian J Pediatr. 71(9):845-7, 2004 5- Banikarim C, Chacko MR: Pelvic inflammatory disease in adolescents. Semin Pediatr Infect Dis. 16(3):175-80, 2005 6- Song AH, Advincula AP: Adolescent chronic pelvic pain. J Pediatr Adolesc Gynecol. 18(6):371-7, 2005

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Vaginal Bleeding
Vaginal bleeding in the pre-menstrual female infant is cause for concern both medically and socially. Differential diagnosis of vaginal bleeding in this age group includes estrogen stimulation, vulvovaginitis, tumors of the lower genital tract, ovarian tumors, foreign bodies, or trauma. Transplacental estrogen stimulation can cause self-limited vaginal bleeding in newborns during the first two weeks of life. Vulvovaginitis is the most common gynecological infection in children caused by ascending enteric organisms due to poor hygiene and is managed with systemic antibiotics. Condylomas can cause painless vaginal bleeding. Tumors of the genital tract associated with vaginal bleeding include hemangiomas of the vulva, arteriovenous malformation of the uterus, rhabdomyosarcoma botryoid (the most common malignant tumor of the low genital tract in young females), endodermal sinus tumors of the vagina and endometrial carcinomas. Functional ovarian or adrenal tumors that produce estrogen can be associated with sexual precocity and vaginal bleeding. Foreign bodies in the vagina of a small girl produce local inflammation resulting in a foul smelling discharge which can be serosanguineous. The debris (foreign body) is often wads of toilet paper. Redundant urethral mucosa may prolapse through the urethral meatus and present as a friable polypoid lesion. Finally genital injury is a major cause of vaginal bleeding including those associated with child sexual abuse.

References: 1- Imai A, Horibe S, Tamaya T: Genital bleeding in premenarcheal children. Int J Gynaecol Obstet. 49(1):41-5, 1995 2- Merritt DF: Evaluation of vaginal bleeding in the preadolescent child. Semin Pediatr Surg. 7(1):35-42, 1998 3- Aribarg A, Phupong V: Vaginal bleeding in young children. Southeast Asian J Trop Med Public Health. 34(1):208-12, 2003 4- Sugar NF, Graham EA: Common gynecologic problems in prepubertal girls. Pediatr Rev. 27(6):213-23, 2006 5- Striegel AM, Myers JB, Sorensen MD, Furness PD, Koyle MA: Vaginal discharge and bleeding in girls younger than 6 years. J Urol. 176(6 Pt 1):2632-5, 2006

Vocal Cord Paralysis

Unilateral or bilateral vocal cord paralysis (VCP) in children can be associated with ventricular septal defects, enlargement of the auricle, abnormalities of the great vessels, patent ductus arteriosus, or during operations for division of cervical tracheo-esophageal fistulas and surgery for congenital heart abnormalities or the neck (thyroidectomy). Most cases are iatrogenic with the left recurrent laryngeal nerve with a longer anatomic course being affected more often. VCP is the second most common cause of neonatal stridor. The baby with unilateral VCP will show a weak,
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breathy cry, aspiration and cyanotic attacks with choking during feeding. Bilateral damage produces abduction of the cords, constant stridor and cyanotic attacks needing a tracheostomy. Diagnosis is made with direct video-laryngoscopy. Management strategies should be individualized and focus on maintenance of a safe and stable airway, acquisition of intelligible speech, and deglutition without aspiration. Unilateral VCP treatment is conservative including thickened feeding and anti-reflux measures. Laryngeal incompetence can be managed with injectable collagen. Irrespective of cause, morbidity associated with unilateral VCP is minimal. Although tracheotomy is not required, careful airway observation is important. Should tracheostomy be constructed vocal cord lateralization procedures with partial arytenoidectomy afford the highest operation-specific decannulation rate.

References: 1- Benjamin BN, Gray SD, Bailey CM: Neonatal vocal cord paralysis. Head Neck. 15(2):169-72, 1993 2- Zbar RI, Smith RJ: Vocal fold paralysis in infants twelve months of age and younger. Otolaryngol Head Neck Surg. 114(1):18-21, 1996 3- de Jong AL, Kuppersmith RB, Sulek M, Friedman EM: Vocal cord paralysis in infants and children. Otolaryngol Clin North Am. 33(1):131-49, 2000 4- Hartnick CJ, Brigger MT, Willging JP, Cotton RT, Myer CM 3rd: Surgery for pediatric vocal cord paralysis: a retrospective review. Ann Otol Rhinol Laryngol. 112(1):1-6, 2003 5- Patel NJ, Kerschner JE, Merati AL: The use of injectable collagen in the management of pediatric vocal unilateral fold paralysis. Int J Pediatr Otorhinolaryngol. 67(12):1355-60, 2003 6- Miyamoto RC, Parikh SR, Gellad W, Licameli GR: Bilateral congenital vocal cord paralysis: a 16-year institutional review. Otolaryngol Head Neck Surg. 133(2):241-5, 2005

Stings
The insects that inflict more venous stings than any other in children are the bees and ants. Stings from bees and wasps produce a local tissue reaction with a wheal and flair. Symptoms develop within twenty minutes of the sting and include urticaria, syncope and respiratory distress. Most serious sequelae is anaphylaxis which occur when the child has been previously inoculated. More than 500 stings are needed to cause death in a child. Management is local and systemic. The venom can be removed if the event has less than 20 minutes. Cold compresses will reduce pain associated with the sting and baking soda helps with the itching. Systemic support includes airway control, alpha agonists medication, inhaled beta agonist for bronchospasm and calcium for muscle spasms. Best prophylaxis is reducing exposure. Fire ants' sting can produce edema, pruritus, erythema, pain and burning with a characteristic wheal. Wound is cleaned with soap and water. Rarely systemic management is needed.

References: 1- Stawiski MA: Insect bites and stings. Emerg Med Clin North Am. 3(4):785-808, 1985 2- Solley GO: Allergy to stinging and biting insects in Queensland. Med J Aust. 153(11-12):650-4, 1990 3- Schultze-Werninghaus C, Wahn U, Niggemann B: Evaluation of the risk of anaphylactic reactions by wasp venom-extract challenges in children. Pediatr Allergy Immunol. 10(2):133-7, 1999 4- Cohen PR: Imported fire ant stings: clinical manifestations and treatment. Pediatr Dermatol. 9(1):44-8, 1992 5- Nguyen SA, Napoli DC: Natural history of large local and generalized cutaneous reactions to imported fire ant stings in children. Ann Allergy Asthma Immunol. 94(3):387-90, 2005

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