Congenital segmental dilatation of the colon is a very rare entity of unknown etiology, characterized by a localized dilatation of a bowel segment of the colon of variable length and an abrupt transition between the normal and dilated intestine. It can affect any part of the colon, with the rectosigmoid colon being the most commonly affected site. The clinical and radiological features may resemble that of Hirschsprung disease, but differ in that the normal ganglion cells are found in the dilated and normal segment of the colon. We performed laparoscopic-assisted transanal endorectal pull-through for segmental dilatation of rectosigmoid colon in an 8-year-old boy with chronic constipation since the age of 5 months.
Child* ; Colon* ; Constipation ; Dilatation* ; Ganglion Cysts ; Hirschsprung Disease ; Humans ; Intestines ; Male

We experienced a case of a 4-year-old girl presenting with vomiting, abdominal pain and mobile mass in the periumbilical area. On physical examination, the abdomen was soft and a 10x10 cm sized, painless firm mobile round mass was palpated in the periumbilical area. An abdominopelvic computed tomography scan showed a huge hypodense mass with encapsulation and multiple septation in the left lower quadrant of the abdomen, compressing the intestinal loops. At operative exploration, she was found to have a soft, yellowish round mass originating from the ileal mesentery, situated 10 cm proximal to the ileocecal valve. The mass was completely excised together with the adjoining ileum, and bowel continuity was restored. Histopathologic diagnosis revealed a mesenteric lipoma, composed of mature adipocytes with no evidence of necrosis or malignancy. The postoperative course was uneventful.
Abdomen ; Abdominal Pain ; Adipocytes ; Child ; Humans ; Ileocecal Valve ; Ileum ; Lipoma ; Mesentery ; Necrosis ; Physical Examination ; Preschool Child ; Vomiting

PURPOSE: To compare the efficacy of a 20-gauge and an 18-gauge needle in sono-guided percutaneous automated gun biopsy for establishing the specific diagnosis of renal parenchymal disease in pediatric kidneys. MATERIALS AND METHODS: In 60 pediatric patients with renal parenchymal diseases, percutaneous sono-guided gun biopsy was performed by an experienced radiologist. In two groups of 30 patients, regardless of their age, two needle passes were performed, using alternately an 18-gauge or a 20-gauge biopsy needle. The core of renal tissue thus obtained was examined with light, immunofluorescent or electron microscopy by the renal pathologist. The mean number of intact glomeruli of whole tissue core per biopsy, as seen on the light microscopy, and post-bioptic complications were compared between the two different needle size groups. RESULTS: The number (mean+/-1 standard deviation) ofglomeruli obtained per biopsy was 17+/-8 in the 18-gauge needle group, and 14+/-5 in the 20-gauge group. Between two groups, there was no major post-bioptic complication requiring specific treatment, nor a statistically significant difference in the frequency of minor complications. CONCLUSION: Even though more glomeruli were obtained with an 18-gauge needle, the number obtained with a 20-gauge needle also permitted adequate pathologic examination. Both an 18-gauge and a 20-gauge needle may thus be suitable for renal biopsy in pediatric patients.
Biopsy* ; Diagnosis ; Humans ; Kidney ; Microscopy ; Microscopy, Electron ; Needles*

Four children with solid and papillary epithelial neoplasm of the pancreas are reported. Three were girls. Mean age at operation was 12 years and 7 months (10-13 years). Clinical presentation included nausea, vomiting, and apalpable mass. One had hemoperitoneum due to tumor rupture. In two cases, tumors were in the body of the pancreas, and one the body and tail, and in one,the tail. Mean diameter of the tumors was 10.8 cm (8-15cm). Surgical procedures were distal pancreatectomy and splenectomy in 2 cases, distal pancreatectomy in one, and subtotal pancreatectomy and splenectomy in one. Mean follow-up period was 61 months (6-121 months). Three patients are still alive without any recurrence. However, in the one case of ruptured tumor, portal vein thrombosis and liver metastasis developed after subtotal pancreatectomy and splenectomy during the course of postoperative adjuvant chemotherapy.
Chemotherapy, Adjuvant ; Child* ; Drug Therapy ; Female ; Follow-Up Studies ; Hemoperitoneum ; Humans ; Liver ; Nausea ; Neoplasm Metastasis ; Neoplasms, Glandular and Epithelial* ; Pancreas* ; Pancreatectomy ; Recurrence ; Rupture ; Splenectomy ; Venous Thrombosis ; Vomiting

Four children with solid and papillary epithelial neoplasm of the pancreas are reported. Three were girls. Mean age at operation was 12 years and 7 months (10-13 years). Clinical presentation included nausea, vomiting, and apalpable mass. One had hemoperitoneum due to tumor rupture. In two cases, tumors were in the body of the pancreas, and one the body and tail, and in one,the tail. Mean diameter of the tumors was 10.8 cm (8-15cm). Surgical procedures were distal pancreatectomy and splenectomy in 2 cases, distal pancreatectomy in one, and subtotal pancreatectomy and splenectomy in one. Mean follow-up period was 61 months (6-121 months). Three patients are still alive without any recurrence. However, in the one case of ruptured tumor, portal vein thrombosis and liver metastasis developed after subtotal pancreatectomy and splenectomy during the course of postoperative adjuvant chemotherapy.
Chemotherapy, Adjuvant ; Child* ; Drug Therapy ; Female ; Follow-Up Studies ; Hemoperitoneum ; Humans ; Liver ; Nausea ; Neoplasm Metastasis ; Neoplasms, Glandular and Epithelial* ; Pancreas* ; Pancreatectomy ; Recurrence ; Rupture ; Splenectomy ; Venous Thrombosis ; Vomiting

Spontaneous gastric perforation in the newborn is a rare disease that requires early diagnosis and prompt surgical treatment. Between 1988 and 2001 at the Department of Pediatric Surgery, Kyungpook National University Hospital, 9 cases of spontaneous gastric perforation were treated. Seven were males and two females. The mean gestational age and birth weight were 36.7 weeks and 2,455 grespectively. All patients presented with severe abdominal distention and pneumoperitoneum on cross table lateral film of the abdomen. Perforations were located on the anterior wall along the greater curvature of the stomach in six and on the posterior wall along the greater curvature in two. One case showed two sites of perforation on the anterior and posterior wall along the greater curvature. Six patients were managed with debridement and primary closure and the others with debridement and partial gastrectomy. Peritoneal drainage was not performed. There were four deaths; two from sepsis due to leakage from the anastomotic site, one as a result of acute renal failure, and the other by associated respiratory distress syndrome. Spontaneous gastric perforation in the newborn is usually located along the greater curvature. Elevated intragastric pressure is a possible cause of the perforation. Poor prognosis is related to associated diseases and prematurity.
Abdomen ; Acute Kidney Injury ; Birth Weight ; Debridement ; Drainage ; Early Diagnosis ; Female ; Gastrectomy ; Gestational Age ; Gyeongsangbuk-do ; Humans ; Infant, Newborn* ; Male ; Pneumoperitoneum ; Prognosis ; Rare Diseases ; Sepsis ; Stomach

There have been a few reports of familial anorectal malformations extending over more than one generation. We experienced a case of a family with 3 members spanning 2 generations affected with isolated low type anorectal malformations. They had same low type of anorectal malformations. In all 3 patients, a perianal anoplasty was performed.
Family Characteristics ; Humans

Chronic obstructive pulmonary disease (COPD) is characterized by persistent airflow limitation that is usually progressive. It is a major cause of morbidity and mortality worldwide, leading to substantial and increasing economic and social burden. Palliative care for COPD patients aims to reduce symptoms and exacerbations and improve exercise tolerance and quality of life. It is difficult to make a prognosis for COPD patients due to the variable illness trajectory and advanced care of patients. However, severity of breathlessness, assessment of lung function impairment, and frequency of exacerbations can help to identify palliative care needs and determine effective methods to mitigate symptoms, which is discussed in this paper. In these patients, it is recommended to provide individualized palliative care along with curative/restorative care at the onset of COPD symptoms. Before launching a palliative care system in Korea, it is necessary to prepare pulmonary rehabilitation resources, patient-centered communication, timely palliative responsiveness, and a program for effective advanced care planning. A multidisciplinary approach involving collaboration with not only the respiratory and palliative care teams but also primary care offers a new model of care for these patients and should be considered with a priority.
Cooperative Behavior ; Dyspnea ; Exercise Tolerance ; Hospice Care ; Hospices* ; Humans ; Korea ; Lung ; Mortality ; Palliative Care* ; Primary Health Care ; Prognosis ; Pulmonary Disease, Chronic Obstructive* ; Quality of Life ; Rehabilitation ; Respiratory Therapy

Median raphe cyst (MRC) of the perineum is rare congenital midline cyst of the male genitalia. MRC is thought to be caused by congenital alterations in the embryologic development of the male genitalia during fetal life. MRC can be found on the midline position between the urethral meatus and the anus. The lesion can be cystic, but sometimes it looks like an elongated configuration called a raphe canal. Diagnosis in childhood is particularly rare because they are usually asymptomatic, but some cases have reportedly been identified after infection. Although conservative treatment can be possible in small asymptomatic lesions, the treatment of choice is simple excision followed by primary closure in symptomatic cases. We describe here the case of 2-year-old boy presented at our institution with a 10-month history of anomaly of the perineal median raphe, which was treated by surgical excision.
Anal Canal ; Child, Preschool* ; Diagnosis ; Genitalia, Male ; Humans ; Male* ; Perineum

No abstract available.
Endodermal Sinus Tumor ; Follow-Up Studies ; Germ Cells ; Liver ; Neoplasms, Germ Cell and Embryonal