The Korean Association of Pediatric Surgeons (KAPS) performed the second nationwide survey on biliary atresia in 2011. It was a follow-up study to the first survey, which was performed in 2001 for the retrospective analysis of biliary atresia between 1980 and 2000. In the second survey, the authors reviewed and analyzed the clinical data of patients who were treated for biliary atresia by the members of KAPS from 2001 to 2010. A total of 459 patients were registered. Among them, 435 patients primarily underwent the Kasai operation. The mean age of patients who underwent the Kasai operation was 66.2±28.7 days, and 89.7% of those patients had type III biliary atresia. Only five patients (1.4%) had complications related to the Kasai operation. After the Kasai operation, 269 (61.8%) of the patients were re-admitted because of cholangitis (79.9%) and varices (20.4%). One hundred and fifty-nine (36.6%) of the patients who underwent the Kasai operation subsequently underwent liver transplantation. The most common cause of subsequent liver transplantation was persistent hyperbilirubinemia. The mean interval between the Kasai operation and liver transplantation was 1.1±1.3 years. Overall the 10-year survival rate after the Kasai operation was 92.9% and the 10-year native liver survival rate was 59.8%. We had 23 patients for primary liver transplantation without the Kasai operation. The mean age patients who underwent primary liver transplantation was 8.6±2.9 months. In summary, among the 458 Kasai-operation and liver-transplantation patients, 373 lived, 31 died, and 54 were unavailable for follow up. One-third of the patient who survived have had complications correlated with biliary atresia. In comparison with the first survey, this study showed a higher survival rate and a greater number of liver transplantation.
Fundoplication is a common surgical procedure for gastroesophageal reflux Disease (GERD). Recently the procedure has been performed with increased frequency laparoscopically. The aim of this study is to compare laparoscopic Nissen fundoplication (LNF) and open Nissen fundoplication (ONF) for GERD in children. We studied retrospectively the 88 pediatric patients who underwent the Nissen fundoplication for GERD as primary antireflux surgery from 1994 and 2009. ONF was performed in 34 cases and LNF was in 54 cases. 58 patients have neurologic impairment. Time to initial food intake after the surgery were reduced in the LNF group (
The purpose of this study is to analyse clinical impact of specific MRI findings in liver in patients of long-term survivors after Kasai portoenterostomy (KPE). Twenty-eight patients who were underwent KPE were followed up more than 5 years. Macro-regenerative nodule (MRN) and beaded-duct dilatation (BDD) were considered as important findings in liver MRI. The association between these findings in MRI and clinical indicator, serum bilirubin level and history of cholangitis were evaluated. Sixteen patients (57.1%) were shown MRN in liver MRI. There were 14 patients(50%) whose MRI showed BDD. Serum total and direct bilirubin were 3.6mg/dL and 1.8mg/dL respectively in positive MRN group whereas 1.4mg/dL and 0.7mg/dL in negative MRN group (
Unreduced small bowel intussusception requires operative treatment although the rate of spontaneous reduction is 60 to 70%. The aim of this study is to compare clinical characteristics and outcome between spontaneous reduction and operation group and to analyze factors related to decisions to treat small bowel intussusceptions. The records of 25 patients with small bowel intussusceptions treated in Seoul National University Children's Hospital from January 1999 to August 2009 were reviewed respectively. Spontaneous reduction group (n=12, 48%) had signs and symptoms of vomiting, abdominal pain, currant jelly stool, abdominal distension, fever, increased CRP but no rebound tenderness. One of them had been diagnosed with Henoch-Schonlein purpura and no one displayed pathologic leading point by image study. Operation group (n=13, 52%) consisted of patients who had primary surgery. Their signs and symptoms were similar to spontaneous reduction group. Seven of them had underlying diseases such as Crohn' disease, ALL, Lymphoma, Peutz-Jeghers syndrome (n=3), post-transplanted state of liver and 2 of them displayed Peutz-Jeghers polyp and Meckel's diverticulum as pathologic leading point by preoperative ultrasonography. Mean relieve interval (interval between onset of symptoms and reduction/operation) was 1.78 days in spontaneous reduction group and 2.25 days in operation group (p=0.341). Seven of operation group had manual reduction and 6 out of 7 received segmental resection of the small bowel. No one of them underwent manual reduction and all of them underwent segmental resection were found to have pathologic leading points [Peutz-Jeghers polyp (n=3), Meckel's diverticulum (n=2), lymphoma (n=1)] during operation. In conclusion, 48% of small bowel intussusceptions resolved spontaneously. Patients' symptoms and relieve intervals were not related to the operative decisions. We therefore recommend significant factors for determining treatment plan such as change of clinical symptoms, underlying disease or pathologic leading point by imaging.
Congenital thoracic malformations such as intra- and extra-pulmonary sequestration, cystic adenomatoid malformation, congenital pulmonary airway malformation, malinosculation, bronchogenic cyst, reduplication cyst, and foregut cyst are frequently detected on routine prenatal ultrasound. There are some controversies about treatment for postnatally persistent pulmonary sequestration. Some authors recommend expectant long term follow up but most authors advocate elective surgical excision because of complication such as respiratory distress, infection, intrathoracic bleeding, haemoptysis, cardiac failure, and potential risk of malignancy. We experienced 2 cases of prenatally diagnosed extrapulmonary sequestration which were located in the subdiaphragmatic retroperitoneum. Resections were performed at 2 months and 4 months of age using intraabdominal approach. There were no complications. In conclusion, if the prenatally diagnosed extrapulmonary sequestration remained postnatally, early operation might reduce morbidity related to extrapulmonary sequestration and parental anxiety without any postoperative complication.
Forme fruste choledochal cyst (FFCC) is one of the diverse types of choledochal cyst with little or no dilatation of the extrahepatic bile duct (EHBD). It is considered that FFCC has to do with the pancreatobiliary malunion (PBM). In children, 3 to 6 millimeters of EHBD is assumed to be normal. Even though there is no clear-cut definition, FFCC is likely to be associated with bile duct dilatation less than 10 millimeters. Almost all cases have PBMs and symptoms of the pancreatitis or cholangitis. We experienced a case of FFCC in a 4-year-old boy. His EHBD measures 10mm diameter. He had symptoms of pancreatitis and elevated hepatic transaminases. The pancreatobiliary common channel was 28 millimeters. He underwent EHBD resection and Roux-en-Y hepaticojejunostomy and was discharged with no specific complications.
Biliary atresia (BA) is an uncommon neonatal surgical disease that has a fatal outcome if not properly treated. The survival rates of the patients with native liver after Kasai's operation in countries outside Japan are not so good. We reviewed the results of 22 cases of biliary atresia treated in Kosin University Hospital between October 1987 and March 2001. There were 13 males and 9 females aged from 21 to 106 days (mean 52 days). There were 3 cases of Type I (13.6%), and 3 of Type II (13.6%), and 16 Type III (72.7%). The operative methods were resection of the common bile duct remnant and cyst followed by Roux-en-Y hepaticojejunostomy in 3 cases for Type I BA; Kasai I in 15 cases, Kasai II in 1 case, and Ueda's operation in 3 cases for Types II and III BA. There was no death within the first 30 days after operation. We were able to follow 21 of the 22 patients (95.4%) for more than 5 years. The actual 5 year survival rate (YSR) was 40.9%. One Type I case received a living-related liver transplantation at 6 years of age because of the multiple intrahepatic stones and liver cirrhosis. Five YSR after biliostomy group (Kasai II and Ueda op.) was 75% (3/4) while that of Kasai I was 20% (3/15). One case had no bile duct in the resected fibrotic plaque on microscopic review and died 8 months after Kasai I operation, would have been a strong candidate for early liver transplantation. From the above result, our conclusions are as follows; (1) early liver transplantation should be considered for cases of no bile duct after pathologic examination of the resected specimen, (2) measures to prevent postoperative cholangitis and prevention of postoperative liver cirrhosis are needed, (3) liver transplantation program should be available for failed cases.
Conventional treatment of Hirschsprung's disease consists of initial colostomy followed by pull-through operation. But, the treatment of Hirschsprung's disease has been changed along with the development of new surgical technique. Since 1995, endo-GIA has been available at our hospital and one stage Duhamel operation has been performed for neonatal Hirschsprung's disease. Between May 1995 and April 2006, 26 neonates have been treated with one stage pull-through operation by one pediatric surgeon at HanYang University Hospital. The sex ratio was 4.2:1 with male predominance. Clinical findings included abdominal distension (96.2 %), vomiting (50.0 %), delayed passage of meconium (46.2 %), constipation (23.1 %), and enterocolitis (15.4 %). Twenty two cases (84.6 %) were short-segment and 4 cases (15.4 %) were long-segment disease, of which 2 cases were total colon aganglionosis. One of the two patients with total colonic aganglionosis had double transition zones - distal ileum and hepatic flexure of the colon. The average age at operation was 14.56 ± 8.77 days and the average weight at operation was 3.26±0.66 kg. Primary Duhamel operations were performed in 25 patients and Soave-Boley operations was performed in one patient. The endo-GIA 35 (Ethicon, USA) was used from 1995 until 1997, and after that endo-GIA 60 (USSC, USA) was used. The average Duhamel operation time was 88.57 ± 22.80 minutes. Wound abscess (n = 2) and septum formation (n =1) occurred after Duhamel operation. Bowel function was normalized in 59 % within 3 months and in 95% within 1 year after operation. There was no mortality after one stage pull-through operation in neonate.
Biliary atresia (BA) is the result of fibrosing destructive inflammatory process affecting intrahepatic and extrahepatic bile ducts, which lead to cirrhosis and portal hypertension. Kasai portoenterostomy has been the standard operative procedure in biliary atresia. Recently, there has been remarkable increase in the survival rate in cases of BA. However, long-term survivors are not clearly evaluated in Korea. To define long-term prognosis factors of patients who underwent surgery for BA, a retrospective study was undertaken of 10 (37 %) patients surviving more than 10 years among 27 patients who underwent one of Kasai procedures between 1981 and 1995. Hepatomegaly was present in 4 and splenomegaly in 7 patients. Serum bilirubin was normalized at 1 year after operation. Aspartate aminotransferase (AST, GOT), Alanine aminotransferase(ALT, GPT) were normalized at 12 years and alkaline phosphatase(ALP) was normalized at 13.5 years. Cholangitis developed mainly within 5 years after operation so close follow up is needed. Life long follow-up is needed because of progressive deterioration of liver function even after 10 years.
Abdominal cystic lymphangiomas arising from the mesentery, omentum and retroperitoneum are rare and occasionally confused with other cause of acute abdomen. Sixteen children who underwent surgery for abdominal cystic lymphangioma between 1984 and 2005 at the Division of Pediatric Surgery, Keimyung University Dongsan Medical Center were evaluated retrospectively. There were 9 boys and 7 girls. Age ranged from 12 days to 13 years (mean age : 4.7years). The cysts were located in the omentum (4 cases),mesentery (9 cases : jejunum : 3, colon : 6). retroperitoneum (2 cases) and pelvic cavity (one case). The cyst content was hemorrhagic (8 cases), serous (5 cases), and inflammatory (2 cases), and chyle (one). Prenatal diagnosis was made in 3. The clinical features were variable, but abdominal pain, mass, and abdominal distention in order. There were 3 emergency operations in patients with complicated cyst, who were suspected of having panperitonitis and volvulus preoperatively. Complete excision was accomplished in all cases. There was one mortalityin a newborn with E.coli sepsis. Intestinal obstruction developed in 2 cases in long-term follow-up. No recurrence was observed. Although intraabdominal lymphangioma arising from mesentery, omentum and retroperitoneum are rare, it should be considered as a possible cause of acute abdomen.
The purpose of this study is to review the operative management and outcome of operation for Crohn's disease. The medical records of 17 patients who underwent operations due to Crohn's disease at Seoul National University Children's Hospital from January of 1988 to June of 2005 were reviewed and retrospective study was carried out. The male-to-female ratio was 1.8: 1. The median age at the onset of symptom and the time of diagnosis was 9 years 6 months and 11 years 6 months respectively. The median interval time from diagnosis to operation was 2 years and 1 month (0 month~8 years). Ileocolic or ileocecal region was the most common site of involvement. The indications of operation were intractable symptoms (8 cases) and obstruction or stricture (7 cases). The median postoperative hospitalized days were 14.4 days (8~35 days). Five patients (29%) experienced postoperative complications. Symptom free state or symptom relief was observed in 11 cases after surgery and 6 cases had wax and wane course of aggravation and improvement. In pediatric Crohn's disease patients who present with intractable symptoms despite medical treatment or develop surgical complications, symptom free state or symptom relief can be achieved by minimal resection of involved lesion.
Improvement in prenatal ultrasonography is leading to diagnose choledochal cyst before birth and before onset of classical symptom more frequently. But, there is a controversy about optimal timing for cyst excision of prenatally diagnosed asymptomatic choledochal cyst. To identify the most appropriate timing for surgery in prenatally diagnosed choledochal cysts, we analyzed 6 patients who had operation for choledochal cysts within 30days after birth at the division of Pediatric Surgery, Samsung Medical Center and Inha University School of Medicine, from June 1995 to June 2002. Males were four and females 2, the mean age at operation was 11.2 days, and the median age 8.0 days. The range of gestational ages of the antenatal diagnosis of bile duct dilatation was 24 weeks to 32 weeks, mean was 38.3 weeks, and mean birth weight was 3,298.3 g. After birth, abdominal ultrasonography, hepatobiliary scintigraphy, and magnetic resonance cholangiopancratography (MRCP) were performed. Mean age at operation was 11.2 days. All patients had the cyst excision and Roux- en-Y hepaticojejunostomy. Immediate postoperative complication was not found. During the median follow-up period of 41 months, one patient was admitted due to cholangitis, and the other due to variceal bleeding. Early operative treatment of asymptomatic newborn is safe and effective to prevent developing complications later in life.
A re-pull-through operation for Hirschsprung's disease is performed when the primary operation has failed because of the remnant or acquired aganglionosis, anastomotic stricture and/or fistula. The purpose of this study is to review our experience of the re-pull-through procedure for Hirschsprung's disease. From May 1978 to July 2003, 26 patients who underwent re-pull-through operations at the Department of Pediatric Surgery, Seoul National University Children's Hospital, were studied retrospectively by means of chart review as well as telephone interview. The mean age at primary operation and re-operation were 11months (2 months - 10 years) and 43 months (1 year - 23 years 3 months), respectively. Initial operation for Hirschsprung's disease was Duhamel's procedure in 17, Swenson's in 6 and Soave's 3. Causes of failure of primary operation were remains of secondary aganglionic segment (n=23), vascular arcade injury (n=1), rectoperineal fistula (n=2, due to mesenteric torsion and poor blood supply). Mean interval between the primary operation and the re-operation was 34 months (6 months-22 years). Reoperation methods were Soave's in 12, Duhamel's in 8, APSP (abdomino-posterosagittal pull-through procedure) in 5, and Swenson's in 1 case. In 2 cases of repeated rectoperineal fistula or rectourethral fistula, re-APSP were performed 3 times, respectively. A total of 29 re-pull-through operations were performed. Postoperative complications were wound infection (n=1), adhesive ileus (n=1), rectoperineal fistula (n=3), rectourethral fistula (n=2), and death due to pulmonary embolism (n=1). Mean follow up period of reoperation was 78 months (1 month-23years). In current state, 2 patients have an ileostomy because of repeated rectoperineal fistula and rectourethral fistula. Of the remaining 23 patients, 21(91 %) are totally continent with or without minimal soiling. Reoperation for Hirschsprung's disease was effective and safe for the patients complicated to the initial pull-through operation.
Diffuse cavernous hemangioma of the rectosigmoid is a rare lesion usually presented in children and young adult, often with life threatening hemorrhage. The clinical diagnosis is difficult and often delayed because of lack of awareness of the clinical features. We report a case of diffuse cavernous hemangioma of the rectosigmoid in a 12-year-old boy who had undergone suture ligation under the impression of hemorrhoid at his age 3. Sphincter-saving coloanal pull through procedure were applied as the surgical treatment. Sclerotherapy was needed with recurrent rectal bleeding 6 months after the pull through operation. The patient is well at 12 months follow-up.
Preauricular sinus and preauricular skin tag are common childhood congenital anomalies. It is important for the pediatric surgeon to be familiar with the embryology and differentiation of head and neck structure to accurately diagnose and treat these lesions. Seventy-eight patients with preauricular sinus and twenty-eight with preauricular skin tag treated in the Department of pediatric surgery at Hanyang University Hospital from January 1981 to May 2002 were reviewed to determine relative frequency, clinical classification and appropriate treatment. The male to female ratio of preauricular sinus was 1:1.2, and preauricular skin tag was 1:1. The most commonly presenting age of sinus and skin tag was before 5 year (62.8%) and before 1 year (53.6%). Twenty nine of 78 cases of preauricular sinuses were on the left, 25 on the right and 24 bilateral. Sings of infectio were infection were seen in 73.0% of patients with preauricular sinus at operation. Only 31.3% of lesions were infected in patients less than one year of age, but 89.5% between 3-5 year and 100% between 5-8 year. Cartilage was present in five patients with preauricular skin tag. Although re-operation due to wound infection was necessary in four cases, no recurrences were found. The preauricular sinus is a common anomaly in childhood, and has had a relatively high recurrence rate. But most of the recurrence were due to incomplete resection because of combined infection. Initial proper diagnosis and early operation are very important. Identification of the exact anatomical location of sinus tract is necessary because total excision of the lesions including those tracts is the only way to prevent recurrence.
The aim of this study was to evaluate the posterior sagittal anorectoplasty (PSARP) as a re-do operation in patients who failed initial repair of anorectal malformation. Nine patients (4 boys and 5 girls) who had previous failed surgery for anorectal malformation underwent secondary operations through posterior sagittal approach. The main reasons of surgery were constipation (n=3) and persistent anatomical derangement in spite of previous correction surgery (n=6). In addition to constipation, the former group (n=3) had various anatomical defects, and the latter group (n=6), of course, had constipation in some degrees. Patients ranged in age from 2 to 19 years (median 3 years) with only one over the age of 6 years. The primary procedures included PSARP (n=8) and anoplasty (n=1). The rectum was mobilized from surrounding structures through posterior sagittal approach and anatomical defects were corrected. The rectum underwent reconstruction, which involved relocation of the rectum and anus within the limits of the intact muscle complex. Patients underwent follow-up for periods ranging from 6 to 77 months (mean 37 months) after surgery. Anatomical corrections of all the defects were successfully fulfilled in 9 patients. All the patients were satisfied with the functional results after redo-PSARP compared with the preoperative defecatory function. This study suggests that (1) some of the patients with troublesome constipation may have anatomical defects, prominent or hidden, (2) surgeons should suspect the possibility of anatomical defect as the cause of incontinence and (3) preoperative thorough investigation to reveal the anatomical defects should be included in estimating patients with severe incontinence after previous surgery and planning the correction for failed previous surgery as well.
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.
Pediatric surgeons are familiar with the posterior sagittal approach to the rectum at sacrococcygeal area and well oriented with the anatomy because of the Penal procedure for imperforate anus. The author utilized the posterior vertical elliptical incisions in 12 cases of sacrococcygeal teratoma since 1987. For presacral tumor(type IV)2, the incision was exactly same as the posterior sagittal procedure for imperforate anus. But the out-growing(type I) or dumbbell-shaped(type II & III) tumors, a vertical elliptical incision was required. For the laterally deviated tumors, a vertical and half-chevron incision was utilized in one case, but an unbalanced vertical elliptical incision was acceptable for the remaining two cases, with shrinkage of the overlying skin. In dumbbell-shaped tumors(type II & III), the narrow waist of the tumor was at the level of the levator muscle, which formed a muscle-belt on the tumor waist. A careful dissection to save the muscle-belt seemed to be the most important point during this procedure, utilizing the nerve-stimulator. After complete removal of the tumor and the coccyx, the levator muscles and the skin were closed in vertical fashion along the midline. For the caudally extending tumors in 3 cases, the muscle complex was divided in midline. Nothing by mouth and total parenteral nutrition was maintained for 1 week and then laxatives were given for 2 weeks in order to give the sphincters rest. Operative scars were acceptable resembling natural vertical midline folds, and the sphincter function was continent in all cases. In conclusion, vertical elliptical incision in sacrococcygeal teratoma is recommended because of the acceptable scar, functional restoration, and because it is a familiar procedure particularly for the pediatric surgeons who are accustomed performing posterior sagittal approach for imperforate anus.
First
Prev
