Clinical characteristics and treatment of esophageal atresia: a single institutional experience / 대한외과학회지

PURPOSE: Treatment for esophageal atresia has advanced over several decades due to improvements in surgical techniques and neonatal intensive care. Subsequent to increased survival, postoperative morbidity has become an important issue in this disease. The aim of our study was to analyze our experience regarding the treatment of esophageal atresia. METHODS: We reviewed and analyzed the clinical data of patients who underwent surgery for esophageal atresia at Severance Children's Hospital from 1995 to 2010 regarding demographics, surgical procedures, and postoperative outcomes. RESULTS: Seventy-two patients had surgery for esophageal atresia. The most common gross type was C (81.9%), followed by type A (15.3%). Primary repair was performed in 52 patients. Staged operation was performed in 17 patients. Postoperative esophageal strictures developed in 43.1% of patients. Anastomotic leakages occurred in 23.6% of patients, and recurrence of tracheoesophageal fistula was reported in 8.3% of patients. Esophageal stricture was significantly associated with long-gap (> or =3 cm or three vertebral bodies) atresia (P = 0.042). The overall mortality rate was 15.3%. The mortality in patients weighing less than 2.5 kg was higher than in patients weighing at least 2.5 kg (P = 0.001). During the later period of this study, anastomotic leakage and mortality both significantly decreased compared to the earlier study period (P = 0.009 and 0.023, respectively). CONCLUSION: The survival of patients with esophageal atresia has improved over the years and the rate of anastomotic leakage has been significantly reduced. However, overall morbidities related to surgical treatment of esophageal atresia still exists with high incidence.

Cerebral Toxoplasmosis Combined with Disseminated Tuberculosis

A 24-year-old man presented with mental change, fever, abdominal pain, tenderness and palpable mass on the lower abdomen. He was a non-Korean engineer and did not accompany a legal guardian, so medical history taking was difficult due to his mental status. Brain magnetic resonance imaging showed multiple rim-enhanced lesions of the brain, and abdominal computed tomography showed huge paraspinal abscess. Chest X-ray and computed tomography showed poorly defined nodular opacities. We initially thought that this patient was infected with toxoplasmosis with typical cerebral image finding and immunoglobulin laboratory finding of cerebrospinal fluid and serum study. The abdominal abscess was confirmed as tuberculosis through the pathologic finding of caseous necrosis. We used anti-tuberculosis medication and anti-toxoplasmosis medication for almost 4 months, and then his clinical state and radiological findings were considerably improved.

Sudden Death Caused by Enterocolitis after Duhamel's Operation for Hirschsprung's Disease

Hirschsprung's disease is a common functional obstructive disease in infants, and a lot of knowledge and experience have been accumulated about this disease. But the exact genesis of Hirschsprung's disease is still unknown, as well as there is a lack of knowledge about the pre- or postoperative complications. Enterocolitis is a common and serious complication that can cause death. The authors experienced sudden death with three cases that were treated with Duhamel's operation under the confirmed diagnosis of the Hirschsprung's disease. To prevent such sudden and unexpected death, we report here on some of the medical procedure that we performed for these 3 patients.

Acute Appendicitis in Children: Comparison between Present and 10 Year Ago

Appendicitis is the most common surgical emergency in childhood and the technologic advances of modern medicine have affected the diagnosis and treatment of appendicitis. This study is to evaluate the differences in diagnosis and treatment of appendicitis between present and 10 year ago. The authors retrospectively reviewed the medical records of patients who underwent appendectomy under the diagnosis of the acute appendicitis from July 1993 to June 1995 (Group A, n = 78) and from July 200 to June 2005 (Group B, n = 105). There are no differences between group A and B in mean age (8.5 +/-3.6 vs. 9.3 +/-3.1 year), duration of symptoms (3.0 +/-3.2 vs. 2.6 +/-3.8 days), and postoperative hospital stay(6.6 +/-4.8 vs. 5.8 +/-3.6 days). Preoperative abdominal ultrasonogram and/or computed tomogram was performed in 7 patients (9.0 %) of group A and in 51 patients (58.5 %) of group B. Thirty-six patients (34.3 %) of group B underwent laparoscopic appendectomy, but none in group A. Incidence of a histologically normal appendix decreased from 15.8 % in group A to 4.8 % in group B (p =0.018). This study suggests that utilization of abdominal ultrasonogram or computed tomogram in preoperative evaluation become more popular and surgical treatment of acute appendicitis become more minimally invasive. The rate of negative appendectomy was also reduced compared with 10 year ago.

Anti-apoptosis Gene, Survivin Expression and Prognosis of Neuroblstoma

PURPOSE: The prognostic significance of survivin expression (SE), which has been reported to be an inhibitor of apoptosis protein, was examined in order to identify a more accurate prognostic grouping of neuroblastomas. METHODS: Thirty-seven tumor specimens were obtained between 1992 and 2002. The SE level was examined by immunohistochemical techniques using paraffin-embedded tissues, and was scored as being positive when more than 5% of the cells reacted with the anti-survivin antibody. The outcome of the stratified potential prognostic groups was evaluated according to age, location, stage, Shimada class and SE. The correlation between the SE level and the prognostic factors were analyzed using a univariate assessment. The predictive value of SE in the prognosis was determined using a multivariate assessment. RESULTS: The median follow up period was 23 months. The 5-year overall survival rate was significantly reduced with an advanced stage, adrenal primary, unfavorable Shimada class and positive SE (P=.01, .01, .02 and .00). A positive SE was correlated with old age, advanced stage and an unfavorable Shimada class (P=.03, .00 and .03). The relative risk of the SE positive group was 5.20 (P=.01). Survivin was expressed in 73% of recurred cases, but in only 31% of non-recurred cases (P=.04). CONCLUSIONS: Survivin expression is a valid independent prognostic factor for a neuroblastoma, which correlates withthe disease progression and a poor prognosis.

Usefulness of MR Cholangiography in the Evaluation of Neonatal Cholestasis: Comparison with 99mTc DISIDA Scan

PURPOSE: To evaluate the diagnostic validity of MR cholangiography as a second-line imaging tool following sonography in the evaluation of neonatal cholestasis, we compared MR cholangiography with 99mTc DISIDA scan. MATERIALS AND METHODS: We retrospectively evaluated sonography, 99mTc DISIDA scan and MR cholangiography in twenty-two neonates and infants (age range, 23-103 days; mean age, 57 days) presenting with conjugated hyperbilirubinemia. Of the 22 patients, 15 were diagnosed as biliary atresia by operative cholangiography and liver biopsy and six as neonatal hepatitis by imaging finding and clinical data. Remaining one patient was diagnosed as neonatal hepatitis by operative cholangiography and liver biopsy. Two independent observers for each study were assigned to review the images of 99mTc DISIDA scan and MR cholangiography without giving the final diagnosis or other clinical data. Diagnostic accuracy and interobserver variability for each study were evaluated. RESULTS: On 99mTc DISIDA scan, biliary atresia was mistaken for neonatal hepatitis in eight patients and vice versa in four patients. On MR cholangiography, it was mistaken biliary atresia as neonatal hepatitis and vice versa in each two patients. Sensitivity, specificity, accuracy, positive and negative predictive values of 99mTc DISIDA scan were 48%, 47%, 48%, 66% and 30%, respectively, and those of MR cholangiography were 90%, 71%, 84%, 87% and 81%, respectively. Interobserver variabilities for 99mTc DISIDA scan and MR cholangiography were 0.62 and 0.85, respectively. CONCLUSION: In the evaluation of patients with neonatal cholestasis, it would be advisable to use MR cholangiography, having superior diagnostic accuracy to 99mTc DISIDA scan, as a second-line imaging tool following sonography.

Congenital Tracheomalacia Associated with Esophageal Atresia

This is a case of tracheomalacia associated with esophageal atresia. An 11-month-old- male boy presented with a life-threatening apneic spell after correction of esophageal atresia (Gross type C). After complete exclusion of the other possible causes of the apneic spell, the presumptive diagnosis of tracheomalacia was made with fluoroscopy and 3-dimensional chest CT. The final diagnosis was made with rigid bronchoscopy under spontaneous respiration. The aortopexy was performed with intraoperative bronchoscopic examination. The postoperative period was unremarkably uneventful. The patient was discharged 9 days after the aortopexy and has remained well to date (5 months after the aortopexy).

Inhibition of Tumor Growth and Angiogenesis by KJ3, Betulinic Acid, and Fumagillin in Mouse Neuroblastoma

The antiangiogenic effects of novel agent KJ3, Betulinic acid, and Fumagillin on the neovascularization were studied by examining ultrastructural alterations in the vasculature of synthetic gelform and mouse neuroblastoma C1300. Small pieces of gelform with 0.4 % agar were introduced subcutaneously (s.c.) in 7 week old male CH3/HeJ mice. After the LD50s were determined by FACS analysis, a third of LD50 of three drugs were injected either locally or intraperitoneally every other day for 14 days. A/J mice were inoculated s.c. with the C1300 neuroblastoma cell line, then either saline or three drugs were injected in the same manner. The antiangiogenic effects of three drugs were studied by measuring the histologic changes in tumors, and immunostaining for CD34, VIII/vWF, CD105, and thymidine phosphorylase. In the drug treated groups, the number of vessels in gelform experiments and C1300 neuroblastoma experiments were lower than the corresponding values in the control. The histologic findings were significantly different in drug treated groups on day 7, but these were not significant on day 14. These results imply that antiangiogenic agents were effective when the tumor burden is minimal.

Proper Treatment for Megacolon after Various Anoplasties for Anorectal Malformation

PURPOSE: The megacolon after repairing an anorectal malformation is not a rare complication, and there is much controversiy on the causes, the treatment of choice and the results after a longterm follow-up. We present 5 cases of a megacolon after the repair of an anorectal malformation, which were controlled with either a surgical resection or conservative treatment. METHODS: Five patients with a megacolon after the repair of an anorectal malformation were studied. A retrospective chart review was done and fecal continence was evaluated with an individual interview. RESULTS: All five patients initially underwent conservative treatment with laxatives and/or enemas. One Patient responded well to conservative treatment and the diameter of the bowel reduced to normal size. Another patient responded to conservative treatment after correcting the location of the anus. Three patients needed a surgical resection and one of those needed a further procedure to correct the anal location. After the surgical resection of the megacolon and/or correction of the anus (one out of the three patients), they soon reported an almost normal bowel habit. CONCLUSION: The first step in treating a megacolon after repairing an anorectal malformation was conservative treatment. However patients without an adequate response to conservative treatment are best managed with a surgical resection. The cause of the megacolon is now under investigation and the lack of adequate management after repair is one of the subjects.

The Impacts of the Prenatal Ultrasonogram on the Pediatric Surgery

PURPOSE: Prenatal diagnosis of congenital anomalies provides the information for the perinatal treatment, which can be beneficial to the patients. Yonsei University is one of the largest tertiary referral centers in Korea and its achievement in pediatric surgery is representative figure of pediatric surgery in Korea. This achievement is used to assess the impacts of prenatal ultrasonograms on the doutcomes of prenally diagnosed anomalies in the neonates. METHODS: Between 1991 and 2000, 41,458 prenatal ultrasonograms were performed on the pregnant women and the fetal abdominal abnormalities were suspected in 165 fetuses. Of these, 87 fetuses were delivered and the abnormalities were finally confirmed. Theses 87 fetuses was the basis of this study in terms of their prenatal and final diagnosis with the outcomes. RESULTS: Among 87 fetuses, 17 cases were terminated in relation to the maternal health or multiple anomalies. Of the remaining 70 fetuses, 55 patients survived. Among the prenatal diagnoses of 87 fetuses, final diagnosis were made from 75 fetuses and the accuracy of the prenatal diagnosis was found to be 60.0% (45/75). Surgical correction was necessary in 44 cases to confirm the diagnosis and of these, 40 patients survived after the surgical correction. CONCLUSION: Prenatal diagnosis of the congenital anomalies will improve postnatal outcomes by proper surgical management. However, it can affect the rate terminations of pregnancies even though its accuracy is not so high. To advance the knowledge of the fetal pathophysiology, pediatric surgeons must play an important role in the prenatal diagnosisin relation to the postnatal treatment of the anomalies.

The Results of Surgical Treatment for Suppurative Cervical Lymphadenitis in Children

PURPOSE: The results of surgical treatment for acute suppurative cervical lymphadenitis in children were evaluated with literature reviews. METHODS: Children under 15-year old who required operative management for acute suppurative cervical lymphadenitis between January 1996 and December 2000 were evaluated with retrospective manner. The clinical characteristics, pathologic results and microbiologic studies were reviewed. RESULTS: 37 patients were treated with surgical methods. 36 patients recovered fully after surgical intervention without any recurrence and one patient expired of sepsis. CONCLUSION: The classical treatment, incision and drainage, is one of the treatment of choice for the recovery from suppurative cervical lymphadenitis in children.

A Case of Wilms Tumor with Thrombi which Invaded the Right Ventricule

Wilms tumor is the second most common malignant retroperitoneal tumor. Inferior vena cava and right atrial involvement is found in about 4-10% and 0.5-3% of cases, respectively. But, right ventricular involvement has not been reported. We experienced a case of Wilms tumor with right ventricular invasion in a 2 year-old male who presented intermittent hematuria and abdominal pain. Computed tomogram and echocardiogram showed a homogeneous mass extended to right ventricle via inferior vena cava. He received pre-operative chemotherapy followed by operation, radiation therapy including heart, and post-operative chemotherapy.

Morphometric Evaluation of PGP9.5 and NCAM Expressing Nerve Fibers in Colonic Muscle of Patients with Hirschsprung's Disease

A quantitative assessment of the density of the protein gene product 9.5 (PGP9.5), the neural cell adhesion molecule (NCAM), and the low-affinity nerve growth factor receptor (NGFR) expressing nerve fibers in the circular muscle layer in the colon was carried out by morphometric analyses from 13 patients with Hirschsprung's disease (HD). The difference in the nerve fiber density between the ganglionic and aganglionic segments was compared by calculating the ratio of the sum of the areas occupied by positively stained nerve fibers per unit area of the muscle after immunohistochemical staining on paraffin embedded tissue sections using computer software. There was an obvious difference in the density of the PGP9.5 stained nerve fibers between the ganglionic (0.0380 +/- 0.0171) and aganglionic segments (0.0143 +/- 0.01661). The NCAM-positive nerve fibers were fewer in number than those of both the PGP9.5-positive fibers and NCAM-positive fibers, which were also markedly lower in number in the aganglionic segment (0.0066 +/- 0.0076) than in the ganglionic segment (0.0230 +/- 0.0195). Immunostaining for low-affinity NGFR revealed much fainter staining in the ganglionic and aganglionic segment without a statistically significant difference in their density. Considering the fact that PGP9.5 is a very sensitive marker for nerve fibers, the results of this study reaffirm the innervation failure of the proper muscle in HD. The decreased NCAM expression level in the aganglionic segment appears to be caused not by the selective down-regulation of NCAM expression among the nerve fibers but by a markedly reduced number of nerve fibers.

Laparoscopic Meckel's Diverticulectomy in Children

Meckel's diverticulum is one of the common causes of gastrointestinal bleeding in the pediatric patient requiring laparotomy. Two children with Meckel's diverticulum have been successfully treated by laparoscopic excision. Both patients recovered without incident and were discharged at 3 and 5 days after surgery. The authors believe that laparoscopic diverticulectomy is a safe, effective, and minimal invasive treatment of Meckel's diverticulum in children.

Experimental Congenital Diaphragmatic Hernia in a Fetal Lamb Model (I)

PURPOSE: The purpose of this study was to construct a fetal animal model of congenital diaphragmatic hernia in a lamb under domestic environments. METHODS: Left-sided diaphragmatic hernias were created in seven fetal lambs at about 85 days' gestation (experimental group). Three other fetal lambs did not receive any surgical procedure and served as control group. Four of these lambs (3 in the experimental group and 1 in the control group) were delivered after a sufficient intrauterine period. The morphological changes of lung development were compared between the two groups. RESULTS: Creation of diaphragmatic hernia resulted in marked hypoplasia in fetal lung development. In this experiment, the maternal mortality was 33.3%, and the fetal mortality was 60% which are relatively high as compared with previous reports. CONCLUSION: From this data, the authors concluded that experimental fetal diaphragmatic hernia can be established although the technique for the measurement of gestational age, anesthesia and postoperative care should be improved to overcome domestic inexperience in using the lamb as an experimental animal.

The development of fetal surgery

The history of fetal surgery features an absolute dependency upon the possibility of diagnosis before birth. Powerful new imaging methods, the techniques of sampling amniotic fluid and fetal tissue, and modern molecular genetics for the prenatal diagnosis of various congenital diseases have removed the veil of secrecy from the fetus. Even though most prenatally detected congenital malformations can be managed after maternal transport, a few simple anatomic defects require fetal surgery, albeit with predictably poor results. The understanding of intrauterine physiology and pathophysiology in several congenital malformations has been worked out in animal model study, and the natural history of congenital defects revealed by prenatal observations on human fetuses. Selection criteria for intrauterine intervention have been developed. Over the last two decades, surgical techniques for open and endoscopic fetal surgery have been defined and anesthesia and tocolysis for fetal surgery improved. As we enter the 21st century, this field of surgery will surely expand.

Succesful Treatment of Multiseptated Liver Abscess by Percutaneous Drainage with Urokinase

Liver abscess is a rare disease but shows a high mortality rate. The standard treatment is complete drainage of the abscess through surgery or insertion of a percutaneous catheter and proper antibiotic treatment. However, successful percutaneous drainage in a multiseptated abscess has several drawbacks, such as the necessity for inserting more than one catheter for proper drainage, a necessity for repetitive cathterization and moreover, the ultimate failure of effective drainage causing surgery to become inevitable. The authors used 80,000 IU urokinase three times per day over 5 days to lyse the fibrous septum in a 9-year-old child with multiseptated liver abscess. Catheter drainage with urokinase under radiologic intervention yielded successful a outcome in the treatment of a multiseptated liver abscess.

The Usefulness of Magnetic Resonance Cholangiography in the Diagnosis of Biliary Atresia

PURPOSE: To evaluate the usefulness of magnetic resonance cholangiography(MRC) for the diagnosis of biliary atresia in infantile cholestatic jaundice. METHODS: Fifty consecutive infants with cholestatic jaundice underwent single-shot MRC for 3 years. The radiologic diagnosis of non-biliary atresia with MRC was based on visualization of the common bile duct and common hepatic duct. The diagnosis of biliary atresia was based on non-visualization of either the common bile duct or common hepatic duct. The final diagnosis of biliary atresia or non-biliary atresia was made with operations or clinical follow-up until jaundice resolved. RESULTS: MRC could clearly visualized the gallbladder, cystic duct, common hepatic duct, common bile duct, both intrahepatic ducts and second order intrahepatic ducts in small neonates and infants. MRC had accuracy of 98%, sensitivity of 100% and specificity of 96% for diagnosis of biliary atresia as the cause of infantile cholestatic jaundice. CONCLUSIONS: MRC is a very reliable noninvasive imaging study for diagnosis of biliary atresia in infants with cholestatic jaundice.

A Successful Slide Cricotracheoplasty in Congenital Cricotracheal Stenosis

Slide tracheoplasty, as a treatment for congenital tracheal stenosis, has been recently reported to have good results and quite a number of advantages as compared with conventional tracheoplasties. The aim of this study is to report a new surgical technique modified from the slide tracheoplasty, "the slide cricotracheoplasty" for the congenital cricotracheal stenosis. A girl was born by Cesarean section and the diagnosis of esophageal atresia (Gross type C) and cricotracheal stenosis (30% of total length of trachea) was established. Esophageal atresia was successfully corrected at the 8th day of life. At the 31st day of life, corrective surgery for congenital cricotracheal stenosis, the slide cricotracheoplasty, was performed with success. Slide cricotracheoplasty is almost the same procedure as slide tracheoplasty except for two technical features. First the cricoid cartilage was split on its anterior surface. Second the split cricoid cartilage was fixed to pre vertebral fascia to maintain enough space to accommodate the sliding caudal segment of trachea because of the stiffness of the cricoid cartilage. We believe that the sliding cricotracheoplasty is a new surgical technique for congenital cricotracheal stenosis that has similar results and advantages as the sliding tracheoplasty.

The results of combined therapeutic modalities for hepatoblastoma

In hepatoblastoma, encouraging cure rates have been achieved with recent advances in chemotherapy and surgical techniques. The aim of this study is to evaluate the role of combined therapeutic modalities and surgical resection in hepatoblastoma. Fifteen cases of hepatoblastoma were treated from January 1993 to August 2000. Six patients had resectable tumors at initial diagnosis. All underwent surgical resection and in four patients postoperative adjuvant chemotherapy was needed. Nine out of 15 patients had unresectbale tumors at initial diagnosis, and preoperative chemotherapy was applied. There was one operative mortality and 14 patients showed good prognosis after surgery. Although various treatment modalities should be combined for the unresectable hepatoblastoma, surgical resection remains the major curative procedure.