A 38-year-old woman was hospitalized in August 2007. This visit was her fifth episode of acute pancreatitis. Computed tomography revealed a cystic structure located near the antrum. Communication between this structure and the pancreatic duct was revealed by endoscopic retrograde cholangiopancreatography. Ultrasonography revealed that the cyst wall had a layered structure. Thus, we regarded it as a gastric duplication cyst. We thought that the gastric duplication cyst communicating with an aberrant pancreatic duct was responsible for the recurrent acute pancreatitis. In August 2008, a cyst gastrostomy was performed between the gastric duplication cyst and the stomach. No recurrence of acute pancreatitis has since occurred.
Background: Congenital hyperinsulinism (CH) is a rare disease, characterized by severe hypoglycemia induced by inappropriate insulin secretion from pancreatic beta-cells in neonate and infant. Hirschsprung's disease (HD) is also a rare disease in which infants show severe bowel movement disorder. We herein report an extremely rare case of combined CH and HD. Case presentation: The patient was a full-term male infant who showed poor feeding, vomiting, and hypotonia with lethargy on the day of birth. He was transferred to tertiary hospital after a laboratory analysis revealed hyperinsulinemic hypoglycemia. The patient showed remarkable abdominal distension without meconium defecation. An abdominal X-ray showed marked dilatation of the large bowel. He was diagnosed with CH (nesidioblastosis) associated with suspected HD. He was initially treated with an intravenous infusion of high-dose glucose with the intermittent injection of glucagon. This was successfully followed by treatment with diazoxide and octreotide (a somatostatin analog). At 8 months of age, HD was confirmed by the acetylcholinesterase staining of a rectal mucosal biopsy specimen, and a transanal pull-through operation was performed to treat HD. At 14 months of age, subtotal pancreatectomy was performed for the treatment of focal CH located in the pancreatic body. His postoperative course over the past 12 years has been uneventful without any neurologic or bowel movement disorders. Conclusions: Although it is extremely rare for CH to be associated with HD, associated HD should be considered when a patient with CH presents severe constipation.
The clinical characteristics and natural history of 55 cases with antenatally diagnosed fetal uropathy were investigated. Percutaneous aspiration of the fetal pelvic or vesical urine was performed to decompress progressive unilateral hydronephrosis in 2 cases and to evaluate renal function in another 2 cases of bilateral hydronephrosis. As the postnatal diagnosis, upper urinary tract dilatation (hydronephrosis or hydronephroureter, 33 cases) and renal dysplasia (15 cases) made up 87% of all cases. A combination of hydronephrosis in one kidney and renal dysplasia in the other was also found in another 2 cases. Among 35 cases with upper urinary tract dilatation, 27 cases demonstrated pelviureteric junction stenosis and surgical intervention was necessary in 15 cases. In 17 cases with renal dysplasia, spontaneous regression was observed in only 3 cases and surgical intervention by means of percutaneous nephrostomy and nephrectomy was performed in 4 and 6 cases, respectively.
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