No abstract available.
Animals ; Rats* ; Regeneration*

Persistent cloaca in a female newborn is one of the most complex and challenging developmental malformations. The incidence is about 10% of all anorectal anomalies. Treatment of cloacal malformations has evolved during the past 40 years; however, it still remains one of the most difficult operations. In 1997, Pe a reported that a new technical variant called "Total urogenital mobilization" We also repaired cloacal anomaly using "Total urogenital mobilization" in 3 patients. The operations were performed between age 15- and 28-month. The length of the common channels was 2.5-3.0 cm. Two cases had double vaginas and one of them also had double uterus. The operation time was 4-5 hours and no major complications occurred. A satisfactory functional and cosmetic results were obtained.
Cloaca ; Female ; Humans ; Incidence ; Infant, Newborn ; Uterus ; Vagina

Beckwith-Wiedemann sydrome is a multisystemic pattern of congenital anomalies with overgrowth. This syndrome is first described independently by Beckwith in 1963 and by Wiedemann in 1964. There is wide spectrum of clinical manifestations, including prenatal or postnatal overgrowth, neonatal hypoglycemia, macroglossia, visceromegaly, omphalocele, hemihypertrophy and a predisposition for embryonal tumors, most frequently Wilms' tumor. We experienced a case of Beckwith-Wiedemann syndrome who developed left adrenal cortical neoplasm of indeterminate malignant potential.
Beckwith-Wiedemann Syndrome ; Hernia, Umbilical ; Hypoglycemia ; Macroglossia ; Wilms Tumor

No abstract available.
Breast* ; Child* ; Humans

Prenatally diagnosed neuroblastomas have been reported in increasing numbers over the past several years. The vast majority are in favorable stages of the disease (stage I, II, IV-S). The authors experienced one case of stage IV-S neuroblastoma of the adrenal gland with liver metastasis, which regressed spontaneously after removal by adrenalectomy. This patient was noticed to have an abdominal mass at prenatal ultrasonography performed at 36weeks of gestation. This tumor was a neuroblastoma of the left adrenal gland with multiple liver metastases. Left adrenalectomy and liver biopsy were performed at 3 months of age. Thirty-eight months after surgery, an MRI demonstrated that the hepatic metastatic lesions had completely regressed without chemotherapy or radiation.
Adrenal Glands ; Adrenalectomy* ; Biopsy ; Drug Therapy ; Humans ; Liver* ; Magnetic Resonance Imaging ; Neoplasm Metastasis* ; Neuroblastoma* ; Pregnancy ; Prenatal Diagnosis ; Ultrasonography, Prenatal

Choledochal cyst is rare in the Western countries, but common in the Oriental countries. The reported complicatioins of choledochal cyst are ascending cholangitis, recurrent pancreatities, progressive biliary cirrhosis, portal hypertension, stone in the cyst, and malignant in the biliary tract. Bile peritonitis secondary to rupture is one of the rarest complications of choledochal cyst, and its reported incidence was 1.8% (Yamaguci, 1980) to 18% (Karnak et al, 1997). The exact cause of perforation of choledochal cyst is unknown, but an anomalous arrangement of the pancreatobiliary ductal system with a long common channel may contribute to the formation of choledochal cyst and even perforation of cyst.Authors reviewed 4 cases (14.2%) of bile peritonitis among 28 cases of choledochal cyst in infants from Jan. 1983 to Jan. 1998. Their ages ranged from 6 months to 3 years and three of them were female. Abdominal distension, pain, and vomiting were common symptoms, and clinical jandice and palpable mass were present in one case. Pre-operative laboratory investigations showed elevated serum bilirubin, serum AST and serum ALT in 3 cases, and elevated serum amylase in one case. The perforation sites were located on the common bile duct at its junction with the cystic duct in 2 cases, distal cyst wall in 1 case and left hepatic duct at its junction with cyst in 1 case. The types of choledochal cysts according to Todani's classification (1977) were as follows;Type IVa was in 3 cases, type I was in 1 case. The results of operative cholangiogram according to new Komi's classification (1992) were as follows;Type Ia was 2 cases, type IIb 1 case, and type III 1 case. One stage primary cyst excision and hepaticojejunostomy(Roux-en Y type) was done in 3 cases, and two staged operation in 1 case. All patients have recovered unevenfully after surgery and discharged at post -operative 9.8th day averagely. Authors concluded that the primary choledochal cyst excision with biliary recontinuity was a safe surgical procedure in ruptured choledochal cyst in infants.
Amylases ; Bile* ; Biliary Tract ; Bilirubin ; Cholangitis ; Choledochal Cyst* ; Classification ; Common Bile Duct ; Cystic Duct ; Female ; Hepatic Duct, Common ; Humans ; Hypertension, Portal ; Incidence ; Infant* ; Liver Cirrhosis, Biliary ; Peritonitis* ; Rupture ; Vomiting

A case of esophageal atresia associated with malrotation and segmental dilatation of the ileum is described. To our knowledge, this is the first such report of this combination of features. The prenatal sonogram showed polyhydramnios. Esophageal atresia was diagnosed 3 days after birth. The diagnosis of malrotation and segmental dilatation of the ileum was made using a barium enema and an explolaparotomy 10 days after the first operation. The facts indicate that abnormal findings on a prenatal sonography and esophageal atresia may be associated with many congenital anomalies should be considered.
Barium ; Diagnosis ; Dilatation* ; Enema ; Esophageal Atresia* ; Ileum* ; Parturition ; Polyhydramnios

Precocious puberty is difficult to define because of the marked variation in the age at which puberty begins normally, onset of puberty before 8 years of age in girls and 9 years in boys may be considered precocious. The etiology of precocious puberty in boys is usually idiopathic, but can result from adrenal and testicular tumors. The hepatoblastoma that produces hCG is a very rare functioning tumor known to cause precocious puberty in boys. Recently, author experienced one case of virilizing adrenal cortical adenoma in 22 month-old boy, one case of adrenal cortical carcinoma in 28 month-old boy, and one case of virilizing hCG-producing hepatoblastoma in 7 year-old boy and reviewed literatures.
Adolescent ; Adrenocortical Adenoma ; Adrenocortical Carcinoma ; Female ; Hepatoblastoma ; Humans ; Infant ; Male ; Puberty ; Puberty, Precocious ; Testicular Neoplasms

Segmental intestinal dilatation is rare, which causes symptom of bowel obstruction and requires resection. The resection is not only diagnostic but also curative procedure. Recently, author experienced 2 cases of segmental dilatation of the ileum due to focal agenesis of the intestinal muscularis in 7 day & 4 day-old female neonates. The postoperative recovery was excellant in the first case after resection of dilated ileum(l5cm in length) and end to end anastimosis, and discharged at 20th day. But in the second case, the passage disturbance was not relieved after resection of dilated ileum(30cm in length), and author re-resected 80cm more of dilated proximal ileum at 2 weeks after the first operation. This baby discharged after diarrhea control with Loperin on I month after the second operation. Final histologic examination showed I) normal population of ganglion cells in both narrowed & dilated ileum in both cases, 2) focal abscence of muscularis propria in both cases, 3) relative hypertrophy of inner circular muscle layer and thinned, multiple fragmented outer longitudinal muscle layer in case 2.
Diarrhea ; Dilatation ; Female ; Ganglion Cysts ; Humans ; Hypertrophy ; Ileum ; Infant, Newborn

Children tend to ingest foreign bodies. The majority present in children between the ages of 6 months and 3 years. We experienced 2 cases of unusual gastrointestinal complications caused by ingested foreign bodies. First case was a 10-month-old male with intestinal perforation due to two pieces of ingested magnetic beads. Second case was a 7-month-old girl with esophageal stricture due to an ingested particle of plastic toy.
Child ; Eating ; Esophageal Stenosis ; Female ; Foreign Bodies ; Humans ; Infant ; Intestinal Perforation ; Male ; Plastics ; Play and Playthings