Choledochoceles: are they choledochal cysts?

OBJECTIVE: The aim of this analysis was to report a multidisciplinary series comparing choledochoceles to Todani Types I, II, IV, and V choledochal cysts. SUMMARY BACKGROUND DATA: Choledochoceles have been classified as Todani Type III choledochal cysts. However, most surgical series of choledochal cysts have reported few choledochoceles because they are managed primarily by endoscopists. METHODS: Surgical, endoscopic, and radiologic records were reviewed at the Riley Children's Hospital and the Indiana University Hospitals to identify patients with choledochal cysts. Patient demographics, presenting symptoms, radiologic studies, associated abnormalities, surgical and endoscopic procedures as well as outcomes were reviewed. RESULTS: A total of 146 patients with "choledochal cysts" including 45 children (31%) and 28 with choledochoceles (18%) were identified, which represents the largest Western series. Patients with choledochoceles were older (50.7 vs. 29.0 years, P < 0.05) and more likely to be male (43% vs. 19%, P < 0.05), to present with pancreatitis (48% vs. 24%, P < 0.05) rather than jaundice (11% vs. 30%, P < 0.05) or cholangitis (0% vs. 21%, P < 0.05), to have pancreas divisum (38% vs. 10%, P < 0.01), and to be managed with endoscopic therapy (79% vs. 17%, P < 0.01). Two patients with choledochoceles (7%) had pancreatic neoplasms. CONCLUSIONS: Patients with choledochoceles differ from patients with choledochal cysts with respect to age, gender, presentation, pancreatic ductal anatomy, and their management. The association between choledochoceles and pancreas divisum is a new observation. Therefore, we conclude that classifications of choledochal cysts should not include choledochoceles.

Gastrointestinal tumors in children and adolescents.

Tumors of the pediatric gastrointestinal tract are extremely rare. Their infrequent presentation at treatment centers has not allowed for the development of standardized treatment protocols and prospective review. The most prevalent gastrointestinal neoplasms and malignancies are described, including gastrointestinal lymphoma, colorectal carcinoma, carcinoid tumors, gastrointestinal stromal tumors, leiomyomas, juvenile polyps, inflammatory pseudotumors, gastric tumors, and Peutz-Jeghers polyposis syndrome. Current recommendations for the medical and surgical management of these tumors are reviewed and summarized for this vast group of gastrointestinal neoplasms in children.

[The effects of interleukin-11 and epidermal growth factor on residual small intestine after experimental extensive small bowel resection]. / Az interleukin-11 és az epidermális növekedési faktor trophicus hatásának vizsgálata kisérletes kiterjesztett vékonybél-reszekció után.

BACKGROUND/PURPOSE: Interleukin 11 (IL-11) is a multifunctional cytokine derived from bone marrow, which has a trophic effect on small bowel epithelium. This study compares the effects of IL-11 with epidermal growth factor (EGF), a growth factor known to enhance small bowel adaptation. METHODS: Forty Sprague-Dawley rats (90-100 g) underwent an 85% mid-small bowel resection with primary anastomosis on day 0. Rats were divided into four treatment groups: controls (group I) received bovine serum albumin (BSA), group II received IL-11, 125 mg/kg subcutaneously (SC) twice daily, group III received EGF, 0.10 mg/g SC bid, and group IV received EGF and IL-11 in the above doses. Half of the animals (five per group) were killed on day 4 of therapy, and the rest on day 8. Animals were evaluated for weight, mucosal length, and bowel wall muscle thickness on days 4 and 8, and expression of proliferating cell nuclear antigen (PCNA) in intestinal crypt and smooth muscle cells on day 8. RESULTS: Body weight was similar at day 4 and 8. Mucosal thickness in groups II (IL-11) and IV (IL-11 and EGF) was significantly increased at days 4 and 8 compared with controls (group I) and EGF (group III, P < 0.001). Muscle thickness was significantly increased in the EGF and combined group IV compared with the BSA controls and IL-11 groups (P < 0.001). Thirty-two percent of the mucosal crypt cells in group I stained positive for PCNA, whereas 51%, 53%, and 60% stained positive in groups II (IL-11), III (EGF), and IV (IL-11 and EGF), respectively. In groups I and II, 2% and 1.7% of the myocytes stained positive for PCNA, whereas 11.2% and 5.2% in group III and IV. CONCLUSIONS: These data suggest that IL-11 has a trophic effect on small intestinal enterocytes, causing cell proliferation and increased mucosal thickness. EGF has a more generalized effect causing proliferation of both enterocytes and myocytes. IL-11, with or without EGF may be a useful adjunct in treatment of short bowel syndrome.

[Pancreatic intraepithelial neoplasia (PanIN) and intraductal papillary mucinous neoplasms (IPMN)]. / A pancreas intraepithelialis neoplasiája (PanIN) és intraductalis papillaris mucinosus neoplasiái (IPMN).

Pancreatic Intraepithelial Neoplasia (PanIN1-3) and Intraductal Papillary Mucinous Neoplasms (IPMNs) putative precursors of and associated to pancreatic cancer represent a distinct, however pathologically heterogenous entity. Recently a new classification and nomenclature was established. We analysed the clinicopathologic and cytogenetic characteristics of six patients with PanIN and two with IPMN undergoing curative surgical resection. The most valuable tool in the diagnosis of these preinvasive neoplasms is ERCP combined with brush cytology. Conventional laboratory blood tests, tumor markers (CEA, CA19-9) were within normal values and imaging modalities were informative in only one third of the cases, however, not pathognostic. Based on our hypothesis that tumor ploidy pattern correlates with biological behaviour, malignant potential and prognosis, we measured the nuclear DNA content, ploidy, synthetic (S) phase or proliferative fractions of each neoplasm by flow cytometry. Each neoplasm demonstrated diploid stemline, with a low mean S-phase or proliferative fraction (3,77%) and mean DNA index (DI) of 0.96. The diploid DNA pattern and the low proliferative activity are consistent with the nonaggressive biological behavior of intraepithelial and intraductal neoplasms and, in part, explain their favorable prognosis.

Evaluation of perioperative cholangiography in one thousand laparoscopic cholecystectomies.

INTRODUCTION: We analyzed a teaching institution's experience with intraoperative cholangiography (IOCG) and endoscopic retrograde cholangiopancreatography (ERCP) and established an algorithm for their timing and use. METHODS: The records of all patients undergoing LC during a five year period were reviewed. Patients with a history of jaundice or pancreatitis, abnormal bilirubin, alkaline phosphatase, serum glutamicoxaloacetic transaminase, or radiographic evidence suggestive of choledocholithiasis were considered "at risk" for common bile duct stones (CBDS). The remaining patients were considered to be at low "risk". RESULTS: LC was attempted on 1002 patients during the study period and successfully completed on 941 (94% of the time). The major complication rate was 3.1% and the common bile duct injury rate 0.1%. Eighty eight (9.5%) patients underwent ERCP, 67 in the preoperative period and 19 in the postoperative period. IOCG was attempted in 272 (24%) patients and completed in 234 for a success rate of 86%. Intraoperative cholangiography (IOCG) and preoperative endoscopic retrograde cholangiopancreatography (ERCP) were equivalent in the detection of CBDSs. Twelve of the 21 patients (57%) with IOCG positive for stones underwent successful laparoscopic clearance of the common duct, and did not require postop. ERCP. No patients were converted to an open procedure for common bile duct exploration. Because postoperative ERCP was 100% successful in clearing the common duct, reoperation for retained common bile duct stones was not necessary. CONCLUSION: IOCG is an alternative procedure to ERCP for patients at risk with biochemical, radiological, or clinical evidence of choledocholithiasis. The incidence of CBDS in low-risk patients is 1.7%, a risk that does not warrant routine cholangiography. Preoperative ERCP is recommended in cases of cholangitis unresponsive to antibiotics, suspicion of carcinoma, and biliary pancreatitis unresponsive to supportive care. Although IOCG leads to a similar percentage of nontherapeutic studies as preoperative ERCP, it often allows for one procedure therapy.

Surgical considerations in cystic fibrosis: a 32-year evaluation of outcomes.

BACKGROUND: Information concerning long-term operative outcomes in patients with cystic fibrosis (CF) is relatively sparse in the operative literature. METHODS: A retrospective review of CF patients with operative conditions was performed (1972-2004) at a tertiary children's hospital to analyze outcomes including long-term morbidity and survival. RESULTS: A total of 226 patients with CF presented with an operative diagnosis (113 men, 113 women). A total of 422 operations were performed in 213 patients (94%). The mean age at operation was 4.1 +/- 6.2 years (range, 1 d to 26 y) and 109 were neonates. Fifteen of 42 (36%) babies with simple meconium ileus (MI) were treated nonoperatively with hypertonic enemas, 27 of 42 and all 45 patients with complicated MI required operation, including 15 with jejunoileal atresia (17%). Seventeen of 27 (63%) patients with meconium ileus equivalent had MI as neonates; 7 of 27 (26%) required operation. Eight of 9 (89%) with fibrosing colonopathy required operation. Organ transplantation was required in 21 patients. Follow-up evaluation was possible in 204 of 213 (96%) patients. The duration of follow-up evaluation was 14.9 +/- 8.5 years (range, 2 mo to 35 y). Operative morbidity was 11% at 1 year, 2% at 2 to 4 years, 1% at 5 to 10 years, and less than 1% at more than 10 years. There were 24 deaths (11%); 22 followed CF-related pulmonary complications and included 8 of 16 (50%) children with pneumothorax. CONCLUSIONS: Long-term survival in CF patients has improved significantly (89%), with many surviving into the fourth decade. MI may predispose to late complications including meconium ileus equivalent and fibrosing colonopathy. Pneumothorax in CF patients is an ominous predictor of mortality. Children with CF are living longer and are good candidates for operation, but require long-term follow-up evaluation because of ongoing exocrine dysfunction.

Surgical management of duodenal injuries in children.

BACKGROUND: The goal of this study was to review current injury characteristics, severity, intervention, and outcome of duodenal injuries from a single, pediatric trauma facility. METHODS: A retrospective review was performed of duodenal injuries in children less than 16 years of age from 1990 to 2000. RESULTS: Twelve children had duodenal injuries as a result of blunt abdominal trauma. Six injuries were the result of motor vehicle crashes. Nonaccidental trauma (2) and contact injury (4) provided the remaining cases. Diagnosis was achieved by abdominal computed tomography. Severity of duodenal injury included grade I (1), II (8), and III (3). Seven patients had associated visceral or neurologic injuries. Average Injury Severity Score was 18. Duodenal repair was required in 9 of the 10 patients explored. Treatment included observation (3); primary repair, alone, (2) or with proximal decompression (4); and pyloric exclusion with gastrojejunostomy (3). Exclusion techniques had fewer complications (0% vs 57%) and fewer hospital days (19 vs 23). CONCLUSIONS: Blunt abdominal trauma remains the most prevalent mechanism for pediatric duodenal injuries. Patients undergoing pyloric exclusion for severe duodenal trauma had a lesser morbidity and a shorter hospital stay in this small series. Pyloric exclusion remains an alternative for the treatment of severe duodenal injuries in selected children.

Long-term results of treatment of Hirschsprung's disease.

Many children with Hirschsprung's disease (HD) have a good outcome following surgical treatment, but long-term follow-up studies have identified a number of concerns. Analysis of long-term function in children after surgical management is difficult. The most commonly encountered problems include constipation, incontinence, enterocolitis and the overall impact of the disease on lifestyle (quality of life). Other complications are less frequent. Each of these problems will be discussed.

Laparoscopic splenectomy has become the gold standard in children.

Splenectomy is frequently required in children with various hematologic disorders. The reported advantages of laparoscopic splenectomy (LS) include less pain, shorter hospital stay, and improved cosmesis. This report evaluates the outcome of children undergoing LS at a single children's facility. One hundred twelve children underwent LS by the lateral approach between August 1995 and February 2001. Indications for LS were hereditary spherocytosis in 58, idiopathic thrombocytopenic purpura in 21, sickle cell disease in 19, and other conditions in 14. LS alone was completed in 89 children and LS and cholecystectomy (LSC) in 20. Three required conversion to open splenectomy. Accessory spleens were identified in 19. Complications included ileus (four), acute chest syndrome (four), bleeding (two), pneumonia (one), and diaphragm perforation (one). There was no mortality. An accessory spleen was missed in one child with recurrent anemia. Average operative time for LS was 106 minutes and for LSC 135 minutes. Operative time for LS decreased with experience but the difference was not significant. Average length of stay was 1.51 days (range 1-11) and was longer in sickle cell disease (2.47 days) versus hereditary spherocytosis (1.29 days) and idiopathic thrombocytopenic purpura (1.16 days). We conclude that LS is safe and effective in children with hematologic disorders and is associated with minimal morbidity, zero mortality, and a short length of stay.

Results of a longitudinal study of rigorous manuscript submission guidelines designed to improve the quality of clinical research reporting in a peer-reviewed surgical journal.

BACKGROUND/PURPOSE: In an effort to improve the reporting quality of clinical research, the Journal of Pediatric Surgery instituted specific reporting guidelines for authors beginning June 2006. This study was conducted to evaluate whether these guidelines improved reporting of observational studies. METHODS: The Guidelines for the Reporting of Clinical Research Data (Guidelines) included 23 criteria in 3 subcategories: Methods, Results, and More than one treatment group. Reporting quality was evaluated by determining the percentage of criteria met. Seventy-three articles before implementation and 147 articles after implementation were independently assessed by 2 reviewers. RESULTS: Mean global composite scores increased from 72.2 pre-Guidelines to 80.1 post-Guidelines (P < .0001). Scores increased in each subcategory: Methods, 71.9 to 78.6 (P < .0001); Results, 77.2 to 83.0 (P = .002); and More than one treatment group, 40.0 to 70.6 (P = .0003). Post-Guidelines implementation scores have increased over time. CONCLUSIONS: The introduction of the Guidelines resulted in significant improvement in the quality of reporting in the Journal. The low cost vs the benefit suggests that the Guidelines can be an effective way to improve reporting quality in nonrandomized studies. We encourage further efforts to increase inclusion of reporting criteria as well as evaluation and improvement of the Guidelines. We suggest that editors of other surgical publications consider implementing analogous guidelines.

William H. Harridge Memorial Lecture: "Progress and its unintended consequences".

BACKGROUND: Because of the development of many clinical and technological advances, significant progress has been made in the management of many neonatal and childhood conditions. The improved survival of many infants and children who formerly died has been accompanied by a number of unintended consequences. METHODS: A review is conducted of the progress achieved in 2 groups of patients requiring pediatric surgical care, newborns (especially the premature) and those afflicted with cancer, as well as the unintended consequences observed in survivors. RESULTS: Dramatic improvements in the survival of premature infants have resulted in an increased number of patients at risk for necrotizing enterocolitis, chronic lung disease, and neuroimpairment, often associated with gastroesophageal reflux and cognitive difficulties. Childhood cancer survivors have many adverse sequelae of intense multidisciplinary therapy affecting multiple systems, including treatment-related second neoplasms. CONCLUSIONS: Remarkable advances in care have transpired, but improved survival may lead to new problems not previously experienced. Appropriate modifications in patient care and long-term follow-up are essential to maintain excellent survival and lessen the adverse consequences of treatment to ensure good quality of life.