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.

Renal concentrating capacity is linked to blood pressure in children with autosomal dominant polycystic kidney disease.

Impaired glomerular filtration rate (GFR) is a risk factor for the development of hypertension in patients with autosomal dominant polycystic kidney disease (ADPKD). However, markers of tubular function were not tested whether they are linked to hypertension or blood pressure (BP) level. The aim of our study was to investigate the relationship between renal concentrating capacity and BP in children with ADPKD. Fifty-three children (mean age 11.8+/-4.4 years) were investigated. Standardized renal concentrating capacity test was performed after nasal drop application of desmopressin, BP was measured by ambulatory BP monitoring (ABPM). Renal concentrating capacity was decreased in 58 % of children. The prevalence of hypertension was significantly higher in children with decreased renal concentrating capacity (35 %) than in children with normal renal concentrating capacity (5 %) (p<0.05). Significant negative correlations were found between renal concentrating capacity, ambulatory BP and number of renal cysts (r = -0.29 to -0.39, p<0.05 to p<0.01). In conclusion, the concentrating capacity is decreased in about half of the patients and is linked to BP. Decreased renal concentrating capacity should be considered.

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.

Serum alpha-glutathione S-transferase as a sensitive marker of hepatocellular damage in patients with cystic fibrosis.

The aim of the study was to evaluate serum a-glutathione S-transferase (s-GSTA) levels in patients with cystic fibrosis (CF) and to compare s-GSTA with other liver function tests and with a hepatic ultrasound scan (US). The cytosolic enzyme, alpha-glutathione S-transferase is predominantly found in the liver and is distributed uniformly in the liver tissue. In our study s-GSTA levels were measured in 37 CF patients aged 1 to 28 years (mean age 10.4 years, 24 males). The control group consisted of 27 patients aged 2 to 17 years (mean age 8.5 years, 18 males). The presence of hepatobiliary abnormalities was assessed by clinical examination, ultrasound scan, s-GSTA, and conventional liver enzymes: alanine aminotransferase (ALT), alkaline phosphatase (ALP), aspartate aminotransferase (AST) and gama-glutamyl transferase (GMT). The calculated 5-95 % range of s-GSTA for the control group was 0.098-2.54 microg/l, for the CF group 0.43-9.76 microg/l. Mean s-GSTA level in the control group was 1.55 microg/l (S.D.=1.57), and 2.05 micro/l (S.D.=2.60) in the CF group. In the group of CF patients, the serum levels were significantly higher than in the control group (P<0.01). No significant correlation existed in the CF group between s-GSTA and conventional liver tests (ALT, AST, ALP and GMT). Four patients in the CF group had hepatobiliary abnormalities detectable by conventional liver tests, s-GSTA and US. Four patients had abnormal s-GSTA, while conventional liver tests and US were normal. One other patient had abnormal hepatic US, but normal standard liver tests and s-GSTA. The study has suggested that a raised s-GSTA level might be a marker of possible pathological changes of the hepatobiliar system in CF patients. Serum GSTA seems to be a more sensitive marker than transaminases for the monitoring of hepatocellular integrity and as an early predictor of hepatic damage.

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.

[Primary lung tumors in childhood]. / Primární nádory plic v detském veku.

The authors give an account of their experience with the treatment of primary lung tumours in children during 1989-1998. Primary lung tumours in children are extremely rare and represent a varied spectrum of pathological conditions (pneumoblastoma, rhabdomyosarcoma, mucoepidermoid carcinoma, pulmonary endodermal tumour and benign tumours). During the mentioned period 10 children were operated on account of primary lung tumours, 5 with malignant tumours and 5 with benign tumours. In these patients during the above period 3 pneumonectomies, 5 lobectomies and 3 segmental resections were made. Chemotherapy was indicated in children with pneumoblastoma and rhabdomyosarcoma. Two patients with pneumoblastoma died, 3 children with malignant and 5 children with benign tumours live without symptoms of the disease.