VOLUME 43, 2014

PSU Volume 43 No 01 JULY 2014                      

Port CVC: Infections

Central venous catheters (CVC) related infections are the most common complication of harboring a central venous catheter with or without a port. This is followed by thrombosis of the catheter/port and mechanical problems. CVC using a port is extremely valuable for the long-term used of chemotherapy in children with malignancies. The most common causative organism of infected port CVC is coagulase negative staphylococcus. Factors that increase the risks of port CVC infections include younger age at the time of a malignant diagnosis, patient with leukemia and intensive chemotherapy with periods of severe neutropenia. It is globally recommended that the patient have an optimal neutropenic level at the time of port placement to decrease the infection risk. The number of port CVC removed due to infection is significantly greater the younger the patient. Other factors identified as increasing the rate of catheter removal due to blood stream infection include periods of hypotension, persistent bacteremia, stem cell transplantation, exit site infection, inappropriate empiric therapy and Candida infections. The use of prophylactic antibiotics is not recommended as an effective management to prevent catheter-associated blood stream infections in children. The use of chlorhexidine-impregnated dressings has shown to be a clinical management that decreases the incidence of bacterial colonization. In the setting of an infection the port CVC should be removed if the child has persistent or relapsing bacteremia in spite of adequate systemic antibiotic therapy, if the blood stream infection is caused by gram negative bacilli or Candida species, and if the patient clinical picture warrants removal.       

1- Elihu A, Gollin G: Complications of implanted central venous catheters in neutropenic children. Am Surg 73(10): 1079-1082, 2007
2- Adler A, Yanily I, Steinberg R, et al: Infectious complications of implantable ports and Hickman catheters in paediatric haematology-oncology patients. J of Hospital Infection 62(3): 356-358, 2006
3- Loh AH, Chui CH: Port-A-Cath insertions in acute leukemia: does thrombocytopenia affects morbidity? J Pediatr Surg 42(7): 1180-1184, 2007
4- Adler A, Yanily I, Solter E, et al: Catheter-associated bloodstream infections in pediatric hematology-oncology patients: factors associated with catheter removal and recurrence. J Pediatr Hematol Oncol 28(1):23-28, 2006
5- Dogar SA, Khan MA: Implantable port devices in paediatric oncology patients: a clinical experience from a tertiary care hospital. J Pak Med Assoc. 63(10): 1248-1251, 2013
6- Garland J, Alex C, Mueller C, et al: A randomized trial comparing povidone-iodine to a chlorhexidine gluconate-impregnated dressing for prevention of central venous catheter infections in neonates. Pediatrics. 107(6): 1431-1436, 2001

Autoimmune Hepatitis

Autoimmune hepatitis (AIH) is a progressive inflammatory disorder of the liver characterized by high level of transaminases, immunoglobulin G, presence of autoantibodies, histological evidence of interface hepatitis with portal plasma cell infiltration, and unknown etiology. Most affected children are females in their adolescent age. Two types of AIH are recognized. Type 1 is the most common and is characterized by positive antinuclear and/or anti-smooth muscle antibody presenting around puberty. Type 2 presents at a younger age and has positive anti-liver kidney microsomal antibodies and/or anti liver cytosol 1 antibodies. Type 2 presents with higher levels of bilirubin and transaminases, along with a more fulminant course of hepatic failure. Clinically children with AIH present with nonspecific symptoms of malaise, nausea/vomiting, anorexia, abdominal pain, followed by jaundice, dark urine and pale stools. Most children have clinical signs of chronic liver disease. Sclerosing cholangitis is often associated with AIH as an overlap syndrome suggesting both are part of the same pathogenic process. AIH responds to immunosuppression (steroids and azathioprine) and treatment should be initiated promptly to avoid progression of disease. Goals of treatment are to reduce or eliminate liver inflammation, to induce remission, improve symptoms, and prolong survival. With early treatment remission occurs in 80% of cases. Cirrhosis is found in 40-80% at diagnosis. Development of end-stage liver disease requiring liver transplant is 10% with most children remaining clinically stable with a good quality of life on long-term treatment. The role of surgery in AIH is provide histologic evidence of progression of disease by open or laparoscopic liver biopsy.

1- Mieli-Vergani G, Heller S, Jara P, Vergani D, et al: Autoimmune hepatitis. J Pediatr Gastroenterol Nutr. 49(2):158-64, 2009
2- Chai PF, Lee WS, Brown RM, McPartland JL, Foster K, McKiernan PJ, Kelly DA: Childhood autoimmune liver disease: indications and outcome of liver transplantation. J Pediatr Gastroenterol Nutr. 50(3):295-302, 2010
3- Mieli-Vergani G, Vergani D: Autoimmune liver diseases in children - what is different from adulthood? Best Pract Res Clin Gastroenterol. 25(6):783-95, 2011
4- Della Corte C, Sartorelli MR, Sindoni CD, Girolami E, Giovannelli L, Comparcola D, Nobili V: Autoimmune hepatitis in children: an overview of the disease focusing on current  therapies. Eur J Gastroenterol Hepatol. 24(7):739-46, 2012
5- Vajro P, Paolella G: Autoimmune hepatitis: current knowledge. Clin Res Hepatol Gastroenterol. 36(3):284-6, 2012
6- Vitfell-Pedersen J, Jargensen MH, Maller K, Heilmann C: Autoimmune hepatitis in children in Eastern Denmark. J Pediatr Gastroenterol Nutr. 55(4):376-9, 2012

Traumatic Bile Leaks

Traumatic bile leaks are a potential complication and sequelae of blunt hepatic injury whether managed operatively or non-operatively occurring exclusively in higher-grade injuries (> Grade III). A bile leak in the setting of trauma occurs after traumatic biliary tract laceration, parenchymal disruption, ductal ischemia, delayed rupture of subcapsular bile collections or persistent leak following hepatorraphy or hepatic resection during laparotomy. The bile leak leads to an intrahepatic biloma or intraperitoneal leakage of bile. The affected child develops symptoms such as nausea, increasing pain, abdominal distension, prolongation of the intestinal ileus and ascites after an initial period of clinical improvement from his hepatic trauma. The first study to confirm the diagnosis should be a HIDA scan due to its high sensitivity. Drainage of the biloma whether image-guided, laparoscopic or during laparotomy should be the first therapeutic intervention once the diagnosis of the biliary leak is made. Properly placed catheters can convert a leak into a controlled bile fistula. If the child continues to leak, an ERCP should be performed along with endoscopic sphincterotomy, nasobiliary drainage and/or stent placement across the papilla. The stent lowers the intrabiliary pressure by keeping the ampulla open. Biliary leaks can take more than six months to resolve. Strict follow-up is required for removal of the indwelling biliary stent as they can dislodge, block and cause pancreatitis.

1-Hollands MJ, Little JM: Post-traumatic bile fistulae. J Trauma. 31(1):117-20, 1991
2- Castagnetti M, Houben C, Patel S, Devlin J, Harrison P, Karani J, Heaton N, Davenport M: Minimally invasive management of bile leaks after blunt liver trauma in children. J Pediatr Surg. 41(9):1539-44, 2006
3- Durakbasa CU, Balik E, Yamaner S, et al: Diagnostic and therapeutic endoscopic retrograde cholangiopancreatography (ERCP) in children and adolescents: experience in a single institution. Eur J Pediatr Surg. 18(4):241-4, 2008
4- Sharma BC, Mishra SR, Kumar R, Sarin SK: Endoscopic management of bile leaks after blunt abdominal trauma. J Gastroenterol Hepatol. 24(5):757-61, 2009
5- Kulaylat AN, Stokes AL, Engbrecht BW, McIntyre JS, Rzucidlo SE, Cilley RE: Traumatic bile leaks from blunt liver injury in children: a multidisciplinary and minimally invasive approach to management.  J Pediatr Surg. 49(3):424-7, 2014

PSU Volume 43 NO 02 AUGUST 2014

Ileocecal Duplication Cyst

Congenital duplications of the gastrointestinal tract are rare and usually detected in the first two years of life or prenatally. The clinical presentation depends on location, size, and  presence of ectopic gastric or pancreatic mucosa. Intestinal duplications lead to volvulus, intussusception, bowel obstruction, bleeding or perforation. They can occur anywhere in the GI tract with the ileum, ileocecal and duodenum segment as the most common sites. Most alimentary tract duplications are cystic (80%–90%), with the remainder being tubular. The presence of gastric or pancreatic mucosa can lead to peptic ulceration, perforation, and hemorrhage. Most of the intestinal duplications share a common muscular wall and blood supply with the native bowel, especially those located in the abdomen, where they lie in the mesenteric border. The management of duplication cysts consists of resection of the segment harboring the duplication with anastomosis unless the duplications encompass a long segment of bowel. Ileocecal duplications are more frequently cystic lesions of varying size, share a common blood supply and wall with the ileum for a few centimeters from the valve and compress the cecum. These malformations manifest a clinical picture usually characterized by early presentation, occlusive feature and potentially deadly outcome. In many reports duplications in the ileocecal area have been managed with resection of the ileocecal valve. Removal of the ileocecal valve can lead to bacterial overgrowth, reduced intestinal transit time and impaired absorption with symptoms like diarrhea, malnutrition, and electrolyte imbalance. This has prompted management of ileocecal duplication cysts performing excision of the cyst together with the common wall of the ileum and restoring ileal wall continuity with transverse anterior enterorrhaphy preserving the functional ability of the ileocecal valve.

1- Tong SC, Pitman M, Anupindi SA: Best cases from the AFIP. Ileocecal enteric duplication cyst:
radiologic-pathologic correlation. Radiographics. 22(5):1217-22, 2002
2- Haratz-Rubinstein N, Sherer DM: Prenatal sonographic findings of congenital duplication of the cecum. Obstet Gynecol. 101(5 Pt 2):1085-7, 2003
3- Foley PT, Sithasanan N, McEwing R, Lipsett J, Ford WD, Furness M: Enteric duplications presenting as antenatally detected abdominal cysts: is delayed resection appropriate? J Pediatr Surg. 38(12):1810-3, 2003
4- Corroppolo M, Zampieri N, Erculiani E, Cecchetto M, Camoglio FS: Intussusception due to a cecal duplication cyst: a rare cause of acute abdomen. Case report. Pediatr Med Chir. 29(5):273-4, 2007
5- Kashiwagi Y, Suzuki S, Watanabe K, Nishimata S, Kawashima H, Takekuma K, Hoshika A:Sudden unexpected death associated with ileocecal duplication cyst and clinical review.  Clin Med Insights Pediatr. 4:25-8, 2010
6- Catalano P, Di Pace MR, Caruso AM, De Grazia E, Cimador M: Ileocecal duplications cysts: Is loss of the valve always necessary? J Pediatr Surg 49: 1049-1051, 2014

Fecal Microbiome Transplant

Fecal microbiome transplant (FMT) also known as stool transplantation is the infusion of human stool, obtained from a healthy donor with no risk factors for transmissible diseases into the gastrointestinal tract of a disease patient. FMT purpose is to normalize the gut flora of the recipient. The human GI microbiota is considered a tissue, not an organ, and is used in FMT to implant in a recipient's GI tract. Routes of instillation for FMT includes retention enemas (most common route), colonoscopy and nasojejunal tubes. Enema administration is effective, cheap, and safe and carries fewer procedural or institutional admission costs. FMT has been used effectively to manage patients with refractory/recurrent pseudomembranous enterocolitis and Clostridium difficile infection with a  success rate of 81% following a single infusion and 94% following a second infusion. No significant adverse events were noted other than mild infusion-related diarrhea and discomfort. FMT has also been used successfully in cases of inflammatory bowel disease (ulcerative colitis and Crohn disease) reducing symptoms, reversing disease and stopping medication, though its use is actually reserved for clinical trials. FMT in the management of inflammatory bowel diseases causes that 76% of subjects experience improvement in symptoms and 63% achieve clinical remission. Other conditions that could benefit from FMT include anorexia nervosa, constipation, diabetes mellitus, eosinophilic disorders of the GI tract, food allergies, irritable bowel syndrome, obesity, neurodegenerative and neurodevelopment disorders, and systemic autoimmunity disorders.  Treatment may consist of a single dose of FMT or multiple doses depending on the condition and response of the patient during treatment. Common treatment-related symptoms include bloating/flatulence, abdominal pain/cramping, diarrhea, blood in stool, and fatigue. The absence of consensus in regards to volume, route, donor screening, safety measures, and the potential lack of medical supervision in children is still being discussed and studied.

1- Kellermayer R: Prospects and challenges for intestinal microbiome therapy in pediatric gastrointestinal disorders. World J Gastrointest Pathophysiol. 4(4):91-93, 2013
2- Borody TJ, Paramsothy S, Agrawal G: Fecal microbiota transplantation: indications, methods, evidence, and future directions. Curr Gastroenterol Rep. 15(8):337, 2013
3- Kunde S, Pham A, Bonczyk S, Crumb T, Duba M, Conrad H Jr, Cloney D, Kugathasan S: Safety, tolerability, and clinical response after fecal transplantation in children and young adults with ulcerative colitis. J Pediatr Gastroenterol Nutr. 56(6):597-601, 2013
4- Allegretti JR, Hamilton MJ: Restoring the gut microbiome for the treatment of inflammatory bowel diseases. World J Gastroenterol. 20(13):3468-74, 2014
5- Samuel BP, Crumb TL, Duba MM: What Nurses Need to Know About Fecal Microbiota Transplantation: Education, Assessment, and Care for Children and Young Adults. J Pediatr Nurs. Feb 7, 2014
6- Borody TJ, Campbell J: Fecal microbiota transplantation: techniques, applications, and issues. Gastroenterol Clin North Am. 41(4):781-803, 2012

Subcostal Hernias

Subcostal hernia is a very rare entity presenting as an isolated defect or a complex multisystem defect, the exact etiology of which is still unknown. They more probably occur secondary to traumatic abdominal wall weakness or previous surgery, than congenital. They most commonly appear in the left subcostal region as a small protruding defect of 2 cm. Protrusion through the hernial content includes stomach and/or small/large bowel. There is risk of incarceration due to the small ring of defect. Associated conditions identified in the few case reports include left absent kidney, deformed thoracic cage, cardiac malformations, agenesis of the müllerian ducts & left ovary, and aplasia cutis congenita. The pathogenesis includes failure of the ectoderm to develop adequately in the area of the hernia that may lead to underlying embryologic defects as a result of disrupted inductive interactions between the abdominal wall layers, which occur during normal development The diagnosis can be confirmed with US, MRI or simple physical examination. The hernial defect has a sac. Management is straightforward and consists of surgical closure of the hernia defect using the surrounding rim of open external abdominal fascia. Recurrence is rare and the prognosis is good.

1- Nicksa GA, Christensen EP, Buchmiller TL: A case report of a congenital left subcostal hernia in a neonate. J Pediatr Surg. 44:1653–1655, 2009
2- Monteagudo J, Ruscher KA, Margolis E, Balazero F, Finck CM: Congenital subcostal hernia with unusual contents. J Pediatr Surg. 45:435–437, 2010
3- Sengar M, Manchanda V, Mohta A, Jain V, Das S:Intercostal variant of lumbar hernia in lumbocostovertebral syndrome: our experience with 6 cases. J Pediatr Surg. 2011 Oct;46(10):1974-7. 4- Lyngdoh TS, Mahalik S, Naredi B, Samujh R, Khanna S: Lumbocostovertebral syndrome with associated VACTERL anomaly. J Pediatr Surg. 45(9):e15-7, 2010
5- Raghu SR(1), Alladi A, Vepakomma D, Siddappa OS, Tilak P: Embryogenesis and types of subcostal hernia--a rare entity. J Pediatr Surg. 48(3):533-537, 2013

PSU Volume 43 NO 03 SEPTEMBER 2014

Near-Infrared Spectroscopy

Near-infrared spectroscopy (NIRS) is a noninvasive monitoring method to measure cerebral and somatic oxygenation during a surgical procedure. NIRS is based on the differential absorption of varying wavelengths of light by hemoglobin as it associates with oxygen. It provides a regional measurement of oxygen content in a localized tissue bed. The main purpose of NIRS is to evaluate regional tissue perfusion and oxygenation continuously. It has been used as standard of care during cardiac surgery to monitor the possible associated neurologic dysfunction from the hemodynamic changes occurring during the procedure. Cerebral hemoglobin oxygen saturation measured with NIRS (rSO2c) is utilized to monitor and titrate brain oxygen delivery preoperatively, during cardiopulmonary bypass, and postoperatively in the pediatric  intensive care unit. Somatic/renal NIRS (rSO2s) has also been proposed, as an estimate of somatic oxygen delivery and as a measure of optimized systemic perfusion, coupled with cerebral NIRS. A near-infrared spectrometer equipped with two independent emittent–sensor pairs is used for simultaneously measurement of rSO2c and rSO2s by cerebral and splanchnic sensors applied to the forehead and renal region, respectively. For example, during thoracoscopy pleural absorption of CO2 causes acidosis which in turn causes of vasoconstriction and reduced cerebral oxygenation signaled as such by the cerebral NIRS sensor placed in the head. Low intraoperative cerebral NIRS values are associated with worse neurological outcomes. Also, perioperative periods of diminished cerebral oxygen delivery, as indicated by rSO2c, are associated with one-year brain MRI abnormalities among infants undergoing reparative heart surgery. NIRS has recently been used in cases of non-cardiac neonatal surgery helping understand neonatal physiological changes occurring in these patients.

1- Berens RJ, Stuth EA, Robertson FA, Jaquiss RD, Hoffman GM, Troshynski TJ, Staudt SR, Cava JR, Tweddell JS, Bert Litwin S: Near infrared spectroscopy monitoring during pediatric aortic coarctation repair. Paediatr Anaesth. 16(7):777-81, 2006
2- Hirsch JC, Charpie JR, Ohye RG, et al. Near-infrared spectroscopy: what we know and what we need to know—a systematic review of the congenital heart disease literature. J Thorac Cardiovasc Surg 137:154–9, 2009
3- Durandy Y, Rubatti M, Couturier R: Near Infrared Spectroscopy during pediatric cardiac surgery: errors and pitfalls. Perfusion. 26(5):441-6, 2011
4- Holtby H, Skowno JJ, Kor DJ, Flick RP, Uezono S: New technologies in pediatric anesthesia. Paediatr Anaesth. 22(10):952-61, 2012
5- Abu-Sultaneh S, Hehir DA, Murkowski K, Ghanayem NS, Liedel J, Hoffmann RG, Cao Y, Mitchell ME, Jeromin A, Tweddell JS, Hoffman GM: Changes in cerebral oxygen saturation correlate with S100B in infants undergoing  cardiac surgery with cardiopulmonary bypass. Pediatr Crit Care Med. 15(3):219-28, 2014
6- Conforti A, Giliberti P, Mondi V, et al: Near infrared spectroscopy: Experience on esophageal atresia infants. J Pediatr Surg. 49: 1064-1068, 2014

Choledochal Cyst Type II

Choledochal cysts are dilatation of the extrahepatic and/or intrahepatic biliary tree causing myriad of complications in children. Choledochal cyst type II is the rarest of all types of choledochal cysts commonly described as diverticular malformation of the common bile duct with an otherwise normal intra- and extrahepatic biliary tree. Such diverticular cysts may be either small or very large occurring in less than 2% of all reported cases of choledochal cysts. The pathogenesis of this type of choledochal cyst remains speculative between an acquired and congenital process, with the common channel theory of insertion of the bile duct into the pancreatic duct allowing pancreatic enzymes to reflux into the common bile duct causing inflammation, weakness and eventual fibrosis, as the most plausible explanation. Others believe that the diverticulum is a remnant of an earlier stage of bile development or an end-stage healing of prenatal rupture of the common duct. Symptoms associated with this type of cyst includes abdominal pain, cholangitis, jaundice, fever and pancreatitis.  First diagnostic study should be an abdominal ultrasound. This can be followed by an MRCP to precisely delineate the rare biliary anatomy. The management of choledochal cysts is surgical excision whenever possible. Removal of the diverticulum is an accepted mode of treatment, leaving patent the native extrahepatic biliary tree. This procedure can be performed either open or laparoscopically with good results. If the cyst has a wide opening to the biliary tree or dilatation of the common bile duct is present (mixed variety) excision and bilio-enteric reconstruction is needed. Whatever technique the surgeon chooses, before removing the diverticular cyst, an intraoperative cholangiogram should be done to delineate precisely the surgical anatomy including demonstrating a normal appearing common bile duct with free flow of contrast into the duodenum and proximally into the intrahepatic ductal branches. Long-term follow up is needed to asses surgical outcome, notably complications such as stricture formation or the development of malignancy.

1- Liu DC, Rodriguez JA, Meric F, Geiger JL: Laparoscopic excision of a rare type II choledochal cyst: Case report and review of the literature. J Pediatr Surg. 35(7): 1117-1119, 2000
2- Chan ES, Auyang ED, Hungness ES: JSLS. 13: 436-440, 2009
3- Yamashita H, Otani T, Shioiri T, Takayama T, Kakiuchi C, Todani T, Makuuchi M: Smallest Todani's type II choledochal cyst. Dig Liver Dis. 35(7):498-502, 2003
4- Kaneyama K, Yamataka A, Kobayashi H, Lane GJ, Miyano T: Mixed type I and II choledochal cyst: a new clinical subtype? Pediatr Surg Int. 21(11):911-3, 2005
5- Agarwal N, Kumar S, Hai A, Parfitt R: Mixed type I and II choledochal cyst in an adult. Hepatobiliary Pancreat Dis Int. 8(4):434-6, 2009
6- Hwang SO, Lee TH, Bae SH, Han DJ, Lee HM, Park SH, Kim CH, Kim SJ: Diverticular Choledochal Cyst with a Large Impacted Stone Masquerading as Mirizzi's Syndrome. Case Rep Gastroenterol. 7(1):164-8, 2013

Sacral Nerve Stimulation

Sacral nerve stimulation (SNS), also known as sacral neuromodulation, is a novel well-establish therapy utilized for fecal incontinence in adults. In children, sacral nerve stimulation has proven to be effective in dysfunctional elimination syndrome, dysfunctional voiding, urinary retention, urgency, fecal soiling and recently constipation. SNS has also been found to be a valuable tool for intraoperative guidance in anorectal malformations allowing direct neuromuscular response to be identified helping surgeons operate closer to the individual anatomy. Electrical stimulation of sacral nerve roots has been shown to have a positive effect on peristalsis in the large bowel in patients with spinal cord injury. The procedure consists of placing programmable stimulators subcutaneously which delivers low amplitude stimulation via a lead to the sacral nerves usually assessed through the S3 foramina. Placement of the electrode using local anesthesia is preferred to ensure perfecto positioning based on sensory reaction to stimulation. The most common complaint after the procedure is pain from mechanical pressure or stimulation, and lead migration. SNS has been found recently to improve defecation in adolescent suffering from slow transit constipation refractory to intensive medical therapy. This needs to be confirmed in larger prospective studies with longer follow-up. Experience using SNS in smaller children is lacking.

1-van Wunnick BP, Peeters B, Govaert B, et al: Sacral Neuromodulation Therapy: A promising treatment for adolescent with refractory functional constipation. Dis Colon Rectum. 55: 278–285, 2012
2- Everaert K, Van den Hombergh U: Sacral nerve stimulation for pelvic floor and bladder dysfunction in adults and children. Neuromodulation. 8(3):186-7, 2005
3- Thompson JH, Sutherland SE, Siegel SW: Sacral neuromodulation: Therapy evolution. Indian J Urol. 26(3):379-84, 2010
4- Lansen-Koch SM, Govaert B, Oerlemans D, Melenhorst J, Vles H, Cornips E, Weil  EH, van Heurn E, Baeten CG, van Gemert WG: Sacral nerve modulation for defaecation and micturition disorders in patients with spina bifida. Colorectal Dis. 14(4):508-14, 2012
5- Dudding TC: Sacral nerve stimulation for constipation in children: a caution for a treatment  in development. Dis Colon Rectum. 55(3):237-8, 2012
6- Hasselbeck C, Reingruber B: Sacral nerve stimulation is a valuable diagnostic tool in the management of anorectal and pelvic malformations. J Pediatr Surg. 47(7):1466-71, 2012

PSU Volume 43 NO 04 OCTOBER 2014

Recurrent Adhesive Bowel Obstruction

Adhesive bowel obstruction (ABO) is a significant cause of morbidity following abdominal surgery in children. Readmission to hospital because of adhesions is estimated to affect up to 19% of children within four years of major abdominal surgery with almost 8% needing surgical intervention within the first two years after surgery. The rate of recurrence increases with each surgical procedure performed in the abdomen. The most common procedures observed in patients managed for ABO are appendectomy, colostomy creation/closure, Ladd's procedure, Nissen fundoplication and repair of Congenital diaphragmatic hernia in descending order of occurrence.  Diagnosis of adhesive bowel obstruction is made clinically (bilious vomiting, abdominal distension and obstipation) and radiographically with simple abdominal films or CT-Scan. Clinical predictors of intestinal ischemia include fever, tachycardia, leukocytosis, localized pinpoint tenderness and evidence of complete small bowel obstruction.  Initial conservative management of adhesive bowel obstruction consist of hydration, NPO and nasogastric decompression. When conservative management fails surgery will be needed to release the entrapped bowel. Recurrent episodes of ABO may occur in up to 42% of all patients. For such recurrent situations the surgeon should consider using long-tube (Jones or Thow) intraluminal stenting of the bowel. Stenting can be done through a jejunostomy or retrograde through the base of the appendix. The tube should remain at least for two weeks when new adhesions have formed. Plication (Noble) carries a high incidence of postop complications and has been abandoned. Seprafilm reduces the incidence of ABO in the pediatric patients undergoing laparotomy. Fibrin-sealed plication of the bowel has found to decrease the risk of recurring obstruction.

1- Eeson GA, Wales P, Murphy JJ: Adhesive small bowel obstruction in children: should we still operate? J Pediatr Surg. 45(5):969-74, 2010
2- Inoue M, Uchida K, Otake K, Nagano Y, Ide S, Hashimoto K, Matsushita K, Koike Y, Mohri Y, Kusunoki M: Efficacy of Seprafilm for preventing adhesive bowel obstruction and cost-benefit  analysis in pediatric patients undergoing laparotomy. J Pediatr Surg. 48(7):1528-34, 2013
3- Sprouse LR 2nd, Arnold CI, Thow GB, Burns RP: Twelve-year experience with the Thow long intestinal tube: a means of preventing  postoperative bowel obstruction. Am Surg. 67(4):357-60, 2001
4- Holland-Cunz S(1), Boelter AV, Waag KL: Protective fibrin-sealed plication of the small bowel in recurrent laparotomy.  Pediatr Surg Int. 19(7):540-3, 2003
5- Valkodai RR, Gurusami R, Duraisami V: Postoperative adhesive intestinal obstruction: The role of intestinal stenting. J Indian Assoc Pediatr Surg. 17(1):20-2, 2012
6- Fazel MZ, Jamieson RW, Watsn CJE: Long-term follow-up of the use of the Jones' intestinal tube in adhesive small bowel obstruction. Ann R Coll Surg Engl 91: 50-54, 2009

Accessory Hemidiaphragm

An accessory hemidiaphragm (AHD)  is a very rare thoracic anomaly in children in which the hemithorax is divided by a transverse fibromuscular septum separating the affected  hemithorax into two cavities. It most commonly occurs in the right side and the septum attaches anteriorly and medially to the central tendon of the thoracic diaphragm and pericardium. The septum has serosal lining, is fibrous, but it can contain striated muscle. Lung is trapped under the septum causing hypoplasia or agenesis with anomalous vascular connections. The anomaly is believed to occur due to the result of the lung bud growing into the posthepatic mesenchymal plate which normally forms the greatest part of the diaphragm. The child can develop respiratory, cardiovascular or be asymptomatic. Symptoms in the majority of cases are related either to inability of the trapped lung to expand or because of the previously described anomalous vessels. Though the diagnosis of AHD can be suspected in plain chest films, it will need a CT-Scan or MRI for final confirmation. It is important to describe well the associated cardiovascular anomalies. On bronchoscopy the bronchi to trapped lobes are narrow and they are crowded together toward the back of the affected hemithorax. Management of the symptomatic child with compromised lung function due to AHD consists of excision along with the entrapped lung tissue. This can be performed by open thoracotomy, thoracoscopy or through a subcostal laparotomy depending on the anatomy and extent of the lesion. Simultaneous or metachronous repair of an associated cardiovascular defect might be needed in some patients. Excision of the accessory diaphragm does not necessarily solve the problem of coexisting lung hypoplasia.

1*- Radhakrishnan J, Bean J, Piazza DJ, Chin AC: Accessory hemi diaphragm. J Pediatr Surg. 49(8):1326-1331, 2014
2- Wille L, Holthusen W, Willich E: Accessory diaphragm. Report of 6 cases and a review of the literature. Pediatr Radiol. 4(1):14-20, 1975
3- Becmeur F, Horta P, Donato L, Christmann D, Sauvage P: Accessory diaphragm--review of 31 cases in the literature. Eur J Pediatr Surg. 5(1):43-7, 1995
4- Kimura M, Asao M, Kawano Y, Inakazu T, Hamamoto K, Oda T: Scimitar syndrome with an accessory diaphragm and an absent right superior vena cava. Jpn J Surg. 16(4):284-7, 1986
5- Hart JC, Cohen IT, Ballantine TV, Varrano LF: Accessory diaphragm in an infant.  J Pediatr Surg. 16(6):947-9, 1981
6- Izeldin OM(1), Ahmed ME: Presentations of agenesis of the hemidiaphragm in an adult. Saudi Med J. 29(3):441-3, 2008
*Best review

Peritoneal Dialysis with VP Shunts

Peritoneal dialysis (PD) is the preferred method for end-stage renal disease children needing chronic dialysis support. Ventriculoperitoneal shunts (VPS) are routinely used to managed hydrocephalus especially in children with spina bifida. A small number of children with VPS will need chronic dialysis support using PD. These means two foreign bodies are within the abdominal cavity simultaneously creating fear of ascending (meningitis from peritonitis) or descending (meningitis to peritonitis) infections and VPS dysfunction. Studies have demonstrated a rate  of one peritonitis per 19 months use in children with simultaneous VPS and PD catheters with an absence of meningitis in cases with peritonitis. Regarding the possibility of a higher rate of VPS malfunction with the automated cycler dialysis in the supine position overnight and filling of  less than 1,000 ml/m2 for any day dwell, it seems very unlikely that the intraperitoneal pressure would ever persistently exceed the CSF pressure and inhibit VPS function. Besides the design of the VPS currently in use which allows CSF to flow in one way only, avoids reflux to the ventricles. There is a small inherent risk of chronic encapsulating peritonitis developing with loss of absorptive capacity. Concurrent use of a VPS and PD is a safe and acceptable option in the rare child requiring dialysis and a cerebral fluid shunt with no evidence to support an increased risk of peritonitis, ascending ventricular infections, or shunt dysfunction. Either the VPS and/or the PD catheter can be placed laparoscopically.

1-  Jachimiak B, Jarmoliaski T: [A child with myelomeningocele as a dialytic patient]. Przegl Lek. 3:176-9, 2006
2- Grunberg J, Verocay MC, Rebori A, Pouso J: Comparison of chronic peritoneal dialysis outcomes in children with and without spina bifida. Pediatr Nephrol. 22(4):573-7, 2007
3- Johnson BW, Pimpalwar A: Laparoscopic-assisted placement of ventriculo-peritoneal shunt tips in children with multiple previous open abdominal ventriculo-peritoneal shunt surgeries. Eur J Pediatr Surg. 19(2):79-82, 2009
4- Ram Prabahar M, Sivakumar M, Chandrasekaran V, Indhumathi E, Soundararajan P: Peritoneal dialysis in a patient with neurogenic bladder and chronic kidney disease with ventriculoperitoneal shunt. Blood Purif. 26(3):274-8, 2008
5- Grunberg J, Rebori A, Verocay MC: Peritoneal dialysis in children with spina bifida and ventriculoperitoneal shunt: one center's experience and review of the literature. Perit Dial Int. 23(5):481-6, 2003
6- Dolan NM, Borzych-Duzalka D, Suarez A, Principi I, Hernandez O, Al-Akash S, Alconchar L, Breen C, Fischbach M, Flynn J, Pape L, Piantanida JJ, Printza N, Wong W, Zaritsky J, Schaefer F, Warady BA, White CT: Ventriculoperitoneal shunts in children on peritoneal dialysis: a survey of the International Pediatric Peritoneal Dialysis Network. Pediatr Nephrol. 28(2):315-9, 2013

PSU Volume 43 No 05 NOVEMBER 2014                          

Fingertip Injury

Fingertip injuries are the most common injury to the hand needing evaluation in an emergency department in young children. Though often viewed as relatively minor injury improper management can lead to considerably loss of skilled hand function. Distal fingertip injuries in children are more common in boys, and right and left-hand injuries occur at near equal frequencies despite the majority of the population being right-handed. Crush injury predominates. Most of these injuries can be managed conservatively.  Others will need surgical management. The goals of treatment are to maintain the length of the digit, as well as to provide well-padded, stable yet sensate pain-free skin with long-term functionality. Simple hand x-ray film should be obtained in all cases to determine bone involvement. Possible treatment includes primary closure, healing by secondary intention, skin grafting, local tissue rearrangement, microvascular free tissue transfer or perforator flaps. The PNB classification takes into consideration whether damage included the pulp, nail and/or bone. This will help determine if conservative management is needed for distal pulp isolated injuries or if the nail is involved full-thickness skin grafting is warrant since they contract less, are more durable, and regain sensibility better than split thickness grafts. An excellent donor site for these skin grafts is the hypothenar eminence. In children the absolute size of the defect relative to the size of the finger must be taken into consideration. If it is felt that the defect is too large for secondary intention healing, a full-thickness skin graft is a viable management option. Injuries with fingertip pulp loss and exposed bone necessitate more complex treatment options including local tissue flaps such as simple V-Y, cross finger, homodigital or distal-based thenar flaps. With more severe proximal injury or amputation with viable adequate tissue, reimplantation is an option. Hand surgeons are expert in the management of such injuries.

1- Fitoussi F, Ghorbani A, Jehanno P, Frajman JM, Penneaot GF: Thenar flap for severe finger tip injuries in children. J Hand Surg Br. 29(2):108-12, 2004
2- Salazard B, Launay F, Desouches C, Samson P, Jouve JL, Magalon G: [Fingertip injuries in children: 81 cases with at least one year follow-up]. Rev Chir Orthop Reparatrice Appar Mot. 90(7):621-7, 2004
3- Muneuchi G, Tamai M, Igawa K, Kurokawa M, Igawa HH: The PNB classification for treatment of fingertip injuries: the boundary between  conservative treatment and surgical treatment. Ann Plast Surg. 54(6):604-9, 2005
4- Gellman H: Fingertip-nail bed injuries in children: current concepts and controversies of treatment. J Craniofac Surg. 20(4):1033-5, 2009
5- Al-Anazi AF: Fingertip injuries in paediatric patients - experiences at an emergency centre in
Saudi Arabia. J Pak Med Assoc. 63(6):675-9, 2013
6- Capstick R, Giele H: Interventions for treating fingertip entrapment injuries in children. Cochrane Database Syst Rev. 30;4:CD009808, 2014
*7- Barr JS, Chu MW, Thanik V, Sharma S: Pediatric thenar flaps: a modified design, case series and review of the literature. J Pediatr Surg. 49(9):1433-8, 2014

Unexpected Pathology after Appendectomy

Acute appendicitis is the most common surgical emergency in children, making appendectomy the most frequent worldwide operation performed in the abdomen. Though obstruction of the lumen of the appendix by lymphoid hyperplasia or fecalith is the dominant factor causing appendicitis, other unexpected pathology may be the cause of obstruction of the lumen in less than 2% of all cases. Histopathological evaluation of the removed appendix is essential to determine the etiology of such causes. The most common unusual factors associated with appendicitis include carcinoids tumors, granulomatous disease, enterobiasis, eosinophilia, teniasis, ascariasis, lymphomas other infectious etiologies, melanosis coli, serosal inflammation, and xanthomatous changes. Carcinoids are the most common tumors found in the appendix. They are asymptomatic, difficult to diagnose, and are incidentally found on histopathological examination. Fortunately the carcinoid typically does not develop carcinoid syndrome, recurrence, and metastasis. Appendectomy alone is the only treatment necessary in most cases. Enterobius vermicularis (pinworm) is the most common helminth infection worldwide and a rare cause of appendicitis. After being hatched in the stomach, larvas migrate to the cecum to become adult. It is here that they can occlude the lumen of the appendix causing obstructive and inflammatory symptoms. Adult female pinworms travel at night to the perianal area depositing eggs and causing anal pruritus. After pathological diagnosis the child should receive antihelminthic therapy. Granulomatous disease of the appendix is a very rare cause of appendicitis reported in cases of Crohn's disease, sarcoidosis, foreign body reaction, and infectious processes. Acute eosinophilic appendicitis is the result of an allergic response associated with eosinophilic enteritis, polypoid lesions, intussusception or gastrointestinal bleeding. Other unusual findings in appendicitis such as melanosis coli, infectious processes, xanthomatous changes, lipomatous hypertrophy, and serosal inflammation are rare entities seldom causing complications.

1- Akbulut S, Tas M, Sogutcu N, Arikanoglu Z, Basbug M, Ulku A, Semur H, Yagmur Y: Unusual histopathological findings in appendectomy specimens: a retrospective analysis and literature review. World J Gastroenterol. 17(15):1961-70, 2011
2- Emre A, Akbulut S, Bozdag Z, Yilmaz M, Kanlioz M, Emre R, Sahin N: Routine histopathologic examination of appendectomy specimens: retrospective analysis of 1255 patients.  Int Surg. 98(4):354-62, 2013
3- Yilmaz M, Akbulut S, Kutluturk K, Sahin N, Arabaci E, Ara C, Yilmaz S: Unusual histopathological findings in appendectomy specimens from patients with suspected acute appendicitis. World J Gastroenterol. 19(25):4015-22, 2013
4- Swank HA, Eshuis EJ, Ubbink DT, Bemelman WA: Is routine histopathological examination of appendectomy specimens useful? A systematic review of the literature. Colorectal Dis.13(11):1214-21, 2011
5- Arca MJ, Gates RL, Groner JI, Hammond S, Caniano DA: Clinical manifestations of appendiceal pinworms in children: an institutional experience and a review of the literature. Pediatr Surg Int. 20(5):372-5, 2004
*6- Alemayehu H, Snyder CL, St Peter SD, Ostlie DJ: Incidence and outcomes of unexpected pathology findings after appendectomy. J Pediatr Surg. 49(9):1390-3, 2014

Bowel Length

Assessment of small bowel length determination in children and adults is essential specially in cases of large intestinal resections to determine and manage nutritional problem arising from reduced absorptive capacity. Small bowel length, defined as distance between Treitz and ileocecal valve, correlates with gestational age, sex, weight, height and racial origin of the subjects. For example in  men the small intestine is 8% longer and the colon 14% longer than women. Intestinal length after loss of intestine is best expressed as a percentage of predicted for gestational age rather than the absolute length in centimeters. Between all the compounding variables affecting small bowel length, height has been found to correlate better by determining more exactly such length of the small and large intestine of humans. Height was chosen for practical reasons since the difference between observation and actual measurement is less compared to weight. A simple formula using height in centimeters has been developed for such purpose: Small bowel length (cm) = 6.741 - 80.409/height (cm). Similarly, Colon length (cm) = 0.111 * height (cm). Small bowel length (SBL) does not increase with growth like other anthropometric variables. The SBL/height ratio significantly decreases with growth; however, bowel diameter increases.

1- Gondolesi G, Ramisch D, Padin J, Almau H, Sandi M, Schelotto PB, Fernandez A, Rumbo C, Solar H: What is the normal small bowel length in humans? first donor-based cohort analysis. Am J Transplant. Suppl 4:S49-54, 2012
2- Siebert JR: Small-intestine length in infants and children. Am J Dis Child. 134(6):593-5, 1980
3- Hosseinpour M, Behdad A: Evaluation of small bowel measurement in alive patients. Surg Radiol Anat. 30(8):653-5, 2008
4- Hounnou G, Destrieux C, Desmé J, Bertrand P, Velut S: Anatomical study of the length of the human intestine. Surg Radiol Anat. 24(5):290-4, 2002
5- Touloukian RJ, Smith GJ: Normal intestinal length in preterm infants. J Pediatr Surg. 18(6):720-3, 1983
*6- Struijs MC, Diamond IR, de Silva N, Wales PW: Establishing norms for intestinal length in children. J Pediatr Surg. 44(5):933-8, 2009

PSU Volume 43 NO 06 DECEMBER 2014

Bladder Diverticulum

A bladder diverticulum (BD) is a rare pseudodiverticulum formed by mucosal herniation through an area of weakness in the muscular wall of the bladder typically in a paraureteral location. They are classified as congenital, acquired, iatrogenic or part of a syndrome. They result from congenital disarrangement of the detrusor muscle fibers at the ureterovesical junction or associated with increased intravesical pressure due to bladder-outlet obstruction. Bladder diverticulum can cause chronic urinary infection, urinary retention, pelvic mass or acute urethral obstruction by impinging upon the posterior urethra when they enlarged. BD in childhood almost exclusively occurs in the male. The most common clinical presentation of BD is acute pyelonephritis due to urine stasis within the diverticulum. A high prevalence of neurogenic bladder is present in patients with urinary BD. Long-term presence of a bladder diverticulum can lead to malignancy. VCUG is the most accurate imaging tool in the diagnosis of large bladder diverticula offering information regarding concomitant vesicoureteral reflux and bladder-neck and urethral morphology. Additional imaging methods of diagnosis include intravenous urography, urodynamics studies, nuclear renal scans, CT-Scan and MRI.  Diverticulectomy is the recommended treatment of choice for bladder diverticulum either symptomatic or not. Transvesical diverticulectomy is the recommended technique as it allows complete correction of any accompanying pathology by ureteral reimplantation or bladder-neck incision with minimal complications and no morbidity regarding long-term bladder function. Recurrence of the diverticula after appropriate surgery is usually associated with Ehlers-Danlos, Menkes or William syndrome or other collagen vascular disorders. Most patients recover and progress well after surgical management.

1- Bogdanos J, Paleodimos I, Korakianitis G, Stephanidis A, Androulakakis PA: The large bladder diverticulum in children. J Pediatr Urol. 1(4):267-72, 2005
2- Alexander RE, Kum JB, Idrees M: Bladder diverticulum: clinicopathologic spectrum in pediatric patients. Pediatr Dev Pathol. 15(4):281-5, 2012
3- Appeadu-Mensah W, Hesse AA, Yaw MB: Giant bladder diverticulum: a rare cause of bladder outlet obstruction in children. Afr J Paediatr Surg. 9(1):83-7, 2012
4- Khemakhem R, Ghorbel S, Jlidi S, Nouira F, Louati H, Douira W, Chennoufi F, Bellagha I, Chaouachi B: Management of congenital bladder diverticulum in children: A report of seven cases. Afr J Paediatr Surg. 10(2):160-3, 2013
5- Bhat A, Bothra R, Bhat MP, Chaudhary GR, Saran RK, Saxena G: Congenital bladder diverticulum presenting as bladder outlet obstruction in infants and children. J Pediatr Urol. 8(4):348-53, 2012
6- Oge O(1), Gemalmaz H, Ozeren B: Acute urinary retention in a child caused by a congenital bladder diverticulum. J Pediatr Surg. 37(6):926-7, 2002

Upper Lip Frenulum

Oral frenulum is a bandlike formation of congenital origin located in the midline composed of fibrous, muscular or fibromuscular tissue covered with a mucous membrane. Under normal conditions most oral frenulum do not have pathological consequences, otherwise they can present clinical problems of orthodontic, prosthetic, phonetic or periodontal nature. The upper lip frenulum is an oral mucosal membrane extending from the internal surface of the upper lip to its insertion on the midline of the attached interincisal gingival tissue of the upper maxilla. In children this type of frenulum can cause breastfeeding problems, interincisal diastema, denture related problems, periodontal disease secondary to retained or impacted food, oral hygiene difficulties and impairment of lip mobility and short lip which in turn require orthodontic treatment. The diagnosis is clinical. Management of upper lip frenulum causing this different problems is surgical. Frenulectomy should be performed in children under general anesthesia. Techniques of frenulectomy include simple or rhomboidal excision, V-Y plasty procedure and/or Z-plasty. Simple removal consists of sectioning or transverse sectioning the frenulum with simple suturing. They can leave a scar in the same direction of the frenulum bringing problems with upper lip lengthening and are contraindicated in cases with short lips exhibits an impaired lip seal. In this cases the best technique is Z-plasty. The procedure can be performed using laser being more precise and clean, shorter operative time, eliminating bleeding and not needing suturing since the wound is left opened to heal by secondary intention.   

1- Kondamudi NP, Ayush Gupta, Kaur R: Magnet balls stuck to the frenulum of the lip. J Emerg Med. 2014 Mar;46(3):345-7
2- Kotlow LA: Diagnosing and understanding the maxillary lip-tie (superior labial, the maxillary labial frenum) as it relates to breastfeeding. J Hum Lact. 2013 Nov;29(4):458-64
3- Pie-Sanchez J, Espana-Tost AJ, Arnabat-Dominguez J, Gay-Escoda C: Comparative study of upper lip frenectomy with the CO2 laser versus the Er, Cr:YSGG laser. Med Oral Patol Oral Cir Bucal. 2012 Mar 1;17(2):e228-32
4- Bagga S(1), Bhat KM, Bhat GS, Thomas BS: Esthetic management of the upper labial frenum: a novel frenectomy technique. Quintessence Int. 2006 Nov-Dec;37(10):819-23
5- Wiessinger D, Miller M: Breastfeeding difficulties as a result of tight lingual and labial frena: a case  report. J Hum Lact. 1995 Dec;11(4):313-6
6- Brabant P, Van der Veen JA:[Surgical treatment of the hypertrophic frenum of the upper lip]. Acta Stomatol Belg. 1988 Sep;85(3):179-83

Ceftriaxone Biliary Pseudolithiasis

Ceftriaxone is a semisynthetic third generation cephalosporin with a wide spectrum of powerful antimicrobial activity administered in one daily dose. If administered in high doses ceftriaxone can induce reversible gallbladder sludge, which can mimic true cholelithiasis clinically and sonographically. The term pseudolithiasis is coined to denote the reversible, benign character of this complication upon discontinuation of ceftriaxone therapy. Ceftriaxone associated pseudolithiasis is related to the high concentration and precipitation of this drug in the gallbladder as an insoluble calcium-ceftriaxone complex salt. Approximately 40% of ceftriaxone is excreted unmetabolized into the bile exceeding its solubility potential.  The other 60% is excreted in the urine. Pseudolithiasis develops in 25 to 50% of children receiving ceftriaxone. The time for pseudolithiasis to develop and resolve completely is unrelated to age, sex or type of infection. Fasting is unrelated to development of pseudolithiasis. A few cases might also develop nephrolithiasis as well. Pseudolithiasis is usually asymptomatic and diagnosed using ultrasound. Cholecystectomy, even if symptomatic, in cases of ceftriaxone induced pseudolithiasis is unnecessary. Observation and ultrasound follow-ups will help recognize the dissolution of the biliary lithiasis. When gallstone and/or sludge are detected in the gallbladder in children by ultrasonographic examination, the administration of ceftriaxone must be sought beyond other causative factors.

1- Alemayehu H, Desai AA, Thomas P, Sharp SW, St Peter SD: Ceftriaxone-induced pseudolithiasis in children treated for perforated appendicitis. Pediatr Surg Int. 30(3):323-6, 2014
2- von Martels JZ, Van de Meeberg EK, Holman M, Ligtenberg JJ, Ter Maaten JC: Pseudolithiasis after recent use of ceftriaxone: an unexpected diagnosis in a child with abdominal pain. Am J Emerg Med. 31(8):1294.e5-6, 2013
3- Meng D, Cao Y, Fu J, Chen R, Lu L, Tu Y: Sonographic assessment of ceftriaxone-associated biliary pseudolithiasis in Chinese children. J Int Med Res. 38(6):2004-10, 2010
4- Biner B, Oner N, Celtik C, BostancioÄŸlu M, Tunçbilek N, Güzel A, KarasalihoÄŸlu S: Ceftriaxone-associated biliary pseudolithiasis in children.  J Clin Ultrasound. 34(5):217-22, 2006
5- Ozturk A, Kaya M, Zeyrek D, Ozturk E, Kat N, Ziylan SZ: Ultrasonographic findings in ceftriaxone: associated biliary sludge and pseudolithiasis in children. Acta Radiol. 46(1):112-6, 2005
6- Ceran C, Oztoprak I, Cankorkmaz L, GumuÅŸ C, Yildiz T, Koyluoglu G: Ceftriaxone-associated biliary pseudolithiasis in paediatric surgical patients.  Int J Antimicrob Agents. 25(3):256-9, 2005

Journal Club