VOLUME 35, 2010

PSU Volume 35 No 01 JULY 2010

Portal Vein Thrombosis

Extrahepatic non-cirrhotic portal vein thrombosis (PVT) is the second most frequent cause of portal hypertension in the world. In extrahepatic portal vein obstruction forward hepatopedal flow from the superior mesenteric vein and splenic vein is impeded due to obstruction of the portal vein which results in development of prehepatic portal hypertension and cavernomatous replacement of the portal vein. The causes of PVT can be split into four groups: direct injury to the portal vein and consequent thrombus formation as it occurs in omphalitis or umbilical catheterization; congenital malformation of the portal system associated with other cardiovascular disorders; indirect factors that predispose to thrombus formation in the portal system (sepsis, dehydration, hypercoagulability); and idiopathic. Children with PVT present with esophageal varices and bleeding, symptoms of secondary hypersplenism (splenomegaly) and growth retardation. Anomalies associated with PVT include heart and large vessel malformation and biliary (cholelithiasis). With time the child develops collateral circulation around the PVT along with coagulation disorders, ascites and biliary cirrhosis. Diagnosis of PVT is established with Doppler ultrasound and angio CT Scan. Variceal bleeding is managed with medication or endoscopic sclerotherapy. Persistent bleeding following endoscopic treatment, prominent splenomegaly with symptomatic hypersplenism, growth retardation, and symptomatic portal biliopathy may need surgical portosystemic shunt decompression the mesenteric-portal Rex shunt if the umbilical portion of the intrahepatic left PV and the superior mesenteric veins are patent. Otherwise, a splenorenal shunt is warranted.

1- Mack CL, Superina RA, Whitington PF: Surgical restoration of portal flow corrects procoagulant and anticoagulant deficiencies associated with extrahepatic portal vein thrombosis. J Pediatr. 2003 142(2):197-9, 2003
2- Gehrke I, John P, Blundell J, Pearson L, Williams A, de Ville de Goyet J: Meso-portal bypass in children with portal vein thrombosis: rapid increase of the intrahepatic portal venous flow after direct portal hepatic reperfusion. J Pediatr Surg. 38(8):1137-40, 2003
3- Chiu B, Superina R: Extrahepatic portal vein thrombosis is associated with an increased incidence of cholelithiasis. J Pediatr Surg. 39(7):1059-61, 2004
4- Schettino GCM, Fagundes EDT, Roquete MLV et al: Portal vein thrombosis in children and adolescents. Jornal de Pediatria. 82(3): 171-178, 2006
5- Maksoud-Filho JG, GonÃalves ME, Cardoso SR, Gibelli NE, Tannuri U: Long-term follow-up of children with extrahepatic portal vein obstruction: impact of an endoscopic sclerotherapy program on bleeding episodes, hepatic function, hypersplenism, and mortality. J Pediatr Surg. 44(10):1877-83, 2009
6- Sharif K, McKiernan P, de Ville de Goyet J: Mesoportal bypass for extrahepatic portal vein obstruction in children: close to  a cure for most! J Pediatr Surg. 45(1):272-6. 2010

Congenital Hyperinsulinism

Congenital hyperinsulinism (CH) is the most frequent cause of severe, persistent hypoglycemia in neonates and young infants leading to seizures, developmental delay, and permanent brain damage. The inappropriate oversecretion of insulin is responsible for profound hypoglycemia which require aggressive treatment to prevent severe and  irreversible brain damage. CH is a heterogeneous disorder with two histopathological lesions, diffuse and focal which are clinically indistinguishable. Focal CH is characterized by a sporadic somatic islet-cell hyperplasia. Diffuse CH corresponds to a functional abnormality of insulin secretion in the whole pancreas and involves several genes with different transmissions. Most cases are caused by mutations in genes coding for either of the two subunits of the beta-cell K(ATP) channel (ABCC8 and  KCNJ11). In the diffuse form the hyperinsulinism is due to a recessive mutation of both alleles of these genes. To differentiate the focal from the diffuse form 18 F DOPA PET and CT scan is needed. Initial management of CH consist of diazoxide therapy. Focal lesions are  effectively treated by limited pancreatic resection while diffuse lesions which are unresponsive to drug or dietary treatment require extensive laparoscopic or open pancreatectomy. Pancreatic beta-cell dysfunction persists following subtotal pancreatectomy of diffuse CH.

1- de Lonlay P, Touati G, Robert JJ, Saudubray JM: Persistent hyperinsulinaemic hypoglycaemia. Semin Neonatol. 7(1):95-100, 2002
2- Fournet JC, Junien C: The genetics of neonatal hyperinsulinism. Horm Res. 59 Suppl 1:30-4, 2003
3- Lindley KJ, Spitz L: Surgery of persistent hyperinsulinaemic hypoglycaemia. Semin Neonatol. 2003 Jun;8(3):259-65.
4- Bax KN, van der Zee DC: The laparoscopic approach toward hyperinsulinism in children. Semin Pediatr Surg. 16(4):245-51, 2007
5- Palladino AA, Bennett MJ, Stanley CA: Hyperinsulinism in infancy and childhood: when an insulin level is not always enough. Clin Chem. 54(2):256-63, 2008
6- Al-Shanafey S, Habib Z, AlNassar S: Laparoscopic pancreatectomy for persistent hyperinsulinemic hypoglycemia of infancy. J Pediatr Surg. 44(1):134-8, 2009

Dermatopathic Lymphadenopathy

Adenopathies are a source of concern for both physicians and patients during the pediatric age. Diagnosis can be suggested by fine needle aspiration cytology, but other times complete lymph node removal is warranted. Most adenopathies in children are benign in nature. Dermatopathic lymphadenopathy (DPL) is characterized by non-neoplastic lymph node enlargement with reactive process and is generally caused by chronic inflammatory skin disease. DPL is usually caused by viral, bacterial, or other specific infections (tattoo). The morphologic features helpful in the diagnosis of DPL on fine needle aspiration cytology are melanin-laden macrophages with variable pigment; large, histiocytic clusters with blood vessels at the center; characteristic histiocytes, with elongated vesicular nuclei, nuclear grooves, crumpled and convoluted nuclei and pseudonucleoli; and absence of or very few tingible body macrophages. DPL is a harmless cause of enlarged lymph nodes rarely difficult to differentiate from mycosis fungoides or lymphoma.

1- Hamazaki M, Taniguchi K, Murata H: Immunohistological study of reactive follicular hyperplasia of lymph node in childhood. Rinsho Byori. 40(4):423-8, 1992
2- Sudilovsky D, Cha I: Fine needle aspiration cytology of dermatopathic lymphadenitis. Acta Cytol. 42(6):1341-6, 1998
3- Iyer VK, Kapila K, Verma K: Fine needle aspiration cytology of dermatopathic lymphadenitis.
Acta Cytol. 42(6):1347-51, 1998
4- Zirkin HJ, Avinoach I, Edelwitz P: A tattoo and localized lymphadenopathy: a case report. Cutis. 67(6):471-2, 2001
5- Winter LK, Spiegel JH, King T: Dermatopathic lymphadenitis of the head and neck. J Cutan Pathol.34(2):195-7, 2007
6- Good DJ, Gascoyne RD: Atypical lymphoid hyperplasia mimicking lymphoma. Hematol Oncol Clin North Am. 23(4):729-45, 2009

PSU Volume 35 No 02 AUGUST 2010

Pancreatic Neuroendocrine Tumors

Neuroendocrine tumors of the upper gastrointestinal tract are mainly located in the pancreas, stomach or duodenum. Pancreatic neuroendocrine tumors (PNET) are rare tumors found in the pancreas that can be either functional or non-functional. PNET are usually sporadic, but may also be associated with genetic syndromes such as Von Hippel-Lindau and tuberous sclerosis. PNET are non-functional in their majority. Functional PNET can secrete insulin, gastrin, glucagon, somatostatin and vasoactive intestinal polypeptide. Non-functional PNET can secrete non-active substance such as neurotensin or chromogranin A. PNET are classified as well differentiate tumor and well differentiated or poorly differentiated carcinomas. Most are already metastatic by the time they are diagnosed with liver the most common site followed by regional lymph nodes. Surgery with curative intent is the mainstay of treatment for localized or loco-regional disease. PNET in children should preferably undergo an organ-sparing resection. Surgery as well as other forms of local treatment like transarterial chemoembolization or radiofrequency ablation can also improve prognosis in patients with liver metastases. For the inoperable cases, cytotoxic therapy with compounds like streptozotocin, 5-fluorouracil or doxorubicin can achieve modest outcome. Treatment with somatostatin analogues like octreotide has been proven to prolong progression-free survival in patients with metastatic neuroendocrine tumors of midgut origin.

1- Van Nieuwenhove Y, Vandaele S, Op de Beeck B, Delvaux G: Neuroendocrine tumors of the pancreas. Surg Endosc. 17(10):1658-62, 2003
2- Brüwer M, Pahlov-Nejad T, Herbst H, Senninger N, Schürmann G: Neuroendocrine tumors of the gastroenteropancreatic system--reevaluation using the Capella classification. Zentralbl Chir. 128(8):656-62, 2003
3- Langrehr JM, Bahra M, Kristiansen G, Neumann HP, Neumann LM, Plöckinger U, Lopez-Hänninen E: Neuroendocrine tumor of the pancreas and bilateral adrenal pheochromocytomas. A
rare manifestation of von Hippel-Lindau disease in childhood. J Pediatr Surg. 42(7):1291-4, 2007
4- Breysem L, Kersemans P, Vanbeckevoort D, Ectors N, Smet MH: Nonfunctioning neuroendocrine tumor of the pancreas in an 8-year-old girl. JBR-BTR. 90(6):528-31, 2007
5- Yu DC, Kozakewich HP, Perez-Atayde AR, Shamberger RC, Weldon CB: Childhood pancreatic tumors: a single institution experience. J Pediatr Surg. 44(12):2267-72, 2009
6- Dimou  AT, Syrigos KN, Saif MW: Neuroendocrine Tumors of the Pancreas: What's New.  JOP. J Pancreas (Online) 5; 11(2):135-138, 2010

ERCP in Children

Endoscopic retrograde cholangiopancreatography (ERCP) is a very important diagnostic and therapeutic tool for pancreaticobiliary disorders in children and adults. Biliary indications for ERCP include common bile duct stones, choledochal cyst, aberrant biliary anatomy, duct stricture, duct hypoplasia, biliary atresia, postoperative bile leaks and bile duct injury. ERCP can be therapeutic in extracting bile duct stones, performing papilla sphincterotomy, cystoduodenostomy, stricture dilatation and endoprosthesis placement. There is controversy whether ERCP should be used routinely in choledocholithiasis since spontaneous passage of common bile duct stones is common in children. Pancreatic indications for ERCP include recurrent or chronic pancreatitis, sclerosing cholangitis, pancreas divisum, pseudocysts, trauma and choledochocele. Therapeutic options for ERCP in pancreatic disorders include papillotomy and stent placement. General anesthesia will be needed to perform ERCP in young children, mostly between the ages of three and 12 years. The morbidity associated with ERCP is very low and consists of pancreatitis (most common), hemorrhage, duodenal perforation and cholangitis. Mortality is nil. ERCP is useful and safe in children, but previous use of diagnostic ultrasound and MRCP to detect morphologic changes should be encouraged to increase the therapeutic efficacy of ERCP.

1- Vrochides DV, Sorrells DL Jr, Kurkchubasche AG, Wesselhoeft CW Jr, Tracy TF Jr, Luks FI: Is there a role for routine preoperative endoscopic retrograde cholangiopancreatography for suspected choledocholithiasis in children? Arch Surg. 140(4):359-61, 2005
2- Cheng CL, Fogel EL, Sherman S, McHenry L, Watkins JL, Croffie JM, Gupta SK, Fitzgerald JF, Lazzell-Pannell L, Schmidt S, Lehman GA: Diagnostic and therapeutic endoscopic retrograde cholangiopancreatography in children: a large series report. J Pediatr Gastroenterol Nutr. 41(4):445-53, 2005
3- Issa H, Al-Haddad A, Al-Salem AH: Diagnostic and therapeutic ERCP in the pediatric age group.  Pediatr Surg Int. 23(2):111-6, 2007
4- 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
5-Shanmugam NP, Harrison PM, Devlin J, et al: Selective use of endoscopic retrograde cholangiopancreatography in the diagnosis of biliary atresia in infants younger than 100 days. J Pediatr Gastroenterol Nutr. 49(4):435-41, 2009
6- Paris C, Bejjani J, Beaunoyer M, Ouimet A: Endoscopic retrograde cholangiopancreatography is useful and safe in children.  J Pediatr Surg. 45(5):938-42, 2010

Gastrostomy Complications

Gastrostomy is a common and important procedure that have demonstrated to improve growth of children with developmental disabilities. A gastrostomy can be constructed open, percutaneously or laparoscopically. The morbidity associated with gastrostomy is significant. Most common reported complications in order of increase frequency include granulation tissue, tube leakage, accidental tube dislodgement, wound infection, blocked tube, bleeding, gastroesophageal reflux and colocutaneous fistula. Granulation tissue can be managed with topical silver nitrate application and Kenalog oral-base ointment. Tube mechanical problems can be managed with flushing, change of tube as soon as dislodged, avoiding pressure necrosis from tight fit, and performing daily tube rotation.  Wound infection if associated with cellulitis might need systemic antibiotics. Reflux is usually managed medically. Colocutaneous fistula needs emergency operation. The open procedure causes more complications that the percutaneous procedure. Laparoscopic gastrostomy in children avoids the serious complications caused by a blind puncture through the abdominal cavity when performing the percutaneous procedure. The frequency of minor ongoing problems necessitates ongoing support of the child and care of the gastrostomy.

1- Day AS, Beasley SW, Meads A, Abbott GD: Morbidity associated with gastrostomy placement in children demands an ongoing integrated approach to care. N Z Med J. 114(1129):164-7, 2001
2- Cameron BH, Blair GK, Murphy JJ 3rd, Fraser GC: Morbidity in neurologically impaired children after percutaneous endoscopic versus Stamm gastrostomy. Gastrointest Endosc. 42(1):41-4, 1995
3- Jones VS, La Hei ER, Shun A: Laparoscopic gastrostomy: the preferred method of gastrostomy in children. Pediatr Surg Int. 23(11):1085-9, 2007
4- Khattak IU, Kimber C, Kiely EM, Spitz L: Percutaneous endoscopic gastrostomy in paediatric practice: complications and outcome. J Pediatr Surg. 33(1):67-72, 1998
5- Zamakhshary M, Jamal M, Blair GK, Murphy JJ, Webber EM, Skarsgard ED: Laparoscopic vs percutaneous endoscopic gastrostomy tube insertion: a new pediatric gold standard? J Pediatr Surg. 40(5):859-62, 2005
6- Peters RT, Balduyck B, Nour S: Gastrostomy complications in infants and children: a comparative study. Pediatr Surg Int. 26(7):707-9, 2010

PSU Volume 35 No 03 September 2010

Frey Procedure

Chronic pancreatitis, a disease process more commonly found in adults than children, generally follows a progressive course of both pancreatic exocrine (steatorrhea) and endocrine (diabetes) insufficiency. Chronic pancreatitis in children is commonly unresponsive to medical therapy and may result in addiction to pain medication, dietary restrictions, absence from school, and restriction in the life of the child. Two procedures, namely Puestow (longitudinal pancreaticojejunostomy) and Duval (distal pancreatectomy with caudal pancreaticojejunostomy), has varying success depending mostly if the head of the pancreas is adequately decompressed or not. The Frey operation is indicated on patients with chronic pancreatitis who have "head dominant" disease. The Frey procedure consists of an anterior resection of the head of the pancreas preserving the duodenum along with a  longitudinal pancreaticojejunostomy in order to improve decompression of the head of the gland. Frey procedure has proved to provide symptomatic relief and improvement in quality of life in children and adults. Frey procedure is considered as the standard procedure in patients with pancreatic head complications and ductal dilatation associated with chronic pancreatitis. Postoperative complications after Frey procedure are usually septic in nature and likely to occur more often in patients in whom endoscopic pancreatic stenting has been performed before surgical intervention.

1- Frey CF, Child CG, Fry W: Pancreatectomy for chronic pancreatitis. Ann Surg. 184(4):403-13, 1976
2- Izbicki JR, Bloechle C, Knoefel WT, Kuechler T, Binmoeller KF, Broelsch CE: Duodenum-preserving resection of the head of the pancreas in chronic pancreatitis. A prospective, randomized trial. Ann Surg. 221(4):350-8, 1995
3- Amikura K, Arai K, Kobari M, Matsuno S: Surgery for chronic pancreatitis--extended pancreaticojejunostomy. Hepatogastroenterology. 44(18):1547-53, 1997
4- Chaudhary A, Negi SS, Masood S, Thombare M: Complications after Frey's procedure for chronic pancreatitis. Am J Surg. 188(3):277-81, 2004
5- Rollins MD, Meyers RL: Frey procedure for surgical management of chronic pancreatitis in children. 39(6): 817-820, 2004
6- Falconi M, Bassi C, Casetti L, Mantovani W, Mascetta G, Sartori N, Frulloni L, Pederzoli P: Long-term results of Frey's procedure for chronic pancreatitis: a longitudinal prospective study on 40 patients. J Gastrointest Surg. 10(4):504-10, 2006
7- Chiang KC, Yeh CN, Hsu JT, Chen HM, Chen HY, Hwang TL, Jan YY, Chen MF: Pancreaticoduodenectomy versus Frey's procedure for chronic pancreatitis: preliminary data on outcome and pancreatic function. Surg Today. 37(11):961-6, 2007
8- Andersen DK, Frey CF: The evolution of the surgical treatment of chronic pancreatitis. Ann Surg. 251(1):18-32, 2010

MRCP in Pancreatic Trauma

Magnetic resonance cholangiopancreatography (MRCP) is a diagnostic method that uses three-dimensional data sets for projection images, as well as arbitrary cross-sectional images, of the pancreatic and biliary ducts. MRCP is simple, comfortable, and requires no contrast media or radiation. Secretin administration improves ductal visualization, particularly of nondilated ducts. MRCP may be the diagnostic method of choice when ERCP is contraindicated or fails. Pancreatic injury has a high morbidity and mortality. The integrity of the main pancreatic duct is the most important determinant of  prognosis. In the setting of blunt abdominal pancreatic trauma MRCP has a role in assessing pancreatic ductal integrity along with specific complications such as pseudocyst and posttraumatic strictures. Suspicion of ductal injury in MRCP determines the need for subsequent endoscopic retrograde cholangiopancreatogram (ERCP). In this era of conservative management of pancreatic injury in children, ERCP is considered a golden standard for identifying ductal injury offering the possibility of placing a ductal stent as primary treatment. Transpapillary drainage is especially effective in patients who have partial pancreatic duct disruption that can be bridged and is  used to treat post-traumatic pancreatic pseudocysts.

1- Takehara Y: MR pancreatography: technique and applications. Top Magn Reson Imaging. 8(5):290-301, 1996
2- Fulcher AS, Turner MA, Yelon JA, McClain LC, Broderick T, Ivatury RR, Sugerman HJ: Magnetic resonance cholangiopancreatography (MRCP) in the assessment of pancreatic duct trauma and its sequelae: preliminary findings. J Trauma. 48(6):1001-7, 2000
3- Ragozzino A, Manfredi R, Scaglione M, De Ritis R, Romano S, Rotondo A: The use of MRCP in the detection of pancreatic injuries after blunt trauma. Emerg Radiol.10(1):14-8, 2003
4- Houben CH, Ade-Ajayi N, Patel S, Kane P, Karani J, Devlin J, Harrison P, Davenport M: Traumatic pancreatic duct injury in children: minimally invasive approach to management. J Pediatr Surg. 42(4):629-35, 2007
5- de Blaauw I, Winkelhorst JT, Rieu PN, van der Staak FH, Wijnen MH, Severijnen RS, van Vugt AB, Wijnen RM: Pancreatic injury in children: good outcome of nonoperative treatment. J Pediatr Surg. 43(9):1640-3, 2008
6- Bhasin DK, Rana SS, Rawal P: Endoscopic retrograde pancreatography in pancreatic trauma: need to break the mental barrier. J Gastroenterol Hepatol. 24(5):720-8, 2009
7- Rekhi S, Anderson SW, Rhea JT, Soto JA: Imaging of blunt pancreatic trauma. Emerg Radiol. 17(1):13-9, 2010

Pulmonary Blastoma

Pulmonary blastoma (PB) is a very rare type of embryonal malignant lung tumor believe to arise from the primitive interstitial mesenchyme of the lung. Other names coined to this tumor include pulmonary sarcoma, embryonal sarcoma, pulmonary rhabdomyosarcoma, embryonal rhabdomyosarcoma, and malignant mesenchymoma. Most cases present before the age of six years. Three pathologic types are recognized based on their morphologic appearance: Type I is a cystic lesion that is not distinguishable from other cystic lesions of the lungs. Type II is a cystic and solid mass, which may be evident radiologically; and Type III is a solid high-grade sarcoma. It is believed pulmonary blastomas can arise from a preexisting  cystic lung disease such as congenital adenomatoid malformation, pulmonary sequestration, bronchogenic cysts and pneumatocele. CT scan is utilized for diagnosis. A new solid component in an old cystic lesion of the lung arise suspicious that we are dealing with PB and a mandatory resection is warranted.  Total tumor removal (lobectomy) offers the only chance of a good long-term outcome. Adjuvant chemotherapy is reserved for metastatic or residual disease. 

1- Kodaira Y, Akiyama H, Morikawa M, Shimizu K: Pulmonary blastoma in a child. J Pediatr Surg. 11(2):239-41, 1976
2- Seballos RM, Klein RL: Pulmonary blastoma in children: report of two cases and review of the literature. J Pediatr Surg. 29(12):1553-6, 1994
3- Tagge EP, Mulvihill D, Chandler JC, Richardson M, Uflacker R, Othersen HD: Childhood pleuropulmonary blastoma: caution against nonoperative management of congenital lung cysts.  J Pediatr Surg. 31(1):187-9, 1996
4- Lallier M, Bouchard S, Di Lorenzo M, Youssef S, Blanchard H, Lapierre JG, Vischoff D, Tucci M, Brochu P: Pleuropulmonary blastoma: a rare pathology with an even rarer presentation. J Pediatr Surg. 34(7):1057-9, 1999
5- Miniati DN, Chintagumpala M, Langston C, Dishop MK, Olutoye OO, Nuchtern JG, Cass DL: Prenatal presentation and outcome of children with pleuropulmonary blastoma. J Pediatr Surg. 41(1):66-71, 2006
6- Nasr A, Himidan S, Pastor AC, Taylor G,  Kim PC: Is congenital cystic adenomatoid malformation a premalignant lesion for pleuropulmonary blastoma? J Pediatr Surg 45:1086–1089, 2010

PSU Volume 35 No 04 October 2010


Congenital high airway obstruction syndrome (CHAOS) is a term coined in 1994 to development of complete or near-complete obstruction of the fetal airway. Though rare, CHAOS is not always incompatible with life. CHAOS is thought to occur when the upper airway fails to recanalize around the 10th week of gestation causing the obstruction. This obstruction blocks the normal egress of fluid from the lung raising the intratracheal pressure and leading to distension of the tracheobronchial tree and proliferative lung growth. The lung expands, the diaphragm flattens and the heart is compressed in the midline. The elevated intrathoracic pressure causes decreased venous return, fetal cardiac failure with ascites, placentomegaly and hydrops fetalis. Diagnosis is performed with prenatal ultrasound and MRI with findings such as dilated airways distal to the obstruction, large echogenic lungs, flattened diaphragm, fetal ascites and hydrops. Most common cause of CHAOS is laryngeal atresia, followed by subglottic stenosis, laryngeal/tracheal webs or agenesis. The prenatal natural history and postnatal course of CHAOS depends on whether the airway obstruction is complete. Survival depends on emergency tracheostomy immediately after birth with the use of the ex-utero intrapartum treatment (EXIT) procedure. The presence of hydrops is an ominous sign. The presence of a pinpoint laryngotracheal or tracheoesophageal fistula in some fetus with CHAOS will allow airway decompression with resolution of ascites and diaphragmatic eversion.   

1- Hedrick MH, Ferro MM, Filly RA, Flake AW, Harrison MR, Adzick NS: Congenital high airway obstruction syndrome (CHAOS): a potential for perinatal intervention. J Pediatr Surg. 29(2):271-4, 1994
2- DeCou JM, Jones DC, Jacobs HD, Touloukian RJ: Successful ex utero intrapartum treatment (EXIT) procedure for congenital high airway obstruction syndrome (CHAOS) owing to laryngeal atresia.  J Pediatr Surg. 33(10):1563-5, 1998
3- Lim FY, Crombleholme TM, Hedrick HL, Flake AW, Johnson MP, Howell LJ, Adzick NS: Congenital high airway obstruction syndrome: natural history and management. J Pediatr Surg. 38(6):940-5, 2003
4- Shimabukuro F, Sakumoto K, Masamoto H, Asato Y, Yoshida T, Shinhama A, Okubo E, Ishisoko A, Aoki Y: A case of congenital high airway obstruction syndrome managed by ex utero intrapartum treatment: case report and review of the literature. Am J Perinatol. 24(3):197-201, 2007
5- Vaikunth SS, Morris LM, Polzin W, Gottliebson W, Lim FY, Kline-Faith B,  Crombleholme TM: Congenital high airway obstruction syndrome due to complete tracheal agenesis: an accident of nature with clues for tracheal development and lessons in management. Fetal Diagn Ther. 26(2):93-7, 2009
6-Roybal JL, Liechty KW, Hedrick HL, Bebbington MW, Johnson MP, Coleman BG, Adzick  NS, Flake AW: Predicting the severity of congenital high airway obstruction syndrome. J Pediatr Surg 45(8): 1633-1639, 2010

Thoracic Neuroblastoma

Neuroblastoma is the most common malignant solid tumor in children with almost 20% of them arising in the thorax. Thoracic neuroblastomas have a better prognosis than those occurring in other parts of the body. Most cases present before the age of one year. Mediastinal neuroblastoma becomes symptomatic earlier and can be detected in an earlier favorable stage. The cell of origin is the dorsal root sympathetic ganglion cell. In what respect biology markers, thoracic neuroblastomas have favorable biological profiles such as DNA index greater than one, significant lower N-myc amplification, LDH level < 1500 and low ferritin levels. Clinically the child might demonstrate cough, dyspnea, wheezing, neurogenic cord compression, dancing eyes' syndrome and Horner syndrome. For diagnosis the chest films suggest the presence of a posterior mediastinal mass. CT scan is sensitive predicting chest wall involvement. MRI is very sensitive for predicting lymph node extension, intraspinal extension and chest wall involvement. Management consists of complete surgical excision with adjuvant chemotherapy.  Thoracoscopic resection of neurogenic tumors achieved similar local control and disease-free survival when compared with open resections. Results that were accompanied by shorter hospital stay and decreased blood loss.

1- Young DG: Thoracic neuroblastoma/ganglioneuroma.  J Pediatr Surg. 1983 Feb;18(1):37-41
2- Adams GA, Shochat SJ, Smith EI, Shuster JJ, Joshi VV, Altshuler G, Hayes FA, Nitschke R, McWilliams N, Castleberry RP: Thoracic neuroblastoma: a Pediatric Oncology Group study. J Pediatr Surg. 28(3):372-7, 1993
3- Morris JA, Shcochat SJ, Smith EI, Look AT, Brodeur GM, Cantor AB, Castleberry RP: Biological variables in thoracic neuroblastoma: a Pediatric Oncology Group study. J Pediatr Surg. 30(2):296-302, 1995
4- Petty JK, Bensard DD, Partrick DA, Hendrickson RJ, Albano EA, Karrer FM: Resection of neurogenic tumors in children: is thoracoscopy superior to thoracotomy? J Am Coll Surg. 203(5):699-703, 2006
5- Demir HA, Yalçin B, Büyükpamukçu N, Kale G, Varan A, Akyüz C, Kutluk T, Büyükpamukçu M: Thoracic neuroblastic tumors in childhood. Pediatr Blood Cancer. 54(7):885-9, 2010
6- Fraga JC, Aydogdu B, Aufieri R, Silva GV, Schopf L, Takamatu E, Brunetto A, Kiely E, Pierro A: Surgical treatment for pediatric mediastinal neurogenic tumors. Ann Thorac Surg. 90(2):413-8, 2010
7- Malek MM, Mollen KP, Kane TD, Shah SR, Irwin C: Thoracic neuroblastoma: a retrospective review of our institutional experience with comparison of the thoracoscopic and open approaches to resection. J Pediatr Surg. 45(8):1622-1626, 2010

Anal Achalasia

Internal anal sphincter achalasia (IASA) is a condition with similar clinical presentation to Hirschsprung's disease, but with ganglion cells present in the rectal biopsy. Theories on pathogenesis include nitregic nerve depletion, defective innervation of the neuromuscular junction and altered distribution of interticial cells of Cajal, considered the pacemakers of the bowel. Children with anal achalasia present with severe constipation with or without soiling. Diagnosis of IASA is made with anorectal manometry which shows the absence of the rectoinhibitory reflex upon rectal balloon inflation with presence of ganglion cells and normal acethycholinesterase activity in a rectal biopsy. Management consists of posterior internal sphincter myotomy. Intrasphincteric injection of Clostridium botulinum toxin has also been tried causing a local and six-month transient denervation of the sphincter. The majority of patients with internal anal sphincter achalasia can be treated successfully by internal sphincter myectomy.  In the long term, a significant number of patients have been found to suffer from soiling-related social problems.

1- De Caluwé D, Yoneda A, Akl U, Puri P: Internal anal sphincter achalasia: outcome after internal sphincter myectomy. J Pediatr Surg. 36(5):736-8, 2001
2- Messineo A, Codrich D, Monai M, Martellossi S, Ventura A: The treatment of internal anal sphincter achalasia with botulinum toxin. Pediatr Surg Int. 17(7):521-3, 2001
3- Heikkinen M, Lindahl H, Rintala RJ: Long-term outcome after internal sphincter myectomy for internal sphincter achalasia. Pediatr Surg Int. 21(2):84-7, 2005
4- Chumpitazi BP, Fishman SJ, Nurko S: Long-term clinical outcome after botulinum toxin injection in children with nonrelaxing internal anal sphincter.Am J Gastroenterol. 104(4):976-83, 2009
5- Doodnath R, Puri P: Long-term outcome of internal sphincter myectomy in patients with internal anal sphincter achalasia. Pediatr Surg Int. 25(10):869-71, 2009
6- Doodnath R, Puri P: Internal anal sphincter achalasia. Semin Pediatr Surg. 18(4):246-8, 2009

PSU Volume 35 No 05 November 2010

Neonatal Liver Hemorrhage

Hemorrhage from the liver in the neonatal period is a very serious and lethal injury. Neonatal liver bleeding can occur spontaneously during a surgical procedure, the result of birth trauma,  associated with sepsis, coagulopathy or  an acquired liver malformation. Breech delivery can be associated with spontaneous blunt hemorrhage into the liver, adrenals, kidney or spleen. Symptoms of abdominal distension, a contiguous mass effect in the right upper quadrant, anemia and shock appear 48 hours after the event. Blunt hepatic hemorrhage has also been reported within 24 hours of birth in babies carrying a congenital cavernous subcapsular liver hemangioma due to the tensile strength of this part of the liver. Performing surgery in very small premature babies with necrotizing enterocolitis can be associated with intraoperative spontaneous bleeding from the liver. In these cases severe liver hemorrhage could be stabilized by early liver tamponade using absorbable thrombostatic sponges and polyglactin mesh, argon beam coagulation, thrombin and fibrin glue application or topical hemostatic agents. Other times recombinant factor VIIa has been needed to manage such events.  Significant increased fluid requirements and persistent hypotension are ominous findings in the preoperative period and important predictors of the development of liver hemorrhage in necrotizing enterocolitis. Since bleeding occurs near the falciform ligament it is believe that it acts as a fulcrum  on which the expanding liver would tear.

1- Strear CM, Graf JL, Albanese T et al: Successful Treatment of Liver Hemorrhage in the Premature Infant. J Pediatr Surg 33(6): 849-851, 1998
2- Pumberger W, Kohlhauser C, Mayr M, Pomberger G: Severe liver hemorrhage during laparotomy in very low birthweight infants. Acta Paediatr. 91(11):1260-2, 2002
3- Metzelder ML, Springer A, August C, Willital GH: Neonatal hemoperitoneum caused by a congenital liver angioma. J Pediatr Surg. 39(2):234-6, 2004
4- Amodio J, Fefferman N, Rivera R, Pinkney L, Strubel N: Idiopathic intraparenchymal hematoma of the liver in a neonate. Pediatr Radiol. 34(4):358-61, 2004
5- Foss K: A case report of a low-birth-weight infant with a subcapsular liver hematoma and  spontaneous bowel perforation. Adv Neonatal Care. 4(2):67-78, 2004
6- Filan PM, Mills JF, Clarnette TD, Ekert H, Ekert P: Spontaneous liver hemorrhage during laparotomy for necrotizing enterocolitis: a potential role for recombinant factor VIIa. J Pediatr. 147(6):857-9, 2005
7- Raghavan M, Stansfield J: Spontaneous liver hemorrhage during laparotomy in a preterm infant. Paediatr Anaesth. 18(7):671-2, 2008

Solitary Rectal Ulcer Syndrome

Solitary rectal ulcer syndrome (SRUS) is a very rare cause of rectal bleeding in children. It is more commonly found in adults. Often goes unrecognized or misdiagnosed. In common with adults there is often a long duration of symptoms prior to diagnosis. The etiology of SRUS is multifactorial. Significant factors include ischemia with trauma due to dysfunctional defecation associated with anterior rectal prolapse. Some form of rectal prolapse is commonly found during videoproctography. Clinically the child manifests with bright rectal bleeding, constipation, mucous discharge, tenesmus, perineal and lower abdominal pain. Most cases are males. Endoscopic findings include ulcerated, polypoid, or plaque-like lesions characteristically found in the anterior rectal wall. Histology shows fibrous obliteration of the lamina propria with disorientation of smooth muscle fibers and hypertrophy of the muscularis mucosa with distorted crypts. The diagnosis is establish using endoscopic biopsy, proctography and anorectal manometry. Most children respond well to conservative therapy. Initial management of SRUS consist of bulk laxatives, high fiber diet,  biofeedback, behavior modification or Argon plasma coagulation. Surgical management is reserved for intractable cases and consist of local excisional procedures and/or rectopexy.

1-Godbole P, Botterill I, Newell SJ, Sagar PM, Stringer MD: Solitary rectal ulcer syndrome in children. JR Coll Surg Edinb 45(6): 411-414, 2000
2- Kiriştioğlu I, Balkan E, Kiliç N, Doğruyol H: Solitary rectal ulcer syndrome in children. Turk J Pediatr. 2000 Jan-Mar;42(1):56-60
3- Gabra HO, Roberts JP, Variend S, Shawis RN: Solitary rectal ulcer syndrome in children. A report of three cases. Eur J Pediatr Surg. 2005 Jun;15(3):213-6.
4- MartÃn de Carpi J, Vilar P, Varea V: Solitary rectal ulcer syndrome in childhood: a rare, benign, and probably misdiagnosed cause of rectal bleeding. Report of three cases. Dis Colon Rectum. 2007 Apr;50(4):534-9
5- Keshtgar AS: Solitary rectal ulcer syndrome in children. Eur J Gastroenterol Hepatol. 2008 Feb;20(2):89-92.
6-Somani SK, Ghosh A, Avasthi G, Goyal R, Gupta P: Healing of solitary rectal ulcers with multiple sessions of argon plasma coagulation. Dig Endosc. 2010 Apr;22(2):107-11

Granulomatous Appendicitis

Granulomatous inflammation of the appendix is a very rare form of appendicitis in children and adults. It is usually associated with a secondary systemic disease process such as Crohn's disease, foreign body reaction, tuberculosis, sarcoidosis, schistosomiasis, or Yersinia pseudotuberculosis. With absence of the above disorders it is termed primary or idiopathic granulomatous appendicitis. Patients' presented with pain in the right lower quadrant of the abdomen frequently associated with a mass and a protracted preoperative course. Most children undergo primary appendectomy. Prominent histologic features included epithelioid granulomas with lymphoid cuffing, central necrosis, transmural inflammation with lymphoid aggregates, mucosal ulceration, and cryptitis. Few children develop Crohn's later in life and postoperative fistulas occur very infrequently. Delayed or interval appendectomy specimens often have a characteristic inflammatory pattern that includes granulomas, xanthogranulomatous inflammation, mural fibrosis/thickening, and transmural chronic inflammation, changes that may be misinterpreted as Crohn disease. The prognosis of idiopathic granulomatous appendicitis is favorable.

1- Allen DC, Biggart JD: Granulomatous disease in the vermiform appendix. J Clin Pathol. 36(6):632-8, 1983
2- Timmcke AE: Granulomatous appendicitis: is it Crohn's disease? Report of a case and review of
the literature. Am J Gastroenterol. 81(4):283-7, 1986
3- Mazziotti MV, Marley EF, Winthrop AL, Fitzgerald PG, Walton M, Langer JC: Histopathologic analysis of interval appendectomy specimens: support for the role of interval appendectomy. J Pediatr Surg. 32(6):806-9, 1997
4- Lamps LW, Madhusudhan KT, Greenson JK, Pierce RH, Massoll NA, Chiles MC, Dean PJ, Scott MA: The role of Yersinia enterocolitica and Yersinia pseudotuberculosis in granulomatous appendicitis: a histologic and molecular study. Am J Surg Pathol. 25(4):508-15, 2001
5- Guo G, Greenson JK: Histopathology of interval (delayed) appendectomy specimens: strong association with granulomatous and xanthogranulomatous appendicitis. Am J Surg Pathol. 27(8):1147-51, 2003
6- Yayla D, Alpman BN, Dolek Y: Granulomatous appendicitis in a 12-year-old boy. J Pediatr Surg. 45(9):e27-9, 2010

PSU Volume 35 NO 06 December 2010

Pneumatosis Cystoides Intestinalis

Pneumatosis cystoides intestinalis (PCI) is a condition characterized by formation of multiple gas-filled cysts located within the wall (subserosa or submucosa) of the gastrointestinal tract. They pneumatosis can be located in any point of the GI tract. In neonates, PCI is associated with necrotizing enterocolitis. Infant and older children can develop PCI associated with immunosuppression (bone marrow transplant or chemotherapy), collagen vascular disease, aganglionosis, colonoscopy, steroid therapy, bacterial infection with Clostridium species, viral infection with rotavirus and cytomegalovirus, short bowel syndrome, bowel obstruction, and congenital heart disease. Following a mechanical (increase intraluminal pressure), bacterial (producing gas) and biochemical (high partial pressure of hydrogen) theory of origin of the pneumatosis, the exact mechanism is still unknown. Clinical presentation includes abdominal distension, bloody diarrhea, bilious vomits, lethargy and hypotension. Management consists of aggressive fluid therapy, frequent clinical and radiological examinations, systemic antibiotics, bowel rest and nutritional support. Worrisome CT findings include thickening of bowel wall, free fluid and periintestinal stranding. Surgery is indicated if the child develops progressive deterioration, free air, metabolic acidosis, severe hemorrhage, obstruction or signs of peritoneal irritation.    

1- West KW, Rescorla FJ, Grosfeld JL, Vane DW: Pneumatosis intestinalis in children beyond the neonatal period. J Pediatr Surg. 24(8):818-22, 1989
2- Reynolds HL Jr, Gauderer MW, Hrabovsky EE, Shurin SB: Pneumatosis cystoides intestinalis in children beyond the first year of life: manifestations and management. J Pediatr Surg. 26(12):1376-80, 1991
3- D'Agostino S, Fabbro MA, Musi L, Bozzola L: Pneumatosis cystoides intestinalis: a rare cause of nonsurgical pneumoperitoneum in an infant. J Pediatr Surg. 35(7):1106-8, 2000
4- Fenton LZ, Buonomo C: Benign pneumatosis in children. Pediatr Radiol. 30(11):786-93, 2000
5- Kurbegov AC, Sondheimer JM: Pneumatosis intestinalis in non-neonatal pediatric patients. Pediatrics. 108(2):402-6, 2001
6- Olson DE, Kim YW, Ying J, Donnelly LF: CT predictors for differentiating benign and clinically worrisome pneumatosis intestinalis in children beyond the neonatal period.Radiology. 253(2):513-9, 2009

Cells of Cajal

The interstitial cell of Cajal (ICC) are the smooth muscle pacemakers cells of spontaneous motility in the gut. The ICC network is widely distributed within the submucosal, intramuscular and intermuscular (between the circular and longitudinal muscles) layers of the gut wall. ICC serves as electrical pacemakers, provides pathways for the active propagation of slow waves, are mediators of enteric motor neurotransmission and play a role in afferent neural signaling. This motor neurotransmission occurs through specialized synapses that exist between enteric nerve terminals and ICC. Digestive motility  consists of non-propulsive mixing (segmental) and propulsive (peristalsis) movement. Electron microscopy and immunochemistry have demonstrated reduced number, density  and structural abnormalities in the ICC of gut motility conditions such as achalasia, gastroesophageal reflux, gastroparesis, infantile pyloric stenosis, segmental dilatation of the intestine, chronic intestinal pseudo-obstruction, Hirschsprung's disease, neuronal intestinal dysplasia, internal anal sphincter achalasia and slow transit constipation. Gastrointestinal stromal tumors originate from the ICC. In the gut musculature, ICC and mast cells are the only cells that have prominent c-kit expression. c-Kit is a transmembrane protein kinase which has as ligand stem cell factor and is involved in cell development in a variety of cell lineages.

1- Ward SM, Sanders KM: Involvement of intramuscular interstitial cells of Cajal in neuroeffector
transmission in the gastrointestinal tract. J Physiol. 576(Pt 3):675-82, 2006
2- Streutker CJ, Huizinga JD, Driman DK, Riddell RH: Interstitial cells of Cajal in health and disease. Part I: normal ICC structure and function with associated motility disorders. Histopathology. 50(2):176-89, 2007
3- Rolle U, Piaseczna-Piotrowska A, Puri P: Interstitial cells of Cajal in the normal gut and in intestinal motility disorders of childhood. Pediatr Surg Int. 23(12):1139-52, 2007
4- Negreanu LM, Assor P, Mateescu B, Cirstoiu C: Interstitial cells of Cajal in the gut--a gastroenterologist's point of view. World J Gastroenterol. 14(41):6285-8, 2008
5- Burns AJ, Roberts RR, Bornstein JC, Young HM: Development of the enteric nervous system and its role in intestinal motility during fetal and early postnatal stages. Semin Pediatr Surg. 18(4):196-205, 2009
6- Mostafa RM, Moustafa YM, Hamdy H: Interstitial cells of Cajal, the Maestro in health and disease. World J Gastroenterol. 14;16(26):3239-48, 2010
7- Okada T, Sasaki F, Honda S, Cho K, Matsuno Y, Itoh T, Kubota KC, Todo S: Disorders of interstitial cells of Cajal in a neonate with segmental dilatation of the intestine.  J Pediatr Surg. 45(6):e11-4, 2010

Postoperative Ileus

Ileus refers to any obstruction of the intestine. Postoperative ileus occurs after a major surgical procedure and is an important cause of postoperative discomfort and prolonged hospital stay. Operations that involve large incision, extensive bowel manipulation and peritoneal irritatives such as blood and pus more commonly results in postop ileus. Ileus is characterized by lack of coordinated  and reduced peristalsis. The patients complain of cramping, abdominal pain and nausea. No test confirms or excludes the diagnosis of ileus. Ileus that fail to resolved after the fifth postop day might be caused by an associated abscess, anastomotic leak, inflammation, intussusception or early adhesion. Ileus is resolving when the patient passes flatus or a bowel movement. Ileus resolves when the patient tolerates oral feeding without significant abdominal symptoms. After surgery the small bowel recovers first, the stomach next and the colon last within a period of 3-5 days. Sympathetic nerve activity due to peritoneal irritation reduces acetylcholine release and inhibits bowel motility.  Traditional treatment includes bowel rest and NG suction. Novel approach includes early enteral feeding, minimal to none NG suction, chewing gum (sham feeding), thoracic epidural catheter, ketorolac, intraoperative steroids and use of laparoscopy. 

1- Burd RS, Cartwright JA, Klein MD: Factors associated with the resolution of postoperative ileus in newborn infants. Int J Surg Investig. 2(6):499-502, 2001
2- Mattei P, Rombeau JL: Review of the pathophysiology and management of postoperative ileus.
World J Surg. 30(8):1382-91, 2006
3- Zhang Q, Zhao P: Influence of gum chewing on return of gastrointestinal function after gastric
abdominal surgery in children. Eur J Pediatr Surg. 18(1):44-6, 2008
4- Noble EJ, Harris R, Hosie KB, Thomas S, Lewis SJ: Gum chewing reduces postoperative ileus? A systematic review and meta-analysis. Int J Surg. 7(2):100-5, 2009
5- Cavuşoğlu YH, Azili MN, Karaman A, Aslan MK, Karaman I, Erdoğan D, Tütün O: Does gum chewing reduce postoperative ileus after intestinal resection in children? A prospective randomized controlled trial. Eur J Pediatr Surg. 19(3):171-3, 2009

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