No abstract available.
Twins, Conjoined*

No abstract available.
Hirschsprung Disease*

No abstract available.

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

No abstract available.
Female ; Mosaicism* ; Ovary*

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*

A fetal rhabdomyomatous nephroblastoma is considered to be a predominantly monophasic mesenchymal variant of Wilms' tumor, which acts less aggressively than a conventional Wilms' tumor despite its much larger size. Bilaterality of this tumor in a nine month-old girl, however, may negatively affect the overall prognosis. A radical nephrectomy for bulky masses in the left kidney and a partial nephrectomy for right kidney with five small tumor masses was performed at the same time. Two small tumor masses in the upper part of right kidney were left behind because of preserving minimal renal functional capacity. Pathological study revealed a mixed type of nephroblastoma which was composed predominantly of mesenchymal components with fetal rhabdomyomatous differentiation. After post-operative chemotherapy with vincristine, actinomycin D and adriamycin, and radiotherapy(2,130 rad), residual tumor masses became a single tumor 5 cm in diameter and well demarcated, which was resected at 15 months after first operation when the size and renal function of remained right kidney was appropriate to resect out the residual tumor. The tumor resected out at second operation was entirely composed of scattered differentiated fetal skeletal muscle cells in the fibrovascular tissue. Only a few entrapped epithelial components were seen but no blastemal cornponents were present. Follow up abdominal CT and ultrasonographic examinations revealed no evidence of tumor recurrence. The girl has developed normally without disease.

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

Lymphangiomas or cystic hygromas are quite commonly seen in children. During a 22-year period, from January 1980 to December 2001, 117 patients with lymphangioma were treated and followed in the Department of Pediatric Surgery at Hanyang University Hospital. The male-to-female ratio was 1.9:1 (77:40) with a male preponderance. As for the age incidence at time of diagnosis, 10 (8.5 %) patients were noted under 1 month of age, 37 (31.6 %) were between 1 month and 1 year of age, 12 (10.3 %) between 1 and 2 years of age, so 59 (50.4 %) were under 2 years of age. Sixty one (52.1 %) lymphangioma-cases were located in the neck, including one case at the nape. Axilla was the second in frequency and the rest were scattered at various sites. The intrascrotal lymphangioma is very rare but we have experienced one case of primary intrascrotal lymphangioma. Eighteen (15.4 %) cases were located in the intraabdominal area, 10 in the mesentery, 2 in the greater omentum and 6 in the retroperitoneum. The chief complaints of intraabdominal lymphangioma were abdominal pain, intestinal obstruction, inguinal hernia, palpable mass, and/or abdominal distension. Among 77 histologically proven cases, 14 cases were cavernous lymphangiomas and the rest were cystic lymphangiomas. Bleeding in the lymphangioma was noted in 20 (17.1 %) cases of all. As for the treatment, a complete excision was performed in 77 (65.8 %) patients and AgNO3 sclerotherapy after incomplete excision was performed in 23 (19.7 %). Picibanil (OK-432) sclerotherapy was performed in 17 (14.5 %) patients. Recurrence rate was 7.7 % and mortality occurred in one case who had a large neck lymphangioma extending into the mediastinum.
Abdominal Pain ; Axilla ; Child* ; Diagnosis ; Hemorrhage ; Hernia, Inguinal ; Humans ; Incidence ; Intestinal Obstruction ; Lymphangioma* ; Lymphangioma, Cystic ; Male ; Mediastinum ; Mesenteric Cyst ; Mesentery ; Mortality ; Neck ; Omentum ; Picibanil ; Recurrence ; Sclerotherapy