Living-donor Sequential ABO-incompatible Kidney Transplantation after Liver Transplantation in a Patient with Alcoholic Liver Cirrhosis and End-stage Renal Disease / 대한이식학회지

A 47-year-old man developed chronic alcoholic liver cirrhosis and end-stage renal disease. He underwent blood-type-compatible liver transplantation with a graft from his daughter. After 8 months, sequential ABO-incompatible (ABOi) kidney transplantation was performed, with his brother as the donor (A to O). The patient had anti-A antibody titers (1:256). We performed pretransplant desensitization, including administration of rituximab, mycophenolate mofetil, tacrolimus, and prednisolone 2 weeks before the scheduled transplantation, and plasmaphresis (PP) and administered an intravenous immunoglobulin injection. The patient underwent PP before kidney transplantation until the anti-A antibody titer was <1:8. The patient achieved normal renal function within 4 posttransplantation days. Postoperative bleeding (diffuse hemorrhage) requiring additional blood transfusions and radiological intervention (drainage procedure) occurred 9 days after transplantation. The patient was discharged on day 20 of hospitalization. Nine months after the kidney transplantation, the recipient's and donor's liver and kidney functions were normal. ABOi renal transplantation after liver transplantation can be successfully performed in patients with high baseline anti-ABO antibody titers after preconditioning with rituximab and PP, and quadruple immunosuppressive therapy. However, caution is required regarding an increased risk of bleeding complications.

The Prognosis of Gastroschisis and Omphalocele / 소아외과

PURPOSE: Gastroschisis and omphalocele are major anterior abdominal wall defects. The purpose of this study was to analyze the clinical differences and mortalities of gastroschisis and omphalocele in Asan Medical Center. METHODS: A retrospective review of the medical records was conducted of 103 cases of gastroschisis and omphalocele from September 1989 to February 2013 in Asan Medical Center in Korea. RESULTS: There were 43 cases (41.7%) of gastroschisis and 60 cases (58.3%) of omphalocele. There was a female predominance in both gastroschisis (60.5%) and omphalocele (58.3%). The average gestational age at delivery was 36.7+/-0.4 weeks for both groups. The mean birth weights were 2,381.9+/-80.6 g for gastroschisis and 2,779.4+/-82.8 g for omphalocele (p=0.001). Mean maternal ages in the gastroschisis and omphalocele groups were 27.5+/-0.7 years and 30.5+/-0.7 years, respectively (p=0.002). Associated malformations were documented in 13 infants (30.2+/-) with gastroschisis and 46 infants (76.7+/-) with omphalocele (p<0.001). All of gastroschisis patients except one underwent surgery including 31 primary repairs and 11 staged repairs. Fifty-two infants with omphalocele underwent surgery-primary repair in 41 infants and staged repair in 11 infants. Among 103 cases, 19 cases (18.4%) expired. Mortality rates of gastroschisis and omphalocele were 23.3% (10/43 cases) and 15.0% (9/60 cases), respectively (p=0.287). The main causes of death were abdominal compartment syndrome (6/10 cases) in gastroschisis, respiratory failure (4/9 cases) and discharge against medical advice (4/9 cases) in omphalocele. CONCLUSION: Gastroschisisis was associated with younger maternal age and lower birth weight than omphalocele. Associated malformations were more common in omphalocele. The mortality rates did not make a statistical significance. This might be the improvement of treatment of cardiac anomalies, because no patient died from cardiac dysfunction in our study. Furthermore, abdominal compartment syndrome might be the main cause of death in gastroschisis.

The surgical experience for retroperitoneal, mesenteric and omental cyst in children / 대한외과학회지

PURPOSE: Intra-abdominal cystic masses originating from the retroperitoneum, mesentery or omentum are very rare and mostly benign tumors, but sometimes present as a complicated cyst encasing the major organs. METHODS: We analyzed the clinical findings, histologic diagnosis, and surgical outcomes in children who underwent operation for retroperitoneal, omental, and mesenteric cyst from 1998 to 2010, retrospectively. RESULTS: Twenty-three patients (male, 12; female, 11) underwent the operation at a median age of 46 months (range, 9 days to 16 years). Among them, 17 cysts presented one or two symptoms such as abdominal mass, abdominal pain or abdominal distension. The median duration of symptoms was 7 days (range, 1 day to 365 days). Five were detected prenatally. Ten cysts were found in retroperitoneum, 8 in the omentum and 5 in the mesentery. The median diameter was 13 cm (range, 3 to 30 cm). Twenty cysts were completely removed. Five mesenteric cysts required bowel resection and anastomosis. Three of retroperitoneal cysts were impossible to complete excise because of location and extensiveness. Pathologically, 20 cysts were lymphangioma and 3 were pseudocyst. The morbidity was one of adhesive ileus and the mortality was one who had extensive retroperitoneal cyst with mesenteric cyst. He died from sepsis. During follow-up period, there was no recurrence. CONCLUSION: Preoperative diagnosis and localization for these cysts are very difficult. Complete excision was possible in almost all cases despite the size, bringing a favorable outcome. The possibility of this disease entity should be considered as the cause of acute abdomen.

Clinical Experience with Colonic Atresia

Colonic atresia (CA) is the rare cause of intestinal obstruction, and diagnosis of CA is difficult. But only few research has been performed, so little information has been available. The purposes of this study was to analyze the clinical findings of CA so that help physicians make decision properly. Children with CA who were treated at the division of pediatric surgery at Asan Medical Center in the period from January 1989 to December 2011 were evaluated retrospectively. A total of 6 children were treated with CA. These accounted for 2.7% of all gastrointestinal atresias managed in Asan Medical Center. Only one child was premature and low birth weight, the others were fullterm neonates and showed normal birth weight. Vomiting and abdominal distension were common symptoms and simple X-ray and barium study were used for diagnose of CA. But only 66.7% of the babies were diagnosed as CA pre-operatively. And 2 children out of 6 underwent re-operation due to missed CA at the time of the first operation. In aspect of types of atresia, the type IIIa were two, type IV were two, type I was one case, and one child showed rectal stenosis due to rectal web. Various operations were done according to individual findings and associated diseases. The 50% (n=3) of children underwent the primary anastomosis and the others (n=3) underwent colostomy first and staged operation later for missed CA or associated disease. All of them were recovered any significant complications. Therefore, the prognosis of CA is satisfactory if diagnosis and surgical management could be made properly. But because of the low incidence of CA, delay of diagnosis and treatment may occur. To prevent delay of diagnosis, we suggest prompt evaluation of doubtful infant and careful inspection of distal patency of bowel including whole colon and rectum when operating patients with intestinal atresia at any level.

Adrenocortical tumors in children 18 years old and younger / 대한외과학회지

PURPOSE: Pediatric adrenocortical tumors (ACTs) are rare. We reviewed findings in 8 children, 18 years of age or younger, diagnosed with ACT in our institution over the past 15 years. METHODS: We retrospectively reviewed 8 children with ACTs treated between 1996 and 2010. RESULTS: Three girls and 5 boys were treated for ACTs; their median age at presentation was 144 months (range, 28 months to 18 years). Seven patients showed signs of endocrine dysfunction, 4 with Cushing syndrome, 2 with virilization, and 1 with hyperaldosteronism. One patient, with symptoms of hematuria, underwent a computed tomography scan, which showed an adrenal mass. The median duration of symptoms prior to resection was 6 months (range, 1 to 24 months). Five patients had adenomas and 3 had carcinomas. All underwent complete resection of the tumor, with laparoscopic adrenalectomy performed on 3 patients with adenoma and 1 with carcinoma. The median tumor weight was 12.5 g (range, 1 to 130 g) and the median tumor volume was 18.3 cm3 (range, 2.2 to 299.2 cm3). At a median follow-up of 5.1 years (range, 4 months to 15 years), all 8 patients remain alive with no recurrence of disease. CONCLUSION: The characteristics of pediatric ACTs vary considerably. Laboratory findings, clinical hormonal features, and tumor size could not distinguish adenomas from carcinomas before surgery. Complete tumor resection was successful, with no tumor recurrence. However, the small number of patients and short follow-up period limit assessments of prognosis.

Single Port Laparoscopic Appendectomy in Pediatric Patients: A Preliminary Experience

PURPOSE: The authors retrospectively compared single-port laparoscopic appendectomy (SPLA) with conventional laparoscopic appendectomy (CLA) in terms of the perioperative outcomes in pediatric patients. METHODS: Cases of laparoscopic appendectomy between December 2009 and July 2010 were retrospectively reviewed. The demographic data, operating time, pathology of the appendix, hospital stay and surgical morbidities were compared. RESULTS: Ten patients underwent SPLA and 57 underwent CLA. The mean age of the CLA and SPLA groups were 11.2 and 10.6 years, respectively. The pathology of the appendix showed that relatively more severe cases were included in the CLA group. There were 2 cases of a post operative intra-abdominal abscess and 2 cases of an umbilical wound infection in the SPLA group. Therefore, the SPLA group showed a significantly higher surgical morbidity rate (p=0.003) and hospital stay (p=0.05). The mean operation time of the SPLA and CLA group was 88.1+/-30.1 and 71.6+/-26.3 minutes, respectively; the mean operation time of the SPLA group was slightly longer (p=0.054). On the other hand, surgical morbidity and the mean operation time showed a decreasing tendency in the latter half of 10 cases. CONCLUSION: The initial experience of SPLA in pediatric patients shows a higher complication rate and longer hospital stay than CLA. On the other hand, the complication rates and operation time have been decreasing. The clinical outcomes are expected to improve after going through a learning curve.

Operative Management of Complicated Meckel's Diverticulum Laparotomy and versus Laparoscopic Assisted Surgery

Meckel's diverticulum (MD) has various clinical presentations and due to the limitation of imaging studies, pre-operative diagnosis is a challenge in pediatric patients. Recently, laparoscopic exploration has been suggested as a favorable method for the diagnosis and treatment of complicated MD. We investigated the results of laparoscopic-assisted surgery compared with open technique. We retrospectively studied patients who underwent resection of complicated MD at our institute from 1997 to 2010 and compared 11 treated by laparoscopic-assisted diverticulectomy (LD) with 11 treated by open diverticulectomy (OD) for complicated MD. Operation time was not significantly different in the two groups. Hospital stay and time to diet were not significantly different. Two patients were re-admitted due to mechanical ileus in the LD group. None of patients in either group needed re-operation. Considering the possibility of false-positive results with imaging studies and the cosmetic benefit, laparoscopic-assisted surgery is a safe and effective treatment modality to diagnose and treat complicated Meckel's diverticulum.

Clinical Experience of Currarino Syndrome

Currarino syndrome is a hereditary syndrome characterized by the triad of a sacral bony defect, presacral mass and anorectal malformation. We retrospectively reviewed 13 Currarino syndrome patients who were treated in our center between 1997 and 2010. Demographic data, initial symptoms, initial diagnosis, pathologic diagnosis of presacral mass, associated anomalies and managements were analyzed. There were 8 boys and 5 girls. Four patients were diagnosed as Currarino syndrome immediately after birth with failure of passage of meconium and abdominal distension. Four patients underwent surgery for imperforate anus immediately after birth and were diagnosed as Currarino syndrome later and underwent reoperation. Three patients were diagnosed during work-up and management with of the tentative diagnosis of Hirschsprung's disease. Diagnosis of the remaining two patients was at the age of 26 months and 9 years and anorectal malformation was not associated. Twelve patients showed hemi-sacrum and one patient showed bilateral sacral subtotal agenesis. Two patients without anorectal malformation underwent presacral mass excision, untethering of spinal cord and repair of myelomeningocele. Six out of 8 patients, excluding 3 that expired or were lost to follow up, with anorectal malformation underwent colostomy, presacral mass excision, untethering of spinal cord, repair of myelomeningocele, posterior sagittal anorectoplasty and colostomy repair. One patient underwent only posterior sagittal anorectoplasty after colostomy. One waits the scheduled operation only with Hegar dilatation. Pathologic examation of presacral masses showed myelomeningoceles in 4 patients, lipomyelomeningoceles in 3 patients and dermoid cyst in one patient. Teratoma was combined in 2 patients. Eight patients needed neurosurgical operation for spinal cord problems. Seven patients had urologic anomalies and two of them underwent operation. Currarino syndrome should be considered as a differential diagnosis in pediatric patients with abdominal distension, constipation and anorectal malformation. For proper evaluation and treatment, a multi-disciplinary approach is recommended.

Extracorporeal Membrane Oxygenation in Neonates with Congenital Diaphragmatic Hernia: a Preliminary Experience

Extracorporeal membrane oxygenation (ECMO) has been utilized in congenital diaphragmatic hernia (CDH) patients with severe respiratory failure unresponsive to conventional medical treatment. We retrospectively reviewed 12 CDH patients who were treated using ECMO in our center between April 2008 and February 2011. The pre ECMO and on ECMO variables analyzed included gestational age, sex, birth weight, age at the time of ECMO cannulation, arterial blood gas analysis results, CDH location, timing of CDH repair operation, complications and survival. There were 9 boys and 3 girls. All patients were prenatally diagnosed. Mean gestational age was 38.8 +/- 1.7 weeks and mean birth weight was 3031 +/- 499 gram. Mean age at the time of ECMO cannulation was 29.9 +/- 28.9 hours. There were 4 patients who survived. Survivors showed higher 5 min Apgar scores (8.25 +/- 0.96 vs. 7.00 +/- 1.20, p=0.109), higher pre ECMO mean pH (7.258 +/- 0.830 vs. 7.159 +/- 0.986, p=0.073) and lower pre ECMO PaCO2 (48.2 +/- 7.9 vs. 64.8 +/- 16.1, p=0.109) without statistical significance. The hernia was located on the left side in 10 patients and the right side in 2 patients. The time interval from ECMO placement to operative repair was about 3~4 days in 5 early cases and around 24 in the remaining cases. There were 3 cases of post operative bleeding requiring re operation and 2 cases of abdominal compartment syndrome requiring abdominal fascia reopening. ECMO catheter reposition was required in 4 cases. Three cases of arterial or venous thrombosis were detected and improved with follow up. Our data suggests that ECMO therapy could save the lives of some neonates with CDH who can not be maintained on other treatment modalities. Protocolized management and accumulation of case experience might be valuable in improving outcomes for neonates with CDH treated with ECMO.

Clinical Experience of H-type Tracheoesophageal Fistula

Tracheoesophageal fistula without esophageal atresia (H-type TEF) is a congenital anomaly that is characterized by a fistula between the posterior wall of the trachea and the anterior wall of the esophagus, not accompanied by esophageal atresia. The purpose of this study is to investigate the clinical characteristics, diagnostic time, the side of cervical approach and short term result after surgery by searching medical records of patients treated for H-type TEF. The search was done at University of Ulsan, Department of Pediatric Surgery of Asan Medical Center, and the total number of patients from May 1989 to December 2010 was 9 with M:F ratio of 1:2. The median gestational age was 39(+6) (32(+6)~41(+0)) wks. Seven out of nine patients were born at term and the other two were born premature. The clinical presentation was aspiration pneumonia, difficulty in feeding, chronic cough, vomiting, abdominal distension and growth retardation. The symptoms presented right after birth. The diagnosis was made with esophagography and the median time of diagnosis was 52 days of life. The majority of surgical corrections were performed within two weeks of diagnosis (median; 15d, range; 1d - 6m). Six patients had associated anomalies, and cardiac anomalies were most common. The cervical approach was utilized in all cases (right 2, left 7). Transient vocal cord palsy and minor esophageal leakage complicated two cases. Although the diagnosis of H-type TEF was difficult and often delayed, we had a good short term result. The left cervical approach was preferred.

Clinical experience with persistent cloaca / 대한외과학회지

PURPOSE: Persistent cloaca is one of the most severe types of anorectal malformation. Appropriate initial drainage is difficult due to their various malformations and hydrocolpos or dilated urinary bladder. Corrective surgery also differs among individual patients. We describe our experiences with the surgical management of children with persistent cloaca. METHODS: We retrospectively reviewed 16 children diagnosed with persistent cloaca at Asan Medical Center. RESULTS: Sixteen patients were managed in their neonatal period. Twelve patients had enlarged bladder or vagina at birth. Three patients, who did not undergo cystostomy or vaginostomy at first operation, had earlier complications after surgery or required drainage tube insertion. One patient who did not undergo hydrocolpos drainage died of sepsis and complications. Nine patients underwent corrective surgery; posterior sagittal anorectovaginourethroplasty using the Pena method. Three patients required additional operations due to complications after surgery. CONCLUSION: Patients found to have anatomical malformations before colostomy, as well as hydrocolpos and bladder enlargement, require a vaginostomy with or without a cystostomy to reduce complications. Follow-up is required in patients with hydrocolpos and bladder enlargement to determine whether vaginal drainage improves dilated bladder. Continuous long-term follow-up examination is required to determine the long-term results of corrective surgery.

Outcomes of Pancreas Transplantation: Single-Center Experience in Korea / 대한내과학회지

BACKGROUND/AIMS: Pancreas transplantation (PT) as the ultimate treatment for insulin-dependent diabetes has been the subject of debate clinically. Marked improvements in patient and graft survival, and decreases in postoperative morbidity have been achieved due to technical refinements, improved immunosuppressants, and better postoperative management. Here, we report our 18-year experience with PT performed at our institute. METHODS: All recipients who underwent deceased donor or living donor PT between July 1992 and December 2009 were included. We reviewed the medical records, including operation records, progress, and laboratory findings during follow-up. Graft and patient survival were analyzed using the Kaplan-Meier method. RESULTS: In total, 119 cases of pancreas transplantation were performed between July 1992 and December 2009 at our institute. Indications for pancreas transplantation were type I diabetes in 93 (78.2%) patients and type II diabetes in 16 (13.4%) patients. The transplanted pancreas was obtained from a deceased donor in 108 cases (90.8%) and a living donor in 11 cases (9.2%). Median follow-up duration was 39.3 months posttransplantation (range 0~176 months). Overall graft survival rates at 1, 5, and 10 years were 81.6%, 63.4%, and 57.1%, respectively. Following the introduction of tacrolimus as an immunosuppressant in 1999, graft survival at 1, 5, and 10 years was 89.1%, 72.9%, and 66.2%, and overall patient survival at 1, 5, and 10 years was 93.0%, 86.0%, and 86.%, respectively. CONCLUSIONS: Considering the quality of life and long-term patient survival, PT is an effective treatment strategy in non-obese diabetic patients requiring insulin regardless of the type of diabetes.

Analysis of Complicationin Pediatric Patients with Hickman Catheters

Hickman catheters are tunneled central venous catheters used for long-term venous access in children with malignancies. The appropriate management for various kinds of catheter related complications has become a major issue. We retrospectively analyzed the clinical, demographic, and surgical characteristics in 154 pediatric hemato-oncology patients who underwent Hickman catheter insertion between January 2005 and December 2009. There were 92 boys and 62 girls. The mean age at surgery was 7.6+/-5.1 years old. The mean operation time was 67.4+/-21.3 minutes and C-arm fluoroscopy was used in 47(30.5%). The causes of Hickman catheter removal were termination of use in 82 (57.3%), catheter related bloodstream infection in 44(30.8%), mechanical malfunction in 11(7.7%), and accidents in 6(4.2%). Univariate and multivariate analysis for associated factors with catheter related bloodstream infection showed that there were no statistically significant associated factors with catheter related infection complications. All cases except two showed clinical improvement with catheter removal and relevant antibiotics treatment. The mean catheter maintenance period in patients of catheter removal without complications was 214.9+/-140.2 days. And, The mean catheter maintenance period in patients of late catheter related bloodstream infection was 198.0+/-116.0 days. These data suggest that it is important to remove Hickman catheter as soon as possible after the termination of use. When symptoms and signs of complications were noticed, prompt diagnostic approach and management can lead to clinical improvements.

The Experience of Operative Management in Jejunoileal Atresia

PURPOSE: Intestinal atresia is a common cause of neonatal intestinal obstruction. Recently, the survival rate has been increasing from development of prenatal diagnosis, total parenteral nutrition (TPN) and neonatal intensive care. We evaluated the complication rate and cause of mortality after operative management for jejunoileal atresia. METHODS: We reviewed 62 patients (36 males, 26 females) with jejuno-ileal atresia who underwent operation from 1998 to 2007. RESULTS: There were 37 patients with jejunal atresia and 25 with ileal atresia. The average gestational age was 256+/-16.6 days and birth weight was 2,824+/-620 g. Prenatal diagnosis was performed in 45 patients (72.6%) around gestational age 27 weeks. Within 2nd day after birth, 44 patients (71%) underwent operation. Half of the jejunoileal atresia was type IIIa and type I was in 8, type II was in 3, type IIIb was in 12, and type IV was in 8. The operative treatment was resection & anastomosis in 59 patients and enterotomy & web excision in 3. They started feeding at 12.4+/-11.5 days after operation on average. The average duration of TPN was 26.7+/-23.5 days, and the incidence of cholestasis was 30.6%. Hospital days averaged 36.8+/-26 days. Early complication occurred in 14 patients (intestinal obstruction in 5, sepsis in 4, wound problem in 3, anastomosis leakage in 1, and intraabdominal abscess in 1). Late complication occurred in 7 patients (anastomosis stricture in 4 and intestinal obstruction in 3). There was only one case of mortality due to short bowel syndrome after re-operation for adhesive ileus. CONCLUSION: The operation for intestinal atresia was successful and aggressive management contributed to a low mortality rate.

The Experience of Non-surgical Treatment for Infantile Hepatic Hemangioendothelioma

PURPOSE: Hepatic hemangioendothelioma (HET) is a rare benign vascular tumor in infants. The clinical manifestations are variable from asymptomatic to life-threatening heart failure or Kasabach-Merritt syndrome (KM SD). We report our non-surgical treatment experience for infantile HET. METHODS: We analyzed 15 infants (5 males, 10 females) of HET retrospectively, from January 1989 to December 2007. RESULTS: All except 2 were full-term babies and median birth weight was 3,140 g. The median age at diagnosis was 15 days of life (0~157 days). Three of 5 patients diagnosed prenatally presented heart failure symptoms and the remaining 2 showed KM SD. Among 6 neonates, 3 showed cyanosis due to congestive heart failure. Nine patients showed multiple nodules at both liver lobes. Three asymptomatic patients were followed with radiologic examinations, thus HETs regressed spontaneously in two and decreased in one. Four cases that showed hepatomegaly or increase in size of subcutaneous hemangioma, were treated with prednisolone. All patients improved from tumor related symptoms and tumor size decreased. Four patients (2 patients of heart failure, 1 with rapid progressive hepatomegaly and 1 with congenital heart disease) received interferon-alpha. Among them, 3 showed regression of tumor and the remaining 1 showed decrease of tumor size. Two patients who suffered from severe heart failure received prednisolone and interferon-alpha, and then recovered. In the 2 patients with KM SD, we tried hepatic artery embolization with medical treatment. One survived but the other is not followed after failure of embolization. CONCLUSION: We experienced that many patients who had clinical symptoms associated with HET very early in their life treated with an aggressive nonsurgical treatment in symptomatic patients showed favorable outcome.

Malignant Ovarian Tumor in Children

Malignant ovarian tumors in children are very rare, and consist of about 1% of all childhood malignant tumors. The purpose of this study is to examine the clinical characteristics, treatment, and prognosis for children with malignant ovarian tumors. We retrospectively reviewed the medical records of children under 15 years of age with malignant ovarian tumors who had been treated surgically at Asan Medical Center between 1989 and March 2009. There were 32 patients, ranged in age at surgery from 2 to 15 years (mean; 10.4 years). The median follow-up period was 64.7 months (from 1 month to 188 months). Pathologic diagnosis were; immature teratoma (n=10), mixed germ cell tumor (n=10), and dysgerminoma (n=6). Tumor stage was classified by the staging system of the International Federation of Gynecology and Obstetrics (FIGO). The number of patients in stage I, II, III, and IV were 24 (75%), 2 (6.2%), 4 (12.5%), and 2 (6.1%), respectively. The tumor recurred in 4 patients. Seven patients of group 1 did not receive postoperative adjuvant chemotherapy, and in three of them, the tumor recurred. Twenty-five patients (group 2) underwent postoperative adjuvant chemotherapy, and there was only one recurrence. One patient who did not receive postoperative adjuvant chemotherapy and expired 10 months after operation because of tumor recurrence and distant metastasis. The overall 5-year event free survival (EFS) was 84.2%: group 1 in 44.4%, and group 2 in 95.7%. Tumor recurrence was related to the postoperative adjuvant chemotherapy (p=0.004). In conclusion, proper surgical procedures with relevant postoperative adjuvant chemotherapy might improve clinical results in children with malignant ovarian tumors.

Treatment of Pulmonary Sequestration with Thoracoscopic Approach

Pulmonary sequestration (PS) is a rare congenital malformation of the lower respiratory tract. The anomaly is characterized by absence of communication with the tracheobronchial tree and isolated blood supply from an anomalous systemic vessels. With the utilization of antenatal ultrasound, the diagnosis of asymptomatic neonatal PS has increased. Treatment options include observation, arterial embolization and surgical resection. The aim of the present study is to review the clinical course of PS and to share our experience with thoracoscopic resection. A total of 96 patients with PS were treated at Asan Children's Hospital between 1999 and 2010. The diagnosis of PS was established by CT in the cases managed by observation or embolization, and by tissue pathology in the surgical cases. Medical records and radiographic images were retrospectively reviewed. Thirty-nine patients were managed by embolization and 30 patients by surgery. The remaining 27 patients have been under observation without any procedures. Among 27 observation patients, 1 patient regressed completely and 10 patients were lost to follow up. Of the 39 embolizations patients, 2 had their lesion regress and sepsis was suspected after embolization. In 1 patient, the microcoil migrated to the iliac artery during the embolization procedure, and another patient developed renal abscess caused by renal artery embolization. Among 30 surgical cases, resection by thoracotomy was performed in 27 at the Department of Thoracic Surgery, and thoracoscopic resection in 3 at the Division of Pediatric Sugery. Only one wound complication ocurred. We conclud that surgical excision should be recommended for pulmonary sequestration, whether the sequestration is symptomatic or not because of the risk of infection, the low rate of natural regress, poor compliance, severe complications after embolization, and to exclude other pathology. In summary, thoracoscopic resection of the pulmonary sequestration is feasible, efficacious, safe and cosmetically superior even in neonatal period.

Infantile Fibrosarcoma in Neonate

Infantile fibrosarcoma is a rare malignant soft tissue tumor occurring especially in newborn and young children under 2 years. We experienced three cases of infantile fibrosarcoma presenting in the neonatal period. Case 1 presented with a multiseptated cystic mass on his left thigh at birth that was diagnosed as lymphangioma. After picibanil injection, we noted the size of the mass doubled and a solid lesion was prominent in the magnetic resonance image. Case 2 was found to have a reddish mass on his lower back mimicking hemangioma. Over 2 weeks, the mass grew rapidly with internal hemorrhaging. Case 3 was noted to have an encircling mass around the splenic flexure, which developed into congenital bowel obstruction. All of the tumors were resected completely, but microscopic resection margin was not clear in two patients. The two patients received adjuvant chemotherapy and all patients are well without evidence of recurrence.

An 18-year experience of tracheoesophageal fistula and esophageal atresia / 소아과

PURPOSE: To determine the clinical manifestations and outcomes of patients with tracheoesophageal fistula (TEF) and esophageal atresia (EA) born at a single neonatal intensive care unit. METHODS: A retrospective analysis was conducted for 97 patients with confirmed TEF and EA who were admitted to the neonatal intensive care unit between 1990 and 2007. RESULTS: The rate of prenatal diagnosis was 12%. The average gestational age and birth weight were 37(+2) weeks and 2.5+/-0.7 kg, respectively. Thirty-one infants were born prematurely (32%). Type C was the most common. The mean gap between the proximal and distal esophagus was 2 cm. Esophago-esophagostomy was performed in 72 patients at a mean age of 4 days after birth; gastrostomy or duodenostomy were performed in 8 patients. Forty patients exhibited vertebral, anorectal, cardiac, tracheoesophageal, renal, limb (VACTERL) association with at least 2 combined anomalies, and cardiac anomaly was the most common. The most common post-operative complications were esophageal stricture followed by gastroesophageal reflux. Balloon dilatation was performed for 1.3 times in 26 patients at a mean age of 3 months. The mortality and morbidity rates were 24% and 67%, respectively, and the most common cause of death was sepsis. The weight of approximately 40% patients was below the 10th percentile at 2 years of age. CONCLUSION: Mortality and morbidity rates of patients with TEF and EA are high as compared to those of infants with other neonatal surgical diseases. Further efforts must be taken to reduce mortality and morbidity and improve growth retardation.

The Experience of Surgical Treatment of Necrotizing Enterocolitis

PURPOSE: Necrotizing enterocolitis (NEC) is a severe inflammatory disorder of the intestine, causing high mortality and morbidity. We investigated the single center experience about the operative indication, treatment method, and mortality in NEC. METHODS: The medical records of infants (<1 year old) who underwent the operation due to complications of NEC at the Asan Medical Center from Jan 1997 to Dec 2007 were retrospectively reviewed. RESULTS: Among 49 patients (M:F = 34:15), 37 underwent the operation at acute phase of NEC, average 26.43+/-35.43 days after birth (3~168), due to pneumoperitoneum in 23, clinical deterioration in 12 and abdominal mass in 2. Average gestational age was 234.64+/-38.27 days (161~279) and birth weight was 2,061.38+/-999.49 g (563~3,740). The extent of necrosis was classified grossly as focal in 14 cases, multifocal in 14 and panintestinal in 9 and the operative methods were enterostomy in 30 patients, resection and anastomosis in 6 and open drainage in 1. Thirteen patients (35.1%) were expired - 8 (21.6%) died of necrotizing enterocolitis and 5 died of other causes. The other 12 patients underwent operation for stricture after NEC at average 81.17+/-77.22 days after birth (32~317). Average gestational age was 240.83+/-34.4 days (173~280) and birth weight was 2,089.83+/-862.47 g (710~3,200). Eight patients underwent resection and anastomosis including stricture and 4 patients underwent enterostomy. CONCLUSION: Resection and enterostomy was the preferred procedure but resection and anastomosis did not increase morbidity or mortality. Quite a number of patients suffered from the stricture after NEC.