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PEDIATRIC SURGERY UPDATE VOLUME 37, 2011

PSU Volume 37 No 01 JULY 2011
Necrotizing Pancreatitis
Necrotizing pancreatitis (NP) refers to a fulminant disease stage of the pancreas that occurs in 20% of all patients that develop acute pancreatitis increasing morbidity and mortality significantly. The initial etiology of the pancreatitis in children is in order of incidence trauma, drug-induced, biliary disorders (gallstones), infectious, metabolic and congenital. These conditions can lead to pancreatic autodigestion by enzyme activation. Symptoms include abdominal pain, vomiting, fever, elevated amylase and lipase with leukocytosis. Ultrasound typically shows a diffusely enlarged hypoechogenic pancreas. Contrast-enhanced CT Scan is the gold standard for diagnosing necrotizing pancreatitis. The affected portions of the pancreas in NP fails to enhanced due to disruption of the normal microcirculation. Initial management of NP consists of intensive medical support and prevention of infection (systemic and po non-absorbable antibiotics). With persistent biliary obstruction in the face of pancreatitis, ERCP should be used in combination with sphincterotomy to relieve the obstruction. Thirty to 70% of patients with NP develop a local pancreatic infection which triple the mortality. Infected peripancreatic collection should be percutaneously aspirated and drained. Surgery with necrosectomy should be delayed as long as possible and has no proven role in sterile necrosis. Complications associated with NP include persistent infection, hemorrhage, pancreatic fistula, duodenal obstruction and pancreatic insufficiency. The mortality is significant.
References 1- Takeda K, Matsuno S, Sunamura M, Kobari M: Surgical aspects and management of acute necrotizing pancreatitis: recent results of a cooperative national survey in Japan. Pancreas. 16(3):316-22, 1998 2- Sakorafas GH, Tsiotou AG: Etiology and pathogenesis of acute pancreatitis: current concepts. J Clin Gastroenterol. 30(4):343-56, 2000 3- Krueger WA, Unertl KE: Selective decontamination of the digestive tract. Curr Opin Crit Care. 8(2):139-44, 2002 4- Takeda K, Mikami Y, Fukuyama S, Egawa S, Sunamura M, Ishibashi T, Sato A, Masamune A, Matsuno S: Pancreatic ischemia associated with vasospasm in the early phase of human acute necrotizing pancreatitis. Pancreas. 30(1):40-9, 2005 5- Babu BI, Sheen AJ, Lee SH, O'Shea S, Eddleston JM, Siriwardena AK. Open pancreatic necrosectomy in the multidisciplinary management of postinflammatory necrosis. Ann Surg. 251(5):783-6, 2010 6- Wittau M, Scheele J, Glz I, Henne-Bruns D, Isenmann R: Changing role of surgery in necrotizing pancreatitis: a singlecenter experience.Hepatogastroenterology. 57(102-103):1300-4, 2010

Chilaiditi Syndrome
Chilaiditi syndrome refers to the abdominal symptoms that arise when a piece of bowel interposition between the liver and the diaphragm. Chilaiditi syndrome is a rare anomaly which occurs in up to 0.28 percent of the population and a source of abdominal problems requiring emergency or elective surgery. Hepatodiaphragmatic interposition of the transverse colon or small intestine can cause Chilaiditi syndrome. Though usually asymptomatic, symptoms can range from intermittent abdominal pain, vomiting, bloating, constipation to acute bowel obstruction. A few
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children have developed respiratory distress. The plain chest and abdominal films are diagnostic. This condition can be mistaken for pneumoperitoneum. US can help avoid confusion when pneumoperitoneum is suspected. The presence of hepatodiaphragmatic interposition of the intestine requires no specific treatment in the absence of symptoms. Volvulus of the transverse colon, history of prior abdominal surgery and colon redundancy can be associated with Chilaiditi syndrome. Surgery is typically reserved for cases of catastrophic colonic volvulus or perforation because of the syndrome. In cases of severe Chilaiditi syndrome refractory to medical treatment, a minimally invasive colopexy should be considered as a possible treatment option.
References: 1- Risaliti A, De Anna D, Terrosu G, Uzzau A, Carcoforo P, Bresadola F: Chilaiditi's syndrome as a surgical and nonsurgical problem. Surg Gynecol Obstet. 176(1):55-8, 1993 2- Sato M, Ishida H, Konno K, Hamashima Y, Naganuma H, Komatsuda T, Ishida J, Watanabe S: Chilaiditi syndrome: sonographic findings. Abdom Imaging. 25(4):397-9, 2000 3- White JJ, Chavez EP, Souza J: Internal hernia of the transverse colon-Chilaiditi syndrome in a child. J Pediatr Surg. 37(5):802-4, 2002 4- Barroso Jornet JM, Balaguer A, Escribano J, Pagone F, Domenech J, del Castillo D: Chilaiditi syndrome associated with transverse colon volvulus: first report in a paediatric patient and review of the literature. Eur J Pediatr Surg. 13(6):425-8, 2003 5- Saber AA, Boros MJ: Chilaiditi's syndrome: what should every surgeon know? Am Surg. 71(3):261-3, 2005 6- Keles S, Artac H, Reisli I, Alp H, Koc O: Chilaiditi syndrome as a cause of respiratory distress. Eur J Pediatr. 165(6):367-9, 2006 7- Blevins WA, Cafasso DE, Fernandez M, Edwards MJ: Minimally invasive colopexy for pediatric Chilaiditi syndrome. J Pediatr Surg. 46(3):e33-5, 2011

Gastric Electrical Stimulation

Gastric electrical stimulation (GES) has recently been developed as an alternative in the management of medically-refractory intractable nausea and gastroparesis associated with diabetes mellitus, postviral illness, idiopathic or postsurgical. The technique consists of placing two electrodes in the anterior seromuscular wall of the stomach hook to a subcutaneously placed stimulator using either an open or laparoscopic approach. Temporary percutaneously placed gastric stimulation electrodes using gastroscopy helps decide whether the patient will be a responder to the use permanent gastric electrical stimulation. The antiemetic effect of GES is mainly mediated by vagal afferent pathways. High-frequency GES has beneficial effects on symptoms in children with diabetic or idiopathic gastroparesis and severe nausea with vomiting. There is a significant improvement in symptoms over a prolonged period, and there are no adverse effects of the GES.
References: 1- Vandenplas Y, Hauser B, Salvatore S: Current pharmacological treatment of gastroparesis. Expert Opin Pharmacother. 5(11):2251-4, 2004 2- 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 3- Hyman P, Schropp K, Sarosiek I, Forster J, Lin Z, Gertken J, McCallum R: Feasibility and safety of gastric electrical stimulation for a child with intractable visceral pain and gastroparesis. J Pediatr Gastroenterol Nutr. 49(5):635-8, 2009 4-Andersson S, Ringstrm G, Elfvin A, Simrn M, Lnroth H, Abrahamsson H: Temporary percutaneous gastric electrical stimulation: a novel technique tested in patients with non-established indications for gastric electrical stimulation. Digestion. 83(1-2):3-12, 2011 5-Elfvin A, Gthberg G, Lnroth H, Saalman R, Abrahamsson H: Temporary percutaneous and permanent gastric electrical stimulation in children younger than 3 years with chronic vomiting. J Pediatr Surg. 46(4):655-61, 2011.

PSU Volume 37 NO 02 AUGUST 2011

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Neoappendicostomy
The use of the appendix as a conduit (appendicostomy) for the management of fecal incontinence caused by anorectal malformations, spina bifida and other diagnosis, has markedly improved the quality of life of children. Using this approach the child can regularly irrigate his large bowel and maintain socially continent for long periods of time. It also creates independence care as the child learns how to maintain clean using the prograde enemas regimen. When the appendix is not available due to previous surgical removal, a neoappendicostomy using a flap of cecum or ascending colon can be constructed. Other alternatives are using the sigmoid, transverse or descending colon. The flap must feed from the mesenteric side of the bowel to avoid ischemia. The neoappendicostomy created is wrap by a segment of colon creating a valve mechanism to reduce the leakage rate. Other workers have found the wrap and fixation unnecessary. The two main complications of the procedure are stricture formation and leakage. The button cecostomy is another common alternative for the child with no appendix available. These buttons can be placed open, laparoscopically, percutaneously or endoscopically. Complications of the buttons include dislodgement, granulation tissue, leakage, pain and ulcer formation. Incidental appendectomy should be discouraged in children with the potential to develop fecal incontinence in the future.
References: 1- Koivusalo A, Pakarinen M, Rintala RJ: Are cecal wrap and fixation necessary for antegrade colonic enema appendicostomy? J Pediatr Surg. 41(2):323-6, 2006 2- Kim J, Beasley SW, Maoate K: Appendicostomy stomas and antegrade colonic irrigation after laparoscopic antegrade continence enema.J Laparoendosc Adv Surg Tech A. 16(4):400-3, 2006 3- Koivusalo AI, Pakarinen MP, Pauniaho SL, Rintala RJ: Antegrade continence enema in the treatment of congenital fecal incontinence beyond childhood. Dis Colon Rectum. 51(11):1605-10, 2008 4-Bowkett BD, Kelly EW: Mucosal colonic tube fistula with antireflux wrap for antegrade colonic enema. Pediatr Surg Int. 25(6):507-11, 2009 5- Lawal TA, Rangel SJ, Bischoff A, Pena A, Levitt MA: Laparoscopic-assisted malone appendicostomy in the management of fecal incontinence in children. J Laparoendosc Adv Surg Tech A. 21(5):455-9, 2011 6- Chatoorgoon K, Pena A, Lawal T, Hamrick M, Louden E, Levitt MA: Neoappendicostomy in the management of pediatric fecal incontinence. J Pediatr Surg. 46(6): 1243-1249, 2011

Vascular Malformations Male Genitalia

Vascular malformations involving the male genitalia can cause considerable stress, adversely impact a child self-image, cause pain and alter urinary, reproductive and sexual function. Vascular malformations of male genitalia are more common than vascular tumors. They are divided in slow and fast flow lesions. The most common slow-flow vascular malformations affecting this anatomic area are lymphatics, venous or a combination of both. Fast-flow lesions are more commonly arteriovenous defects. These malformations clinically present with swelling, pain, fluid leakage, and infection. Other specific symptoms include hematuria, chylous reflux, dysuria, bladder outlet obstruction, erectile dysfunction and ambiguous genitalia. Diagnosis is made with a combination of physical exam and imaging. Indications for management of vascular malformations include bleeding, infection, fluid leakage, dysfunction and cosmetic concern. Management of vascular malformation includes surgical excision, pharmacotherapy, sclerotherapy, embolization, or a combination. The most common specific malformation is the infantile hemangioma. Though treatment is usually expectant there is place for use of steroid, propanolol and surgical excision. Lymphatic malformations in the groin and cord can be confused with an inguinal hernia. Combined slow-flow malformations are usually associated with soft tissue and skeletal hypertrophy.
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References: 1- Go S, Tokiwa K, Imazu M, Higuchi K, Iwai N: Arteriovenous malformation of the penis in an infant. J Pediatr Surg. 35(7):1130-1, 2000 2- Marrocco-Trischitta MM, Nicodemi EM, Stillo F: Sclerotherapy for venous malformations of the glans penis. Urology. 57(2):310-3, 2001 3- Morrison SC, Reid JR: Continuing problems with classifications of vascular malformations. Pediatr Radiol. 37(6):609, 2007 4- Papali AC, Alpert SA, Edmondson JD, Maizels M, Yerkes E, Hagerty J, Chaviano A, Kaplan WE: A review of pediatric glans malformations: a handy clinical reference. J Urol. 180(4 Suppl):1737-42, 2008 5-Kulungowski AM, Schook CC, Alomari AI, et al: Vascular anomalies of the male genitalia. J Pediatr Surg. 46(6):12141221, 2011

Appendix Diverticulum
Diverticulum of the appendix is a very rare disorder. Two types have been described. The true or congenital type in which all layers of the wall of the appendix are found in the wall of the diverticulum. The lesion is in the antimesenteric border and may be single or multiple. The other type most commonly found is the false or acquired diverticulum in which the muscular layer is absent from the wall. Acquired diverticulum are found on the mesenteric border and most commonly multiple. Appendix diverticulum can develop inflammation and perforation. Clinical symptoms range from chronic right lower quadrant abdominal pain to acute appendicitis. With inflammation of the diverticulum the most common diagnosis entertained is acute appendicitis. Even at operation the diagnosis of a diverticulum causing the inflammation can be missed. The congenital variety brings problems in the pediatric age, while the acquired form is more proper of the adult population. The management in all cases is appendectomy. Asymptomatic diverticulosis of the appendix found incidentally warrants appendectomy. Children with cystic fibrosis have a high incidence of appendix diverticulosis.
References: 1- Trollope ML, Lindenauer SM: Diverticulosis of the appendix: a collective review. Dis Colon Rectum. 17(2):200-18, 1974 2- George DH: Diverticulosis of the vermiform appendix in patients with cystic fibrosis. Hum Pathol. 18(1):75-9, 1987 3- Konen O, Edelstein E, Osadchi A, Shapiro M, Rathaus V: Sonographic appearance of an appendiceal diverticulum. J Clin Ultrasound. 30(1):45-7, 2002 4- Rakovich G: Diverticulosis of the appendix. Dig Surg. 23(1-2):26, 2006 5- Barc RM, Rousset J, Maignien B, Lu M, Prime-Guitton CH, Garcia JF: Diverticula of the appendix and their complications: value of sonography (review of 21 cases). J Radiol. 86(3):299-309, 2005 6- Majeski J: Diverticulum of the vermiform appendix is associated with chronic abdominal pain. Am J Surg. 186(2):129-31, 2003 7- Yates LN: Diverticulum of the vermiform appendix. A review of 28 cases. Calif Med. 116(1):9-11, 1972

PSU Volume 37 NO 03 SEPTEMBER 2011

HBO Therapy
Hyperbaric oxygen (HBO) therapy is a conservative therapy that involves inhaling up to 100% oxygen at a pressure greater than one atmosphere in a pressurized chamber design for such purpose. Most typical indications for hyperbaric oxygen therapy involve the use of hyperbaric pressures above two atmospheres. Such pressures are needed to manage conditions such as carbon monoxide poisoning and improve wound healing. HBO therapy for children is safe, even at pressures above two atmospheres. Some surgical conditions were concomitant HBO therapy in
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children has shown favorable results include crush injury, traumatic ischemia, electrical injuries, compartment syndrome, clostridial myonecrosis, necrotizing fasciitis, chemotherapy-induced neutropenia fasciitis, compromised skin graft, ischemic flaps, and refractory osteomyelitis. In order to see the favorable effects of HBO therapy, it is better to start the treatment within the first 2448 hours following injury. An increased oxygen concentration during HBO therapy promoted spontaneous wound healing. The needs of the pediatric patient, especially the critically ill, require specific skills and equipment inside the hyperbaric chamber. Close collaboration between the pediatrician and the hyperbaric medicine physician is essential to ensure adequate care for infants and children.
References: 1- Waisman D, Shupak A, Weisz G, Melamed Y: Hyperbaric oxygen therapy in the pediatric patient: the experience of the Israel Naval Medical Institute. Pediatrics. 102(5):E53, 1998 2- Johnston DL, Waldhausen JH, Park JR: Deep soft tissue infections in the neutropenic pediatric oncology patient. J Pediatr Hematol Oncol. 23(7):443-7, 2001 3- Rossignol DA, Rossignol LW, James SJ, Melnyk S, Mumper E: The effects of hyperbaric oxygen therapy on oxidative stress, inflammation, and symptoms in children with autism: an open-label pilot study. BMC Pediatr. 16;7:36, 2007 4- Pandey A, Gangopadhyay AN, Upadhyaya VD: Necrotising fasciitis in children and neonates: current concepts. J Wound Care. 17(1):5-10, 2008 5- Ambiru S, Furuyama N, Aono M, Otsuka H, Suzuki T, Miyazaki M: Analysis of risk factors associated with complications of hyperbaric oxygen therapy. J Crit Care. 23(3):295-300, 2008 6- Takac I, Kvolik S, Divkovic D, Kalajdzic-Candrlic J, Puseljic S, Izakovic S: Conservative surgical management of necrotic tissues following meningococcal sepsis: case report of a child treated with hyperbaric oxygen. Undersea Hyperb Med. 37(2):95-9, 2010

Anterior Mediastinal Mass

The differential diagnosis of an anterior mediastinal mass in a child includes in order of increased frequency a lymphoma, teratoma, thymoma or suprasternal goiter. Histopathologic analysis of an anterior mediastinal mass of unknown origin is essential for treatment decision. An anterior mediastinal mass can risk airway compromised. General anesthesia should be avoided in children with tracheal cross sectional area or peak expiratory flow rate less than 50% of predicted for age and sex, or severe narrowing and occlusion of a main stem bronchi. Diagnosis can be established in most patients by open biopsy or image-guided core needle biopsy under local anesthesia. Other cases might need aspiration of pleural effusion or bone marrow biopsy for diagnosis. Children that present with symptoms of dyspnea at rest, orthopnea, respiratory distress, or stridor are at risk of respiratory collapse. In severe cases when symptoms of respiratory collapse are present and the diagnosis cannot be obtained, a short course of steroid therapy can reduce the size of the mass and improve the child clinically. The Chamberlain procedure provides excellent access to the anterosuperior mediastinum for biopsy of obscure mediastinal mass lesions in childhood with a low rate of complications. Should general anesthesia be needed, spontaneous ventilation is preferred.
References: 1- Shamberger RC: Preanesthetic evaluation of children with anterior mediastinal masses. Semin Pediatr Surg. 8(2):61-8, 1999 2- Lam JC, Chui CH, Jacobsen AS, Tan AM, Joseph VT: When is a mediastinal mass critical in a child? An analysis of 29 patients. Pediatr Surg Int. 20(3):180-4, 2004 3- Salas Valverde S, Gamboa Y, Vega S, Barrantes M, Gonzalez M, Zamora JB: Diagnosis of anterior mediastinal mass lesions using the Chamberlain procedure in children. Pediatr Surg Int. 24(8):935-7, 2008 4- Perger L, Lee EY, Shamberger RC: Management of children and adolescents with a critical airway due to compression by an anterior mediastinal mass. J Pediatr Surg. 43(11):1990-7, 2008 5- Stricker PA, Gurnaney HG, Litman RS: Anesthetic management of children with an anterior mediastinal mass. J Clin Anesth. 22(3):159-63, 2010
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Cleft Lift Procedure

Sacrococcygeal pilonidal sinus disease is a chronic disorder that recurs even after adequate surgical procedures. Recurrence and chronicity are attributed to the depth of the gluteal cleft, moist bacteria-laden conditions within the airless cleft, incomplete excision of the diseased tissue, midline suture line, and closure tension. For the past years the cleft lift procedure has been applied to this disorder as a means of reducing recurrence. This technique involves excision of the chronic disease with a parasagittal elliptical resection and primary closure of the defect with a lateralbased flap, resulting in a tension-free closure that flattens the natal cleft and shifts the healing point laterally off of the midline. The flap technique aims at removing all the inflamed deep tissues, and the creation of a thick fat flap that reduces the depth of the airless cleft while decreasing the recurrence rate significantly. The cleft lift procedure does not involve wide excision of the deep inflamed tissue. The cleft lift procedure is a superior treatment method of pilonidal disease in adolescents, resulting in improved wound healing and lower likelihood of recurrent disease. Shaving, pulling hairs from the pores, and careful cleaning may be enough for earliest disease. The mystery of unhealed pilonidal disease is solved by understanding that the shape of the cleft and the conditions generated within that cleft inhibit epidermal closure and are the reason for the failure to heal.
References: 1- Bascom J, Bascom T: Failed pilonidal surgery: new paradigm and new operation leading to cures. Arch Surg. 137(10):1146-50, 2002 2- Bascom J, Bascom T: Utility of the cleft lift procedure in refractory pilonidal disease. Am J Surg. 193(5):606-9, 2007 3- Rushfeldt C, Bernstein A, Norderval S, Revhaug A: Introducing an asymmetric cleft lift technique as a uniform procedure for pilonidal sinus surgery. Scand J Surg. 97(1):77-81, 2008 4- Abdelrazeq AS, Rahman M, Botterill ID, Alexander DJ: Short-term and long-term outcomes of the cleft lift procedure in the management of nonacute pilonidal disorders. Dis Colon Rectum. 51(7):1100-6, 2008 5- Dudink R, Veldkamp J, Nienhuijs S, Heemskerk J: Secondary healing versus midline closure and modified bascom natal cleft lift for pilonidal sinus disease. Scand J Surg. 100(2):110-3, 2011 6- Gendy AS, Glick RD, Hong AR:A comparison of the cleft lift procedure vs wide excision and packing for the treatment of pilonidal disease in adolescents. J Pediatr Surg. 46(6): 1256-59, 2011

PSU Volumen 37 NO 04 OCTOBER 2011

Ventriculo-Gallbladder Shunts
When dealing with hydrocephalus in children the preferred method of drainage by neurosurgeons is the use of ventriculo-peritoneal shunting. Ventriculoperitoneal shunting may become dysfunctional due to repeated infections, intraabdominal adhesions, peritonitis, multiple abdominal surgical procedures, cerebrospinal fluid cysts or loculations, and other anatomic causes. When the peritoneum is useless as absorptive medium for CSF fluid, other alternatives previously used include the central venous system (atrium), pleural cavity, gallbladder, stomach, ureter and even fallopian tubes. The ventriculo-cholecystic (gallbladder) shunt is an attractive alternative when the intraperitoneal and intravascular route is not longer available. Ultrasound and nuclear studies of the gallbladder should be performed preop to determine absence of sludge or stones and adequate
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bile emptying. A fenestrated piece of distal shunt within the gallbladder lumen connected by a metal device to the proximal shunt catheter will provide the placement of the purse string in the gallbladder fundus. The gallbladder remains functional and revisions free. In several series involving the use of ventriculo-gallbladder shunts the long-term patency rate is above 75%. Infection and obstruction are the most common complications.
References: 1- West KW, Turner MK, Vane DW, Boaz J, Kalsbeck J, Grosfeld JL: Ventricular gallbladder shunts: an alternative procedure in hydrocephalus. J Pediatr Surg. 22(7):609-12, 1987 2- Stringel G, Turner M, Crase T: Ventriculo-gallbladder shunts in children. Childs Nerv Syst. 9(6):331-3, 1993 3- Ketoff JA, Klein RL, Maukkassa KF: Ventricular cholecystic shunts in children. J Pediatr Surg. 32(2):181-3, 1997 4- Pal K, Jindal V: Ventriculo cholecystic shunt in the management of hydrocephalus. Indian Pediatr. 44(6):435-7, 2007 5- Aldana PR, James HE, Postlethwait RA: Ventriculogallbladder shunts in pediatric patients. J Neurosurg Pediatr. 1(4):2847, 2008 6- Girotti ME, Singh RR, Rodgers BM: The ventriculo-gallbladder shunt in the treatment of refractory hydrocephalus: a review of the current literature. Am Surg. 75(8):734-7, 2009

Esophageal Lung
Esophageal lung refers to a very rare congenital bronchopulmonary foregut malformation where there is an anomalous connection of a main stem bronchus with the esophagus or stomach instead of the trachea. Esophageal lung anomaly usually occurs in the right side with no sex predilection. This anomaly arises when independent collections of cells with respiratory potential arise from the primitive esophagus caudal to the normal lung bud or when part of the lung bud originates from the dorsal esophagus instead of the ventral laryngotracheal tube. Associated anomalies consist of esophageal atresia, tracheoesophageal fistula, and cardiac defects. In cases of esophageal atresia, is the distal esophagus where the esophageal lung is connected through a bronchus arising from it. The most common clinical presentation is recurrent lower respiratory tract infections, atelectasis, and bronchiectasis arising from this lung which is usually hypoplastic and unilobar. Diagnosis is made with contrast study of the esophagus, trachea and CT Scan. The esophageal lung receives its blood supply from the pulmonary artery different from a sequestration where the blood supply comes directly from an aortic branch. Drainage occurs via the inferior pulmonary veins. Management of esophageal lung consists of resection of the destroyed hypoplastic lung with repair of the esophageal communication. In early cases where the esophageal lung is not destroyed, it has been reimplanted to the trachea.
References: 1- Leithiser RE Jr, Capitanio MA, Macpherson RI, Wood BP: "Communicating" bronchopulmonary foregut malformations. AJR Am J Roentgenol. 1986 Feb;146(2):227-31 2- Srikanth MS, Ford EG, Stanley P, Mahour GH: Communicating bronchopulmonary foregut malformations: classification and embryogenesis.J Pediatr Surg. 1992 Jun;27(6):732-6. 3- Jamieson DH, Fisher RM: Communicating bronchopulmonary foregut malformation associated with esophageal atresia and tracheo-esophageal fistula. Pediatr Radiol. 1993;23(7):557-8. 4- Michel JL, Revillon Y, Salakos C, De Blic J, Jan D, Beringer A, Scheinmann P: Successful bronchotracheal reconstruction in esophageal bronchus: two case reports. J Pediatr Surg. 1997 May;32(5):739-42 5- Tsugawa J, Tsugawa C, Satoh S, Nishijima E, Muraji T, Takamizawa S, Kanegawa K, Akasaka Y: Communicating bronchopulmonary foregut malformation: particular emphasis on concomitant congenital tracheobronchial stenosis.Pediatr Surg Int. 2005 Nov;21(11):932-5 6- Sugandhi N, Sharma P, Agarwala S, Kabra SK, Gupta AK, Gupta DK: Esophageal lung: presentation, management, and review of literature. J Pediatr Surg. 2011 Aug;46(8):1634-7

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Histiocytosis
Histiocytosis refers to a general term use for a group of syndromes that involves an abnormal increase in the number of immune cells known as histiocytes. The three majors type of histiocytosis are Langerhans's cell (histiocytosis X), malignant histiocytosis syndrome (T-cell lymphoma), and non-Langerhans's histiocytosis (hemophagocytic syndrome). Histiocytosis X is the most common, and is type of autoimmune condition in which the immune cells attack the body by mistake. Extraimmune cells may form tumors which affect the bones, the skulls and other areas of the body. Most cases are children within the ages of one to 15. Up to 50% of patients with either single or multiorgan manifestation of Langerhans' cell histiocytosis initially present with cutaneous symptoms. Symptoms depend on the system affected such as abdominal pain, bone pain, irritability, fever, swollen lymph nodes, etc. Children above age five often have bone involvement. Punch-out lesions in bone x-ray are characteristics. Management of Histiocytosis X consists of immunosuppression, chemotherapy and radiotherapy. A localized self-limited cutaneous form of the disease can be managed with complete surgical excision. Three types of skin lesions usually occur: nodules (common), scaling, or crusted papules and soft, yellow papular xanthomas.
References: 1- Chu T: Langerhans cell histiocytosis. Australas J Dermatol. 42(4):237-42, 2001 2- Janka GE, Schneider EM: Modern management of children with haemophagocytic lymphohistiocytosis. Br J Haematol. 124(1):4-14, 2004 3- Gasent Blesa JM, Alberola Candel V, Solano Vercet C, Laforga Canales J, Semler C, Prez AntolMR, RodrguezGalindo C: Langerhans cell histiocytosis. Clin Transl Oncol. 10(11):688-96, 2008 4- Abla O, Egeler RM, Weitzman S: Langerhans cell histiocytosis: Current concepts and treatments. Cancer Treat Rev. 36(4):354-9, 2010 5- Querings K, Starz H, Balda BR: Clinical spectrum of cutaneous Langerhans' cell histiocytosis mimicking various diseases.Clinical spectrum of cutaneous Langerhans' cell histiocytosis mimicking various diseases. Acta Derm Venereol. 86(1):39-43, 2006 6- Hussein MR: Skin-limited Langerhans' cell histiocytosis in children. Cancer Invest. 27(5):504-11, 2009

PSU Volume 37 No 05 NOVEMBER 2011

Chest Wall Tumors
Primary chest wall tumors (PCWT) in children are rare, with the majority being malignant. They originate from the skeletal chest wall or surrounding soft tissue. Benign chest wall tumors consist of osteochondroma, lipoblastoma, osteoblastoma bone cyst and eosinophilic granuloma. Resection provides cure in most cases. Malignant PCWT includes primitive neuroectodermal tumor (formerly Ewing's sarcoma), rhabdomyosarcoma, chondrosarcoma, neuroblastoma, leiomyosarcoma, fibrosarcoma and osteogenic sarcoma. Both leukemia and lymphoma can present as chest wall masses. Diagnosis of PCWT includes simple chest film, CT Scan and MRI studies. An initial biopsy by either core-needle or open is warranted since many of these tumors are amenable to preoperative adjuvant therapy. Those tumors that respond to preop chemotherapy will make later chest wall resection less morbid and more feasible. During resection of the tumor the surgeon should open one to two interspace higher and away from the primary rib involved. Negative microscopic margins are required for malignant tumors when feasible. To avoid paradoxical motion of the rib after resection of two or more ribs, rigid chest reconstruction with mesh is needed. Methylmethacrylate cement sandwich between tow layers of polypropylene mesh produces this effect very well. The mesh should extend over the edge of the rigid part of the sandwich to allow
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suturing with non-absorbable material. Complications include recurrence, respiratory insufficiency, scoliosis and chest wall deformity. In general prognosis is good.
References: 1- Dang NC, Siegel SE, Phillips JD: Malignant chest wall tumors in children and young adults. J Pediatry Surg.34(12):1773-8, 1999 2- Wong KS, Hung IJ, Wang CR, Lien R: Thoracic wall lesions in children.Pediatr Pulmonol. 37(3):257-63, 2004 3- Soyer T, Karnak I, Ciftci AO, Senocak ME, Tanyel FC, Bykpamuku N: The results of surgical treatment of chest wall tumors in childhood. Pediatry Surg Int.22(2):135-9, 2006 4- van den Berg H, van Rijn RR, Merks JH: Management of tumors of the chest wall in childhood: a review. J Pediatry Hematol Oncol.30(3):214-21, 2008 5- La Quaglia MP: Chest wall tumors in childhood and adolescence. Semin Pediatry Surg. 17(3):173-80, 2008 6- Gonfiotti A, Santini PF, Campanacci D, Innocenti M, Ferrarello S, Caldarella A, Janni A: Malignant primary chest-wall tumours: techniques of reconstruction and survival. Eur J Cardiothorac Surg. 38(1):39-45, 2010

Pancreatic Abscess
A pancreatic abscess is a localized collection of purulent material with little or no necrosis in the region of the pancreas which is delineated by a wall of collagen and granulation tissue. It is also known as walled-off pancreatic necrosis (WOPN). In contra position, an infected pancreatic pseudocyst is a localized collection of infected fluid in the region of the pancreas, and like an abscess is also walled off by a membrane of collagen and granulation tissue. Both conditions are seen as complications after acute pancreatitis. The child develops abdominal pain, fever and leukocytosis. Blood cultures are positive. The degree of enzyme elevations does not correlate with the degree of necrosis. The presence of air in necrotic tissue in a pseudocyst is specific for infection. The diagnosis is established with contrast enhanced CT-Scan. Management consists of systemic antibiotics, bowel rest, gastric suction and enteral nutrition. Primary drainage is the treatment of choice for a wall-off pancreatic necrosis. Endoscopic ultrasound with transgastric drainage is another option. WOPN can lead to several complications such as fistula formation, recurrent pancreatitis, bowel obstruction or death. Acute surgery may be needed in cases of perforation, major bleeding or when the child is not responding to therapy.
References: 1- Papachristou GI, Takahashi N, Chahal P, Sarr MG, Baron TH: Peroral endoscopic drainage/debridement of walled-off pancreatic necrosis. Ann Surg. 245(6):943-51, 2007 2- Stamatakos M, Stefanaki C, Kontzoglou K, Stergiopoulos S, Giannopoulos G, Safioleas M: Walled-off pancreatic necrosis. World J Gastroenterol. 16(14):1707-12, 2010 3- Cheung MT, Li WH, Kwok PC, Hong JK: Cheung MT, Li WH, Kwok PC, Hong JK. J Hepatobiliary Pancreat Sci. 17(3):338-44, 2010 4- Gardner TB, Coelho-Prabhu N, Gordon SR, Gelrud A, Maple JT, Papachristou GI, Freeman ML, Topazian MD, Attam R, Mackenzie TA, Baron TH: Direct endoscopic necrosectomy for the treatment of walled-off pancreatic necrosis: results from a multicenter U.S. series.Gastrointest Endosc. 73(4):718-26, 2011 5- Brun A, Agarwal N, Pitchumoni CS: Fluid collections in and around the pancreas in acute pancreatitis. J Clin Gastroenterol. 45(7):614-25, 2011 6- Gluck M, Ross A, Irani S, Lin O, Hauptmann E, Siegal J, Fotoohi M, Crane R, Robinson D, Kozarek RA: Endoscopic and percutaneous drainage of symptomatic walled-off pancreatic necrosis reduces hospital stay and radiographic resources. Clin Gastroenterol Hepatol. 8(12):1083-8, 2010

Pyogenic Granuloma
Pyogenic granuloma, also known as lobular capillary hemangioma, is a common benign acquired vascular lesion of the skin and mucous membranes in the pediatric age group. Mean age of
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presentation is six years with most cases being males. Mean lesional size is 6.5 cm. Pyogenic granulomas are rapidly developing vascular nodules characterized by an erythematous, domeshaped papule that bleeds easily most commonly located in the head and neck area, followed by trunk, upper extremity and lower extremity. The etiology of pyogenic granuloma is unknown, but proposed agents include trauma, infection, and preceding dermatoses. The preponderance of pyogenic granulomas occurs in the skin (80%), with the remaining ones in the oral cavity and conjunctiva. Most lesions are effectively managed with full-thickness skin excision and linear closure. Another alternative is shave excision followed by laser photocoagulation or cryotherapy. Topical Imiquimod 5% cream has also been used. Shaving and cream therapy has a higher recurrence rate.
References: 1- Patrice SJ, Wiss K, Mulliken JB: Pyogenic granuloma (lobular capillary hemangioma): a clinicopathologic study of 178 cases. Pediatr Dermatol. 8(4):267-76, 1991 2- Kirschner RE, Low DW: Treatment of pyogenic granuloma by shave excision and laser photocoagulation. Plast Reconstr Surg. 104(5):1346-9, 1999 3- Pagliai KA, Cohen BA: Pyogenic granuloma in children. Pediatr Dermatol. 21(1):10-3, 2004 4- Lin RL, Janniger CK: Pyogenic granuloma. Cutis. 74(4):229-33, 2004 5- Giblin AV, Clover AJ, Athanassopoulos A, Budny PG: Pyogenic granuloma - the quest for optimum treatment: audit of treatment of 408 cases. J Plast Reconstr Aesthet Surg.60(9):1030-5, 2007. 6- Tritton SM, Smith S, Wong LC, Zagarella S, Fischer G: Pyogenic granuloma in ten children treated with topical imiquimod.Pediatr Dermatol. 26(3):269-72, 2009

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Single Site Laparoscopic Surgery
As technological surgical advances continue to occur in the field of laparoscopic surgery in search of the scarless operation, single site umbilical laparoscopic procedures have emerged. A single multi-port with several holes is introduced through the navel, instead of several small incisions. The working space in children is small and space is needed for instrument triangulation and retraction. Nevertheless, single site umbilical laparoscopic surgery is performed in children for such procedures as cholecystectomy, appendectomy, intussusception reduction, splenectomy, nephrectomy, inguinal hernia repair, fundoplication, gastrostomy, orchiopexy and pyloromyotomy. Due to lack of triangulation roticulating and curved instruments have been developed for single port procedures. Most roticulating instruments are disposable and need a learning curve for safe use. Alternatives to roticulating instruments are rigid bent instruments which can be reused and are low cost. The shaft of the telescope comes longer to avoid clashing with the surgeons hand. Retraction can be achieved with the used of magnets. This is achieved with magnetic intracorporeal graspers and an extracorporeal magnet that is manipulated over the abdominal wall to adjust and control the instruments. Laparoscopic surgery in children is moving toward achieving this goal of scarless surgery.
References: 1- Woldrich JM, Holmes N, Palazzi-Churas K, Alagiri M, DeCambre M, Kaplan G, Chiang G: Comparison of laparoendoscopic single-site, conventional laparoscopic, and open nephrectomy in a pediatric population. Urology. 78(1):74-7, 2011 2- Garey CL, Laituri CA, Ostlie DJ, Snyder CL, Andrews WS, Holcomb GW 3rd, St Peter SD: Single-incision laparoscopic surgery in children: initial single-center experience. J Pediatr Surg. 46(5):904-7, 2011 3- Kawauchi A, Naitoh Y, Miki T: Laparoendoscopic single-site surgery for pediatric patients in urology. Curr Opin Urol.
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21(4):303-8, 2011 4- Ostlie DJ: Single-site umbilical laparoscopic appendectomy. Semin Pediatr Surg. 20(4):196-200, 2011 5- Padilla BE, Dominguez G, Millan C, Martinez-Ferro M: The use of magnets with single-site umbilical laparoscopic surgery. Semin Pediatr Surg. 20(4):224-31, 2011 6- Krpata DM, Ponsky TA: Instrumentation and equipment for single-site umbilical laparoscopic surgery. Semin Pediatr Surg 20(4): 190-195, 2011

Acquired Tracheoesophageal Fistula

Acquired tracheoesophageal fistula (TEF) is a rare clinical disease encountered in the pediatric age. Most cases are the result of a benign condition. Complications resulting from such conditions as prolonged mechanical ventilation, indwelling tracheal or esophageal stents, granulomatous mediastinal infection, trauma, iatrogenic injuries, ingested foreign body and caustic ingestion are the common cause for development of an acquired TEF. The most common cause of acquired TEF in children is foreign body impaction. Onset of symptoms can be immediate with respiratory difficulty, subcutaneous air, pneumothorax or mediastinum. It can be chronic with coughing, choking, respiratory infections or dysphagia. Increased tracheal secretions, air leak around the tube cuff, suctioning of gastric contents through the tracheotomy, abdominal distention, and air leakage from a nasogastric or gastrostomy tube are among the signs that should suggest the potential diagnosis of TEF. Diagnostic evaluation should include chest film, water soluble esophagogram, bronchoscopy and esophagoscopy. Initial management should consist of stabilization and prevention of airway contamination by the fistula. This is followed by gastrostomy and jejunostomy to vent and feed the child respectively. Reducing inflammation, managing pulmonary infection and improving nutrition can hasten spontaneous closure in a 4-6 week period. Failure of conservative management requires surgical intervention. This includes cervical or thoracic closure of the TEF with interposition muscle if necessary. Other larger fistulas will require more complex procedures.
References: 1- Rahbar A, Farha SJ: Acquired tracheoesophageal fistula. J Pediatr Surg. 1978 Aug;13(4):375-6 2- Szold A, Udassin R, Seror D, Mogle P, Godfrey S: Acquired tracheoesophageal fistula in infancy and childhood. J Pediatr Surg. 26(6):672-5, 1991 3- Birman C, Beckenham E: Acquired tracheo-esophageal fistula in the pediatric population. Int J Pediatr Otorhinolaryngol. 10;44(2):109-13, 1998 4- Imamolu M, Cay A, Koucu P, Ahmetolu A, Sarihan H: Acquired tracheo-esophageal fistulas caused by button battery lodged in the esophagus. Pediatr Surg Int. 20(4):292-4., 2004 5- Grisel JJ, Richter GT, Casper KA, Thompson DM: Acquired tracheoesophageal fistula following disc-battery ingestion: can we watch and wait? Int J Pediatr Otorhinolaryngol. 72(5):699-706, 2008 6- Yalcin S, Ciftci AO, Karnak I, et al: Management of acquired tracheoesophageal fistula with various clinical presentations. J Pediatr Surg 46(10): 1887-1892, 2011

Prevention CVC Infections

Central venous catheter (CVC) are an indispensable tool in the management of acute and chronic diseases in children. The most common complication of use of CVC is bloodstream infection. Recent evidence-based studies and metaanalysis have found: 1) chlorhexidine skin prep and impregnated dressing can reduce CVC colonization and bloodstream infection. Chlorhexidine in neonates and premature infants can cause skin irritation and systemic absorption. 2) use of heparin and antibiotic impregnated CVC can decrease CVC colonization and bloodstream infection. 3) Ethanol and Vancomycin lock therapy can reduce the incidence of CVC infection. Vancomycinhome.coqui.net/titolugo/PSU37.html#33761 11/12

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heparin lock is associated with asymptomatic hypoglycemia. 4) Benefit of systemic prophylactic antibiotic at the time of CVC insertion is unclear.5) With regard to site of insertion, subclavian placement has a reduced incidence of CVC colonization.6) CVC infection in the setting of shortterm use should undergo catheter removal with systemic antibiotics for 7-14 days. 7) Patient in long-term CVC use who develop infection with S. Aureus, gram-negative bacilli, Candida, clinical deterioration or persistent relapsing bacteremia should undergo immediate catheter removal and defined course of systemic antibiotics, except in rare circumstances when no alternate venous access is available.
References: 1- O'grady NP, Alexander M, Dellinger EP, Gerberding JL, Heard SO, Maki DG, Masur H, McCormick RD, Mermel LA, Pearson ML, Raad II, Randolph A, Weinstein RA; Healthcare Infection Control Practices Advisory Committee: Guidelines for the prevention of intravascular catheter-related infections. Am J Infect Control. 30(8):476-89, 2002 2- Levy I, Katz J, Solter E, Samra Z, Vidne B, Birk E, Ashkenazi S, Dagan O: Chlorhexidine-impregnated dressing for prevention of colonization of central venous catheters in infants and children: a randomized controlled study. Pediatr Infect Dis J. 24(8):676-9, 2005 3- Gilbert RE, Harden M: Effectiveness of impregnated central venous catheters for catheter related blood stream infection: a systematic review. Curr Opin Infect Dis. 21(3):235-45, 2008 4- Mouw E, Chessman K, Lesher A, Tagge E: Use of an ethanol lock to prevent catheter-related infections in children with short bowel syndrome. J Pediatr Surg. 43(6):1025-9, 2008 5- O'Grady NP, Alexander M, Burns LA, Dellinger EP, Garland J, Heard SO, Lipsett PA, Masur H, Mermel LA, Pearson ML, Raad II, Randolph AG, Rupp ME, Saint S; Healthcare Infection Control Practices Advisory Committee (HICPAC): Guidelines for the prevention of intravascular catheter-related infections. Clin Infect Dis. 52(9):e162-93, 2011 and Summary of recommendations: Guidelines for the Prevention of Intravascular Catheter-related Infections. Clin Infect Dis. 52(9):108799, 2011 6- Huang EY, Chen C, Abdullah F, et al: Strategies for the prevention of central venous catheter infections: an American Pediatric Surgery Association Outcomes and Clinical Trials Committee systematic review. J Pediatr Surg 46(10): 2000-2011, 2011

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