Recently, amniotic fluid has gained attention as one of the potential sources for cell therapy and tissue engineering because it has characteristics of multipotent stem cells. However, current knowledge about what types of cells are naturally found in amniotic fluid is still limited. In this study, we aimed to investigate whether human amniotic fluid contains cells that have characteristics of respiratory cells. Samples of human amniotic fluid (5 mL per sample) obtained from amniocenteses were cultured with small airway growth medium (SAGM). Cells were grown until the third passage and the presence of type II alveolar cells were characterized by inverted microscopy, immunofluorescence, and reverse transcription polymerase chain reaction (RT-PCR). On inverted microscopy, cultured cells showed typical polygonal and cobblestone-like epithelial morphology. The morphology of cells was not changed after selection and passing. Immunofluorescence analysis demonstrated that the isolated cells stained positive for surfactant protein C (SPC), specific marker for type II alveolar cells. Cells also stained positive for TTF-1 protein but negative for CD 31 and vimentin. RT-PCR analysis of cells showed expression of SPC mRNA. This study has demonstrated that respiratory cells can be isolated and identified from human amniotic fluid cultured in SAGM medium. Our results may provide the basis for further investigations of amniotic fluid.
Choledochal cyst is a congenital dilatation of the bile duct. Intrahepatic bile duct dilatation of type IVa by Todani's classification at the time of diagnosis resolved spontaneously after cyst excision and hepaticojejunostomy in many cases. It should be distinguished from the true cystic dilatation of the intrahepatic ducts, which tends to persist, albeit after some regression. We therefore studied postoperative intrahepatic duct dilatation changes in choledochal cyst. A total of seventy-six choledochal cysts were managed at the Division of Pediatric Surgery, Department of Surgery, Samsung Medical Center from May 1995 to December 2005. The ratio of males to females was 1:2.8. Preoperative radiologic diagnosis by Todani's classification was Type I (n=52, 68.4 %), II (n=1, 1.3 %), IVa (n=23, 30.3 %). Among fifty-five patients with intrahepatic bile duct dilatation we were able to follow up forty-eight by ultrasonography. Twenty-two patients were type IVa, and twenty-six patients were type I and showed intrahepatic duct dilatation. Mean follow-up duration was 35.3 months (9~105 months). Complete regression of dilated intrahepatic duct was observed in fifteen patients of type IVa and twenty-four patients of type I. Incomplete regression of dilated intrahepatic duct was observed in six patients in type IVa and two patients in type I. Only one patient in type IVa showed no change in ductal dilatation during a follow-up period of 15 months. We conclude that true type IVa is much less frequent than what was diagnosed preoperatively by imaging study. Therefore in type IVa patients who are diagnosed preoperatively the decision to perform liver resection should be carefully considered. Postoperative long term follow up of choledochal cyst with intrahepatic bile duct dilation is needed.
The survival of Very Low Birth Weight (VLBW) infants has been improved with the advancement of neonatal intensive care. However, the incidence of accompanying gastrointestinal complications such as necrotizing enterocolitis has also been increasing. In intestinal perforation of the newborn, enterostomy with or without intestinal resection is a common practice, but there is no clear indication when to close the enterostomy. To determine the proper timing of enterostomy closure, the medical records of 12 VLBW infants who underwent enterostomy due to intestinal perforation between Jan. 2004 and Jul. 2007 were reviewed retrospectively. Enterostomy was closed when patients were weaned from ventilator, incubator-out and gaining adequate body weight. Pre-operative distal loop contrast radiographs were obtained to confirm the distal passage and complete removal of the contrast media within 24-hours. Until patients reached oral intake, all patients received central-alimentation. The mean gestational age of patients was 26+2 wks (24+1~33+0 wks) and the mean birth weight was 827 g (490~1450 g). The mean age and the mean body weight at the time of enterostomy formation were 15days (6~38 days) and 888 g (590~1870 g). The mean body weight gain was 18 g/day (14~25 g/day) with enterostomy. Enterostomy closure was performed on the average of 90days (30~123 days) after enterostomy formation. The mean age and the mean body weight were 105 days (43~136 days) and 2487 g (2290~2970 g) at the time of enterostomy closure. The mean body weight gain was 22 g/day after enterostomy closure. Major complications were not observed. In conclusion, the growth in VLBW infants having enterostomy was possible while supporting nutrition with central-alimentation and the enterostomy can be closed safely when the patient's body weights is more than 2.3 kg.
Neuroblastoma is the most common extracranial solid tumor in children. We retrospectively analyzed the results of neuroblastoma treatment of 191 patients (116 males and 75 females) treated between January 1986 and December 2005 at the Department of Pediatric Surgery and the Department of Pediatrics, Seoul National University Children's Hospital. The mean age at diagnosis was 3.1 years (0.1 yrs - 13.5 yrs). Forty-seven patients were under 1 year of age. The mean follow-up period was 57.3 months (24 days - 19.1 yrs). Patients were classified into two groups according to the completeness of resection of the primary tumor; (1) gross total resection (GTR) and (2) incomplete resection (IR). The number of patients in stages I, II, III, IV, IV-S were 17 (8.9 %), 12 (6.3 %), 43 (22.5 %), 114 (59.7 %), 4 (2.1 %), respectively. GTR was achieved in 120 patients and IR in 71 (22 stage III, 47 stage IV, 1 stage IV-S, 1 brain). Overall survival (OS) was 65.2 % and event-free survival (EFS) was 48.6 %. EFS were 100 %, 75 %, 66.8 %, 31.3 %, 75 % at stage I, II, III, IV, IV-S, respectively. There was no significant difference in EFS according to the completeness of resection. EFS was improved in GTR group (p=ns) of stage III, but by contrast, stage IV patients showed worse EFS in GTR group. EFS was improved significantly after the introduction of autologous stem cell transplantation (ASCT) (58.1% vs. 40.6%, p=.029). The EFS improved significantly after the introduction of ASCT in IR group (p=.009) rather than GTR group (p=ns). The EFS of the patients under 1 year of age (N=47) was better than the patients over 1 year of age (N=144) significantly (75.5 % vs. 39.4 %, p=.0034). The prognosis of neuroblastoma was related to the INSS stage and age at diagnosis. The survival of IR group significantly improved after ASCT.
With the widespread use of the obstetrical ultrasound, identification of a fetal suprarenal mass becomes more common. Most of these masses prove to be congenital neuroblastomas (CNB) postnatally. However, the diagnosis is often confused with other benign lesions and the post-natal management remains controversial. The medical records of 13 patients that underwent primary surgical excision for an antenatally detected adrenal CNB, between January 1995 and April 2009, were reviewed retrospectively. The clinical, radiological, surgical, and pathological data on the suprarenal mass were collected. Staging evaluation was performed after histological confirmation of the CNB. Most of the CNBs were stage I (N=11), with 1 stage IV and 1 stage IV-S. Four patients (3 stage I and 1 stage IV-S) had N-myc gene amplification. The stage I patients were cured by surgery alone, and stage IV patients underwent 9 cycles of adjuvant chemotherapy and currently have no evidence of disease after 39 months of follow-up. The patient with stage IV-S is currently receiving chemotherapy. There were no post-operative complications. For early diagnosis and treatment, surgical excision should be considered as the primary therapy for an adrenal CNB detected before birth. The surgery can be safely performed during the neonatal period and provides a cure in most cases. Surgical diagnosis and treatment of CNB is recommended in neonatal period.
This study is a retrospective analysis of 1244 cases of the inguinal hernia in children under the age of fifteen years who were operated at the department of pediatric surgery, Inje University Busan Paik Hospital from March, 1997 to February, 2007. The ratio of male to female was 3.6:1. The type of hernia was indirect in all of the cases. The hernia was on the right side in 656 cases (53.9 %), left side in 467 cases (37.5 %), and bilateral in 121 cases (9.7 %). The hernia presented most frequently in infants under age 12 months; 364 cases (29.2 %). Fifty-nine cases (21.7 %) were in female and 305 cases (31.3 %) in male. There were 428 cases (33.6 %) in 1-3 years age group, 295 cases (23.7 %) in 4-6 years, 112 cases (9.0 %) in 7-9 years, 39 cases (3.1 %) in 10-12 years and 16 (1.2 %) in 13-15 years. The content of hernia sac was small bowel (59 %), omentum (31 %) in males and the ovary and tube (54 %) and small bowel (26 %) in female. The incidence of combined operation at the time was 3.2 %, and consisting of orchiopexy (67.5 %), frenulotomy (12.5 %), appendectomy (10 %), circumcision (5 %), and fistulotomy (5%). The incidence of combined disease was 2.8 % and consisting of undescended testis, Hirschsprung's disease, idiopathic hypertrophic pyloric stenosis, imperforate anus, and congenital heart disease. After unilateral inguinal hernia repairs, contralateral hernias developed in 34 patients. The laterality of the primary site of hernias were left in 19 cases (55.8 %), and right 15 cases (44.1 %). The 936 cases (75.2 %) were operated under general anesthesia; Mask bagging 663 cases (53.2 %), endotracheal intubation 257 cases (20.6 %), and laryngeal mask 16 cases (1.2 %). The remainder 308 cases (24.7 %) were operated under regional caudal anesthesia.
Situs inversus abdominis is a rare congenital condition commonly associated with serious cardiac and splenic malformations. The importance of recognizing the presence of situs inversus abdominis preoperatively is emphasized by the fact that the surgical incision is placed on the incorrect side of the abdomen. A 6 day-old girl was referred to our hospital because of bile stained vomiting. A plain radiography of abdomen and chest showed the heart to be normal position and a reversed "double-bubble" picture with no other gas shadow in the rest of the abdomen. Abdominal computed tomography scan revealed situs inversus with the stomach and polysplenia on the right side and the liver on the left side. A laparotomy confirmed the diagnosis of situs inversus with duodenal atresia. The obstruction was bypassed by constructing a side-to-side duodenoduodenostomy. The postoperative course was uneventful.
Splenic cystic lesion is uncommon in children, and cystic lymphangioma of the spleen has not been reported in Korean pediatric patients. Here we report a case of cystic lymphangioma arising from the spleen in a 16 year-old male. The patient presented with left flank pain for 5 days after blunt trauma to the same site. On physical examination, left abdominal tenderness and a palpable spleen were noted. Abdominal ultrasound and MRI revealed multiple septated macro-cystic mass abutting to the spleen medially, suggestive of cystic lymphangioma of the spleen. Laparotomy revealed a 20 cm sized cyst in the spleen, and 2,000mL of dark-brownish fluid was aspirated from the cyst. Splenectomy was performed. Pathological examination revealed the cystic lymphangioma. Post-operative recovery was uneventful, and the patient was discharged at 7 days after surgery.
Neuroblastoma is the most common extracranial solid tumor in children, and accompanies various clinical symptoms including hypertension. Hypertension is associated with catecholamines secreted from the tumor, and is usually not severe. We report one case of malignant hypertension with cardiac failure in a patient with adrenal neuroblastoma, successfully treated with adrenalectomy. A 3 year-old boy complained of protrusion of the chest wall. Physical examination revealed severe hypertension with cardiac failure. The levels of metabolites of catecholamine were increased in blood (norepinephrine >2000 pg/mL) and urine (norepinephrine 1350.5 ug/day). Abdominal CT showed a 7 cm-sized solid mass arising from the right adrenal gland. After stabilizing the hemodynamics with oral phenoxybenzamine, right adrenalectomy was performed. Pathological diagnosis was a ganglioneuroblastoma. The hypertension and cardiac failure were resolved after tumor removal.
Sertoli-Leydig cell tumor is a rare sex-cord stromal tumor of the ovary. They make up less than 0.5 % of all ovarian tumors. We experienced a case of an ovarian Sertoli-Leydig cell tumor in a 4 year-old girl who presented with nausea, vomiting, and lower abdominal pain of 2 days' duration. On physical examination, there was mild tenderness in the right lower quadrant of the abdomen. Abdominal ultrasonography and computed tomography (CT) scan revealed a pelvic mass measuring 5 × 3 cm that appeared to arise from the right ovary. At exploratory laparotomy, a 6 × 5 × 3 cm solid right ovarian mass without torsion was found. A right salpingo-oophorectomy was performed. The postoperative course was uneventful. The child was discharged 5 days after surgery.
Extraosseous Ewing's sarcoma is a rare primary malignant soft tissue tumor which is histologically identical to Ewing's sarcoma. This tumor tends to involve the soft tissue of the lower extremity and paravertebral region of adolescents and young adults but particularly rare in infants. We recently experienced a case of extraosseous Ewing's sarcoma which presented in the left arm of 4 months infant.