VOLUME 33, 2009

PSU Volume 33 No 01 JULY 2009

Benign Mesothelioma

Benign cystic mesothelioma (BCM) also known as cystic mesothelioma of the peritoneum is a rare abdominal tumor found in young women during their third decade of life. It originates from the pelvic retroperitoneum with a predilection for pelvic visceral serosal surfaces. It a neoplastic tumor with a high tendency for recurrence not associated to asbestos exposure. The tumor produce cysts that are usually intraperitoneal in location but also the chest and pericardium can be affected. Clinically the child presents with progressive painless abdominal distension. Most reliable method of preoperative diagnosis is with aspiration and cellular analysis of peritoneal washing showing abundant mesothelial cells. The role of immunohistochemistry and electron microscopy in diagnosis can be important. CT-Scan provides an idea of the extension of the benign tumor. The differential diagnosis includes a cystic lymphangioma. BCM shows multiplicity, extensive involvement of the peritoneal and serosal surfaces without significant organ invasion. Management of BCM is surgical excision. The cysts appear  almost avascular. Multiple resections are common due to the recurrence rate of the tumor. Prognosis depends on the rate of recurrence and postoperative complications from multiple abdominal procedures.

1- Raafat F, Egan M: Benign cystic mesothelioma of the peritoneum: immunohistochemical and
ultrastructural features in a child. Pediatr Pathol. 8(3):321-9, 1988
2- Hanukoglu A, Gewurtz G, Zaidel L, Krispin M, Fried D: Benign cystic mesothelioma of the peritoneum: the occurrence of an adult entity in a child. Med Pediatr Oncol. 20(2):169-71, 1992
3- McCullagh M, Keen C, Dykes E: Cystic mesothelioma of the peritoneum: a rare cause of 'ascites' in children. J Pediatr Surg. 29(9):1205-7, 1994
4- Scattone A, Pennella A, Giardina C, Marinaccio M, Ricco R, Pollice L, Serio G: Polycystic mesothelioma of the peritoneum. Description of 4 cases. Pathologica. 93(5):549-55, 2001
5- Terry NE, Fowler CL: Benign cystic mesothelioma in a child. J Pediatr Surg. 44(5):e9-11, 2009

Anaplastic Thyroid Cancer

Anaplastic or undifferentiated thyroid cancer occurs very rarely during young adults and children. Most cases of thyroid malignancy are well differentiated papillary and follicular variants. Other times medullary thyroid carcinoma associated with multiple endocrine neoplasia syndromes. Nevertheless it's good to learn what is the characteristic of this unusual disease in young adults and children. Poorly-differentiated thyroid carcinoma is a stage in the development of anaplastic carcinoma from well-differentiated neoplastic transformation of follicular epithelium. The BRAFT1799A mutation has been identified. In patients under 40 years of age, encapsulated poorly-differentiated tumors are more frequent with a trabecular histological pattern alone with a much smaller size (30 mm). Pathologic criteria used in the diagnosis of poorly or undifferentiated thyroid carcinoma includes (1) presence of a solid/trabecular/insular pattern of growth, (2) absence of the conventional nuclear features of papillary carcinoma, and (3) presence of at least one of the following features: convoluted nuclei; mitotic activity >or =3 x 10 HPF; and tumor necrosis. Among papillary carcinomas, the frequency of a solid growth pattern, a criterion for classifying a tumor as poorly differentiated, was higher in the Belarus region of Russia than that in Japan. Management of anaplastic thyroid malignancy is total thyroidectomy, since chemotherapy, radiotherapy and radioiodine are of no avail.

1- Datta C, Bhattacharyya S, Ghosh A, Ghosh S: Dedifferentiated papillary carcinoma of thyroid in an adolescent girl--a case report. Indian J Pathol Microbiol. 48(4):496-7, 2005
2- Volante M, Collini P, Nikiforov YE, Sakamoto A, Kakudo K, Katoh R, Lloyd RV, LiVolsi VA, Papotti M, Sobrinho-Simoes M, Bussolati G, Rosai J: Poorly differentiated thyroid carcinoma: the Turin proposal for the use of uniform diagnostic criteria and an algorithmic diagnostic approach. Am J Surg Pathol. 31(8):1256-64, 2007
3- Shirahige Y, Ito M, Ashizawa K, Motomura T, Yokoyama N, Namba H, Fukata S, Yokozawa T, Ishikawa N, Mimura T, Yamashita S, Sekine I, Kuma K, Ito K, Nagataki S: Childhood thyroid cancer: comparison of Japan and Belarus. Endocr J. 45(2):203-9, 1998
4- Kumagai A, Namba H, Mitsutake N, Saenko VA, Ohtsuru A, Ito M, Noh JY, Sugino K, Ito K, Yamashita S: Childhood thyroid carcinoma with BRAFT1799A mutation shows unique pathological
features of poor differentiation. Oncol Rep. 16(1):123-6, 2006
5- Gétaz EP, Shimaoka K, Rao U: Anaplastic carcinoma of the thyroid following external irradiation. Cancer. 43(6):2248-53, 1979

Genital Tumors

Tumors occurring in the vulva and external vaginal orifice are rare to find in female children. In the area of the vulva the most common tumor is either and hemangioma, lymphangioma, lipomas, neurofibromatosis and vulvar intraepithelial neoplasia. Hemangiomas resolve with conservative therapy, while lymphangiomas or other type of tumor will require surgical excision. Vulva intraepithelial neoplasia are associated with cases of sexual abuse and human papilloma viral infections. In the external vaginal orifice bleeding hemangiomas have been previously reported in children. Cavernous hemangiomas with brisk and continuous bleeding will require some form of therapy such as cryosurgery, excision or steroid injection. Interferon has also been used effectively. It is always important to study with imaging (MRI) the extension of the perineal hemangioma. Another lesion of importance in this privilege area is the vaginal rhabdomyosarcoma which presents with protrusion and a bleeding mass. Management consists of a biopsy, chemotherapy followed by surgery if necessary.

1- Breen JL, Bonamo JF, Maxson WS: Genital tract tumors in children. Pediatr Clin North Am. 28(2):355-67, 1981
2- Imai A, Furui T, Tamaya T: Gynecologic tumors and symptoms in childhood and adolescence; 10-years' experience. Int J Gynaecol Obstet. 45(3):227-34, 1994
3- Martelli H, Oberlin O, Rey A, Godzinski J, Spicer RD, Bouvet N, Haie-Meder C, Terrier-Lacombe MJ, Sanchez de Toledo J, Spooner D, Sommelet D, Flamant F, Stevens MC: Conservative treatment for girls with nonmetastatic rhabdomyosarcoma of the genital tract: A report from the Study Committee of the International Society of  Pediatric Oncology. J Clin Oncol. 17(7):2117-22, 1999
4- Groff DB: Pelvic neoplasms in children. J Surg Oncol. 77(1):65-71, 2001
5- Monk BJ, Tewari KS: The spectrum and clinical sequelae of human papillomavirus infection. Gynecol Oncol. 107(2 Suppl 1):S6-13, 2007

PSU Volume 33 No 02 AUGUST 2009

MIS for Children Cancer

Minimal invasive surgical (MIS) procedures through the use of  laparoscopy and thoracoscopy have evolved slowly in the actual management of children with solid abdominal tumors and cancer in other sites of the body. The main indication is biopsy or simply determination of resectability of large abdominal tumors not amenable to immediate surgical therapy, along with thoracoscopic biopsy of lung metastasis. Adequate tissue with minimal surgical trauma can be obtained in most of these children. In the case of neuroblastoma, thoracoscopy has been also useful in resection of residual tumors and primary tumors of the posterior mediastinum. The efficacy of laparoscopic adrenalectomy  for metastatic lesions, benign tumors, and small to medium neuroblastomas (two to 4 cm in diameter) is well established. Trocar site metastasis can be averted by retrieving the specimen within an endobag. Ovarian tumors are amenable to laparoscopic resection following strict protocol observation and managing of tissue extraction. The length of hospital stay,  time to start postoperative feeding,  time to start postoperative chemotherapy and postop discomfort (postoperative pain,  analgesics requirement, postoperative ileus)  is significantly shorter in the group of patients who undergoes MIS procedures. Role of MIS in Wilm's tumor, rhabdomyosarcoma and hepatoblastoma is limited to biopsy and staging. MIS can be used safely and successfully to diagnose children with suspicious solid neoplasms.

1- Sailhamer E, Jackson CC, Vogel AM, Kang S, Wu Y, Chwals WJ, Zimmerman BT, Hill CB, Liu DC: Minimally invasive surgery for pediatric solid neoplasms. Am Surg. 69(7):566-8, 2003
2- Iwanaka T, Arai M, Kawashima H, Kudou S, Fujishiro J, Imaizumi S, Yamamoto K, Hanada R, Kikuchi A, Aihara T, Kishimoto H: Endosurgical procedures for pediatric solid tumors. Pediatr Surg Int. 20(1):39-42, 2004
3- Saad DF, Gow KW, Milas Z, Wulkan ML: Laparoscopic adrenalectomy for neuroblastoma in children: a report of 6 cases. J Pediatr Surg. 40(12):1948-50, 2005
4- Iwanaka T, Kawashima H, Uchida H: The laparoscopic approach of neuroblastoma. Semin Pediatr Surg. 16(4):259-65, 2007
5- Leclair MD, Sarnacki S, Varlet F, Heloury Y: Minimally-invasive surgery in cancer children. Bull Cancer. 94(12):1087-90, 2007
6- Chan KW, Lee KH, Tam YH, Yeung CK: Minimal invasive surgery in pediatric solid tumors. J Laparoendosc Adv Surg Tech A. 17(6):817-20, 2007

Splenic Trauma: Embolization

The spleen is the most commonly injured visceral organ in blunt abdominal trauma in both adults and children. Most children with splenic trauma are managed conservatively and the need for surgical intervention is very rarely utilized. When the need for surgery arises splenic perservation is tried by all means. For such purpose various suture techniques, biomaterials and resorbable protheses are utilized. Splenic artery embolization (SAE) is another useful technique in the management of blunt splenic injury. SAE is performed when patients has the following CT angiographic criteria: (1) extravasation of contrast material extending beyond or within the splenic parenchyma, (2) arterial disruption or major arteriovenous fistula, or both. Major complications occurs in 25% of the SAE-treated patients and included total splenic infarction, splenic atrophy, and postprocedure bleeding. Minor complications are more common and included fever, pleural effusion, and partial splenic infarction. Proximal splenic artery embolization in children may be a safe therapeutic alternative to either conservative or surgical management in spontaneous splenic rupture and even after delayed rupture. Preservation of splenic tissue with a reduced risk of repeated hemorrhage can be obtained with proximal splenic artery embolization. Splenic salvage rate goes beyond the 90% in reported series. 

1- Uroz Tristan J, Poenaru D, Martinez Lagares F, Leclerc S, Sanchis Solera L: Selective splenic artery embolization or use of polyglycolic acid mesh in children with severe splenic trauma. Eur J Pediatr Surg. 5(5):310-2, 1995
2- Hagiwara A, Yukioka T, Ohta S, Nitatori T, Matsuda H, Shimazaki S: Nonsurgical management of patients with blunt splenic injury: efficacy of transcatheter arterial embolization.AJR Am J Roentgenol. 167(1):159-66, 1996
3- Naess PA, Gaarder C, Dormagen JB: Nonoperative management of pediatric splenic injury with angiographic embolization.J Pediatr Surg. 40(11):e63-4, 2005
4- Wu SC, Chen RJ, Yang AD, Tung CC, Lee KH: Complications associated with embolization in the treatment of blunt splenic injury.World J Surg. 32(3):476-82, 2008
5- Raikhlin A, Baerlocher MO, Asch MR, Myers A: Imaging and transcatheter arterial embolization for traumatic splenic injuries: review of the literature. Can J Surg. 51(6):464-72, 2008
6- Maurer SV, Denys A, Lutz N: Successful embolization of a delayed splenic rupture following trauma in a child. J Pediatr Surg. 44(6):E1-4, 2009

Artificial Anal Sphincter

Fecal incontinence is a devastating social and psychological problem in children and adults. Several methods to manage this condition includes biofeedback, bowel mechanical cleansing training, dynamic graciloplasty, permanent stoma, sacral neuromodulation and implantation of an artificial anal sphincter (AAS). The success rate of AAS is approximately 75%. The artificial anal  sphincter restores continence to solid stools in almost all severely incontinent patients, two-thirds of whom achieve practically normal continence. Infection has been the most serious complication, but a number of technical complications (cuff broken, rectal erosion, difficulty in evacuating) related to the device have also occurred and required revisional procedures in up to 60% of the patients. The late complications (infection and skin erosion) are the main cause of device explantation. Although morbidity and the need for revisional surgery are high, after artificial sphincter implantation anal incontinence and quality of life improve significantly. Recently, a novel artificial anal sphincter system with sensor feedback based on transcutaneous energy transmission was developed.

1- Christiansen J: The artificial anal sphincter. Can J Gastroenterol. 14 Suppl D:152D-154D, 2000
2- Lehur PA, Zerbib F, Neunlist M, Glemain P, Bruley des Varannes S: Comparison of quality of life and anorectal function after artificial sphincter implantation. Dis Colon Rectum. 45(4):508-13, 2002
3- Wong WD, Congliosi SM, Spencer MP, Corman ML, Tan P, Opelka FG, Burnstein M, Nogueras JJ, Bailey HR, Devesa JM, Fry RD, Cagir B, Birnbaum E, Fleshman JW, Lawrence MA, Buie WD, Heine J, Edelstein PS, Gregorcyk S, Lehur PA, Michot F, Phang PT, Schoetz DJ, Potenti F, Tsai JY: The safety and efficacy of the artificial bowel sphincter for fecal incontinence: results from a multicenter cohort study. Dis Colon Rectum. 45(9):1139-53, 2002
4- O'Brien PE, Dixon JB, Skinner S, Laurie C, Khera A, Fonda D: A prospective, randomized, controlled clinical trial of placement of the artificial bowel sphincter (Acticon Neosphincter) for the control of fecal incontinence. Dis Colon Rectum. 47(11):1852-60, 2004
5- Tan EK, Vaizey C, Cornish J, Darzi A, Tekkis PP: Surgical strategies for faecal incontinence--a decision analysis between dynamic  graciloplasty, artificial bowel sphincter and end stoma. Colorectal Dis. 10(6):577-86, 2008.
6- CarmonaSafioleas M, Andromanakos N, Lygidakis N: Anorectal incontinence: therapeutic strategy of a complex surgical problem. Hepatogastroenterology. 55(85):1320-6, 2008
7- Zan P, Yan G, Liu H, Luo N, Zhao Y: Adaptive transcutaneous power delivery for an artificial anal sphincter system. J Med Eng Technol. 33(2):136-41, 2009

PSU Volume 33 No 03 SEPTEMBER 2009

Waardenburg Syndrome

Waardenburg syndrome (WS) is an autosomical recessive or dominant trait condition derived from the neural crests and includes sensorineural hearing loss (bilateral or unilateral), and pigmentation disorder of the hair, eyes and skin (iris heterochromia, white forelock, lateral displacement of inner canthi of the eyes). Four types of the syndrome have been described. Though very rare, WS can be associated with Hircshsprung's disease (Shaw-Waardenburg syndrome). Type IV WS is a term used to denote intestinal aganglionosis associated with the Waardenburg complex, consisting of hypopigmented spots on the skin, heterochromia irides, depigmented ocular fundus, telecanthus, and sensory deafness. The aganglionosis associated with WS is usually total colonic with extensive involvement of ileus. Clinically these affected babies present with intestinal obstruction findings such as bilious vomiting, abdominal distension, and inability to feed orally from the first few days of life. Mutations in the endothelin-3 endothelin B receptor, and SOX10 genes have been identified as causative genes of WS. The length of the aganglionic segment decides the prognosis of these children. The longer the affected bowel the worse the prognosis even in the face of restorative surgery (long bowel myectomy-myotomy).

1-Tomiyama H, Shimotake T, Ono S, Kimura O, Tokiwa K, Iwai N: Relationship between the type of RET/GDNF/NTN or SOX10 gene mutations and long-term results after surgery for total colonic aganglionosis with small bowel  involvement. J Pediatr Surg. 36(11):1685-8, 2001
2- Gnananayagam EJ, Solomon R, Chandran A, Anbarasi S, Sen S, Moses PD:Long segment Hirschsprung's disease in the Waardenburg-Shah syndrome.Pediatr Surg Int. 2003 Aug;19(6):501-3, 2003
3- Moore SW: The contribution of associated congenital anomalies in understanding Hirschsprung's disease. Pediatr Surg Int. 22(4):305-15, 2006
4- Jan IA, Stroedter L, Haq AU, Din ZU: Association of Shah-Waardenburgh syndrome: a review of 6 cases. J Pediatr Surg. 43(4):744-7, 2008
5- Espinosa R, Alonso Calderon JL: Neural crest disorders and Hirschsprung's disease. Cir Pediatr. 22(1):25-8, 2009
5- Karaca I, Turk E, Ortac R, Kandirici A: Waardenburg syndrome with extended aganglionosis: report of 3 new cases. J Pediatr Surg. 44(6):E9-13, 2009

Bishop-Koop Anastomosis

The survival of babies born with cystic fibrosis and meconium ileus was improved significantly with the development of intestinal surgical procedures in the second part of the past century. Such is the case of the temporary Bishop-Koop anastomosis (BKA), where the proximally dilated bowel is anastomosed end to side to the distal bowel which in turns is taken out as a stoma. Succus entericus can then pass from the proximal bowel to the distal bowel all the way down to the anus or come out through the stoma if there is a distal functional obstruction. Beside meconium ileus, this anastomosis has been used in children suffering from various other intestinal anomalies such as jejuno-ileal atresia, necrotizing enterocolitis, volvulus, gastroschisis associated with bowel atresia, and apple-peel syndrome. In such situations  the Bishop-Koop anastomosis is a safer procedure than primary end-to-end or end-to-side anastomosis, especially for the management of greatly different intestinal diameters. This occurs in cases of slowly distensible microcolons associated with the above conditions where the BKA is a temporary procedure suitable for the colon to grow properly.

1- Kootstra G, Kamann HL, Okken A, Vander Vliet JA, Zwierstra RP, Slooff MJ, Krom RA, Kuijjer PJ: The Bishop-Koop anastomosis-a find in pediatric surgery. Neth J Surg. 32(3):92-6, 1980
2- Murshed R, Spitz L, Kiely E, Drake D: Meconium ileus: a ten-year review of thirty-six patients. Eur J Pediatr Surg. 7(5):275-7, 1997
3- Wit J, Sellin S, Degenhardt P, Scholz M, Mau H: Is the Bishop-Koop anastomosis in treatment of neonatal ileus still current?. Chirurg. 71(3):307-10, 2000
4- Fleet MS, de la Hunt MN: Intestinal atresia with gastroschisis: a selective approach to management. J Pediatr Surg. 35(9):1323-5, 2000
5- Kumaran N, Shankar KR, Lloyd DA, Losty PD: Trends in the management and outcome of jejuno-ileal atresia. Eur J Pediatr Surg. 12(3):163-7, 2002
6- Siman J, Trnka J: Stoma procedures in the congenital digestive tract malformations. Rozhl Chir. 86(7):347-52, 2007

Fetal Rectal Perforation

Perforation of the extraperitoneal rectal wall in newborns is very rare. Most described cases are term babies. Fetal distress is not noted at the time of delivery. All affected babies have a recognizable abnormality at birth mostly seen during the first five days of birth. The abnormality can be a perineal or buttock lesion, varying from a minor skin blemish, fistula, swelling to a rapidly enlarging aerocele with meconium staining of perineal tissue with subsequent skin rupture or isolated ascites. Diagnosis is suggested with lateral simple films. Meconium is expressed under pressure by fetal peristalsis through a defect in the lower rectal wall into normal tissue planes within the infralevator space to reach a subcutaneous level and up toward the abdominal cavity. No other congenital associated anomaly has been described. The position of the defect at the level of the pelvic floor is consistent with the watershed between the inferior and middle rectal arteries and might suggest a local ischemic insult, though the cause remains unknown. After resuscitation and antibiotherapy, surgery is mandatory. The procedure consists of fecal diversion achieved by sigmoid colostomy; in addition the perineal tissues are debrided and wide drainage is achieved. Prognosis is usually good with normal continence obtained later in most affected cases. Some cases has developed a rectal stenosis amenable to dilatation.

1- Mitsudo SM, Boley SJ, Rosenzweig MJ, Campbell DE: Extraperitoneal pelvic meconium extravasation in a newborn infant. J Pediatr. 103(4):598-600, 1983
2- Davies MR, Cywes S, Rode H: Prenatal perforation of the extraperitoneal part of the rectum, associated with a developmental defect of the pelvic floor. Z Kinderchir. 39(4):271-3, 1984
3- Casaccia G, Giorlandino C, Catalano OA, Bagolan P: Prenatal rectal perforation: an unsuspected cause of isolated ascites. J Perinatol. 26(11):717-9, 2006
4- Sundararajan L, Patel D, Jawaheer G: Antenatal rectal perforation presenting in the neonate. Pediatr Surg Int. 24(5):601-3, 2008
5- Pitcher GJ, Davies MR, Bowley DM, Numanoglu A, Rode H: Fetal extraperitoneal rectal perforation: a rare neonatal emergency. J Pediatr Surg. 2009 Jul;44(7):1405-9

PSU Volume 33 No 04 OCTOBER 2009

US-guided CVC placement

Central venous catheter (CVC) placement is an integral part in management of many medical and surgical conditions in children. Percutaneous placement of CVC in the subclavian or internal jugular vein is a blind procedure using external anatomical landmarks technique. As such, they carry an inherent risk of puncturing the artery or lung parenchyma with its attendant complications associated with variation in venous anatomy and depth of cannulating needle. Doppler ultrasound (US) guided CVC placement permits direct visualization and cannulation of the central veins in the neck (internal jugular vein), specially when placing CVC in very small babies. US-guided CVC placement has also a reduced rate of complications and the rate of needle punctures is also reduced. Reducing the rate of needle punctures reduce the rate of venous thrombosis. The right internal jugular vein is preferred with this technique in most patients. Specific training in interventional radiology is not essential to perform this technique safely and with a low complication rate, but a learning curve is associated with dominating this technique. US-guided CVC placement can be done safely in children of all ages.    

1- Verghese ST, McGill WA, Patel RI, Sell JE, Midgley FM, Ruttimann UE: Ultrasound-guided internal jugular venous cannulation in infants: a prospective comparison with the traditional palpation method. Anesthesiology. 91(1):71-7, 1999
2- MacIntyre PA, Samra G, Hatch DJ: Preliminary experience with the Doppler ultrasound guided vascular access needle in paediatric patients. Paediatr Anaesth. 10(4):361-5, 2000
3- Asheim P, Mostad U, Aadahl P: Ultrasound-guided central venous cannulation in infants and children. Acta Anaesthesiol Scand. 46(4):390-2, 2002
4- Machotta A, Kerner S, Höhne C, Kerner T: Ultrasound-guided central venous cannulation in a very small preterm neonate. Paediatr Anaesth. 15(4):325-7, 2005
5- Chuan WX, Wei W, Yu L: A randomized-controlled study of ultrasound prelocation vs anatomical landmark-guided cannulation of the internal jugular vein in infants and children. Paediatr Anaesth. 15(9):733-8, 2005
6- Arul GS, Lewis N, Bromley P, Bennett J: Ultrasound-guided percutaneous insertion of Hickman lines in children. Prospective study of 500 consecutive procedures. J Pediatr Surg. 44(7):1371-6, 2009


Melanoma is very rare in children with approximately 400 new cases diagnosed yearly in the United States and 2% of all melanoma cases. The incidence of melanoma is increasing worldwide. With accurate diagnosis the outcome for pediatric melanoma is good. Factors associated with an increased risk of melanoma includes white race, female sex, fair complexion, red or blonde hair, light eye color, tendency to burn with ultraviolet light, dysplastic nevi, congenital nevi, increase number of benign nevi, family history and immunosuppression. Early in infancy melanoma arises from transplacental metastasis, congenital or large nevi. Clinical signs suspicious of melanoma include increase or change in lesion size or color, bleeding, irregular border or pigmentation distribution, pruritus or enlarged regional lymph nodes. Trunk and extremity are the most common location. Superficial spreading melanoma is the most common histologic variant. Recurrence is more common in black children and misdiagnosed cases. Increase awareness with biopsy of suspicious lesions confirms the diagnosis while establishing depth of tumor. Excision with sentinel lymph node (SL N) biopsy or complete lymph node dissection is curative and improves stage-specific survival in pediatric melanoma. The sentinel node status correlates with primary tumor depth. Pediatric patients have a higher incidence of SLN metastases than adults yet have a lower incidence of recurrence.

1- Roaten JB, Partrick DA, Pearlman N, Gonzalez RJ, Gonzalez R, McCarter MD: Sentinel lymph node biopsy for melanoma and other melanocytic tumors in adolescents. J Pediatr Surg. 40(1):232-5, 2005
2- Roaten JB, Partrick DA, Bensard D, Pearlman N, Gonzalez R, Fitzpatrick J, McCarter MD: Survival in sentinel lymph node-positive pediatric melanoma. J Pediatr Surg. 40(6):988-92, 2005
3- Strouse JJ, Fears TR, Tucker MA, Wayne AS: Pediatric melanoma: risk factor and survival analysis of the surveillance, epidemiology and end results database. J Clin Oncol. 23(21):4735-41, 2005
4- Livestro DP, Kaine EM, Michaelson JS, Mihm MC, Haluska FG, Muzikansky A, Sober AJ, Tanabe KK: Melanoma in the young: differences and similarities with adult melanoma: a case-matched controlled analysis. Cancer. 110(3):614-24, 2007
5- Lewis KG: Trends in pediatric melanoma mortality in the United States, 1968 through 2004. Dermatol Surg. 34(2):152-9, 2008
6- Gow KW, Rapkin LB, Olson TA, Durham MM, Wyly B, Shehata BM: Sentinel lymph node biopsy in the pediatric population. J Pediatr Surg. 43(12):2193-8, 2008
7- Aldrink JH, Selim MA, Diesen DL, Johnson J, Pruitt SK, Tyler DS, Seigler HF: Pediatric melanoma: a single-institution experience of 150 patients. J Pediatr Surg. 44(8):1514-21, 2009

Rectal Strictures

Rectal strictures in children can occur after inflammatory bowel disease, trauma, or most commonly postoperative following a coloanal anastomosis. Factors that contribute to the formation of a postoperative rectal stricture include ischemia, leakage and infection, inflammatory response to anastomotic material, circular stapler size, and fecal contact with the anastomosis. Stapling devices are associated with a higher rate of postoperative strictures than handsewn anastomosis. Clinically, the patient with a rectal stricture after surgery can develop partial or complete bowel obstruction, frequent bowel movements, lower abdominal fullness, sense of residual stools followed by anal pain, or the stricture is diagnosed after preoperative imaging prior to closing a diverting stoma. Anastomotic colorectal strictures are usually defined as being less than 10 to 12 mm in diameter and are usually short (< 1 cm in length). A radiographic assessment of the stricture length must be done. Depending on the diameter and length of the stricture management might consist of transanal dilatations (manual or by bougie), hydrostatic balloon dilatations, microwave coagulation therapy, transanal incision, excision, or reanastomosis by means of a circular stapler. Use of steroid injection (Kenalog) has also been utilized. Postoperative strictures usually respond well to direct dilatations.   

1- Shimada S, Matsuda M, Uno K, Matsuzaki H, Murakami S, Ogawa M: A New Device for the Treatment of Coloproctostomic Stricture After Double Stapling Anastomoses. Ann Surg 224:603-608, 1996
2- Suchan KL, Muldner A, Manegold BC: Endoscopic treatment of postoperative colorectal anastomotic strictures. Surg Endosc 17: 1110-1113, 2003
3- Garcea G, Sutton CD, Lloyd TD, Jameson J, Scott A, Kelly MJ: Management of benign rectal strictures: a review of present therapeutic procedures.  Dis Colon Rectum. 46(11):1451-60, 2003
4- Pabst M, Giger U, Senn M, Gauer JM, Boldog B, Scweizer W: Transanal treatment of strictured rectal anastomosis with circular stapler device: Simple and safe. Dig Surg 24: 12-14, 2007
5- McKee R, Pricolo V: Stapled revision of complete colorectal anastomotic obstruction. Am Journal Surg 195: 526-527, 2008 
6- Lillehei CW, Leichtner A, Bousvaros A, Shamberger RC: Restorative proctocolectomy and ileal pouch-anal anastomosis in children. Dis Colon Rectum. 52(9):1645-9, 2009

PSU Volume 33 No 05 NOVEMBER 2009

Esophageal Leiomyoma

Leiomyoma is considered the most common benign tumor of the esophagus. More common in adults than children. The mean age of involvement in pediatric patients is 14 years old with more girls affected than boys. Contrary to the predominant solitary and localized nodules found in adults, more than 90% of affected children harbor diffuse lesions. Associated anomalies include hiatal hernia, Alport's syndrome (nephropathy with hematuria, deafness and cataract), genital anomalies, esophageal peptic ulcer, cholelithiasis and rectal prolapse. Children demonstrate symptoms of slowly progressive dysphagia, weight loss, hematemesis, food impact, hiccups and choking. Rarely the tumor might affect the tracheobronchial tree. Diagnostic tests include esophagogram, endoscopy and CT-Scan. Barium swallow reveals a long-segment stricture and CT-Scan demonstrates a circumferential mass lesion in the lower esophagus. Endoscopic ultrasonography has also been used to diagnose the presence of a subepithelial tumor causing extrinsic compressions of the oesophagus. Biopsy through the endoscope is hazardous and is not recommended. Esophageal leiomyoma must be considered in the differential diagnosis of a mediastinal mass or esophageal stricture. Management consists of resection with primary or substitute esophageal reconstruction. Localized lesions may be amenable to enucleation. Diffuse lesions might need esophageal replacement.

1- Bourque MD, Spigland N, Bensoussan AL, Collin PP, Saguem MH, Brochu P, Blanchard H, Reinberg O: Esophageal leiomyoma in children: two case reports and review of the literature. J Pediatr Surg. 24(10):1103-7, 1989
2- Tannuri U, Feferbaun R, Costa RB: Leiomyoma of the esophagus in children - case report and review of the literature. J Pediatr (Rio J) 70(6):365-70, 1994
3-  Levine MS, Buck JL, Pantongrag-Brown L, Buetow PC, Lowry MA, Sobin LH: Esophageal leiomyomatosis. Radiology. 199(2):533-6, 1996
4- Massicot R, Aubert D, Mboyo A, Destuynder O, Queneau PE: Localized esophageal leiomyoma and hypertrophic osteoarthropathy. J Pediatr Surg. 32(4):646-7, 1997
5- Lee H, Morgan K, Abramowsky C, Ricketts RR: Leiomyoma at the site of esophageal atresia repair. J Pediatr Surg. 36(12):1832-3, 2001
6- Gupta V, Lal A, Sinha SK, Nada R, Gupta NM: Leiomyomatosis of the esophagus: experience over a decade. J Gastrointest Surg. 13(2):206-11, 2009

Meningococcal Sepsis

Meningococcal sepsis is a devastating condition caused by the bacteria Neisseria Meningitidis. Meningococcal septicemia can lead to purpura fulminans with subsequent full thickness skin loss and deep muscle damage causing mutilating amputations of hands, digits, lower limbs and toes. Children with this condition are managed with aggressive intravenous fluids, vasoactive drugs, antibiotics, respiratory support and immunoglobulin therapy. The basic process in the pathophysiology of this condition that affects the microvasculature is increased vascular permeability, pathologic vasoconstriction and vasodilatation, loss of thromboresistance, intravascular coagulation and myocardial dysfunction. The lower limbs are predominantly affected. Surgical therapy is typically delayed in these children due to cardiovascular instability. Tissue loss can be extensive and difficult to determine at the outset. Surgical procedures consist of debridement, amputation, skin-grafting and soft-tissue releasing incisions. Recently, early microsurgical arteriolysis, freeing affected blood vessels, has proved a reliable method to decrease the level and reduce the amputation rate observed in these cases. Children requiring surgery for purpura fulminans achieve age-appropriate milestones and are primarily limited by their physical disability related to amputations, scarring and abnormal bone growth.

1- Huang S, Clarke JA: Severe skin loss after meningococcal septicaemia: complications in treatment. Acta Paediatr. 86(11):1263-6, 1997
2- Leclerc F, Leteurtre S, Cremer R, Fourier C, Sadik A: Do new strategies in meningococcemia produce better outcomes? Crit Care Med. 28(9 Suppl):S60-3, 2000
3- Wheeler JS, Anderson BJ, De Chalain TM: Surgical interventions in children with meningococcal purpura fulminans--a review of 117 procedures in 21 children. J Pediatr Surg. 38(4):597-603, 2003
4- Dinh TA, Friedman J, Higuera S: Plastic surgery management in pediatric meningococcal-induced purpura fulminans. Clin Plast Surg. 32(1):117-21, 2005
5- Numanoglu A, Bickler SW, Rode H, Bosenberg AT: Meningococcal septicaemia complications involving skin and underlying deeper tissues--management considerations and outcome. S Afr J Surg. 45(4):142-6, 2007
6- Boeckx WD, Nanhekhan L, Vos GD, Leroy P, Van den Kerckhove E: Minimizing limb amputations in meningococcal sepsis by early microsurgical arteriolysis. J Pediatr Surg. 44(8):1625-30, 2009

All-Terrain Vehicles

All-terrain vehicles (ATV) injuries continue to produce morbidity and mortality in our kids. ATV include, four wheels vehicles, minibikes, golf carts, and go-karts. Children lack the physical strength, cognitive abilities, and fine motor skills to operate ATVs properly. Most cases of injury occur in males in the ages of 11 to 15 years. Children aged 0 to 5 years are more likely than older children to have facial injuries, whereas older children are more likely to sustain lower trunk and leg or foot injuries. Some of the recommendations that several medical organizations have proposed to reduce the injury rate include that laws should prohibit the use of ATV by children younger than 16 years of age, recreational use of ATV should be limited to people who have license to operate other motor vehicles, children between ages 16 and 18 should be supervised by a legal guardian, drivers should complete an approved training course, and operators should always wear a government-approved helmet, eye protection and protective clothing. 

1- Shults RA, Wiles SD, Vajani M, Helmkamp JC: All-terrain vehicle-related nonfatal injuries among young riders: United States, 2001-2003. Pediatrics. 116(5):e608-12, 2005
2- Fonseca AH, Ochsner MG, Bromberg WJ, Gantt D: All-terrain vehicle injuries: are they dangerous? A 6-year experience at a level I trauma center after legislative regulations expired. Am Surg. 71(11):937-40, 2005
3- Alawi K, Lynch T, Lim R: All-terrain vehicle major injury patterns in children: a five-year review in Southwestern Ontario. CJEM. 8(4):277-80, 2006
4- Trauma Committee of the Canadian Association of Pediatric Surgeons: Canadian Association of Pediatric Surgeons' position statement on the use of all-terrain vehicles by children and youth. J Pediatr Surg. 43(5):938-9, 2008
5- Kellum E, Creek A, Dawkins R, Bernard M, Sawyer JR: Age-related patterns of injury in children involved in all-terrain vehicle accidents. J Pediatr Orthop. 28(8):854-8, 2008
6- Burd R: American Pediatric Surgical Association Trauma Committee position statement on the use of all-terrain vehicles by children and youth. J Pediatr Surg. 44(8):1638-9, 2009

PSU Volume 33 No 06 DECEMBER 2009

Subdural Hematoma

Subdural hematoma (SDH) is a type of traumatic brain injury where blood collect within the dura layer of the brain. Subdural bleeding usually results from tears in veins that cross the subdural space. Subdural hematoma requiring surgery in children is unusual. The incidence of SDH in infants is between 12-25 cases per 100,000 children and most detected SDH are due to physical abuse. Radiographically, these are easily distinguishable from a "lens shaped" epidural hematoma. Epidural hematoma is more likely to be accidental. The small unilateral SDH seen with diffuse head injury can be observed. Isolated SDH associated with neurologic deficit or progressive loss of consciousness will need surgical evacuation. Almost 60% of these are associated with a skull fracture. Chronic SDH are encountered in children that have been abused. SDH in the infant has a different pattern from that seen in the older child and adult. It is usually a widespread, bilateral, thin film, unlike the thick, space-occupying and often unilateral clot seen in older children and adults after trauma. Healing of SDH is by formation of a granulating membrane which may confer vulnerability to rebleeding, either spontaneously or after an otherwise innocuous event. SDH has a particular significance as one of the features of the triad (together with retinal hemorrhage and encephalopathy) associated with non-accidental injury (physical child abuse).

1- Swift DM, McBride L: Chronic subdural hematoma in children. Neurosurg Clin N Am. 11(3):439-46, 2000
2- Jayawant S, Parr J: Outcome following subdural haemorrhages in infancy. Arch Dis Child. 92(4):343-7, 2007
3- Case ME: Inflicted traumatic brain injury in infants and young children. Brain Pathol. 18(4):571-82, 2008
4- Case ME: Accidental traumatic head injury in infants and young children. Brain Pathol. 18(4):583-9, 2008
5- Squier W, Mack J: The neuropathology of infant subdural haemorrhage. Forensic Sci Int. 187(1-3):6-13, 2009

Subglottic Stenosis

Subglottic stenosis refers to stenosis of the airway inside the cricoid ring. Subglottic stenosis (SGS) can be congenital or acquired. In either case the child will develop an inspiratory and expiratory (biphasic) stridor. Subglottic stenosis will also have a barking cough and can manifest during a viral illness. The congenital variety of subglottic stenosis is very rare and consists of a soft tissue thickening of the subglottic area, occasionally involving the true vocal cords. Minimal laryngeal inflammation precipitates airway obstruction because the cricoid cartilage is nondistensible. With time as laryngeal growth occurs, the congenital stenosis improves needing surgery less common. The acquired variety of SGS in infants is usually the result of prolonged endotracheal intubation during the neonatal period due to hyaline membranes disease or after surgical procedures. More than the duration of intubation is the continuous movement of the tube from inadequate fixation that produces the chronic inflammation needed for the stenosis to develop. With better neonatal airway care the incidence of acquired subglottic stenosis has significantly decreased. Severe airway obstruction will need tracheostomy followed by laryngotracheal reconstruction. Anterior or multiple cricoid splitting with cartilage graft interpositioning is usually performed. The success rates for these procedures have been shown to be approximately 90%.

1- Schroeder JW Jr, Holinger LD: Congenital laryngeal stenosis. Otolaryngol Clin North Am. 41(5):865-75, 2008
2- Walner DL, Loewen MS, Kimura RE: Neonatal subglottic stenosis--incidence and trends. Laryngoscope. 111(1):48-51, 2001
3-  Sichel JY, Dangoor E, Eliashar R, Halperin D: Management of congenital laryngeal malformations. Am J Otolaryngol. 21(1):22-30, 2000
4- Cotton RT: Management of subglottic stenosis. Otolaryngol Clin North Am. 33(1):111-30, 2000
5- Fraga JC, Schopf L, Forte V: Thyroid alar cartilage laryngotracheal reconstruction for severe pediatric subglottic stenosis. J Pediatr Surg. 36(8):1258-61, 2001
6- Hartnick CJ, Hartley BE, Lacy PD, Liu J, Willging JP, Myer CM 3rd, Cotton RT: Surgery for pediatric subglottic stenosis: disease-specific outcomes. Ann Otol Rhinol Laryngol. 110(12):1109-13, 2001

CHARGE Syndrome

CHARGE syndrome is a specific collection of non-randomly occurring congenital anomalies named for its six major clinical features. Patients of CHARGE syndrome may be presented to ophthalmologists due to ocular coloboma. The acronym CHARGE stands for the major features of this syndrome: Coloboma of the eye, Heart defects, Atresia of the choanae, Retarded growth and development, Genital hypoplasia, and Ear anomalies and/or deafness. CHARGE syndrome occurs in an estimated of one in 8,500 live births. Individuals with CHARGE syndrome who demonstrated a less extensive phenotype (less than three major criteria) were more likely to present with minor cardiovascular malformations, including small atrial or ventricular septal defects (VSD) or patent ductus arteriosus (PDA). Mutations in the CHD7 gene (member of the chromodomain helicase DNA protein family) are detected in more than 75% of patients with CHARGE syndrome. A significant cause of morbidity is severe feeding difficulty, including problems with chewing, swallowing, and gastroesophageal reflux, which are prevalent throughout childhood. Evaluation of associated defects and proper referral for timely management may be critical for patients with CHARGE syndrome.

1- Hsueh KF, Yang CS, Lu JH, Hsu WM: Clinical characteristics of CHARGE syndrome. J Chin Med Assoc. 67(10):542-6, 2004
2- Issekutz KA, Graham JM Jr, Prasad C, Smith IM, Blake KD: An epidemiological analysis of CHARGE syndrome: preliminary results from a Canadian study.  Am J Med Genet A. 133A(3):309-17, 2005
3- Blake KD, Prasad C: CHARGE syndrome.  Orphanet J Rare Dis. 7;1:34, 2006
4- Pagon RA, Graham JM Jr, Zonana J, Yong SL: Coloboma, congenital heart disease, and choanal atresia with multiple anomalies: CHARGE association. J Pediatr. 99(2):223-7, 1981
5- Davenport SL, Hefner MA, Mitchell JA: The spectrum of clinical features in CHARGE syndrome.  Clin Genet. 29(4):298-310, 1986

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