Postoperative Conversion Disorder Presenting as Inspiratory Stridor and Hemiparesis in a Pediatric Patient.

BACKGROUND Postoperative conversion disorder is rare and has been reported. The diagnosis is usually made after all major organic causes have been ruled out. CASE REPORT We describe a case of a 13-year-old female who presented in the post-anesthesia care unit with acute-onset inspiratory stridor and unresponsiveness to verbal or painful stimuli after receiving a general anesthetic for upper endoscopy. Later in the post-anesthesia care unit, she presented with acute-onset right hemiplegia and sensory loss. She was first evaluated for causes of her stridor and unresponsiveness. The evaluation revealed paradoxical vocal cord movement, and all laboratory test values were normal. For her hemiplegia and sensory loss, she was evaluated for stroke with head MRI and CT scans, which were normal. CONCLUSIONS After extensive workup and consideration of multiple etiologies for her presenting signs and symptoms, the most likely diagnosis was conversion disorder.

Abnormal endocrine pancreas function at birth in cystic fibrosis ferrets.

Diabetes is a common comorbidity in cystic fibrosis (CF) that worsens prognosis. The lack of an animal model for CF-related diabetes (CFRD) has made it difficult to dissect how the onset of pancreatic pathology influences the emergence of CFRD. We evaluated the structure and function of the neonatal CF endocrine pancreas using a new CFTR-knockout ferret model. Although CF kits are born with only mild exocrine pancreas disease, progressive exocrine and endocrine pancreatic loss during the first months of life was associated with pancreatic inflammation, spontaneous hyperglycemia, and glucose intolerance. Interestingly, prior to major exocrine pancreas disease, CF kits demonstrated significant abnormalities in blood glucose and insulin regulation, including diminished first-phase and accentuated peak insulin secretion in response to glucose, elevated peak glucose levels following glucose challenge, and variably elevated insulin and C-peptide levels in the nonfasted state. Although there was no difference in lobular insulin and glucagon expression between genotypes at birth, significant alterations in the frequencies of small and large islets were observed. Newborn cultured CF islets demonstrated dysregulated glucose-dependent insulin secretion in comparison to controls, suggesting intrinsic abnormalities in CF islets. These findings demonstrate that early abnormalities exist in the regulation of insulin secretion by the CF endocrine pancreas.

[Pancreas. Part I: congenital changes, acute and chronic pancreatitis]. / Pankreas. Teil I: Angeborene Veränderungen, akute und chronische Pankreatitis.

The pancreas develops from ventral and the dorsal buds, which undergo fusion. Failure to fuse results in pancreas divisum, which is defined by separate pancreatic ductal systems draining into the duodenum. Risk of developing pancreatitis is increased in pancreas divisum because of insufficient drainage. MR cholangiopancreatography (MRCP) is the technique of choice for detecting pancreas divisum non-invasively. Annular pancreas is the result of incomplete rotation of the pancreatic bud around the duodenum with the persistence of parenchyma or a fibrous band encircling (and sometimes stenosing) the duodenum. Acute pancreatitis is usually caused by bile duct stones or alcohol abuse. The Atlanta classification differentiates between mild acute and severe acute pancreatitis associated with organ failure and/or local complications such as necrosis, abscess or pseudocyst. Contrast-enhanced multi-detector row CT is the method of choice to assess the extent of disease. Balthazar et al.'s CT severity index assesses the risk of mortality and morbidity. In acute pancreatitis, the role of MRCP is mainly limited to finding bile duct stones in patients with suspected biliary pancreatitis. Chronic pancreatitis results in relentless and irreversible loss of exocrine (and sometimes endocrine) function of the pancreas. MDCT even shows subtle calcifications. MRCP is the method of choice for non-invasive assessment of the duct. Inflammatory pseudotumor in chronic pancreatitis and groove pancreatitis are difficult to differentiate from pancreatic cancer. In these cases, multiple imaging methods such as MDCT, MRI and endosonography including biopsy may be used to make a diagnosis.

Chronic hereditary pancreatitis with N29I mutation in a Turkish child.

Hereditary pancreatitis, an autosomal dominant disease, is the second most common cause of pancreatitis in children. Here we report a child with recurrent pancreatitis attacks and N29I mutation. Due to the increased risk of pancreatic cancer, taking a detailed past and family history and early diagnosis are important.

Cholecystectomy alone is inadequate for treating forme fruste choledochal cyst: evidence from a rare but important case report.

Treatment of "forme fruste" choledochal cyst (FFCC) where pancreaticobiliary malunion (PBMU) is associated with minimal dilatation of the common bile duct (CBD) remains controversial. PBMU allows pancreaticobiliary reflux to occur, which causes complications such as recurrent pancreatitis and gallbladder cancer. Therefore, some surgeons opt to treat FFCC by cholecystectomy alone in order to prevent gallbladder cancer, with the result that pancreaticobiliary reflux could still occur. Our treatment of choice-excision of both the CBD and the gallbladder, followed by Roux-en-Y hepatico-jejunostomy-can eliminate pancreaticobiliary reflux and prevent complications. Our case, a 2-year-old boy, initially presented to a hospital abroad with recurrent abdominal pain. Endoscopic retrograde cholangiopancreatography showed massive protein plugs impacted in the papilla of Vater and mild CBD dilatation, but PBMU was not identified. Intraoperative cholangiography performed during laparotomy 5 days later suggested PBMU with minimal CBD dilatation. Despite these findings, cholecystectomy with T-tube drainage was performed rather than CBD excision with biliary reconstruction. Postoperative T-tube cholangiography clearly showed PBMU. The T-tube was removed after 2 weeks, and 3 months later the boy was referred to us because of recurrent pancreatitis. We performed CBD excision and Roux-en-Y hepatico-jejunostomy. His postoperative course was uneventful, and he is well after 10 years of follow-up. This case provides strong evidence that CBD excision with biliary reconstruction is mandatory for treating FFCC and, conversely, that cholecystectomy alone is inadequate for treating children with FFCC.

Sphincter of Oddi dysfunction in children with recurrent pancreatitis and anomalous pancreaticobiliary union: an etiologic concept.

BACKGROUND: The exact cause of recurrent pancreatitis among patients with anomalous pancreaticobiliary union is not known. Sphincter of Oddi dysfunction has been implicated as a mechanism. This study evaluated sphincter of Oddi function in children with anomalous pancreaticobiliary union and recurrent pancreatitis and assessed the results of endoscopic sphincterotomy in the management of this condition. METHODS: We retrospectively reviewed 128 endoscopic retrograde cholangiopancreatographic (ERCP) studies performed on children older than 1 year and adolescents with pancreaticobiliary disease. In 64 instances, ERCP was performed because of recurrent pancreatitis. Nine patients underwent sphincter of Oddi manometry followed by endoscopic sphincterotomy, and these patients were included in this study. A basal pressure greater than 35 mm Hg was considered diagnostic for sphincter of Oddi dysfunction. Follow-up data were obtained retrospectively from the patients' relatives and referring physicians. RESULTS: An anomalous pancreaticobiliary union was found in 18 of 64 (28%) patients with recurrent pancreatitis. The 9 patients who underwent sphincter manometry and endoscopic sphincterotomy were 5 girls and 4 boys 2.9 to 17 years of age (mean 7.8 years). A choledochal cyst was found in 7 of these 9 patients. Two patients had anomalous pancreaticobiliary union without common bile duct dilatation. All 9 patients had sphincter of Oddi dysfunction (mean basal pressure 96 +/- 37.8 mm Hg, range 48 to 156 mm Hg). The length of the common channel was 22.8 +/- 5.5 mm, and the length of the sphincter of Oddi segment was 12.1 +/- 1.9 mm (p < 0.001). In all patients the sphincter of Oddi segment was located within the duodenal wall. The mean follow-up period after endoscopic sphincterotomy was 26.4 months (range 18 to 38 months). Eight patients had excellent results defined as absence of symptoms and no subsequent episodes of acute pancreatitis. Treatment of 1 patient was considered moderately successful because the patient still had occasional pain without pancreatic enzyme elevation but no subsequent episodes of acute pancreatitis. One patient had mild postprocedural pancreatitis. CONCLUSIONS: Recurrent pancreatitis and anomalous pancreaticobiliary union are associated with sphincter of Oddi dysfunction in children and adolescents. Endoscopic sphincterotomy is beneficial to these patients.

Pancreaticobiliary maljunction associated with nondilatation or minimal dilatation of the common bile duct in children: diagnosis and treatment.

It is known that the etiology of congenital biliary dilatation (CBD) is closely associated with pancreaticobiliary maljunction (PBMJ). Treatment of CBD today is primary excision of the cyst followed by hepaticoenterostomy. However, PBMJ without dilatation of the biliary tract has recently been reported and its treatment is still controversial. In adults, simple cholecystectomy without biliary reconstruction is often performed, because it usually presents as an anomaly of the gallbladder. Over 30 years, we have encountered 8 patients with PBMJ without dilatation of the common bile duct in 180 pediatric cases of CBD. The presenting symptoms of these patients were those of pancreatitis i.e. abdominal pain associated with elevation of serum amylase levels. Five cases presented with jaundice or a history of pale colored stools. Endoscopic retrograde cholangiopancreatography was performed to confirm the diagnosis. Five (62.5%) of these 8 patients were found to have dilatation of the common channel, which was seen in 50 (29%) of the 172 other cases with biliary tract dilatation. Three cases (37.5%) had proven protein plugs or debris at the level of the common channel, and this was observed in 28 (16%) of the 172 other cases. It is strongly suggested that the manifestation of clinical symptoms in these patients results from stasis or obstruction at the level of the common channel. In children who present with recurrent pancreatitis, PBMJ must be suspected even if the common bile duct appears to be normal. It is difficult to resolve these anomalies without operative repair. The authors consider that radical treatment of PBMJ is required in these children to prevent serious long-term complications.

Does interleukin-2 abrogate peripheral immunologic self-tolerance in vivo?

Transgenic mice that constitutively expressed murine IL-2 in islet beta cells (RIP-IL-2 mice) had pancreatitis from birth which resolved into a peri- and intra-islet infiltrate in adult animals. In spite of the impressive infiltration, these mice did not develop autoimmunity to islet antigens. Neither was autoimmunity found to extrathymic H-2Kb molecules known to induce tolerance by a peripheral mechanism, when IL-2 and H-2Kb were coexpressed in the beta cells. Apparently, IL-2 can only act on activated T cells and is unable to reverse tolerance in T cells that have been made unresponsive through inappropriate antigen presentation in our system.