VOLUME 25, 2005

Volume 25 No 01 JULY 2005


Osteochondroma is the most common benign bone exostosis found in children. Osteochondroma most frequently arise sporadically and as a solitary lesion, but may also arise associated with hereditary multiple exostosis. Hereditary multiple exostosis is an autosomal dominant disorder in which the clinical hallmark is the growth of bony protuberances from long bones causing a variety of orthopedic deformities. In hereditary multiple osteochondromas the prevalence is one in 50,000 individuals. Ten percent of affected children have no family history of multiple exostosis. Median age at the time of diagnosis is three years. Most cases present with an obvious deformity of the forearm, followed by an inequality in the lengths of the limbs, an angular deformity of the knee, or a deformity of the ankle. Symptomatics complications of osteochondroma consists of pain,  fracture, osseous deformity limiting range of motion, vascular injury, neurological compromise, bursa formation and malignant transformation (chondrosarcoma). MRI is the ideal imaging modality in the diagnostic evaluation of symptomatic complications of osteochondromas and often avoids the need for further imaging. Spontaneous resolution of a solitary osteochondroma is rare. Management of symptomatic osteochondromas is surgical excision. Surgical complications associated with excision consist of peroneal neurapraxias, arterial laceration, compartment syndrome and fibular fracture. The surgical risk for the management of osteochondromas is low.

1- Schmale GA, Conrad EU 3rd, Raskind WH: The natural history of hereditary multiple exostoses. J Bone Joint Surg Am 76(7):986-92, 1994
2- Wicklund CL, Pauli RM, Johnston D, Hecht JT: Natural history study of hereditary multiple exostoses. Am J Med Genet 55(1):43-6, 1995
3- Wirganowicz PZ, Watts HG: Surgical risk for elective excision of benign exostoses. J Pediatr Orthop 17(4):455-9, 1997
4- Mehta M, White LM, Knapp T, Kandel RA, Wunder JS, Bell RS. MR imaging of symptomatic osteochondromas with pathological correlation. Skeletal Radiol 27(8):427-33, 1998
5- Ahmed AR, Tan TS, Unni KK, Collins MS, Wenger DE, Sim FH: Secondary chondrosarcoma in osteochondroma: report of 107 patients. Clin Orthop Relat Res (411):193-206, 2003
6- Claikens B, Brys P, Samson I, Baert AL: Spontaneous resolution of a solitary osteochondroma.  Skeletal Radiol 27(1):53-5, 1998

Suture Granuloma

Suture granuloma, commonly known as a stitch abscess, is a fairly common benign complication seen after surgery. Suture material is a foreign body that causes local irritation and tissue necrosis. Suture granuloma can occur many years after the primary surgical procedure. The most common suture causing suture granuloma is braided silk material, a  non-absorbable suture. Monofilament and absorbable sutures carry a lower risk of infection. At skin or subcutaneous level, the granuloma presents as a chronic intermittent indolent infection with a burrow sinus, with no fever or signs of systemic infection. Diagnosis is suggested with ultrasound (hypoechoic lesion). Removal of the suture material is curative. The intra-abdominal presence of foreign material is an important cause of adhesion formation.  Therefore, intra-abdominal contamination with foreign material should be minimized. Suture granulomas mimic neoplasms in clinical appearance. Suture granuloma can occur in the bronchial stump after lung resection, in the lung parenchyma after segmentectomy or as a paravesical mass or abscess after inguinal hernia repair. The paravesical abscess granuloma causes urinary discomfort, swelling, tenderness and microscopic hematuria. Again, definitive treatment is removal of the infected suture material (silk).

1- Brunsvold MA, Reding ME, Kornman KS: Infected suture granuloma: a case report. Int J Oral Maxillofac Implants 6(2):215-7, 1991
2- Lynch TH, Waymont B, Beacock CJ, Wallace DM: Paravesical suture granuloma: a problem following herniorrhaphy. J Urol 147(2):460-2, 1992
3- Nagar H: Stitch granulomas following inguinal herniotomy: a 10-year review. J Pediatr Surg. 28(11):1505-7, 1993
4- Luijendijk RW, de Lange DC, Wauters CC, Hop WC, Duron JJ, Pailler JL, Camprodon BR, Holmdahl L, van Geldorp HJ, Jeekel J: Foreign material in postoperative adhesions. Ann Surg. 223(3):242-8, 1996
5- Nagar H, Kessler A, Graif M: The role of ultrasound in the diagnosis of stitch granulomas following paediatric herniotomy. Pediatr Radiol. 29(11):803-6, 1999
6- Imamoglu M, Cay A, Sarihan H, Ahmetoglu A, Ozdemir O: Paravesical abscess as an unusual late complication of inguinal hernia repair in children. J Urol. 171(3):1268-70, 2004

Vacuum-Assisted Closure

Vacuum-assisted closure (VAC) is a novel method utilized to promote expedite open wound closure. The technique consists of placing an open cell foam into the wound, sealing the site with an adhesive drape while applying subatmospheric pressure (125 mmHg below ambient)  transmitted to the wound in a controlled manner. The technique removes chronic edema and decreases bacterial colonization leading to increased localized blood flow. VAC utilized in acute and chronic open wounds hasten granulation tissue formation. VAC has shown to be cost effective in the management of complex pilonidal sinus disease, sacral and extremity ulcers, sternal and spinal contaminated wounds, traumatic soft tissue wounds, diabetic foot ulcers, vascular insufficiency ulcers and extensive compartmental tissue loss areas. VAC therapy reduces the number of days to healing, reduces hospital stay, affords fewer dressing changes, allows mobility without bulky bandages and faster return to school or work. Further advantages consist of a clean closed system measuring fluid loss, change on a periodic basis rather than daily and enhancement of wound contraction.  Complications with VAC therapy are uncommon.

1- Morykwas MJ, Argenta LC, Shelton-Brown EI, McGuirt W: Vacuum-assisted closure: a new method for wound control and treatment: animal studies and basic foundation. Ann Plast Surg. 38(6):553-62, 1997
2- McGuinness JG, Winter DC, O'Connell PR: Vacuum-assisted closure of a complex pilonidal sinus. Dis Colon Rectum. 46(2):274-6, 2003
3- Sibbald RG, Mahoney J; V.A.C. Therapy Canadian Consensus Group: A consensus report on the use of vacuum-assisted closure in chronic, difficult-to-heal wounds.  Ostomy Wound Manage. 49(11):52-66, 2003
4- Antony S, Terrazas S: A retrospective study: clinical experience using vacuum-assisted closure in the treatment of wounds. J Natl Med Assoc. 96(8):1073-7, 2004
5- Caniano DA, Ruth B, Teich S: Wound management with vacuum-assisted closure: experience in 51 pediatric patients. J Pediatr Surg. 40(1):128-32, 2005
6- Clubley L, Harper L: Using negative pressure therapy for healing of a sternal wound. Nurs Times. 101(16):44-6, 2005

Volume 25 No 02 AUGUST 2005

Gardner Syndrome

Gardner's syndrome refers to a group of children born with familial adenomatous (multiple) polyposis and significant extracolonic manifestations. Familial adenomatous polyposis is an autosomal dominant disorder originating from a germline alteration of the adenomatous polyposis coli gene in the long arm of chromosome 5. The most significant extracolonic manifestation of Gardner's syndrome consists of soft tissue (desmoid) tumors and osteomas. Bowel cancer develops in one-third of patients with Gardner's syndrome from malignant degeneration of the adenomatous polyps. Desmoid tumors are typically benign but locally aggressive slow-growing tumors that surround and compress adjacent vascular structures and viscera. Affected children are asymptomatic until they manifest rectal bleeding or multiple soft/hard tissue tumors. Osteomas appear in the mandible causing dental abnormalities (odontomas, cementomas, cysts, supernumerary teeth). Surveillance by colonoscopy is imperative in affected family members. Development of a subcutaneous fibroma single or multiple that recurs as a desmoid tumor is a sentinel event identifying children with Gardner's syndrome. Management of Gardner's syndrome consists of excision of the soft/hard tissue tumor and removal of the affected colon (proctocolectomy) with preservation of the sphincteric muscle mechanism. Sulindac has been reported to produce drug-induced complete regression of colonic adenomas in Gardner's syndrome.

1- Svanes K, Bjerkeseth T, Ellekjaer E, Gilhuus-Moe O: Gardner's syndrome, diagnosis and treatment. Report on a family. Acta Chir Scand. 145(4):267-72, 1979
2- Naylor EW, Lebenthal E: Early detection of adenomatous polyposis coli in Gardner's syndrome. Pediatrics. 63(2):222-7, 1979
3- Palmer TH Jr: Gardner's syndrome: six generations. Am J Surg. 143(4):405-8, 1982
4- Lev R, Lebenthal E, Rossi T, Lance P: Histochemical and morphological analysis of colonic epithelium from children with Gardner's syndrome and adults bearing adenomatous polyps. J Pediatr Gastroenterol Nutr. 6(3):414-25, 1987
5- Wehrli BM, Weiss SW, Yandow S, Coffin CM: Gardner-associated fibromas (GAF) in young patients: a distinct fibrous lesion that identifies unsuspected Gardner syndrome and risk for fibromatosis. Am J Surg Pathol. 25(5):645-51, 2001
6- Okai T, Yamaguchi Y, Sakai J, Ohtsubo K, Mouri H, Sawabu N: Complete regression of colonic adenomas after treatment with sulindac in Gardner's syndrome: a 4-year follow-up. J Gastroenterol. 36(11):778-82, 2001
7- Campos FG, Habr-Gama A, Kiss DR, Atui FC, Katayama F, Gama-Rodrigues J: Extracolonic manifestations of familial adenomatous polyposis: incidence and impact on the disease outcome. Arq Gastroenterol. 40(2):92-8, 2003
8- Huerta S, Heubner DR, Marcus DR: Mesenteric fibromatosis in a young girl without familial adenomatous polyposis. J Pediatr Surg. 40(5):e33-6, 2005

Turcot Syndrome

Turcot (glioma-polyposis) syndrome refers to the presence of multiple adenomatous polyposis coli associated with glioblastoma multiforme, medulloblastoma, or glioma developing during the pediatric teens. The polyposis in Turcot is associated with a low number of polyps, large polyps over three cm in diameter, and complication by colonic cancer occurring during the second or third decades of life. Turcot syndrome is determined by an autosomal gene with pleiotropic effect and variable expressivity. Children with Turcot syndrome can develop multiple regions of congenital hypertrophy of the retinal pigment epithelium  with areas of surrounding hypopigmentation in the fundi of both eyes, a fact which can help in the diagnosis. Two types of Turcot syndrome have been identified: Type I, also known as true Turcot syndrome (autosomal recessive) with less than 100 intestinal polyps, large size and apt to transform to the malignant tumor. Brain tumor is mainly diagnosed as glioblastoma or astrocytoma and mismatch repair genes might be involved. Type II with  FAP-associated type (autosomal dominant) predisposing to medulloblastoma. Management in both cases is surgical.

1- Itoh H, Ohsato K: Turcot syndrome and its characteristic colonic manifestations. Dis Colon Rectum. 28(6):399-402, 1985
2- Costa OL, Silva DM, Colnago FA, Vieira MS, Musso C: Turcot syndrome. Autosomal dominant or recessive transmission? Dis Colon Rectum. 30(5):391-4, 1987
3- Jamjoom ZA, Sadiq S, Mofti AB, al-Mofleh I, Ajarim D: Turcot syndrome: report of a case and review of the literature. Int Surg. 74(1):45-50, 1989
4- Munden PM, Sobol WM, Weingeist TA: Ocular findings in Turcot syndrome (glioma-polyposis). Ophthalmology. 98(1):111-4, 1991
5- Hamilton SR, Liu B, Parsons RE, Papadopoulos N, Jen J, Powell SM, Krush AJ, Berk T, Cohen Z, Tetu B, et al: The molecular basis of Turcot's syndrome. N Engl J Med. 332(13):839-47, 1995
6- De Vos M, Hayward BE, Picton S, Sheridan E, Bonthron DT: Novel PMS2 pseudogenes can conceal recessive mutations causing a distinctive childhood cancer syndrome. Am J Hum Genet. 74(5):954-64, 2004

Splenic Abscess

Splenic abscess is an uncommon event identified during the pediatric age. Nevertheless, is a potentially fatal disorder if not diagnosed and managed in a timely fashion. Children harboring a splenic abscess present with fever, leukocytosis and left upper quadrant abdominal pain. Simple chest films could be associated with a left pleural effusion or basal atelectasis. Most children with splenic abscess have an associated predisposing medical condition such as sickle cell disease, immune deficiency (HIV), leukemia, aplastic anemia, perforated bowel, typhoid fever, endocarditis, otitis media, appendicitis or trauma. Staphylococci, Salmonella and Escherichia coli are the most common etiologic agents in single abscess, while Candida species predominates in multiple splenic abscess. Many children are septic before the diagnosis is made. Blood cultures are seldom positive. Diagnosis is made with abdominal ultrasound or CT-Scan. Initial management incorporating  the strategy of preserving the spleen consists of CT-guided percutaneous drainage and antibiotics. This combined approach is effective in two-thirds of cases. If the child does not improve, splenectomy follows. Children with immune deficiency and splenic abscess have rapid resolution of symptoms with immediate splenectomy. Some cases with splenic abscess will completely resolve with antibiotics alone.

1- Hadas-Halpren I, Hiller N, Dolberg M: Percutaneous drainage of splenic abscesses: an effective and safe procedure. Br J Radiol. 65(779):968-70, 1992
2- Fernandes ET, Tavares PB, Garcette CB: Conservative management of splenic abscesses in children. J Pediatr Surg. 27(12):1578-9, 1992
3- Smith MD Jr, Nio M, Camel JE, Sato JK, Atkinson JB: Management of splenic abscess in immunocompromised children. J Pediatr Surg. 28(6):823-6, 1993
4- Ooi LL, Leong SS: Splenic abscesses from 1987 to 1995. Am J Surg. 1997 Jul;174(1):87-93
5- Frumiento C, Sartorelli K, Vane D: Complications of splenic injuries: expansion of the nonoperative theorem. J Pediatr Surg. 35(5):788-91, 2000
6- Ng KK, Lee TY, Wan YL, Tan CF, Lui KW, Cheung YC, Cheng YF: Splenic abscess: diagnosis and management. Hepatogastroenterology. 49(44):567-71, 2002
7- Kang M, Saxena AK, Gulati M, Suri S: Ultrasound-guided percutaneous catheter drainage of splenic abscess. Pediatr Radiol. 34(3):271-3, 2004

Volume 25 No 03 SEPTEMBER 2005


Polydactylism (accessory finger or toe) is one of the most common congenital anomaly of hands or feet in children. The prevalence of polydactylism is almost two cases for every each 1000 born alive newborns. Familial occurrence is associated in almost 20% of cases with variable gene penetrance. Polydactylism of the hand is more common than in the foot. In the hand the ulnar accessory finger predominates. A few cases undergo traumatic intrauterine amputation, a condition known as rudimentary polydactylism. Different genes are involved in the pathomorphogenesis of postaxial polydactylism. Polydactyly may be preaxial (medial, thumb side) or tibial (hallux-side), postaxial (lateral) or ulnar (side of the little finger or toe), and central (middle fingers or toes). The duplication may appear at the distal and medial phalanges or at the whole digit. Surgical amputation of the affected finger or toe is indicated for cosmetic reasons or for functional disturbances in wearing shoes respectively. Careful clinical and radiographic evaluation should be made prior to treatment to achieve good functional and cosmetic results. Polydactylism can be diagnosed prenatally and when isolated is associated with good perinatal outcome. Primary suture ligation of accessory  digits in infancy can be associated with later development of neuroma in the stump. Identification and high transection of the accessory digital nerve is essential in the treatment of pedunculated supernumerary digits.

1- Perez-Molina JJ, Alfaro-Alfaro N, Lopez-Zermeno MC, Garcia-Calderon MA: Polydactyly in 26,670 consecutive births. The clinical characteristics, prevalence and risk factors. Bol Med Hosp Infant Mex. 50(11):803-8, 1993
2- Cifuentes L, Nazer J, Huber ME, Ramirez R, Nazer C, Morales I: Polydactyly: a genetic epidemiological study in Santiago, Chile. Rev Med Chil. 124(3):313-8, 1996
3- Bader B, Grill F, Lamprecht E: Polydactyly of the foot. Orthopade. 28(2):125-32, 1999
4- Bromley B, Shipp TD, Benacerraf B: Isolated polydactyly: prenatal diagnosis and perinatal outcome. Prenat Diagn. 20(11):905-8, 2000
5- Leber GE, Gosain AK: Surgical excision of pedunculated supernumerary digits prevents traumatic
amputation neuromas. Pediatr Dermatol. 20(2):108-12, 2003

Postsplenectomy Guidelines

Removal of the spleen in children is considered necessary in some hemolytic diseases and trauma. Hemolytic disease where splenectomy is therapeutic includes idiopathic thrombocytopenic purpura, congenital spherocytosis and Sickle cell disease. Removal of the spleen with its attendant immunologic shortness can cause overwhelming sepsis. Overwhelming postsplenectomy infection is a fulminant process that carries a poor prognosis.The risk of serious infection is highest among young children, in immunologically compromised individuals and in the first few years after splenectomy. Current guidelines for children undergoing elective splenectomy include immunization for pneumococcus, meningococcus and hemophilus at least two weeks prior to the procedure. After emergency splenectomy the immunization protocol is the same, except that the amnestic response of the host is lower. Another guideline for splenectomized children includes the  use of continuous antibiotic prophylaxis (penicillin) until the age of twelve years. Compliance with this antibiotic regimen is poor. It is recommended that parents be advised to bring the postsplenectomy child to the hospital anytime an illness or fever develops that might require an immediate loading dose of an appropriate antibiotic.

1- Posey DL, Marks C: Overwhelming postsplenectomy sepsis in childhood. Am J Surg. 145(3):318-21, 1983
2- Brigden ML, Pattullo AL: Prevention and management of overwhelming postsplenectomy infection--an update. Crit Care Med. 27(4):836-42, 1999
3- Waghorn DJ: Overwhelming infection in asplenic patients: current best practice preventive measures are not being followed. J Clin Pathol. 54(3):214-8, 2001
4- Davies JM, Barnes R, Milligan D; British Committee for Standards in Haematology.  Working Party of the Haematology/Oncology Task Force: Update of guidelines for the prevention and treatment of infection in patients with an absent or dysfunctional spleen. Clin Med. 2(5):440-3, 2002
5- Deodhar M, Kakkar N: An audit of splenectomies in a teaching hospital in North India. Are postsplenectomy guidelines being complied with? J Clin Pathol. 57(4):407-10, 2004
6- Bolton-Maggs PH, Stevens RF, Dodd NJ, Lamont G, Tittensor P, King MJ; General Haematology Task Force of the British Committee for Standards in Haematology: Guidelines for the diagnosis and management of hereditary spherocytosis. Br J Haematol. 126(4):455-74, 2004

Splenic Artery Embolization

Splenic artery embolization as an alternative for splenectomy is a minimally invasive technique that has been utilized for the past 25 years. The procedure is done under local anesthesia, sedation or general anesthesia. Partial splenic artery embolization has been used in cases of thalassemia major to reduce transfusion requirements with variable results. Also in children with secondary hypersplenism or massive spleens due to portal hypertension, variceal bleeding (portal vein thrombosis, biliary atresia and biliary cirrhosis) or myelodysplastic disorders. The leukopenia and thrombocytopenia is corrected temporarily and variceal hemorrhage is ameliorated in most cases. Objective is to embolize at least two-third of the splenic circulation. After splenic artery embolization the child develops prolonged fever, pain in the left hypochondrium, leukocytosis and ileus. If the embolization causes total shutdown of the arterial circulation the child might develop a subcapsular hematoma with effusion, abscess formation or need for open splenectomy. Embolization allows for safe surgical splenectomy. Long term follow-up shows evidence of partial splenic regeneration. Partial splenic embolization is a useful method for reducing serum bilirubin concentrations in patients with hypersplenism following the Kasai procedure for biliary atresia, has also been found  safe and effective minimally invasive treatment for patients with bleeding from a blocked distal splenorenal shunt, and as therapy for post-traumatic splenic artery pseudoaneurysm.

1- Kumpe DA, Rumack CM, Pretorius DH, Stoecker TJ, Stellin GP: Partial splenic embolization in children with hypersplenism. Radiology. 155(2):357-62, 1985
2- Ionescu GO, Daniil C: Splenic artery embolization in hematologic diseases in children. Chir Pediatr. 28(6):285-9, 1987
3- Brandt CT, Rothbarth LJ, Kumpe D, Karrer FM, Lilly JR: Splenic embolization in children: long-term efficacy. J Pediatr Surg. 24(7):642-4, 1989
4- Shah R, Mahour GH, Ford EG, Stanley P: Partial splenic embolization. An effective alternative to splenectomy for hypersplenism. Am Surg. 56(12):774-7, 1990
5- Hickman MP, Lucas D, Novak Z, Rao B, Gold RE, Parvey L, Tonkin IL, Hansen DE: Preoperative embolization of the spleen in children with hypersplenism. J Vasc Interv Radiol. 3(4):647-52, 1992
6- Ando H, Ito T, Nagaya M: Partial splenic embolization decreases the serum bilirubin level in patients with hypersplenism following the Kasai procedure for biliary atresia. J Am Coll Surg. 182(3):206-10, 1996
7- Petersons A, Volrats O, Bernsteins A: The first experience with non-operative treatment of hypersplenism in children with portal hypertension. Eur J Pediatr Surg. 12(5):299-303, 2002
8- Shah SR, Pramesh CS, Deshmukh HL, Mathur SK: Splenic artery embolization for variceal hemorrhage following blocked distal splenorenal shunt. Hepatogastroenterology. 50(52):1167-8, 2003
9- Yardeni D, Polley TZ Jr, Coran AG: Splenic artery embolization for post-traumatic splenic artery pseudoaneurysm in children. J Trauma. 57(2):404-7, 2004

Volume 25 No 03 OCTOBER 2005

Hemolytic-Uremic Syndrome

Hemolytic-Uremic Syndrome (HUS) refers to the constellation of signs and symptoms that occurs after enteric infection with the verotoxic producing bacteria Escherichia Coli 0157:H7. The organisms are carried in the intestines of cattle; partially cooked contaminated hamburger is the single most common vector. E. coli produces a potent cytotoxin that gains access to the circulation, is taken up by glycolipid receptors on intestinal and glomerular endothelial cells causing cell death. Usually the HUS triad consists of an microangiopathic hemolytic anemia, thrombocytopenia and acute renal failure. HUS is the most common cause of acute renal failure in infants and young children. The prodromal phase of HUS starts with fever, vomiting, crampy abdominal pain and bloody diarrhea. In a few occasions the child will develop signs of peritoneal irritation mimicking an acute abdomen. After the prodromal phase the child develops pallor, severe anemia, petechia, oliguria, edema, hypertension and electrolyte disturbances. Due to the associated inflammatory enterocolitis simple abdominal films might show thumbprinting indicative of edema and intestinal mucosal hemorrhage. Treatment of HUS is supportive, but plasma exchange may be useful in selected high-risk subsets. Antibiotics have not yielded any benefit. Plasma infusions and plasma exchange appear to be efficacious. Dialysis is necessary in many children. Surgical complications associated with HUS include bowel perforation (pneumoperitoneum), intussusception or later development of intestinal strictures.

1- Siegler RL: Hemolytic uremic syndrome in children. Curr Opin Pediatr. 7(2):159-63, 1995
2- Gordjani N, Sutor AH, Zimmerhackl LB, Brandis M: Hemolytic uremic syndromes in childhood. Semin Thromb Hemost. 23(3):281-93, 1997
3- Trachtman H, Christen E: Pathogenesis, treatment, and therapeutic trials in hemolytic uremic syndrome. Curr Opin Pediatr. 11(2):162-8, 1999
4- Robson WL: Haemolytic uraemic syndrome. Paediatr Drugs. 2(4):243-52, 2000
5- MacConnachie AA, Todd WT: Potential therapeutic agents for the prevention and treatment of haemolytic uraemic syndrome in shiga toxin producing Escherichia coli infection. Curr Opin Infect Dis. 17(5):479-82, 2004
6- Cleary TG: The role of Shiga-toxin-producing Escherichia coli in hemorrhagic colitis and hemolytic uremic syndrome. Semin Pediatr Infect Dis. 15(4):260-5, 2004

Giant Pigmented Nevus

Giant Pigmented Nevus (GPN) refers to a congenital melanocytic nevi characterized by a diameter of 20 cm or greater in adulthood, or a lesion occupying greater than 2% of the body surface area of an infant or child. Generally, melanocytic nevi is solitary, but smaller satellite lesions may be present within a GPN. The mode of inheritance of GPN is probably multifactorial with a 2:1 female predominance. The lesion is typically brown pigmented, varies in size and shape, contains hair and can be found anywhere on the face, neck, trunk or extremity of the child. GNP extensively involving the extremities can result in reduced growth of the affected limb. GPN has a higher incidence of malignant transformation (melanoma formation) due to the increase number of nevus cells at risk for such transformation. Lifetime incidence of malignant transformation is four to 6%. This capacity of GPN to developed into malignant melanoma is the main reason for opting for prophylactic surgical excision as treatment of choice. Tissues expansion is the primary modality for excision and reconstruction in the face and scalp since it can be started early in life and repeated as required. For the trunk excision with abdominoplasty or skin graft is preferred. In the extremity excision and skin grafting is best option. Dermabrasion or laser therapy cannot ensure complete removal of nevus cells.

1- Borges AF, Lineberger AS: Malignant melanoma without metastasis in a giant nevus. Ann Plast Surg. 12(5):454-60, 1984
2- Bauer BS, Vicari FA: An approach to excision of congenital giant pigmented nevi in infancy and early childhood. J Pediatr Surg. 23(6):509-14, 1988
3- Ruiz-Maldonado R, Tamayo L, Laterza AM, Duran C: Giant pigmented nevi: clinical, histopathologic, and therapeutic considerations. J Pediatr. 120(6):906-11, 1992
4- Rompel R, Moser M, Petres J: Dermabrasion of the congenital nevocellular nevi: experience on 215 patients. Dermatology. 194:261, 1997
5- Hamilton SA, Kirk J, Morris AM: Long-term results of surgical excision and skin grafting for a giant hairy naevus of the face: time for a return to conventional wisdom? Br J Plast Surg. 54(6):543-5, 2001

Alveolar Capillary Dysplasia

Alveolar capillary dysplasia (ACD), also known as acinar dysplasia, is a rare cause of severe and irreversible pulmonary hypertension that usually presents with acute onset respiratory failure during the first 48 hours of life. ACD is characterized by muscularization of pulmonary arterioles, deficient number of alveolar units and pulmonary capillary vessels with thickened interalveolar septa. ACD also shows  abnormally immature parenchymal development in the lungs. The primary pulmonary vascular anomaly is likely to be a failure of fetal lung vascularization dating from the second trimester of fetal life. ACD is uniformly fatal due to inefficient gas exchange. Usual presentation is a term neonate, normal at delivery that develops sudden hypoxia, respiratory acidosis and hypotension within 48 hours of life. Associated anomalies include cardiac, intestinal and GU anomalies. Management consists of mechanical ventilation followed by high frequency ventilation or ECMO. A lung biopsy establishes the histologic diagnosis of this uniformly fatal entity. Open lung biopsy may prevent from using costly, invasive and probably ineffective procedures such as ECMO.

1- Newman B, Yunis E: Primary alveolar capillary dysplasia. Pediatr Radiol. 21(1):20-2, 1990
2- Cullinane C, Cox PN, Silver MM: Persistent pulmonary hypertension of the newborn due to alveolar capillary dysplasia. Pediatr Pathol. 12(4):499-514, 1992
3- Alameh J, Bachiri A, Devisme L, Truffert P, Rakza T, Riou Y, Manouvrier S, Lequien P, Storme L: Alveolar capillary dysplasia: a cause of persistent pulmonary hypertension of the newborn. Eur J Pediatr. 161(5):262-6, 2002
4- Hugosson CO, Salama HM, Al-Dayel F, Khoumais N, Kattan AH: Primary alveolar capillary dysplasia (acinar dysplasia) and surfactant protein B deficiency: a clinical, radiological and pathological study. Pediatr Radiol. 35(3):311-6, 2005
5- Michalsky MP, Arca MJ, Groenman F, Hammond S, Tibboel D, Caniano DA: Alveolar capillary dysplasia: a logical approach to a fatal disease. J Pediatr Surg. 40(6): 1100-1105, 2005

Volume 25 No 05 NOVEMBER 2005


Adhesion formation after abdominal surgery is an expected sequelae which can cause future development of chronic abdominal pain, acute or chronic bowel obstruction or infertility in a minority if patients. Seprafilm is a sodium hyaluronate and carboxymethylcellulose antiadhesion bio adsorbable membrane that is available for use during abdominal surgery. Preclinical studies in animals have shown that Seprafilm is safe and effective in reducing postsurgical adhesions. In adults, seprafilm has shown to be safe and significantly reduces the incidence, extent, and severity of postoperative adhesions to the midline incision compared with no treatment. Has also been found to reduce the formation of postsurgical adhesions in patients with ulcerative colitis or familial polyposis who has undergone restorative proctocolectomy and J-pouch along with gynecologic patients undergoing myomectomy. Seprafilm had no adverse effect on wound healing. Seprafilm adhesion barrier has resulted in a significant reduction of adhesion formation to polypropylene mesh during repair of abdominal wall hernias. The anti-adhesive effects of seprafilm might be caused by increasing the level of peritoneal hydroxyproline levels though experimental evidence suggests the physical properties (barrier, hydroflotation and siliconizing effect) of the membrane are primarily responsible for adhesion prevention. Wrapping the suture or staple line of a fresh bowel anastomosis with seprafilm should be avoided, because the data suggest that this practice may increase the risk of anastomotic leaks.

1- Burns JW, Colt MJ, Burgees LS, Skinner KC: Preclinical evaluation of Seprafilm bioresorbable membrane. Eur J Surg Suppl. (577):40-8, 1997
2- Beck DE: The role of Seprafilm bioresorbable membrane in adhesion prevention. Eur J Surg Suppl. (577):49-55, 1997
3- Baptista ML, Bonsack ME, Delaney JP: Seprafilm reduces adhesions to polypropylene mesh. Surgery. 128(1):86-92, 2000
4- Altuntas I, Tarhan O, Delibas N: Seprafilm reduces adhesions to polypropylene mesh and increases peritoneal hydroxyproline. Am Surg. 68(9):759-61, 2002
5- Beck DE, Cohen Z, Fleshman JW, Kaufman HS, van Goor H, Wolff BG; Adhesion Study Group Steering Committee: A prospective, randomized, multicenter, controlled study of the safety of Seprafilm adhesion barrier in abdominopelvic surgery of the intestine. Dis Colon Rectum. 46(10):1310-9, 2003
6-  Tarhan OR, Eroglu A, Cetin R, Y Nce A, Bulbul M, Altuntas YR: Effects of seprafilm on peritoneal fibrinolytic system. ANZ J Surg. 75(8):690-2, 2005
7- Inoue M, Uchida K, Miki C, Kusunoki M: Efficacy of Seprafilm for reducing reoperative risk in pediatric surgical patients undergoing abdominal surgery. J Pediatr Surg. 40(8):1301-6, 2005

Chronic Granulomatous Disease

Chronic Granulomatous Disease (CGD) is a very rare inherited primary immunodeficiency disease of childhood. Due to defective respiratory burst, phagocytic cells of children with CGD are not able to kill certain bacterias and fungi despite normal chemotaxis and phagocytosis. The proper functioning of the NADPH oxidase of the phagocytic cell is impaired. Children with CGD are rendered susceptible to infection by a group of catalase positive microorganisms due to the inability of phagocytic cells to reduce molecular oxygen and create reactive oxygen metabolites necessary for normal intracellular killing. Catalase positive organisms affecting children with CGD include staph aureus, Escherichia Coli, Serratia, Salmonella, Candida and Aspergillus. Children with CGD present clinically with recurrent abscess involving skin, soft tissue (most common site), lymph nodes, lung, bone and liver that respond poorly to antibiotics needing surgical drainage. Palisading granulomas with central necrosis can be seen in histologic samples. Though four different types of CGD have been described, most cases are X-linked defects. Diagnosis of CGD is made by showing the inability of neutrophils from the patient to undergo respiratory burst after phagocytosis (NBT Test). Fungal infections account for most deaths. Children with CGD should receive long-term Trimethoprim-Sulfamethoxazole prophylaxis. Gamma interferon reduces the number of infection requiring hospitalizations.

1- Frayha HH, Biggar WD: Chronic granulomatous disease of childhood: a changing pattern? J Clin Immunol. 3(3):287-91, 1983
2- Leonard AS, Mulholland MW, Filipovich AH: Surgery of the immunodeficient child. Surg Clin North Am. 65(6):1505-25, 1985
3- Nakhleh RE, Glock M, Snover DC: Hepatic pathology of chronic granulomatous disease of childhood. Arch Pathol Lab Med. 116(1):71-5, 1992
4- Eckert JW, Abramson SL, Starke J, Brandt ML: The surgical implications of chronic granulomatous disease. Am J Surg. 169(3):320-3, 1995
5- San Vicente B, Parri FJ, Castanon M, Sancho MA, Martin Mateos A, Morales L: Chronic granulomatous disease: the surgical aspects. Cir Pediatr. 12(4):148-51, 1999
6- Chen LE, Minkes RK, Shackelford PG, Strasberg SM, Kuo EY, Langer JC: Cut it out: Managing hepatic abscesses in patients with chronic granulomatous disease. J Pediatr Surg. 38(5):709-13, 2003

Primary Peritonitis

Primary peritonitis or spontaneous bacterial peritonitis refers to a diffuse infection of the peritoneum with no obvious focus of infection. Includes peritoneal infection in children with indwelling catheters such as peritoneal cannulas and ventriculo-peritoneal shunts. During the past 20 years the incidence of primary peritonitis has decreased due to the widespread use of systemic antibiotics. The bloodstream is the most common pathway of infection toward the peritoneum. Clinically the child develops high fever, vomiting and generalized peritoneal signs sometimes indistinguishable from acute appendicitis needing urgent surgery. Paracentesis smear or intraoperative gram stain culture when no secondary cause for the peritonitis can be established usually obtains the causative organism of the peritonitis. During exploratory laparotomy no secondary cause is identified and the peritoneal fluid is usually cloudy. Antibiotics should be continued until peritoneal cultures arrive. Most children with primary peritonitis have a smooth recovery.

1- McDougal WS, Izant RJ Jr, Zollinger RM Jr: Primary peritonitis in infancy and childhood. Ann Surg. 181(3):310-3, 1975
2- Fine RN, Salusky IB, Hall T, et al: Peritonitis in children undergoing continuous ambulatory peritoneal dialysis. Pediatrics 71:806, 1983
3- Gaskill SJ, Marlin AE: Spontaneous bacterial peritonitis in patients with ventriculoperitoneal shunts. Pediatr Neurosurg. 26(3):115-9, 1997
4- Hoshii S, Honda M, Itami N, Oh S, Matsumura C, Moriya S, Mori M, Hatae K, Ito Y, Karashima S: Sclerosing encapsulating peritonitis in pediatric peritoneal dialysis patients. Pediatr Nephrol. 14(4):275-9, 2000
5- Mayer MP, Schweizer P: Primary peritonitis in a child caused by Haemophilus parainfluenzae. Pediatr Surg Int. 18(8):728-9, 2002

Volume 25 No 06 DECEMBER 2005

Abdomino-Scrotal Hydrocele

Scrotal hydrocele is fairly commonly seen in infants. Most scrotal hydrocele will disappear during the first year of life of the child as the fluid accumulated in the tunica vaginalis after the processus vaginalis have closed disappears. Abdomino-scrotal hydrocele (ASH), also known as "hydrocele en bissac", is a very rare condition seen in infants consisting of a collection of fluid in the tunica vaginalis extending through the inguinal canal into the abdominal cavity. Clinically, the child with an inguino-scrotal hydrocele has an abdominal mass of variable size and firm consistency characterized by increase in tension of the hydrocele when squeezing the abdominal mass and viceversa. The abdominal compartment can be retroperitoneal or properitoneal. An increasing pressure within the hydrocele is transmitted above the deep inguinal ring because of the inexpansible musculofascial covering of the inguinal canal. The diagnosis of an abdomino-scrotal hydrocele is made with the help of ultrasound or MRI showing the fluid filled cavities in both the abdominal (pelvic) and scrotal compartments in communication. ASH should be differentiated from other cystic tumors of the abdominal cavity such as hydronephrosis, bladder diverticulum, mesenteric cysts and lymphangiomas. ASH has been found to cause obstructive uropathy, reduced blood supply to the testis or hemorrhage. Total excision of the abdomino-scrotal hydrocele through an inguinal approach is the proposed treatment of choice.

1- Burgues PL, Alvarez JA, Hernandez L, Teixidor JL: Abdominoscrotal hydrocele. J Pediatr Surg 21(11):987-8, 1986
2- Khan AH, Yazbeck S: Abdominoscrotal hydrocele: a cause of abdominal mass in children: a case report and review of the literature. J Pediatr Surg 22(9):809-10, 1987
3- Wlochynski T, Wassermann J, Generowicz Z: Abdominoscrotal hydrocele in childhood. J Pediatr Surg 28(2):248-50, 1993
4- Spier LN, Cohen H, Kenigsberg K: Bilateral abdominoscrotal hydrocele: a case report. J Pediatr Surg 30(9):1382-3, 1995
5- Mahomed AA, Stockdale EJ, Varghese J, Youngson GG: Abdominoscrotal hydrocoeles: little place for conservatism. Pediatr Surg Int 13(2-3):186-8, 1998
6- Belman AB: Abdominoscrotal hydrocele in infancy: a review and presentation of the scrotal
approach for correction. J Urol. 165(1):225-7, 2001
7- Celayir AC, Akyuz U, Ciftlik H, Gurbuz A, Danismend N: A critical observation about the pathogenesis of abdominoscrotal hydrocele. J Pediatr Surg 36(7):1082-4, 2001
8- Estevao-Costa J, Morgado H, Soares-Oliveira M, Campos M, Carvalho JL: Hemorrhagic abdominoscrotal hydrocele. A challenging entity. J Pediatr Surg 40: 731-733, 2005

Group B Streptococcus associated Diaphragmatic Hernia

Group B streptococcus (GBS) is a possible cause of chorioamnionitis, endometritis and urinary tract infections in pregnant woman. Maternal transmission of GBS occurs following fever during labor, the rupturing of membranes for more than 18 hours before delivery, prematurity and chorioamnionitis. Group B Beta Hemolytic streptococcal (GBS) infection among newborn infants is usually confined to lung pneumonia, sepsis or meningitis with a high mortality rate. An association between GBS lung infection and late-onset diaphragmatic hernia development has been reported previously. Whenever a child has radiologic evidence of opacification of the diaphragm, persistent atelectasis of the lower lung lobes or an evolving pleural effusion, the diagnosis of late-presentation diaphragmatic hernia should be entertained. The mechanism for the association between GBS and late-presentation right diaphragmatic hernia focus on the abnormal pulmonary compliance produce by the GBS inflammatory process which delay visceral herniation splinting the defect buttressed by the liver. When the GBS inflammation subsides, the intrathoracic pressure reduces promoting the herniation of visceral content through the diaphragm. Diagnosis is made with simple chest films. Management of the late-presenting diaphragmatic hernia is operative after the pneumonia is gone. Prognosis is excellent in most cases.

1- Velaphi S, Siegel JD, Wendel GD Jr, Cushion N, Eid WM, Sanchez PJ: Early-onset group B streptococcal infection after a combined maternal and neonatal group B streptococcal chemoprophylaxis strategy. Pediatrics. 111(3):541-7, 2003
2- McCarten KM, Rosenberg HK, Borden S 4th, Mandell GA: Delayed appearance of right diaphragmatic hernia associated with group B streptococcal infection in newborns. Radiology. 139(2):385-9, 1981
3- Vachharajani AJ, Shah JK, Paes BA: Late-onset left diaphragmatic hernia after group B streptococcal sepsis: An unusual presentation. J Pediatr Surg. 37(6):932-3, 2002
4- Rescorla FJ, Yoder MC, West KW, Grosfeld JL: Delayed presentation of a right-sided diaphragmatic hernia and group B streptococcal sepsis. Two case reports and a review of the literature. Arch Surg. 124(9):1083-6, 1989
5- Handa N, Suita S, Shono T, Kukita J: Right-sided diaphragmatic hernia following group B streptococcal pneumonia and sepsis. J Pediatr Surg. 27(6):764-6, 1992
6- Ashcraft KW, Holder TM, Amoury RA, Hall FK, Rising WD, Hall RT, Sharp RJ: Diagnosis and treatment of right Bochdalek hernia associated with group B streptococcal pneumonia and sepsis in the neonate. J Pediatr Surg. 18(4):480-5, 1983
7- Jain M, Ford WD, Hayward C: Beta haemolytic streptococcal infection does not cause right diaphragmatic eventration.  Pediatr Surg Int. 12(2-3):188-9, 1997

Ribs Exostosis

Exostosis of the ribs is a rare benign condition seen sporadically or most commonly as manifestation of a genetic disorder known as Hereditary Multiple Exostosis (HME). Children  with exostosis of the ribs can develop acute or chronic thoracic pain, hemothorax, pericardial effusion, localized bronchiectasis, brachial plexus palsy and blood vessel entrapment, or create disfiguring bony deformities. Hereditary multiple exostosis is characterized by osteochondroma development in multiple areas of the body. HME is inherited as autosomal dominant, commonly diagnosed by the first decade of life, affecting mainly the long bones of the extremity. The rib exostosis associated with HME can protrude toward the thoracic cavity pleural space and produce hemothorax or pleural effusion due to direct irritation of the diaphragm or pericardium by the sharp growth. Diagnosis is made with CT-Scan. Management consists of resection of the exostosis or the rib either by open thoracotomy or video-assisted thoracoscopy in symptomatic individuals. Prognosis is good in most cases.

1- Teijeira FJ, Baril C, Younge D: Spontaneous hemothorax in a patient with hereditary multiple exostoses. Ann Thorac Surg. 48(5):717-8, 1989
2- Reynolds JR, Morgan E: Haemothorax caused by a solitary costal exostosis. Thorax. 45(1):68-9, 1990
3- Simansky DA, Paley M, Werczberger A, Bar Ziv Y, Yellin A: Exostosis of a rib causing laceration of the diaphragm: diagnosis and management. Ann Thorac Surg. 63(3):856-7, 1997
4- Tang AT, Hulin SJ, Weeden DF: Surgical treatment for an unusual cause of localized bronchiectasis. Ann Thorac Surg. 69(5):1586-7, 2000
5- Alifano M, Morcos M, Molina T, Regnard JF: An unusual cause of hiccup: costal exostosis. Treatment by video-assisted thoracic surgery. Eur J Cardiothorac Surg. 23(6):1056-8, 2003
6- Cowles RA, Rowe DH, Arkovitz MS: Hereditary multiple exostoses of the ribs: an unusual cause of hemothorax and pericardial effusion. J Pediatr Surg 40(7): 1197-1200, 2005

Journal Club