References: 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
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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.
References: 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.
References: 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
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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- Martn-Garca RF, Garca ME, Rosado A: Modified curettage technique for molluscum contagiosum. Pediatr Dermatol. 24(2):192-4, 2007
References: 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
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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.
References: 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- Trken A, Senocak ME, Kotiloglu E, Kale G, Hisnmez 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
References: 1- Ko CW, Kalloo AN: Per-oral transgastric abdominal surgery. Chin J Dig Dis. 2006;7(2):67-70
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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
References: 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- Aydingz 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):359, 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
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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. WerdnigHoffmann 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.
References: 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):34955, 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.
References:
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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- Maguia 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
References: 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
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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.
References: 1- Sakai Y, Okazaki M, Kobayashi S, Ohmori K: Endoscopic excision of large capsulated lipomas. Br J Plast Surg. 49(4):22832, 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):5360, 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
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References: 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
References: 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- Lvy 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
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
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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.
References: 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
References: 1- Little DC, Pratt TC, Blalock SE, Krauss DR, Cooney DR, Custer MD: Patent ductus arteriosus in micropreemies and fullterm infants: the relative merits of surgical ligation versus indomethacin treatment. J Pediatr Surg. 38(3):492-6, 2003
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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
References: 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
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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.
References: 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
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.
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References: 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 pseudoobstruction. 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
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