No abstract available.
Polycystic Kidney Diseases* ; Tuberous Sclerosis*

No abstract available.
Child* ; Colon* ; Humans ; Mucins*

Morgagni hernia is an uncommon finding in adult and rarer still in infants and children, adn they say that Morgagni hernia occur about one in every 300 diaphragmatic hernias diagnosed. This paper presents a 16 months old Korean male infant with symptomatic Morgagni's hernia in whom the diagnosis was established during the course of treatment of upper respiratory tract infection and who were operated successfully. This well nourished patient was admitted with the chief complaints of mid dyspnea, coughing and restlessness for 2 days. During treatment and investigation we detected a peristaltic sound in the right side lung field and confirmed it as Morgagni hernia by chest X-ray and barium swallowing. We performed corrective surgery through a midline supra-umbilical incision. As opened the peritoneum, transverse colon was gushed out, which suspected as herniated intestinal loop. After well mobilization of visible bowel, hernia sac was notified from the anterior portion or right crus of diaphragm. The hernia was reduced very easily. The patient made an uninterrupted recovery and on the 8th post-op. day, he went to home with symptom-free state. We made a brief literature review.
Adult ; Barium ; Child ; Colon, Transverse ; Cough ; Deglutition ; Diagnosis ; Diaphragm ; Dyspnea ; Hernia* ; Hernia, Diaphragmatic ; Humans ; Infant* ; Lung ; Male ; Peritoneum ; Psychomotor Agitation ; Respiratory Tract Infections ; Thorax

This paper presents a case of ganglioneuroblastoma in 3-year-old Korean boy which was arised from right adrenal gland. The tumors arising from the ganglion cells of the peripheral nervous system are ganglioneuroma, neurplastoma and ganglioneuroblastoma. We experienced a case of ganglioneuroblastoma recently which was confirmed histologically and excised successfully. Also we made a brief review of literatures.
Adrenal Glands ; Child, Preschool ; Ganglion Cysts ; Ganglioneuroblastoma* ; Ganglioneuroma ; Humans ; Male ; Peripheral Nervous System

Thyroglossal duct cyst is ectodermal remnant, which may develop along the line of descent of the thyroid gland from the foramen cecum of the tongue to the pyramidal lobe of the thyroid gland. Meticulous dissection of the cyst and duct, along with the body of the hyoid bone is necessary to avoid recurrence. Eighty-one patients with thyroglossal duct cyst treated at Hanyang University Hospital between January 1980 and December 2000 were reviewed to determine the incidence and to analyze the result of management. The male-to-female ratio was 1.4:1 (47:34) with a male preponderance. They are most commonly present at 3-8years (54.2%) of age, but rarely present at infancy. The most common symptom was a painless midline neck mass (76.5%, 62cases). Eighty-one patients underwent modified Sistrunk operation without evidence of recurrence. Eight-nine percent (72 cases) of these lesions were located between thyroid substance and hyoid bone, and 11% (9 cases) were above the hyoid bone. There were 22 infected cysts (27.2%). The Sistrunk operation is a gold standard for treating the thyroglossal duct cysts. For best results in thyroglossal duct cyst surgery, one should make every effort to remove the cyst intact in continuity with the body of the hyoid bone. In our institute, Sistrunk operation modified by the authors showed a good result.
Cecum ; Ectoderm ; Humans ; Hyoid Bone ; Incidence ; Male ; Neck ; Recurrence ; Thyroglossal Cyst* ; Thyroid Gland ; Tongue

Biliary atresia (BA) is very difficult to distinguish from neonatal hepatitis (NH) and its prognosis depends on the age at the time of Kasai operation. Therefore early differentiation between these two conditions is very important. Although various clinical and laboratory tests have been reported to differentiate between them, they are still of limited value. From 1980 to 1999, forty-five infants were referred to our pediatric surgical unit for operation for suspected BA. Eight patients underwent Kasai operation immediately because late diagnosis. These were excluded from the study. The clinical history, physical findings, radiologic and laboratory examinations of 37 cases were analyzed retrospectively. The average age of BA (n=20) was 55.1+/-6.7 days, and that of NH (n=17) was 55.8+/-5.6 days. The sex ratio of BA was 13:7, and that of NH was 14:3. All the patients had obstructive jaundice and acholic stool except 4 BA and 6 NH patients. Acholic stool with yellow component was more frequent in NH. Onset of jaundice was within 2 weeks after birth in 85% of BA, and in 65% of NH. The onset of acholic stool was within 2 weeks after birth in 60% of BA, and in 23.5% of NH. The duration of jaundice and acholic stool of BA were 50.9+/-6.6 days and 41.3+/-8.4 days and those of NH were 40.1+/-3.1 days and 26.6+/-5.4 days respectively. The ultrasonogram and hepatobiliary scan were useful, but not a definitively reliable method for the differentiation of these two diseases. There was no difference in laboratory data. Seventeen cases had NH among 45 referred cases for Kasai operation with the clinical impression of BA, and 4 cases of 17 NH cases needed to be explored to rule out BA. In conclusion, false positive rate of clinical impression of BA was 37.8%, and negative exploration rate was 8.9%. Therefore, careful clinical observation for 1-2 weeks by an experienced pediatric surgeon was very important to avoid unnecessary operation to rule out NH up to the age of 8 - 10 weeks, so long as the stool had yellow component.
Biliary Atresia* ; Delayed Diagnosis ; Diagnosis, Differential ; Hepatitis ; Humans ; Infant ; Jaundice ; Jaundice, Obstructive ; Parturition ; Prognosis ; Retrospective Studies ; Sex Ratio ; Ultrasonography

No abstract available.
Female ; Mosaicism* ; Ovary*

Intestinal obstruction is the commonest cause for emergency surgical intervention in the newborn. An extremely rare cause of intestinal obstruction in the neonate is a congenital abnormality consisting of segmental absence of intestinal musculature with intact serosa and mucosa. This report represents not only the fourth reported case of this anomaly but also the first case associated with long segment Hirschsprung's disease in English-written literatures.
Infant, Newborn ; Humans

Among 60 children with teratoma, forty-three (71.7 percent) were girls and 17 (28.3 percent) boys. Primary sites were sacrococcygeal in 30 patients (50 percent), retroperitoneal in 12 (20 percent), ovarian in 11 (18.3 percent), testicular in 3 (5 percent), and one in each of nasopharyngeal, gastric, hepatic and pancreatic (1.6 percent, respectively). Fifty-five (91.7 percent) teratomas were benign and 5 (8.3 percent) malignant. Malignant teratomas were detected only at sacrococcygeal region (16.7 percent). Older than 2 months of age at diagnosis, presence of urinary and colonic obstructive symptoms, multiple masses and elevated serum alpha-fetoprotein were indicators of malignancy in sacrococcygeal region. Tumor size, presence of calcification, and gross appearance (cystic or solid) did not correlate with malignant nature. Thirteen (21.7 percent) cases were associated with other anomalies. For the immature teratoma, the operative resection without adjuvant chemotherapy was enough. Three malignant cases were survived, one with chemotheapy for 3 years and the others without chemotherapy for 5 and 10 years.
alpha-Fetoproteins ; Carcinoma, Embryonal ; Chemotherapy, Adjuvant ; Child* ; Colon ; Diagnosis ; Drug Therapy ; Endodermal Sinus Tumor ; Female ; Humans ; Sacrococcygeal Region ; Teratoma*

Parenteral nutrition has been an essential part of postoperative care of neonates requiring major surgery soon after birth followed by prolonged period of no allowable enteral feeding. However, TPN via central venous catheters (central TPN), used in increasing trend, still presents significant morbidity. To find out whether TPN via peripheral veins (peripheral TPN) could be used as a viable alternative for postoperative parenteral nutrition in neonates, a clinical study was carried out by a retrospective analysis of 53 neonates subjected to peripheral TPN for more than 7 days after surgery for esophageal atresia with tracheoesophageal fistula, gastroschisis and omphalocele at the Division of Pediatric Surgery, Hanyang University Hospital, from 1983 to 1994. The mean total duration of TPN was 13.3 days (range: 7-58 days), the average daily total fluid intake was 117.6 ml/kg during TPN and 158.6 ml/kg during subsequent oral feeding. The average daily total calorie intake was 57.7 kcal/kg during full strength TPN and 101.3 kcal/kg during oral feeding. The mean urine output was maintained at 3.5 ml/kg/hour during TPN and at 3.6 ml/kg/hour during oral feeding. The increment of body weight observed during TPN was 132gm in TEF, 53gm in gastroschisis and 3gm in omphalocele patients, while loss of body weight was not oberved in each group. The mortality rate was 5.7% (3/53), related to underlying congenital anomalies but not to TPN. The common complications were laboratory findings suggestive of liver dysfunction in 23 cases (43.4%) with no significant clinical symptom or signs in any case, transient pulmonary edema in one case, and generalized edema in one case. The result of the study suggests that peripheral TPN could be used for adequate postoperative nutritional support in neonates requiring about 2 to 3 weeks TPN.
Body Weight ; Central Venous Catheters ; Edema ; Enteral Nutrition ; Esophageal Atresia ; Gastroschisis ; Hernia, Umbilical ; Humans ; Infant, Newborn ; Liver Diseases ; Mortality ; Nutritional Support ; Parenteral Nutrition ; Parenteral Nutrition, Total* ; Parturition ; Postoperative Care ; Pulmonary Edema ; Retrospective Studies ; Tracheoesophageal Fistula ; Veins*