OK-432 Intralesional Injection Therapy for Lymphangioma in Children

Lymphangioma is a congenital malformation of the lymphatic system, commonly seen in the neck. Operation was the treatment of choice but it is difficult to resect the lymphangiomas completely. The aim of this study is to evaluate the result of intralesional injection of OK-432 as a treatment strategy of lymphangioma in children. Medical records of 51 cases of lymphangioma from March 1996 to February 2001 were reviewed retrospectively. Intralesional injection of 0.1 mg OK-432 in 10ml normal saline was performed after the aspiration of as much fluid as possible. The location of the lesion was the face and neck in 26 patients, the chest wall in 14, the extremities in 9, and the abdominal wall in 2. The cystic type was present in 45 patients and the cavernous type in 6. Four postoperative recurrent cases were included. Fluid aspiration from the lesion was impossible in 5 patients. Development of fever after injection was observed in 27 patients and local inflammatory reaction was in 5 patients. There was no scar formation at injection sites. Complete shrinkage was observed in 20 patients, remarkable shrinkage in 23, slight shrinkage in 3, and no response in 5. Cystic type or aspiration-possible cases showed better outcome than cavernous type or aspiration-impossible cases. All of four recurrent cases after surgical excision showed at least remarkable shrinkage. These results indicate that intralesional injection of OK-432 is a safe and satisfactory treatment modality of lymphangiomas in children and might be considered as a treatment of choice, even in recurrent cases.

Evaluation of Prognosis in Esophageal Atresia

The aims of this study were to evaluate the prognostic predictabilities of the risk classifications (Waterston, Montreal, and Spitz), preoperative treatment of pneumonia and the length of esophageal gap in esophageal atresia. Twenty-five cases of esophageal atresia were reviewed retrospectively. The data was analyzed with Likelihood ratio test for trend and Fisher's exact test. Survival rates were 100.0% in group A and B, and 60.0% in group C according to Waterston classification (p=0.027). By Montreal classification, 95.7% in group I and 50.0% in group II (p=0.086). By Spitz classification, 95.5% in group I and 66.7% in group II (p=0.159). The survival rate was 85.7% in the patients with pneumonia preoperatively treated and 94.4% in the patients without pneumonia. The difference was not significant (p=0.490). Survival rate in the patients with long gap (> or = 3cm) was 50.0% and 100.0% in patients with short gap (< 3cm) with significant difference (p=0.020). The Waterston classification could be modified into two groups because the prognosis of both group A and B were the same. Preoperative pneumonia does not seem to be a risk factor. The length of the esophageal gap showed prognostic value.

The Result of Delayed Operation in Congenital Diaphragmatic Hernia

Congenital diaphragmatic hernia (CDH) in the past was considered a surgical emergency requiring immediate operation. Several groups now advocate preoperative stabilization and delayed surgery. The treatment strategy for CDH in this institution is delayed surgery after preoperative stabilization. The aim of this study was to evaluate the results of delayed surgery. A retrospective review of 16 neonates with CDH was performed. Surfactant, conventional mechanical ventilation, high frequency oscillation, and nitric oxide were utilized for preoperative stabilization as necessary. The difference in outcome between two groups differentiated by the duration of the preoperative stabilization periods with mechanical ventilation (8 hours) was determined. Chi-square test was used to analyze the data. There were 7 right-sided hernias and 9 left. The average duration of stabilization was 32.4 hours. Hepatic herniation through the defect was found in 6 cases and all died. The most common postoperative complication was pneumothorax. The mortality rate of the right side hernia was higher than the left (85.7% vs. 33.3%, p=0.036). Mortality rate of the group (N=8) whose preoperative stabilization period was 8 hours or less was better than that (N=6) whose preoperative stabilization period was more than 8 hours (25.0% vs. 83.3%, p=0.031). The overall mortality rate was 56.3%. The better prognosis was noticed in left side hernia, no liver herniation, or shorter preoperative stabilization period.

A Case of Gastric Volvulus

Gastric volvulus is a rare surgical disorder in the pediatric population. We experienced a case of gastric volvulus. A 2-year-old boy was admitted to hospital with abdominal distension. An upper gastrointestinal series showed reversal of the greater and lesser curvatures. Surgical exploration revealed an organoaxial volvulus of the stomach, and anterior gastropexy was performed.

Clinical Analysis of Urachal Anomalies

PURPOSE: Although urachal anomalies are rarely observed clinically, they often give rise to a number of problems, such as infection and late malignant changes. Because of variable clinical presentations, uniform guidlines for evaluation and treatment are lacking. The authors discuss the problems involved in both the diagnosis and the treatment of these anomalies. METHODS: We retrospectively analyzed 15 patients (9 males and 6 females) who had undergone surgery for urachal anomalies from July 1995 to December 1999. RESULTS: The age distribution ranged from 14 days to 38 years old, and the male to female ratio was 1.5:1. The 4 variants of urachal anomalies included a urachal sinus in 7 patients (47%), a urachal cyst in 4 (27%), a patent urachus in 3 (20%), and a bladder diverticulum in 1 (6%). The presenting complaint was periumbilical discharge in 6 patients, fever in 5, umbilical granuloma in 4, low abdominal mass in 3, and low abdominal pain in 1. Eight combined anomalies were seen in 7 patients; 2 umbilical hernias, 1 inguinal hernia, 1 hydrocele, 1 urachal vessel anomaly, 1 vesicoureteral reflux, 1 hydronephrosis, 1 Hirschsprung's disease, and 1 hypertrophic pyloric stenosis. Ultrasound examination disclosed a cyst or a sinus in 5 patients, and CT was performed in 1 case. Excision was performed in all patients, and there was no postoperative complication or recurrence. CONCLUSION: Urachal anomalies most frequently present in infancy or childhood, and the initial presentation is umbilical discharge with infection. Furthermore, the large number of associated genitourinary and gastrointestinal anomalies suggests that a complete work-up for these conditions should be performed. Definitive surgical excision appears to be appropriate for most patients.

Clinical Analysis of Urachal Anomalies

PURPOSE: Although urachal anomalies are rarely observed clinically, they often give rise to a number of problems, such as infection and late malignant changes. Because of variable clinical presentations, uniform guidlines for evaluation and treatment are lacking. The authors discuss the problems involved in both the diagnosis and the treatment of these anomalies. METHODS: We retrospectively analyzed 15 patients (9 males and 6 females) who had undergone surgery for urachal anomalies from July 1995 to December 1999. RESULTS: The age distribution ranged from 14 days to 38 years old, and the male to female ratio was 1.5:1. The 4 variants of urachal anomalies included a urachal sinus in 7 patients (47%), a urachal cyst in 4 (27%), a patent urachus in 3 (20%), and a bladder diverticulum in 1 (6%). The presenting complaint was periumbilical discharge in 6 patients, fever in 5, umbilical granuloma in 4, low abdominal mass in 3, and low abdominal pain in 1. Eight combined anomalies were seen in 7 patients; 2 umbilical hernias, 1 inguinal hernia, 1 hydrocele, 1 urachal vessel anomaly, 1 vesicoureteral reflux, 1 hydronephrosis, 1 Hirschsprung's disease, and 1 hypertrophic pyloric stenosis. Ultrasound examination disclosed a cyst or a sinus in 5 patients, and CT was performed in 1 case. Excision was performed in all patients, and there was no postoperative complication or recurrence. CONCLUSION: Urachal anomalies most frequently present in infancy or childhood, and the initial presentation is umbilical discharge with infection. Furthermore, the large number of associated genitourinary and gastrointestinal anomalies suggests that a complete work-up for these conditions should be performed. Definitive surgical excision appears to be appropriate for most patients.

Sclerotherapy with Pure Ethanol in Congenital Vascular Malformations Preliminary report

Congenital vascular malformations (CVM) have many different clinical presentations, ranging from an asymptomatic birthmark to a life-threatening status. There has been confusion in the classification of these malformations. Two major classification systems are used at the present time: one is the Mulliken and coworkers' system that has been adopted by the International Society for the Study of Vascular Anomalies, and the other is the Hamburg classification that was declared in the 7th Meeting of the International Workshop on Vascular Malformations in Hamburg 1988. The latter is used in this article. BACKGROUND: There are many difficulties in the surgical extirpation of CVM because of their invasiveness, variability, hypervascularity, and evolutibility, especially in a diffuse infiltrating type of CVM. Thus, many endovascular ablative agents have been utilized since 1930s, but there were some handicaps, such as recanalization of previously treated vessels. Since the 1980s, Yakes has reported on much research about the effectiveness of absolute alcohol (98% ethyl alcohol) which incurs permanent vessel wall destruction. AIMS: We performed this study to evaluate the effectiveness of pure ethanol (95-99% ethyl alcohol, Samsung Medical Center, Seoul, Korea) in the treatment of inoperable CVM, to identify the complications that occur and to establish the method of endovascular ablative therapy. MATERIALS AND METHODS: From August 1996 through October 1997, we applied 37 sessions of sclerotherapy using direct puncture technique with pure ethanol to 19 patients with the extratruncular, diffuse infiltrating type of CVM (11 predominantly venous, 8 predominantly arteriovenous shunting)among 250 CVM patients registered at the CVM Clinic of Vascular Center at Samsung Medical Center. RESULTS: Angiographically, the results were excellent in 33 (89%) and good in 3 (9%) sessions. Only one (3%) session failed due to extravasation of the contrast media. The mean follow-up period was only 6 months; 17 (89%) patients were symptomatically improved. Some complications, such as ischemic bullae, deep vein thrombosis, and hematuria, developed, but those patients were recovered without any problem. CONCLUSIONS: The short-term results of pure ethanol sclerotherapy for the diffuse infiltrating type of CVM were good. With more clinical experience, this new treatment modality will be more effective. Further investigations are needed to solve the some complications.

Software for Management of Congenital Vascular Malformation(CVM): A proposal

There is not a unique registry program about the CVM patients in Korea, even in the World. During the business meeting of International Society for the Study of Vascular Anomalies (ISSVA) 1996 in Rome, a sincere discussion took place about the 1988 Hamburg classification of CVM. This was followed by introduction of registry form that could be used universally throughout the world. We developed a CVM registry program calling CVM for world-wide sharing and analysis. This program is a typical database program application system based on Borland's Delphi 1.0 and the database is managed with dBase III+ . It became clear incoporating the Hamburg classification would be natural while expanding the diagnosis and treatment sections. This is a user-friendly, self-explanatory program that hardly needs any tutorial or instruction. Nonthless, for the foolhardy, it comes with manual that explains every aspect of it. Every panel comes with friendly buttons at your service at the stroke of the mouse or the pad. It can be used either on Windows 3.1 or 95, switching back and forth depending on your computer's capability. The program may obtained by diskettes, compact disc or downloaded from the Internet hompage. We will endeavor to upgrade periodcally as necessity arises.

The Clinical Differences of Infantile Hypertrophic Pyloric Stenosis between Premature and Full-term Infants

Infantile hypertrophic pyloric stenosis (IHPS) is known to be prevalent in full-term babies, and relatively rare in prematures. The diagnosis of IHPS in premature infants may be obscured because of the lack of classical symptoms and signs and the absence of the standard criteria for ultrasonic diagnosis. The purpose of this study is to discover the clinical differences of IHPS between premature and full-term infants with pyloric stenosis, and to determine the appropriate diagnostic methods for early diagnosis in prematures. The clinical records of 52 IHPS patients who had been operated from October, 1994 to April, 1997 were reviewed. IHPS in premature infants was 25%. The onset of symptom was 4.7 weeks of age in premature, and 2.9 weeks in full-term babies. Diagnosis was established by typical symptoms, signs, and diagnostic imaging studies. In two premature infants, diagnosis was confirmed by upper gastrointestinal (GI) series, because ultrasonography did not meet the diagnostic criteria. Two premature infants diagnosed as gastroesophageal reflux by esophagography initially, were confirmed to have IHPS by upper GI series. For the diagnosis of IHPS, a new set of criteria for premature babies has to be developed.

Total Colonic Aganglionosis With Skip Area

Early recognition and surgical treatment of Hirschsprung's disease prevents serious mortality and morbidity from enterocolitis and obstruction. Usually this disease is chractrized by a single aganglionic segment of colon extending distally to anal margin. In surgical treatment, the surgeon performs frozen biopsy to confirm whether there are ganglion cells or not. If there are intervening ganglionic area in aganglionic bowel, there are much confusion in diagnosis and treatment. The authors experienced one case of total colonic aganglionosis with skip area. A transverse loop colostomy was made to a 7 day-old male baby with colon perforation due to Hirschsprung's disease. But intestinal obstruction persisted and it took 2 more operations to find out the ture nature of the disease. There were aganglionic segments from anal margin to terminal ileum 3.7cm proximal from ileocecal valve. But the entire transverse colon and appendix were normally ganglionated.

Experiences of Garotid Endarterectomy for Extracranial Carotid Stenosis in Korea

Strokes are responsible for major disability among its survivors, adding immense cost not only in terms of health care, but also in terms of the quality of life. The carotid endarterectomy to prevent strokes is commonly performed in Western countries. But in Asian countries, carotid endarterectomy is not commonly performed even on the symptomatic carotid stenosis patients. 26 male and 5 female patients undergoing carotid endarterectomy due to extracranial carotid stenosis from January 1995 through January 1996 were reviewed. The mean age was 67.2 +/- 6.6 years. 14 cases (45.2%) operated on right side and 17 cases (54.8%) operated on left side. The mean carotid stenosis according to luminal diameter reduction was 81.3 +/- 11.1%. Symptomatic cases, including transient ischemic attack, were 26 cases (83.9%) and five were asymptomatic cases (16.1%). Only two cases did not use carotid shunt during carotid clamping. Eight cases (25.8%) showed an abnormal intraoperative EEG pattern in the carotid shunting group. The average operation time was 173+/-36 minutes. The perioperative mortality was zero. Two postoperatively patients suffered from transient ischemic attack. One patient developed myocardial infarction. Vocal cord palsy developed in three patients. With the Westernization of Korean life style, the incidence of atherosclerosis disease has been on the rise. In this study, we report the experiences of 31 cases of carotid endarterectomy in our institute.

A Study on Survival in the Very Low Birth Weight Infants Received Neonata Intensive Care in Two Years

PURPOSE: Recent progress in neonatal intensive care has led to increased survival of infants weighing less than 1500 gm. Many studies to declining sequelae of intensive care, addition to increasing survival, were made. So, we investigated particulary the relationship between survival and birth weight, gestational age, and factors associated with morbidities. METHOD: The retrospecitve review of medical records was analyzed for 92 VLBW infants(birth weight <1,500gm) who were admitted to the neonatal intensive care unit of Samsung seoul hosptial from 1994 to 1996. We compared the outcomes of 32 VLBW infants between October 1994 and September 1995 (period I), with the outcomes of 60 VLBW infants between October 1995 and September 1996 (period II). RESULTS: 1) The incidence of VLBW infants was 1.01% in period I and 1.55% in period II. The overall survival rate increased to 76.7% in period II, compared with 71.9% in period I. 2) By birth weight, the highest survival rate was 100% at 750gm in peeriod I, and 86.4% at 1000 to 1249gm in period II. The survival rate at birth weight 1000 to 1249 gm increased significantly in period II. According to gestational age, the highest was at 31-32 weeks(85.7%) in period I, and at 29-30 weeks(88.9%) in period II. 3) The male : female ratio was 1:1 in period I versus 1:1.07 in period II. There was no significant difference in sex and mode of delivery. 4) The most common major morbidity occurred in VLBW infants was respiratory distress syndrome (65.6% in period I, 53.3% in period II). 5) There was no difference of neonatal death rate during period I, while 2-3 days after birth was the highest(30.0%) during period II. Major causes of death, during both periods, were infection and respiratory distress syndrome and/or its sequelae. CONCLUSION: This report demonstrates marked increased survival rate than previous other reports and especially during period II. With increasing survival, more attention to neonatal sequelae, including chronic lung disease and neurodevelopmental delay, is required.

Patent Omphalomesenteric Duct Remnants: Report of 4 Cases

The omphalomesenteric duct normally obliterates by the sixth week of intrauterine life. Incomplete obliteration results various abnormalities which may be apparent in the newborn infant. The omphalomesenteric duct may persist as an omphalomesenteric fistula. Morphologically the fistula resembles the ileum, but may contain ectopic gastric, colonic or pancreatic tissue. The infant presents with an umbilical discharge which may be recognizable as small bowel content. Although this malforamtion should be recognizable at birth, 40% of patients are not treated until after the first month of life. In the past 28 months since the Samsung Medical Center opened its doors to public, the authors experienced 4 cases of patent omphalomesenteric duct remnant including one case of T-shaped total prolapse of duct and adjacent ileum. These cases will be discussed with literature review.

A Case of Sliding Hiatal Hernia associated with Bochdalek Hernia Repair

This is a case report of a sliding hiatal hernia with severe gastroesophageal reflux (GER) after repair of congenital diaphragmatic hernia(CDH). It was not possible to determine whether the hiatal hernia is a de novo lesion which was missed at the original operation or a consequence of overzealous repair of the Bochdalek defect at the expense of weakening of the diaphragmatic crura. This case demonstrates that a sliding hiatal hernia can be a cause of severe gastroesophageal reflux that should be managed surgically.

An Intraabdominal Pulmonary Sequestration Containing Congenital Cystic Adenomatoid Malformation

Pulmonary sequestration is a complex anomaly involving the pulmonary parenchymal tissue and its vasculature. It presents as a cystic mass of nonfunctional lung tissue without communication with the tracheobronchial system. Usually, it receives blood supply from anomalous systemic vessels. Therefore, preoperative diagnosis of the pulmonary sequestration is difficult, especially when it is located in the abdomen and combined with congenital cystic adenomatoid malformation (CCAM). We encountered such a mass(CCAM type 2) detected prenatally by ultrasonography. It was a kidney bean shaped, pinkish mass straddling the thorax and abdomen on the right side. Because of the sonographic appearance, neuroblastoma was diagnosed preoperatively. The mass was completely extirpated without difficulty.