- OK-432 therapy is effective for the treatment of macrocystic lymphatic malformations (LMs), but the optimal management of patients with microcystic LMs associated with large chylous pleural effusions or chylous ascites is not resolved. We performed thoracoscopic- or laparoscopic-guided injection of OK-432 for 2 patients with diffuse microcystic LMs accompanied by refractory chylous pleural effusion or chylous ascites. Both cases responded well to OK-432 therapy with improvement/resolution of fluid collections and associated symptoms.
- Anomalous arrangement of the pancreaticobiliary duct is considered to be a high-risk factor for biliary tract malignancy. We report a case of intrahepatic cholangiocarcinoma in a 26-year-old man after total resection of choledochal cyst with anomalous arrangement of the pancreaticobiliary duct at the age of 5 months. He had been doing well after total resection of the choledochal cyst; however, he suddenly presented with a spiky fever and abdominal pain in the right upper quadrant at the age of 26 years.
- Spontaneous rupture and subsequent bile peritonitis are rare complications of choledochal cysts. Of these complications, the formation of a biliary pseudocyst is an unusual form, and its preoperative diagnosis is difficult. In this report, we describe 2 cases showing spontaneous rupture with biliary pseudocyst formation. Inflammatory tissue surrounded those pseudocysts, one of which was adjacent to the perforation and the other formed in the transverse mesocolon apart from the biliary tract. These pseudocysts were removed by careful dissection, and single-stage cyst excision with biliary reconstruction was successfully performed in both cases.
- Hirschsprung's disease is an inherited disorder characterized by the absence of ganglion cells in the distal bowel. Neurocutaneous melanosis is a rare congenital syndrome characterized by proliferation of melanin-producing cells in the skin and leptomeninges. The authors described a newborn patient with neurocutaneous melanosis associated with Hirschsprung's disease. This male baby had congenital hydrocephalus, large and multiple pigmented skin nevi, and severe abdominal distension. He showed marked hydrocephalus at birth and underwent a ventriculo-peritoneal shunt at the age of 5 days.
- The authors describe a newborn patient with mesenchymal hamartoma of the chest wall associated with pulmonary hypoplasia. A massive thoracic tumor was diagnosed by prenatal ultrasonography and magnetic resonance imaging at the 28th week of gestation. She was delivered through cesarean delivery at the 36th gestational week. Respiratory distress because of pulmonary hypoplasia necessitated neonatal intensive care. The tumor extensively involved the left hemithorax including all 12 ribs and the first 10 thoracic vertebrae, resulting in marked deformity of the thorax.
- The authors describe a rare case of mucoepidermal carcinoma of the lung incidentally identified in preoperative assessments for inguinal hernia repair in a 5-year-old girl. This patient was referred for right external inguinal hernia, and a 3.0-cm round-shaped lesion was found in the right lower lung field of a chest x-ray film. She had no respiratory tract complaints, but her serum carcinoembryonic antigen concentration was markedly elevated (21.2 ng/mL). Chest and abdominal computed tomography/magnetic resonance images could not determine the nature of the lesion, but 2-[18F]fluoro-2-deoxy-d-glucose positron emission tomography (FDG-PET) indicated a malignant tumor pattern.