VOLUME 30, 2008

PSU Volume 30 No 01 JANUARY 2008

Esophageal Atresia with Proximal TEF

Esophageal atresia with or without a tracheoesophageal fistula (TEF)  is considered the most common congenital anomaly of the esophagus. Most cases are either esophageal atresia with distal TEF or pure esophageal atresia without a fistula. A gasless abdomen with a coiled nasogastric tube is sufficient evidence to diagnose pure esophageal atresia. Less than 1% of all cases of esophageal atresia have a concomitant proximal TEF. The presence of such anomaly can be suspected with the proximal esophageal stump is filled with air, or if a contrast proximal esophagogram is ordered and the fistulous tract identified. Intraoperatively a proximal TEF can be identified during bronchoscopy or more commonly while dissecting the proximal esophageal stump to obtain length for the anastomosis. Esophageal atresia is initially managed with a feeding gastrostomy to start gastric feeding and obtain a study to determine the gap that exists between the proximal and distal esophageal stumps. In the presence of a proximal TEF causing chronic aspiration of saliva the need for early esophageal continuity arises. The strategy can consist of ligating the TEF and doing an anastomosis under tension, bringing the proximal esophageal stump through an extrathoracic lengthening procedure or utilizing Foker technique of continuous proximal and distal lengthening with later anastomosis. In either cases the rate of ischemia, leak and stricture is high.

1- Yun KL, Hartman GE, Shochat SJ: Esophageal atresia with triple congenital tracheoesophageal fistulae. J Pediatr Surg. 27(12):1527-8, 1992
2- Lessin MS, Wesselhoeft CW, Luks FI, DeLuca FG: Primary repair of long-gap esophageal atresia by mobilization of the distal esophagus. Eur J Pediatr Surg. 9(6):369-72, 1999
3- Kimura K, Nishijima E, Tsugawa C, Collins DL, Lazar EL, Stylianos S, Sandler A, Soper RT: Multistaged extrathoracic esophageal elongation procedure for long gap esophageal atresia: Experience with 12 patients. J Pediatr Surg. 36(11):1725-7, 2001
4- Katsura S, Shono T, Yamanouchi T, Taguchi T, Suita S: Esophageal atresia with double tracheoesophageal fistula--a case report and review of the literature. Eur J Pediatr Surg. 15(5):354-7, 2005
5- Kane TD, Atri P, Potoka DA: Triple fistula: management of a double tracheoesophageal fistula with a third H-type proximal fistula. J Pediatr Surg. 42(6):E1-3, 2007

Dermal Sinus Tract

Dermal sinus tracts (DST) represent incomplete disjunction of the neural tube from cutaneous ectoderm during neurulation leaving a sinus tract of cutaneous tissue attached to the nervous system. A dermal sinus tract can occur at the lumbosacral area (most common location) or less likely at the cranial end of the neural tube, frontally (frontonasal sinus tracts) or in the occiput (occipital sinus tracts). The child presents with a skin dimple, a small dermal mass, a cutaneous hemangioma, skin tag or tuft of hair in these areas. The lumbosacral sinus tract extends through or between the laminas, penetrates the dura and ascend within the thecal sac to end on the dorsal aspect of the spinal cord at the second sacral cord level. A lumbosacral sinus tract can tethered the cord, serve as portal of entry for bacteria leading to recurrent infections (subdural abscess), result in an aseptic meningitis or a dermoid tumor can develop from the tract and compress the spinal cord. Lumbosacral DST can be associated with focal neurological deficit, neurogenic bladder or orthopedics deformities. The diagnosis can be established with MRI in most cases. Management is surgical excision. Simply removing the cutaneous component without addressing the spinal cord malformation is not sufficient therapy. Poor awareness leads to delayed management.

1- Matson DD, Jerva MJ: Recurrent meningitis associated with congenital lumbo-sacral dermal sinus tract. J Neurosurg. 25(3):288-97, 1966
2- Chen CY, Lin KL, Wang HS, Lui TN: Dermoid cyst with dermal sinus tract complicated with spinal subdural abscess. Pediatr Neurol. 20(2):157-60, 1999
3- Gupta DK, Shastank RR, Mahapatra AK: An unusual presentation of lumbosacral dermal sinus with CSF leak and meningitis. A case report and review of the literature. Pediatr Neurosurg. 41(2):98-101, 2005
4- Ramnarayan R, Dominic A, Alapatt J, Buxton N: Congenital spinal dermal sinuses: poor awareness leads to delayed treatment. Childs Nerv Syst. 22(10):1220-4. Epub 2006 Mar 23, 2006
5- Holzmann D, Huisman TA, Holzmann P, Stoeckli SJ: Surgical approaches for nasal dermal sinus cysts. Rhinology. 45(1):31-5, 2007

Molluscum Contagiosum

Molluscum contagiosum is a pox viral infection affecting primarily the skin of infants, children and adults. It causes firm discrete pearly papules that measure between one and four millimeters in diameter. The papules have a characteristic central umbilication with a caseous type of material containing virus-laden cells. One-third of children have symptoms from, or secondary reactions to the infection, including pruritus, erythema and, occasionally, inflammation and pain. Molluscum contagiosum can occur singly or in clusters anywhere on the body, though the trunk is more commonly affected. Spread is usual by direct contact, with genital involvement suggesting the possibility of sexual abuse in the young child. The virus produces a number of substances that block immune response formation in the infected host. Molluscum contagiosum is a benign and self limited disease with most cases resolving within six months to one year irrespective of therapy, though patients with weakened immune systems have increased difficulty in clearing lesions. A single, most effective treatment for either infection has not been defined. Conventional methods attempt to nonspecifically destroy infected tissue. Immunocompetent children can be managed with imiquimod, retinoids, and alpha-hydroxy acids. Surgical management, if undertaken, includes curettage of the central plug, cryosurgery and/or electrodesiccation.

1- Allen AL, Siegfried EC: Management of warts and molluscum in adolescents. Adolesc Med. 12(2):vi, 229-42, 2001
2- Silverberg N: Pediatric molluscum contagiosum: optimal treatment strategies. Paediatr Drugs. 5(8):505-12, 2003
3- Tyring SK: Molluscum contagiosum: the importance of early diagnosis and treatment. Am J Obstet Gynecol. 189(3 Suppl):S12-6, 2003
4- Laxmisha C, Thappa DM, Jaisankar TJ: Clinical profile of molluscum contagiosum in children versus adults. Dermatol Online J. 9(5):1, 2003
5- Dohil MA, Lin P, Lee J, Lucky AW, Paller AS, Eichenfield LF: The epidemiology of molluscum contagiosum in children. J Am Acad Dermatol. 54(1):47-54, 2006
6- Hanna D, Hatami A, Powell J, Marcoux D, Maari C, Savard P, Thibeault H, McCuaig C: A prospective randomized trial comparing the efficacy and adverse effects of four recognized treatments of molluscum contagiosum in children. Pediatr Dermatol. 23(6):574-9, 2006
7- Martín-García RF, García ME, Rosado A: Modified curettage technique for molluscum contagiosum. Pediatr Dermatol. 24(2):192-4, 2007

PSU Volume 30 No 02 FEBRUARY 2008

Mesh Repair Congenital Diaphragmatic Hernia

Congenital diaphragmatic hernia continues to carry a high mortality associated with the presence of pulmonary hypoplasia. Repair of the diaphragmatic defect is usually carried on when the child obtains sufficient hemodynamic stability to tolerate a major surgical procedure. Surgical repair or closure of the defect is either carried out primarily or in case that most of the hemidiaphragm is lacking, using a piece of prosthetic mesh. Primary repair is performed when there is sufficient diaphragm to approximate with low tension, carries a low rate of recurrence and avoids the mechanical and infectious complications associated with implanted prostheses. When the size of the defect can be known preoperatively a split abdominal wall muscle flap through a low abdominal incision can be planned. Mesh repair utilizes several prosthetic materials such as polytetrafluoroethylene (Gore-Tex), polypropylene (Marlex), lyophilized dura, Surgisis or even small intestinal submucosa. Composite patch repair using Gore-Tex/Marlex has also been reported. Overall, recurrence rates after mesh repair is significant greater (almost 50%) than after primary repair. Polypropylene mesh in contact with small bowel carries a high risk of fistula formation reason why Gore-Tex is preferred. Vycryl mesh is not a suitable material for repairing these defects.

1- Saltzman DA, Ennis JS, Mehall JR, Jackson RJ, Smith SD, Wagner CW: Recurrent congenital diaphragmatic hernia: A novel repair. J Pediatr Surg. 36(12):1768-9, 2001
2- Sydorak RM, Hoffman W, Lee H, Yingling CD, Longaker M, Chang J, Smith B, Harrison MR, Albanese CT: Reversed latissimus dorsi muscle flap for repair of recurrent congenital diaphragmatic hernia. J Pediatr Surg. 38(3):296-300, 2003
3- Scaife ER, Johnson DG, Meyers RL, Johnson SM, Matlak ME: The split abdominal wall muscle flap--a simple, mesh-free approach to repair large diaphragmatic hernia. J Pediatr Surg. 38(12):1748-51, 2003
4- Smith MJ, Paran TS, Quinn F, Corbally MT: The SIS extracellular matrix scaffold-preliminary results of use in congenital diaphragmatic hernia (CDH) repair. Pediatr Surg Int. 20(11-12):859-62, 2004
5- Grethel EJ, Cortes RA, Wagner AJ, Clifton MS, Lee H, Farmer DL, Harrison MR, Keller RL, Nobuhara KK: Prosthetic patches for congenital diaphragmatic hernia repair: Surgisis vs Gore-Tex. J Pediatr Surg. 41(1):29-33, 2006
6- Riehle KJ, Magnuson DK, Waldhausen JHT: Low recurrence rate after Gore-Tex/Marlex composite patch repair for posterolateral congenital diaphragmatic hernia. J Pediatr Surg 42(11): 1841-1844, 2007

Solitary Intestinal Fibromatosis

Congenital solitary intestinal fibromatosis (SIF) is a very rare tumor that occurs in the newborn and infant period. It belongs to the group of pediatric fibromatosis. Solitary Intestinal fibromatosis involves the intestinal wall, can produce bowel obstruction or perforation and usually involves most frequently the jejunum and ileum. SIF presents most commonly as a solitary or less commonly  as multiple lesions usually confined to soft tissue and bone. Lesions in the duodenum can cause gastric outlet obstruction indistinguishable from pyloric stenosis. Most cases consist of solitary tumors affecting the small or large bowel. Histologic examination in each case shows a transmural infiltrative spindle cell lesion having the morphologic features of fibromatosis. The spindle cells have no atypia, stain positively for Vimentin and CD34 while negative for muscle cell markers.  Ultrastructural studies reveals the tumor to be composed of myofibroblasts. The tumor most often presents as a polypoid mass protruding into the intestinal lumen causing obstruction. Symptoms are usually bilious vomiting, abdominal distension, malabsorption and obstipation. Management consists of wide local resection of the tumor along with the segment of affected bowel. Some cases have demonstrated spontaneous regression. Solitary lesions carry an excellent prognosis after resection, while multiple lesions carry a worse prognosis.

1- Canioni D, Fekete C, Nezelof C: Solitary intestinal fibromatosis: a rare cause of neonatal obstruction. Pediatr Pathol. 9(6):719-24, 1989
2- Srigley JR, Mancer K: Solitary intestinal fibromatosis with perinatal bowel obstruction. Pediatr Pathol. 2(3):249-58, 1984
3- Türken A, Senocak ME, Kotiloglu E, Kale G, Hiçsönmez A: Solitary intestinal fibromatosis mimicking malabsorption syndromes. J Pediatr Surg. 30(9):1387-9, 1995
3- Arets HG, Blanco C, Thunnissen FB, Heineman E: Solitary intestinal fibromatosis as a cause of bile vomiting in a neonate. J Pediatr Surg. 35(4):643-5, 2000
4- Numanoglu A, Davies J, Millar AJ, Rode H: Congenital solitary intestinal fibromatosis. Eur J Pediatr Surg. 12(5):337-40, 2002
5- Coulon A, McHeik J, Milin S, Levard G, Levillain P, Fromont G: Solitary intestinal fibromatosis associated with congenital ileal atresia. J Pediatr Surg. 42(11):1942-5, 2007

Natural Orifice Transluminal Endoscopic Surgery

Natural orifice transluminal endoscopic surgery (NOTES) is the next frontier in minimal invasive  surgical procedures. NOTES takes advantage of doing a laparoscopic procedure through natural orifices of our body such as mouth (trans-gastric), anus (trans-colonic) or vagina totally eliminating a scar in the abdomen. Theoretically, this approach could reduce postoperative abdominal wall pain, wound infection, hernia formation, and adhesions. So far studies in animal models have demonstrated the feasibility of performing such procedure to remove the gallbladder through either per-oral transgastric or per-anal transcolonic by perforating such viscera and introducing a multichannel locking endoscope to introduce the laparoscopic instruments utilized during actual transperitoneal procedures. The incision done in the perforated viscera is subsequently closed with endoscopic clips, endoloops, or a prototype closure device. In contrast to the transgastric method, a transcolonic approach provides more consistent identification of structures in the upper abdomen and provides better en face orientation and scope stability. There is no bleeding or laceration of adjacent organs. Animal models have been used to demonstrate the possible applications of NOTES, including transgastric peritoneoscopy, tubal ligation, gastrojejunostomy, partial hysterectomy, oophorectomy, nephrectomy and transcolonic exploration, liver biopsy, distal pancreatectomy and cholecystectomy. The first human report in 2007 was a successful trans-vaginal cholecystectomy.

1- Ko CW, Kalloo AN: Per-oral transgastric abdominal surgery. Chin J Dig Dis. 2006;7(2):67-70
2- Pai RD, Fong DG, Bundga ME, Odze RD, Rattner DW, Thompson CC: Transcolonic endoscopic cholecystectomy: a NOTES survival study in a porcine model (with video). Gastrointest Endosc. 64(3):428-34, 2006
3- Fong DG, Pai RD, Thompson CC: Transcolonic endoscopic abdominal exploration: a NOTES survival study in a porcine model. Gastrointest Endosc. 65(2):312-8, 2007
4- Wagh MS, Thompson CC: Surgery insight: natural orifice transluminal endoscopic surgery--an analysis of  work to date. Nat Clin Pract Gastroenterol Hepatol. 4(7):386-92, 2007
5- Marescaux J, Dallemagne B, Perretta S, Wattiez A, Mutter D, Coumaros D: Surgery without scars: report of transluminal cholecystectomy in a human being. Arch Surg. 142(9):823-6, 2007
6- Fritscher-Ravens A, Ghanbari A, Thompson S, Patel K, Kahle E, Fritscher T, Niemann H, Koehler P, Milla P: Which parameters might predict complications after natural orifice endoluminal
surgery (NOTES)? Results from a randomized comparison with open surgical access in pigs. Endoscopy. 39(10):888-92, 2007

PSU Volume 30 No 03 MARCH 2008

Cutaneous Mucinosis

Frequently pediatric surgeons are asked to evaluate a skin disorder in children. Most cases are evaluated and managed by dermatologists, but in a few patients due to the nature of the lesion or age an excisional ir incisional biopsy is required to establish the histologic diagnosis. Such is the case of juvenile cutaneous mucinosis, a very rare disorder of skin found in children. Characteristically cutaneous mucinosis is a nontender hypopigmented plaque-like raised papula which can appear in the trunk or extremity singly or with several other associated lesions. Cutaneous mucinoses are a heterogeneous group of diseases in which mucin accumulates in the skin or within the hair follicle. Mucin is a gelatinous substance composed of glycosaminoglycans, especially hyaluronic acid and dermatan sulfate bound to small quantities of chondroitin sulfate and heparin sulfate. Two groups are identified: the primary cutaneous mucinoses in which the mucin deposit is a distinctive histopathologic feature that manifests as a clinically specific lesion, and the diseases associated with histopathologic mucin deposition as an additional finding (secondary) such as myxedema, lupus erythematosus or scleroderma. The primary juvenile variety occurs in children  characterized by an early age of onset, the presence of plaques and nodules in a characteristic distribution, and rapid onset followed by spontaneous resolution of the lesions within a period of weeks to months.

1- Rongioletti F, Rebora A: Cutaneous mucinosis. Ann Dermatol Venereol. 120(1):75-87, 1993
2- Wadee S, Roode H, Schulz EJ: Self-healing juvenile cutaneous mucinosis in a patient with nephroblastoma. Clin Exp Dermatol. 19(1):90-3, 1994
3- Caputo R, Grimalt R, Gelmetti C: Self-healing juvenile cutaneous mucinosis. Arch Dermatol. 131(4):459-61, 1995
4- Aydingöz IE, Candan I, Dervent B: Self-healing juvenile cutaneous mucinosis. Dermatology. 199(1):57-9, 1999
5- Carder KR, Fitzpatrick JE, Weston WL, Morelli JG: Self-healing juvenile cutaneous mucinosis. Pediatr Dermatol. 20(1):35-9, 2003
6- Nagaraj LV, Fangman W, White WL, Woosley JT, Prose N, Selim MA, Morrell DS: Self-healing juvenile cutaneous mucinosis: cases highlighting subcutaneous/fascial involvement. J Am Acad Dermatol. 55(6):1036-43, 2006

Werdnig-Hoffmann Disease

Spinal muscular atrophy is a common autosomal recessive neuromuscular disorder characterized by degeneration of motor neurons of the spinal cord. Werdnig-Hoffman disease (WHD), the second most common neuromuscular disease in childhood, is a type of spinal muscular atrophy. Werdnig-Hoffmann is subdivided into two groups on the basis of a combination of age of onset, milestones of development and age of survival. Type I has an acute onset before six months of life and the progression of disease is severe with a more uniform poor prognosis and early death. They showed generalized hypotonia, abnormal respiration and could not sit without support. Type II  onset of the disease is between the age of six and 18 months and progression is slower. A gene termed 'survival
of motor neuron' (SMN) has been recognized as the disease-causing gene. SMN encodes a protein located within a novel nuclear structure and interacts with RNA-binding proteins. Pre- or postnatal diagnosis is made with this genetic testing. There is no effective therapy for WHD.Management consists of preventing or treating the complications of severe weakness, such as restrictive lung disease, poor nutrition, orthopedic deformities, immobility, and psychosocial problems. Tracheostomy, gastrostomy and fundoplication with non-invasive mechanical ventilation can help prolong life in WHD.

1- Russman BS, Iannacone ST, Buncher CR, Samaha FJ, White M, Perkins B, Zimmerman L, Smith C, Burhans K, Barker L: Spinal muscular atrophy: new thoughts on the pathogenesis and classification schema. J Child Neurol. 7(4):347-53, 1992
2- Lefebvre S, Burlet P, Liu Q, Bertrandy S, Clermont O, Munnich A, Dreyfuss G, Melki J: Correlation between severity and SMN protein level in spinal muscular atrophy. Nat Genet. 16(3):265-9, 1997
3- Iannaccone ST: Spinal muscular atrophy. Semin Neurol. 18(1):19-26, 1998
4- Stipoljev F, Sertic J, Latin V, Rukavina-Stavljenic A, Kurjak A: Prenatal diagnosis of spinal muscular atrophy type I (Werdnig- hoffmann) by DNA deletion analysis of cultivated amniocytes. Croat Med J. 40(3):433-7, 1999
5- Bach JR, Saltstein K, Sinquee D, Weaver B, Komaroff E: Long-term survival in Werdnig-Hoffmann disease. Am J Phys Med Rehabil. 86(5):339-45, 2007
6- Bach JR: Medical considerations of long-term survival of Werdnig-Hoffmann disease. Am J Phys Med Rehabil. 86(5):349-55, 2007

Treadmill Injury

Injury is the leading cause of preventable death and disability in childhood and early adulthood. Most of us have a treadmill, or jogging machine in our house to stay fit. Treadmills have been found to account for an increase in the number of injuries in our children. Approximately 25,000 children with a median age of 2.5 years are injured on exercise equipment each year.  Jogging machines can cause friction burns, abrasions, blunt trauma, or even amputation. Most of these injuries will require surgical intervention and rehabilitation. Almost all injuries are friction injury due to contact with the moving treadmill belts. Friction injuries that convert into a full thickness burn needing skin grafting surgery. The upper extremity is more commonly affected than the lower extremity with almost 75% of cases involving the palmar aspect of the hand. Most common mechanism is when the machine is in use by an adult and a curious toddler comes toward the running treadmill. Other cases are the older child who has the height to reach and activate the machine sustaining injury when they fall. Prevention modalities include additional manufacture safety features, warning labels, and parental education. Parent supervision during use of this type of machine is paramount. Recommendations include limiting children access, facing the treadmill toward the open room, use a back mirror, and avoiding the use of headsets while on the treadmill.

1- Attalla MF, al-Baker AA, al-Ekiabi SA: Friction burns of the hand caused by jogging machines: a potential hazard to children. Burns. 17(2):170-1, 1991
2- Carman C, Chang B: Treadmill injuries to the upper extremity in pediatric patients. Ann Plast Surg. 47(1):15-9, 2001
3- Borschel GH, Wolter KG, Cederna PS, Franklin GA: Acute management of exercise treadmill-associated injuries in children. J Trauma. 55(1):130-4, 2003
4- Maguiña P, Palmieri TL, Greenhalgh DG: Treadmills: a preventable source of pediatric friction burn injuries. J Burn Care Rehabil. 25(2):201-4, 2004
5- Han T, Han K, Kim J, Lee G, Choi J, Lee J, Jang Y, Oh S: Pediatric hand injury induced by treadmill. Burns. 31(7):906-9, 2005
6- Wong A, Maze D, La Hei E, Jefferson N, Nicklin S, Adams S: Pediatric treadmill injuries: a public health issue. J Pediatr Surg. 42(12):2086-9, 2007

PSU Volume 30 No 04 APRIL 2008

Esophageal Foreign Bodies

The superior esophagus is the narrowest portion of the alimentary tract of children and the most common site for lodge foreign bodies. Due to the nature of infants and toddlers to place objects in their mouth, especially a coin, this represent the most common foreign body identified within the proximal esophagus. The child will develop cough, stridor, choking, drooling, pain and inability to swallow with a lodge esophageal foreign body. Complications secondary to the esophageal foreign body itself include erosion/perforation, stricture, migration, mediastinitis and airway complications. Since aspiration and perforation are immediate complications, the impacted foreign body mandates urgent surgical attention. A simple chest film will delineate the position of the lodge coin. With other type of non-opaque foreign bodies an esophagogram will be needed to help visualized the position and type of obstruction. Rigid esophagoscopy under general anesthesia or flexible esophagoscopy under sedation is the procedure of choice to remove the foreign body, though Foley balloon extraction under fluoroscopic control is an acceptable method of coin extraction with minimal morbidity. Other times the foreign body can be pushed toward the stomach using esophageal bougienage. Children younger than one years, those with a widened tracheoesophageal interface, not a smooth object or more than one week after ingestion seems to be at highest risk for esophageal edema, failure of balloon extraction and complications.

1- Ozguner IF, Buyukyavuz BI, Savas C, Yavuz MS, Okutan H: Clinical experience of removing aerodigestive tract foreign bodies with rigid endoscopy in children. Pediatr Emerg Care. 20(10):671-3, 2004
2- Waltzman ML, Baskin M, Wypij D, Mooney D, Jones D, Fleisher G: A randomized clinical trial of the management of esophageal coins in children. Pediatrics. 116(3):614-9, 2005
3- Little DC, Shah SR, St Peter SD, Calkins CM, Morrow SE, Murphy JP, Sharp RJ, Andrews WS, Holcomb GW 3rd, Ostlie DJ, Snyder CL: Esophageal foreign bodies in the pediatric population: our first 500 cases. J Pediatr Surg. 41(5):914-8, 2006
4- Waltzman ML: Management of esophageal coins. Curr Opin Pediatr. 18(5):571-4, 2006
5- Tokar B, Cevik AA, Ilhan H: Ingested gastrointestinal foreign bodies: predisposing factors for complications in children having surgical or endoscopic removal. Pediatr Surg Int. 23(2):135-9, 2007
6- Weissberg D, Refaely Y: Foreign bodies in the esophagus. Ann Thorac Surg. 84(6):1854-7, 2007


Lipomas are benign tumors accounting for 6% of soft-tissue tumors found in the pediatric age. They are ubiquitous and can occur anywhere in the body with a predilection for the trunk. Lipomas develop as painless, gradually enlarging swellings, soft to palpation and with demarcated borders. These neoplasms are slow growing and may reach great proportions without producing significant symptoms depending on location. Mediastinal lipomas produce respiratory distress, while spinal cord lipomas are associated with significant neurological symptoms. Ultrasound will uncover the homogenous nature of the tumor, while CT or MRI will demonstrate the relation of the tumor with surrounding structures. Infiltration of surrounding structure rather than displacement suggests a malignant variant known as liposarcoma. Definite diagnosis can only be obtained by pathologic examination which must differentiate between lipoma, lipoblastoma, liposarcoma or myxoma. Needle aspiration biopsy can provide the diagnosis. Management consists of surgical resection to establish the diagnosis, alleviate symptoms if present and avoid local recurrence. Endoscopic excision or liposuction of large capsulated lipomas can be appropriate treatment and effective from a cosmetic point of view.

1- Sakai Y, Okazaki M, Kobayashi S, Ohmori K: Endoscopic excision of large capsulated lipomas. Br J Plast Surg. 49(4):228-32, 1996
2- Mahomed AA, Beale P, Puri P: Mediastinal lipoma in children. Pediatr Surg Int. 13(2-3):218-9, 1998
3- de Jong AL, Park A, Taylor G, Forte V: Lipomas of the head and neck in children. Int J Pediatr Otorhinolaryngol. 43(1):53-60, 1998
4- Ilhan H, Tokar B: Liposuction of a pediatric giant superficial lipoma. J Pediatr Surg. 37(5):796-8, 2002
5- Inampudi P, Jacobson JA, Fessell DP, Carlos RC, Patel SV, Delaney-Sathy LO, van Holsbeeck MT: Soft-tissue lipomas: accuracy of sonography in diagnosis with pathologic correlation. Radiology. 233(3):763-7, 2004
6- Grandbois L, Vade A, Lim-Dunham J, Al-Masri H: MRI findings of an intermuscular lipoma in a 2-year-old. Pediatr Radiol. 36(9):974-6, 2006

Anal Canal Duplication

Anal canal duplication is a very rare congenital malformation: the most distal and least common duplication of the digestive tube. It can be confused with other types of anorectal pathology  including hemorrhoids, fistula-in-ano, and perirectal abscess. Anal canal duplications are usually located posterior presenting as a one to 2 mm perineal opening just behind the anus in the midline. The tract runs along the posterior aspect of the anal canal without communication with the anorectum. Most  cases are females. Simple perineal inspection makes the diagnosis. Older children will present with  localized infection or pruritus. Most children are asymptomatic. Fistulography reveals a tubular structure or a cystic structure behind the normal anal canal whose length varies from 10 to 30 mm. Associated malformations include sacrococcygeal teratomas, dermoid cysts, sacral dysgenesis, hindgut anomalies and lumbosacral myelomeningocele. Non-invasive preoperative investigations consisting of pelvic X-ray, US examination, barium enema and fistulography, are sufficient in most cases; MRI is reserved to evaluate the presence of associated anomalies. Surgical treatment through a posterior sagittal approach or simple mucosectomy restores a normal perineal aspect without sequelae and avoids future complications like those described in other types of digestive duplications namely infection, ulceration, bleeding, and malignant changes during later adult life. Histology shows squamous epithelium on the surface of the fistula and columnar epithelium and goblet cells in the base, which confirms the diagnosis of an anal-canal duplication. Prognosis is good after surgery.

1- Jacquier C, Dobremez E, Piolat C, Dyon JF, Nugues F: Anal canal duplication in infants and children--a series of 6 cases. Eur J Pediatr Surg. 11(3):186-91, 2001
2- Ochiai K, Umeda T, Murahashi O, Sugitoh T: Anal-canal duplication in a 6-year-old child. Pediatr Surg Int. 18(2-3):195-7, 2002
3- Choi SO, Park WH: Anal canal duplication in infants. J Pediatr Surg. 38(5):758-62, 2003
4- Tiryaki T, Senel E, Atayurt H: Anal canal duplication in children: a new technique. Pediatr Surg Int. 22(6):560-1, 2006
5- Lisi G, Illiceto MT, Rossi C, Broto JM, Jil-Vernet JM, Lelli Chiesa P: Anal canal duplication: a retrospective analysis of 12 cases from two European pediatric surgical departments. Pediatr Surg Int. 22(12):967-73, 2006

PSU Volume 30 No 05 MAY 2008

Enterocutaneous Fistulas

Enterocutaneous fistulas (ECF) are serious complications associated with high morbidity and mortality. Most ECF occurs after surgery or trauma, while other times Crohn's disease, necrotizing enterocolitis, intra-abdominal abscess, malignant disease and radiotherapy are the culprits. ECF can be classified as low output (less than five ml/kg/day), or high output (greater than five ml/kg/day). Postoperative ECF results from infection and breakdown of an anastomosis, bowel injury, deserosalization of bowel, suture-lines defects, tight sutures with ischemic necrosis, injury to mesenteric vessels, poor hemostasis, adhesive ischemia, volvulus and bowel loop caught in a fascial suture. Postoperative ECF can be also classified as early (those that occur within 48 hours after surgery and are associated to a technical error), and late (occurring 48 hours after the procedure) and associated with low ischemia time. It is vital to identify the source and route of the ECF tract by imaging techniques (UGIS, Barium enema, CT Scan or MRI) and whether the patient has distal obstruction. Management consists of reducing the septic state by adequate draining, hydration, correction of electrolyte imbalances, parenteral antibiotics, somatostatin-14 trial, bowel rest, parenteral nutrition, cutaneous protection, and surgical correction using resection with anastomosis or bypass procedures if the ECF fails to respond to conservative measures.

1- Fekete CN, Ricour C, Duhamel JF, Lecoultre C, Pellerin D: Enterocutaneous fistulas of the small bowel in children (25 cases). J Pediatr Surg. 13(1):1-4, 1978
2- Lévy E, Frileux P, Cugnenc PH, Honiger J, Ollivier JM, Parc R: High-output external fistulae of the small bowel: management with continuous enteral nutrition. Br J Surg. 76(7):676-9, 1989
3- Falconi M, Pederzoli P: The relevance of gastrointestinal fistulas in clinical practice: a review. Gut 49: iv2-iv10, 2002
4- Gonzalez-Pinto I, Moreno Gonzalez E: Optimizing the treatment of upper gastrointestinal fistulas. Gut 49: iv21-iv28, 2002
5- Jamil M, Ahmed U, Sobia H: Role of somatostatin analogues in the management of enterocutaneous fistulae. J Coll Physicians Surg Pak. 14(4):237-40, 2004
6- Ahmad RR, Fawzy SY: Enterocutaneous fistula. Causes and management. Saudi Med J. 28(9):1408-13, 2007


Pneumothorax is the presence of air in the pleural cavity. Results from either a tear in the visceral or parietal pleura. Pneumothorax can be spontaneous (primary or secondary), or acquire. The most common cause of primary spontaneous pneumothorax is rupture of an apical subpleural bleb of the lung, usually a thin adolescent male who suddenly develops chest pain and shortness of breath. Secondary spontaneous pneumothorax occurs after hyaline membrane disease, meconium aspiration, cystic fibrosis, or AIDS. Acquired pneumothorax is more common than spontaneous usually the result of blunt or penetrating trauma, iatrogenic after central line placement, thoracentesis, lung biopsy, barotrauma from mechanical ventilation and laparoscopic procedures. Diagnosis of pneumothorax is done with simple chest films. Complex cystic lung conditions will need chest CT scan for diagnosis. The purpose of management is to evacuate the air in the pleura and expand adequately the lung. Small pneumothorax (less than 20%) can be managed with observation and oxygen therapy. Tube thoracostomy is recommended for pneumothorax larger than 20%. The tube is removed when the lung has expanded completely and the air leak is no longer present for at least 24 hours. Surgical treatment is indicated using video assisted thoracic surgery (VATS) when air leaks continues for more than 72 hours, there is incomplete lung expansion or pneumothorax recurs after adequate management.

1- Liu HP, Yim AP, Izzat MB, Lin PJ, Chang CH: Thoracoscopic surgery for spontaneous pneumothorax. World J Surg. 23(11):1133-6, 1999
2- Shaw KS, Prasil P, Nguyen LT, Laberge JM: Pediatric spontaneous pneumothorax. Semin Pediatr Surg. 12(1):55-61, 2003
3- Ozcan C, McGahren ED, Rodgers BM: Thoracoscopic treatment of spontaneous pneumothorax in children. J Pediatr Surg. 38(10):1459-64, 2003
4- Choudhary AK, Sellars ME, Wallis C, Cohen G, McHugh K: Primary spontaneous pneumothorax in children: the role of CT in guiding management. Clin Radiol. 60(4):508-11, 2005
5- Qureshi FG, Sandulache VC, Richardson W, Ergun O, Ford HR, Hackam DJ: Primary vs delayed surgery for spontaneous pneumothorax in children: which is better? J Pediatr Surg. 40(1):166-9, 2005
6- Butterworth SA, Blair GK, LeBlanc JG, Skarsgard ED: An open and shut case for early VATS treatment of primary spontaneous pneumothorax in children. Can J Surg. 50(3):171-4, 2007

Patent Ductus Arteriosus

Patent ductus arteriosus (PDA) is a fetal vessel that connects the main pulmonary trunk with the descending aorta distal to the origin of the left subclavian artery. Most term infants have the PDA closed by three weeks of age, while premature takes longer to close. In terms infants, PDA can lead to death from congestive heart failure (tachypnea, tachycardia, poor feeding, slow weight gain and recurrent pulmonary infections). In premature, the PDA can lead to sepsis, renal failure, necrotizing enterocolitis, metabolic acidosis and death. PDA murmur is systolic. Indications for closure include  congestive heart failure, bacterial endocarditis, failure to close during the first year of life. In premature the PDA can be medically closed with pharmacologic indomethacin therapy (prostaglandin inhibition). If medical therapy fails surgical closure is needed. Surgical closure (division is preferred for term and older children, while ligation is reserved for premature) can be done through a left lateral thoracotomy, a medial sternotomy or from inside the pulmonary artery. Other alternatives include percutaneous catheter closure or thoracoscopic closure of the PDA. Some postoperative complications include bleeding, damage to phrenic, vagus or recurrent laryngeal nerve and chylothorax. In general prognosis is good.

1- Little DC, Pratt TC, Blalock SE, Krauss DR, Cooney DR, Custer MD: Patent ductus arteriosus in micropreemies and full-term infants: the relative merits of surgical ligation versus indomethacin  treatment. J Pediatr Surg. 38(3):492-6, 2003
2- Wyllie J: Treatment of patent ductus arteriosus. Semin Neonatol. 8(6):425-32, 2003
3- Van Overmeire B, Chemtob S: The pharmacologic closure of the patent ductus arteriosus. Semin Fetal Neonatal Med. 10(2):177-84, 2005
4- Arora R: Transcatheter closure of patent ductus arteriosus. Expert Rev Cardiovasc Ther. 3(5):865-74, 2005
5- Hermes-DeSantis ER, Clyman RI: Patent ductus arteriosus: pathophysiology and management. J Perinatol. 26 Suppl 1:S14-8, 2006
6- Herrera C, Holberton J, Davis P: Prolonged versus short course of indomethacin for the treatment of patent ductus arteriosus in preterm infants. Cochrane Database Syst Rev. 18;(2):CD003480, 2007


PSU Volume 30 No 06 JUNE 2008

Doxycycline Sclerotherapy

Lymphangiomas or cystic hygromas are congenital lymphatic vessels developmental anomalies affecting primarily the head and neck region of newborns. They manifest as multiple lymphatic fluid cysts involving muscle, fascia, blood vessels and nerves. This anatomic fact makes surgical excision very dangerous in respect to mutilating complications. Alternatively, sclerotherapy has progressively become the therapy of choice in many such cases. OK-432, bleomycin, fibrin glue sealant  and recently doxycycline are the most commonly used sclerosing agents. Lymphangiomas are classified as either macrocystic, microcystic or mixed. Pretreatment imaging to classify the lymphangioma is mandatory, and MRI is the modality of choice for the most definitive evaluation and classification of these cysts. Macrocystic and mixed lesions respond well to percutaneous sclerotherapy, while microcystic disease is usually managed with surgical excision. Doxycycline is an inexpensive and available tetracycline with widespread use as effective sclerosant in other parts of the body (pleura, pericardium and postoperative lymphoceles). A recent study confirmed doxycycline efficacy in managing macrocystic and mixed lymphatic malformations of the head and neck in children without significant side effects (4). Aspiration and sclerotherapy is done at the operating room under general anesthesia  using ultrasound cyst guidance.

1- Molitch HI, Unger EC, Witte CL, vanSonnenberg E: Percutaneous sclerotherapy of lymphangiomas. Radiology. 194(2):343-7, 1995
2- Wimmershoff MB, Schreyer AG, Glaessl A, Geissler A, Hohenleutner U, Feuerbach SS, Landthaler M: Mixed capillary/lymphatic malformation with coexisting port-wine stain: treatment
utilizing 3D MRI and CT-guided sclerotherapy. Dermatol Surg. 26(6):584-587, 2000
3- Mabrut JY, Grandjean JP, Henry L, Chappuis JP, Partensky C, Barth X, Tissot E: Mesenteric and mesocolic cystic lymphangiomas. Diagnostic and therapeutic management Ann Chir. 127(5):343-9, 2002
4- Nehra D, Jacobson L, Barnes P, Mallory B, Albanese CT, Sylvester KG: Doxycycline sclerotherapy as primary treatment of head and neck lymphatic malformations in children. J Pediatr Surg 43(3): 451-460, 2008

Pancreaticobiliary Maljunction

Pancreaticobiliary maljunction (PBM) or common channel is defined as communication of the common bile duct and pancreatic duct outside the sphincter of Oddi (main papilla). Etiology of PBM is caused by a disturbance in the embryonic connections (misarrangement) of the choledochopancreatic duct system in the early embryo, whereby the terminal bile duct joins with a branch of the ventral pancreatic duct system, including the main pancreatic duct. This malformation causes mixing of bile with pancreatic secretions resulting in choledochal cysts, recurrent pancreatitis, biliary tract carcinoma and formation of protein plugs. The frequency of gallbladder cancer in patients with PBM without congenital biliary duct dilatation is very high making some consider prophylactic cholecystectomy or resection of the extrahepatic bile duct for carcinogenesis prevention. The diagnostic criteria for PBM are the radiological and anatomical detection of the extramural location of the junction of the pancreatic and biliary ducts in the duodenal wall done by MRCP or ERCP. Clinical features of PBM are intermittent abdominal pain, with or without elevation of pancreatic enzyme levels; and obstructive jaundice, with or without acute pancreatitis, while the clinical features of PBM patients with congenital cystic duct dilatation are primary bile duct stone and acute cholangitis. Objective in management of PBM is prevention of the reciprocal reflux of bile and pancreatic juice in the pancreas and the bile duct system. To achieve these aims the surgical (resection and bilioenteric reconstruction) or endoscopic approach (papillotomy) is utilized.

1- Funabiki T, Matsubara T, Ochiai M, Marugami Y, Sakurai Y, Hasegawa S, Imazu H: Surgical strategy for patients with pancreaticobiliary maljunction without choledocal dilatation. Keio J Med. 46(4):169-72, 1997
2- Matsumoto Y, Fujii H, Itakura J, Matsuda M, Nobukawa B, Suda K: Recent advances in pancreaticobiliary maljunction. J Hepatobiliary Pancreat Surg. 9(1):45-54, 2002
3- Hamada Y, Tanano A, Takada K, Watanabe K, Tokuhara K, Sato M: Magnetic resonance cholangiopancreatography on postoperative work-up in children with choledochal cysts. Pediatr Surg Int. 20(1):43-6, 2004
4- Ohuchida J, Chijiiwa K, Hiyoshi M, Kobayashi K, Konomi H, Tanaka M: Long-term results of treatment for pancreaticobiliary maljunction without bile duct dilatation. Arch Surg. 141(11):1066-70, 2006
5- Ohama K, Ishikawa N: Dilatation of the intrahepatic bile duct associated with congenital anomalous junction of the cystic duct--a new disease entity. J Pediatr Surg. 41(12):1996-8, 2006
6- Terui K, Yoshida H, Kouchi K, Hishiki T, Saito T, Mitsunaga T, Takenouchi A, Tsuyuguchi T, Yamaguchi T, Ohnuma N: Endoscopic sphincterotomy is a useful preoperative management for refractory pancreatitis associated with pancreaticobiliary maljunction. J Pediatr Surg. 43(3):495-9, 2008
7- Okada T, Sasaki F, Honda S, Naitou S, Onodera Y, Todo S: Usefulness of axial planes of helical computed tomography for diagnosis of pancreaticobiliary maljunction in early infants with negative findings on magnetic resonance cholangiopancreatography. J Pediatr Surg. 43(3):579-82, 2008


Gastroparesis (GP) is a gastric disorder associated to symptoms of gastric retention or altered gastric emptying without evidence of mechanical obstruction. GP in children is usually associated with diabetes mellitus, postviral syndrome or after surgical procedures. Symptoms associated with gastroparesis include nausea, vomiting, early satiety, postprandial fullness and abdominal pain. Establishing the diagnosis of GP is difficult and requires UGIS, gastric scintigraphy, electrogastrography  and endoscopy with biopsy. Initial management consists of dietary modification, prokinetic agents  (cisapride), antiemetic therapy and pain control. More severe symptoms may need enteral or parenteral nutritional support. Endoscopic injection of botulinum toxin to the pyloric muscle can help. Surgery therapy might consist of pyloromyotomy, pyloroplasty (gastric emptying procedure), gastrostomy tube, jejunal feeding tube or gastrectomy procedures. Recently gastric electrical stimulation pacemaker placement has improved children with gastroparesis.

1- Sigurdsson L, Flores A, Putnam PE, Hyman PE, Di Lorenzo C: Postviral gastroparesis: presentation, treatment, and outcome. J Pediatr. 131(5):751-4, 1997
2- Katz S, Lazar L, Erez I, Kaufman Z: Subtotal gastrectomy in a teenager with gastroparesis. J Pediatr Surg. 34(3):509-11, 1999
3- Michaud L, Guimber D, Carpentier B, Sfeir R, Lambilliotte A, Mazingue F, Gottrand F, Turck D: Gastrostomy as a decompression technique in children with chronic gastrointestinal obstruction. J Pediatr Gastroenterol Nutr. 32(1):82-5, 2001
4- Smith DS, Williams CS, Ferris CD: Diagnosis and treatment of chronic gastroparesis and chronic intestinal pseudo-obstruction. Gastroenterol Clin North Am. 32(2):619-58, 2003
5- Chelimsky TC, Chelimsky GG: Autonomic abnormalities in cyclic vomiting syndrome. J Pediatr Gastroenterol Nutr. 44(3):326-30, 2007
6- Islam S, Vick LR, Runnels MJ, Gosche JR, Abell T: Gastric electrical stimulation for children with intractable nausea and gastroparesis. J Pediatr Surg. 43(3):437-42, 2008

Journal Club