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

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

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

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

Congenital anomalies in the head and neck region such as preauricular sinus and skin tag, thyroglossal duct cyst, branchial anomaly, cystic hygroma and dermoid cyst are common in pediatric population. It is important for pediatricians and pediatric surgeons to be familiar with the embryology and the anatomical characterics of these lesions in order to diagnose and treat properly. Three hundred nineteen patients with congenital head and neck anomalies treated at Hanyang University Hospital between 1980 and 1999 were reviewed to determine the relative frequency of the anomalies and to analyze the method of management. Eight-four patients(25.1%) of 335 lesions had preauricular sinus and skin tag, 81 patients(24.2%) had thyroglossal duct cyst, 81 patients(24.2%) had branchial anomaly, 58 patients(17.3%) had cystic hygroma, 31 patients(9.2%) had dermoid cyst. The male-to-female ratio was 1.4:1. The thyroglossal duct cyst was most commonly present at 3-5years, however branchial anomaly was commonly diagnosed in children younger than 1 year. Preauricular sinus shoeed familial tendency in three patients and bilaterality is 33.8%. Most head and neck anomalies in children had clinical and anatomical characterics. A careful history and physical examination were very useful for diagnosis and proper management. The initial surgery should be done by experienced pediatric surgeonsl since the recurrence rate after incomplete surgical excision could be high.
Child* ; Dermoid Cyst ; Diagnosis ; Embryology ; Head* ; Humans ; Lymphangioma, Cystic ; Neck* ; Physical Examination ; Recurrence ; Skin ; Thyroglossal Cyst

No abstract available.
Female ; Mosaicism* ; Ovary*

The embryological and anatomical features of urachal anomalies have been well defined. Because of the variable clinical presentation, uniform guideline for evaluation and treatment are lacking. Although urachal remnants are rarely observed clinically, they often give rise to a number of problems such as infection and late malignant changes. Therefore, a total assessment of the disease with a particular focus on embryology, anatomy and clinical symptoms, as well as the most advisable management, is called for. Twenty six patients with urachal remnants were treated at the Department of Pediatric Surgery from August 1980 to June 1998. Of these 26, 9 were classified as patent urachus, 11 as urachal sinus, 4 urachal cyst, 1 urachal diverticulum and 1 alternating sinus. The group consisted of 11 males and 15 females. The age distribution was 20 neonates, 3 infants, 2 preschoolers and 1 adult. Infection was the most frequent complication and Staphylococcus aureus was the predominant causative microorganism. Fistulogram was performed in 4 cases and ultrasound examination disclosed cysts or sinus in 7 cases. Excision was performed in 24 patients and incision and draniage in 2 cases as a primary treatment. There was no postopreative complication or recurrence.
Adult ; Age Distribution ; Diverticulum ; Embryology ; Female ; Humans ; Infant ; Infant, Newborn ; Male ; Recurrence ; Staphylococcus aureus ; Ultrasonography ; Urachal Cyst ; Urachus

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*

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*