Duodenum-sparing technique of head resection in solid pseudopapillary tumor of the pancreas in children.

AIM OF STUDY: Aim of the study was to assess the complications and long-term results in children operated on for solid pseudopapillary tumor of the pancreas (SPTP) between 1993-2008 at the authors' institution with a focus on a novel duodenum-sparing technique to treat tumors of the head of the pancreas. METHODS: Retrospective analysis was performed of patient data including demographics, diagnostic measures, the operative technique focusing on tumor of the head of the pancreas, complications and long-term results. RESULTS: There were 13 patients (12 girls and one boy) with an average age of 14 years (9-17.5 years) at operation. In 7 patients the tumor was localized in the head of the pancreas, in 4 patients in the tail, and in 2 patients both the body and tail were involved. Patients with body and tail involvement underwent distal pancreatic resection. In 6 patients with head involvement a duodenum-sparing resection of the head and end-to-end anastomosis of the excluded jejunal loop either to the corpus or tail of the pancreas were performed. One girl underwent a modified Whipple operation. She developed a biliary fistula which closed after three weeks with endoscopic stenting. One patient with head resection developed a biliary fistula which closed after two weeks of stenting. One patient who underwent resection of the pancreatic head complained of recurrent abdominal pain one year postoperatively. All patients are alive without tumor recurrence at 6 months to 16 years after operation. CONCLUSION: SPTP is a rare pancreatic tumor with a low degree of malignancy. No perioperative chemotherapy is necessary. Therefore duodenal resection in cases of SPTP in the head of the gland seems too invasive and mutilating. The authors consider the duodenum-sparing technique to be more appropriate for the developing organism of a child. Favorable short and long-term results support this opinion.

Surgical management of major pancreatic injury in children.

INTRODUCTION: Major or complicated pancreatic trauma in children is uncommon and management strategies remain controversial. The aim of this study was to evaluate our experience with both early and delayed surgery in these pediatric cases. METHODS: We carried out a retrospective analysis of data of pediatric patients with major or complicated pancreatic injury operated on between January 1994 and December 2005 in our pediatric trauma center. RESULTS: Thirteen children (9 boys and 4 girls) with a mean age of 8.5 years (range 3 - 16 years) were operated for major or complicated pancreatic injury. The extent of injury was: grade II (major contusion without duct injury or tissue loss) in 4 children; grade III (distal transection) in 5 children and grade IV injury (proximal transection) in four patients. Pseudocyst developed in 8 children: 4 with grade II injury, 2 with grade III injury and 2 with grade IV injury (one with abdominal pseudocyst and one with an abdominal and a mediastinal pseudocyst). Early diagnosis and operation was achieved in 5 cases, while delayed diagnosis and operation occurred in 8. Three children underwent cystogastrostomy; 6 had a spleen-sparing distal pancreatectomy and 4 had resection with Roux-en-Y jejunostomy drainage. Endoscopic retrograde cholangiopancreaticography (ERCP) was the most useful diagnostic tool in assessing ductal injury. There were no deaths or long-term morbidity in our group of patients. CONCLUSIONS: Our results support the view that early operation is important in ductal pancreatic injury. We recommend transferring children with a suspected ductal injury to a tertiary center with experience in both pediatric ERCP and pancreatic surgery.

Porcelain gallbladder with extrahepatic bile duct obstruction in a child.

An extrahepatic bile duct obstruction was diagnosed in a 13-year-old boy presenting with pruritus, abdominal pain and jaundice. Several weeks after sphincterotomy and biliary stenting via endoscopic retrograde cholangiopancreaticography which relieved the obstruction, the patient was operated on. Severe fibrosis encased the extrahepatic biliary tract, so only cholecystectomy was performed because planned hepaticojejunoanastomosis could jeopardize the vascular supply to the liver. Histopathology showed calcification of the gallbladder wall and chronic fibroproliferative changes in the surrounding tissue. The stricture of extrahepatic biliary duct resolved after 3 years of repeated replacement of stents. The stenting was thereafter terminated. In the following 3 years no dilation of intrahepatic bile ducts and no laboratory signs of cholestasis recurred and the now 19-year-old boy is doing well. Neither a case of porcelain gallbladder with extrahepatic bile duct obstruction in a child nor a successful treatment of the obstruction by long-term stenting has been described in the literature yet.

[Living relative donor for liver transplantation in paediatric recipient]. / Transplantace jater dítete od zivého príbuzenského dárce.

Paper refers the first in Czech Republic liver transplantation in a child with the donor being the close living relative - the father. Indication was the chronic liver failure caused by biliary atresia after the Kasai procedures, which enabled the child to survive two years. Liver segments II. -III were transplanted with favourable postoperative development. Within the following twenty months the nutrition status and the psychomotor development of the child significantly improved. Authors discuss present situation and results of the paediatric liver transplantation in the world.

[Trichobezoar-Rapunzel syndrome--case report]. / Trichobezoár-Rapunzelin syndrom--kazuistika.

Bezoars consist of swallowed foreign materials or indigestible organic matter and form a mass in gastrointestinal tract, usually in stomach. Trichobezoars are formed by swallowed hair, they are rare, and occur often in patients with psychiatric disorders. They may present with malabsorption, weight loss, abdominal pain, and signs of gastrointestinal obstruction or even perforation. Gastric trichobezoar with a tail reaching small intestine is called Rapunzel syndrom. Authors present a case of Rapunzel syndrome in a 13-year-old girl with only a short history of mild abdominal pain in who a gastric trichobezoar weighting 700g and measuring 24 x 16 x 10 cm was removed from laparotomy.

[Results of treatment of esophageal atresia]. / Soucasné výsledky lécby atrézie jícnu.

BACKGROUND: The aim of the study was to analyze morality, incidence of complications and influence of various prognostic factors on the outcome of patients with esophageal atresia. A retrospective analysis of the records of 109 consecutive cases of esophageal atresia and/or tracheoesophageal fistula operated in 1992-2001 was undertaken. Primary anastomosis was achieved in 68 (62.4%) patients, in 7 (6.4%) procedures aimed at esophageal substitution in future were performed and in 34 (31.2%) delayed anastomosis was primarily planned. METHODS AND RESULTS: Major associated anomalies were found in 55 (50.4%), cardiac anomalies in 19 (17.4%), and more than one associates anomaly in 16 (14.7%) patients. The most frequent surgical complication was anastomotic leak in 11.9%, septic complications were the most frequent and occurred in 66 (60.5%) patients. Gastroesophageal reflux was diagnosed in 30.3% and treated surgically in 14.7% of infants. The overall mortality was 20.2% (22 patients). The most frequent cause of death was sepsis and/or pneumonia. Long gap esophageal atresia was not found to be a significant factor in mortality. Factors significantly increasing mortality were birth weight below 2000g (OR 3.94), cardiac anomalies (OR 3.95), and mechanical ventilation before the operation (OR 2.97). CONCLUSIONS: Analysis of the outcome suggests that improved strategy in treating infants with multiple anomalies and more frequent attempts at primary anastomosis even in children in sub optimal general condition at diagnosis could decrease mortality.

Alimentary tract duplications in children: high incidence of associated anomalies.

BACKGROUND: Alimentary tract duplications (ATDs) are rare congenital anomalies affecting about 1 in 4500 newborns with a male/female ratio of 1.2 : 1. The presence of associated, sometimes complex congenital anomalies can pose a challenge to surgeons treating patients with ATDs. The aim of this work was to study the characteristics of ATDs and associated anomalies in our patients. METHODS: Retrospective study. Medical charts of 24 patients (7 males, 17 females) diagnosed with ATD during the years 1990 - 2001 at the Department of Paediatric Surgery, Motol Children's Hospital in Prague, Czech Republic were reviewed. RESULTS: Sixteen patients (67 %) were diagnosed at ages younger than 3 years. Eight cases were foregut duplications, 9 were midgut, and 7 hindgut anomalies. In 13 patients (54 %) associated malformations were documented. Eight patients (33 %) developed postoperative complications, 1 patient died. Complications were more frequent in the group of patients with associated anomalies (53 % vs. 9 %). CONCLUSIONS: Associated congenital malformations are frequently present in patients with ATDs. The complexity of the surgical repair of these anomalies may contribute to higher morbidity and mortality rates. A thorough examination to identify and characterise the presence of ATDs and associated congenital defects would provide appropriate diagnostic and surgical approaches for these patients.

[Current results in treatment of omphalocele and gastroschisis]. / Soucasné výsledky lécby omfalokély a gastroschízy.

OBJECTIVE: The aim of the study was to evaluate current possibilities of treatment of newborns with abdominal wall defects: omphalocele and gastroschisis. SETTING: Department of Pediatric Surgery, 2nd Medical Faculty Charles University and Faculty Hospital Motol, Prague. METHODS: A retrospective cohort study of the treatment results in newborns with defect of abdominal wall. RESULTS: Thirty eight newborns with omphalocele and gastroschisis were treated at the authors department in the period of 1995-2002. Primary closure of defect was possible in 23 newborns (60%), Goretex patch or "silo" technique was used in 13 patients (34%), while two newborns (6%) were not operated on. The overall survival in children with omphalocele was 73%, being 89% in children without chromosomal anomalies. Six infants died: three with trisomy 13, one with trisomy 18, one infant died of sepsis and one had primary pulmonary hypertension and congenital heart defect. The survival in children with gastroschisis was 94%, one child died of multiorgan failure during sepsis. CONCLUSION: Nowadays prenatal diagnosis of gastroschisis or omphalocele is a recommendation for complex examination and consultation in a specialized center. The prognosis for most isolated defects of abdominal wall is good as far as survival and quality of life are concerned.

[Treatment of the Hirschsprung's disease (HD) at the Department of Pediatric Surgery of Charles University, 2nd Faculty of Medicine, from 1979 to 2004]. / Lécba Hirschsprungovy choroby na Klinice detské chirurgie 2. LF UK a FN Motol v letech 1979-2004.

BACKGROUND: Survey of surgical techniques, strategy and results of the treatment of patients with Hirschsprung's disease (HD) at the Department of Pediatric Surgery of Charles University, 2nd Faculty of Medicine in Prague from 1979 to 2004 is presented. METHODS AND RESULTS: Paper summarises a twenty-five years long retrospective study of medical records of 274 patients who were operated on 1979-2004. 173 (67,3 %) pts had classical rectosigmoid (CRA) and/or long colonic aganglionosis (LCA). 19 (6,4 %) pts had total colonic aganlionosis with small bowel involvement (TCA). 72 (26,3 %) pts suffered from ultra-short rectal aganglionosis (URA). 74 pts with CRA and LCA operated on 1979-1991 underwent Kasai's colorectoplasty. 8 (10,8 %) of them had anastomotic leak, 7 (9,5 %) pts had anastomotic stricture, 2 (2,7 %) pts had postoperative enterocolitis (PEC) and 10 (13,5 %) had chronic constipation. In 6 pts an additional partial sphincteromyectomy of the internal anal sphincter SFME) had to be done. In period 1991-2004 93 pts underwent Swenson's procedure with (SFME). Anastomotic leak occurred in 3 (3,2 %) pts. Anastomotic stricture, PEC and/or chronic constipation were not registered. In 6 pts with anastomotic leak from both groups Soave re-do pull-through was done. In 2003-2004, 10 pts with CRA underwent a transanal resection of aganglionic segment with Swenson's colorectoplasty. One pt developed anal stricture. 3 of 19 pts with TCA operated on in 1979-1990 underwent classic Martin's long side to side ileo-recto-colic anastomosis. In 16 pts of 1991-2004 only short ileo-recto anastomosis according to Kasai and/or Swenson were done. In 93 pts suffering from URA a Lynn's SFME was performed. Three-stage procedure for the last time was used in 3 pts in 1984. Two- and one-stage procedures are still used. CONCLUSIONS: A number of postoperative complications decreased, a three-stage procedure was abandonned and the operation of HD is currently performed in newborn. In CRA a TAR replaced the laparatomy and in TCA the short ileo-rectal anastomosis is performed only.

[Transanal resection of the recto-sigmoid--the future in the treatment of classic Hirschsprung's disease?]. / Transanální resekee rektosigmoidea--budoucnost v lécení klasické Hirschsprungovy choroby?

INTRODUCTION: The technique of transanal resection (TAR) of aganglionary part in rectosigmoid, published in 1998, has gradually become a standard operation technique in the treatment of Hirschsprung's disease (H.d.). Since the TAR technique for H.d. is not yet generally widespread and has not been used in the Czech Republic yet, the authors intended to share their own experience and early results obtained by this surgical technique. COHORT OF PATIENTS: Seven patients (five boys and two girls) suffering from the classical recto-sigmoid form have been operated on since June 2003. One girl was affected with the Down's syndrome. The age of the patients at the time of operation was between one and 24 months. SURGICAL TECHNIQUE: The extent of intestinal resection was determined on the basis of per-operation biopsy. The length of the rectrosigmoid resection was between 25 and 35 cm. The colorectal anastomosis with partial sphincteromyectomy of internal anal sphincter was performed by the technique according to Swenson. The antibiotic prophylaxis was secured by three doses of cefoxitin and one dose of isepamycin. RESULTS: Intestinal passage reestablished 8-12 hours after the surgery, and complete oral intake began on day 3 or 4 after the operation. All patients healed up without complications, the period of hospitalization was seven days on the average. The final diagnosis of H.d. was confirmed by biopsy examination of the dissected portion in all cases. CONCLUSION: TAR is univocally the method of first choice in the surgery of classical Hirsprung's disease when compared with laparotomy or laparoscopy.

[Surgery of congenital defects--present trends]. / Chirurgie vrozených vad--soucasné trendy.

The objective of the present work is a review of contemporary therapeutic possibilities of neonates with serious developmental defects. Neonatal surgery of congenital developmental defects was in recent decades favourably influenced by the development of neonatology and prenatal diagnosis. Nowadays prenatally diagnosed congenital anomaly is not a primary indication for termination of pregnancy but for comprehensive examination and consultation in a specialized centre. The definite decision regarding continuation of pregnancy or its termination is up to fully informed parents. In the majority neonatal surgery is not surgery of urgent operations, adequately stabilized neonates are operated while ensuring comprehensive extrasurgical care. The prognosis as regards survival and quality of life is favourable for the majority of isolated congenital defects. During the period 1994-2001 at the authors' departments a 73% survival of neonates with an isolated diaphragmatic hernia was recorded, 87% with omphalocele, 93% with gastroschisis, 90% with oesophageal atresia, 97% with anorectal atresia and 100% with intestinal atresia. With declining mortality, in the foreground of interest are problems of early definitive correction of long-term results and quality of life. With regard to the low incidence of different congenital anomalies and the contemporary birth rate in the Czech Republic its is justified to concentrate selected defects in centres which are able to provide comprehensive prenatal and early postnatal treatment.

Idiopathic fibrosing pancreatitis presenting with obstructive jaundice in a child.

Idiopathic fibrosing pancreatitis (IFP) is a rare cause of obstructive jaundice in children. The obstruction is caused by circumferential compression of the intrapancreatic portion of the common bile duct (CBD). Various forms of biliary decompression, including surgical sphincteroplasty, choledochoduodenostomy, and Roux-en-Y choledochojejunostomy are employed as a treatment. We report a 14-year-old boy with IFP who was successfully treated by a temporary drainage of compressed CBD with the help of a stent placed by endoscopic retrograde cholangiopancreatography (ERCP). The diagnosis of IFP was confirmed histologically after open surgery. The patient remains well after 3.5 years of follow-up with no evidence of pancreatic insufficiency. This report offers an additional case of IFP causing obstructive jaundice treated by temporary internal drainage of CBD, which is safer and less invasive than biliodigestive surgical decompression.

Nonoperative therapy of the posttraumatic biliary fistula in adolescents.

Posttraumatic biliary fistula is one of the complications of major hepatic injury. Conventional treatment has consisted of surgical intervention with hepatic debridement, ductal repair, and controlled drainage. The authors present two boys, 15 and 17 years of age, with biliary fistulas resulting after major hepatic injury. Biliary fistulas were successfully treated nonoperatively with sphincterotomy and endoscopically inserted small-caliber stents. The duration of time between stent placement and closure of the fistulas were 2 and 8 days, respectively.2.5 years after injury, both patients are without problems. These cases demonstrate the effectiveness of nonoperative management of traumatic biliary fistulas in adolescents.

Endoscopic retrograde cholangiopancreatography in infants and children.

BACKGROUND: Although many reports describe the use of diagnostic endoscopic retrograde cholangiopancreatography (ERCP) in children, few mention therapeutic application of this technique in pediatric patients with pancreatic or biliary disease. METHODS: We report our 4-year experience of 80 ERCPs performed in 59 children for obstructive jaundice or cholestasis with dilatation of the biliary tree (32 children), biliary atresia (11), recurrent pancreatitis (8), and blunt trauma to the abdomen (8). RESULTS: The patients' ages ranged from 5 weeks to 18 years. The appropriate duct was cannulated in 94% of cases. Common bile duct sphincterotomy was performed in 35 patients and pancreatic duct sphincterotomy in one. Multiple procedures were done in 16 patients where biliary stents were inserted; in one patient with chronic pancreatitis and pancreaticolithiasis, pancreatic stent was inserted. Four patients developed mild pancreatitis, one had moderate pancreatitis and one had leak of contrast, which was treated by administration of clear fluids orally for one day. One patient with benign stenosis of the hepatic duct developed cholangitis after migration of the stent into the bowel lumen. CONCLUSION: Diagnostic and therapeutic ERCP can be done as safely and effectively in pediatric patients as in adults.

[Extracorporeal membrane oxygenation in the treatment of severe pulmonary hypertension in a neonate after surgery for laparoschisis]. / Mimotelní membránová oxygenace v lécbe tezké plicní hypertenze u novorozence po operaci laparoschízy.

The authors describe the case of newborn with laparoschisis in whom severe idiopathic pulmonary hypertension during postoperative period developed and initiation of extracorporeal membrane oxygenation (ECMO) to maintain circulatory stability and adequate oxygenation was necessary. ECMO was performed for 75 hours with maximum extracorporeal support up to 50% of cardiac output (Biomedicus pump BP 50, Jostra oxygenator M8). Patient was successfully weaned and switched to conventional ventilation and nitric oxide inhalation with consequent extubation. No bleeding complications were observed during ECMO in connection with surgical repair of laparoschisis.

[Esophageal reconstruction using the stomach in children]. / Náhrada jícnu zaludkem v detském veku.

The authors present an account of their experience with oesophageal replacement by gastric transposition in 10 children during the period between November 1992 and January 1999. Indications for oesophageal replacement was in seven children atresia of the oesophagus at the age of 9-36 months and in three children (2.5, 3 and 14 years) a long stricture of the oesophagus after caustic injury which did not respond to balloon dilatation. Replacement after caustic injury was made in children aged 2.5, 3 and 14 years. In eight patients the replacement was made without thoracotomy, by blunt resection of the mediastinum, in two patients after caustic injury thoracotomy had to be performed. In three patients after surgery a temporary fistula developed at the site of anastomosis which healed spontaneously. Eight patients are in a satisfactory condition after surgery, one patient five months after operation swallows with difficulty, his birth weight was 1500 g. One female patient died one year after oesophageal replacement, apparently due to aspiration and respiratory arrest. Gastric transposition is a considerate and relatively simple method in child age. The functional results are favourable.