This report present the result of the national survey of pediatric surgeons' preferences on diagnosis and treatment of Hirschsprung's disease(HD) carried out in 1993. The questionnaires were sent to twenty-seven members of the Korean Association of Pediatric Surgeons (KAPS) working in twenty-four institutions. The questionnaires were designed to determine the individual surgeon's preference for the methods of diagnosis and treatment of the disease. Twenty-three pediatric surgeons from twenty institutions returned completed forms. The total number of patients diagnosed with HD in 1992 was 190 in this group. The estimated incidence of HD was 1/3,900. The most important symptom was delayed meconium passing and the most preferred diagnostic procedure was barium study. Anorectal manometric examination was carried out by 13 pediatric surgeons and 19 confirmed the diagnosis before operation by rectal biopsy, 12 with full-thickness biopsy and 7 with suction. Frozen section biopsy during operation was done by 22 surgeons. Eight surgeons did one stage operation if the age of the patient is suitable. Definitive operation was usually done at the age of 6 to 11 months. The most preferred operation was Duhamel procedure done by 19. Enterocolitis was the most serious complication of HD. Most of patients had normal continence within 6 to 12 months after operation. The follow-up period was less than 6 years in 16 surgeons. The results were presented at the 9th annual meeting of KAPS in June of 1993. This is the first national survey of HD and it can provide guidelines of diagnosis and treatment of Hirschprung's disease even though it is not a detailed study of patient data.
Barium ; Biopsy ; Diagnosis ; Enterocolitis ; Follow-Up Studies ; Frozen Sections ; Hirschsprung Disease ; Humans ; Incidence ; Korea ; Meconium ; Suction ; Surgeons

To understand the current status of neonatal surgery in Korea, a suvey was made among the 27 members of Korean Association of Pediatric Surgeons. Response rate among surgeons was 78 perecnt, eighteen hospitals participated in this study. Five hundred and three cases of neonatal surgical patients were analyzed. In Korea, about 50% of cases were treated at the hospital in the capital city area. Regional number of patients were closely related to the regional population. Imperforate anus(19%), atresia/stenosis of gut(12%), and Hirschsprung's diseases(12%) were sitting at the top in the list. Majority of operation was done within the first week of life, especially during the first 24 hour period. Eighty per cent was major or so called index cases. Mortality in so-called index cases was 17%. High mortality was observed in patients with diaphragmatic hernia(47%), gastrointestinal perforation(65%) and esophageal atresia(28%). Low birth weight babies showed higher mortality in gastro -intestinal perforation, esophageal atresia and abdominal wall defect. These were compared to 1993 survey of Japanese Society of Pediatric Surgeons.
Abdominal Wall ; Asian Continental Ancestry Group ; Esophageal Perforation ; Humans ; Infant, Low Birth Weight ; Infant, Newborn ; Korea ; Mortality ; Surgeons

Introduction: This was a retrospective study aimed to investigate the perioperative outcomes of long construct minimally invasive spinal stabilisation (MISt) using percutaneous pedicle screws (PPS) versus conventional open spinal surgery in the treatment of spinal fracture in ankylosing spondylitis (AS) and diffuse idiopathic skeletal hyperostosis (DISH). Material and Methods: Twenty-one patients with AS and DISH who were surgically treated between 2009 and 2017 were recruited. Outcomes of interest included operative time, intra-operative blood loss, complications, duration of hospital stay and fracture union rate. Results: Mean age was 69.2 ± 9.9 years. Seven patients had AS and 14 patients had DISH. 17 patients sustained AO type B3 fracture and 4 patients had type B1 fracture. Spinal trauma among these patients mostly involved thoracic spine (61.9%), followed by lumbar (28.6%) and cervical spine (9.5%). MISt using PPS was performed in 14 patients (66.7%) whereas open surgery in 7 patients (33.3%). Mean number of instrumentation level was 7.9 ± 1.6. Mean operative time in MISt and open group was 179.3 ± 42.3 minutes and 253.6 ± 98.7 minutes, respectively (p=0.028). Mean intra-operative blood loss in MISt and open group was 185.7 ± 86.4ml and 885.7 ± 338.8ml, respectively (p<0.001). Complications and union rate were comparable between both groups. Conclusion: MISt using PPS lowers the operative time and reduces intra-operative blood loss in vertebral fractures in ankylosed disorders. However, it does not reduce the perioperative complication rate due to the premorbid status of the patients. There was no significant difference in the union rate between MISt and open surgery.

Delayed post-operative spinal epidural haematoma (DPSEH) is diagnosed when the onset of symptoms is more than three days from the index surgery. DPSEH is a rare but serious complication of spinal surgery. Missed diagnosis will result in irreversible neurological deficit which may lead to permanent disabilities. We report two cases of DPSEH who presented with worsening neurological deficit four days after the index surgery. Magnetic resonance imaging (MRI) showed the presence of an epidural haematoma compressing the spinal cord. Surgical evacuation of haematoma were performed for both patients. Both patients experienced neurological improvement. Surgeons should have high index of suspicion to identify delayed onset of spinal epidural haematoma (SEH) and timely intervention should be taken to avoid irreversible neurological damage.

The first national survey on esophageal atresia and tracheoesophageal fistula was conducted to access the current status of its incidence. clinical manifestation, preoperative diagnosis and management, type of its anomaly, associated anomalies, and surgical results and course. The 43 members of the Korea Association of Pediatric Surgeons received questionnaires and registration forms to be filled out on each patient who were born during the three years from January I, 1992 through Decestricurember 31, 1994. Questionnaires composed of six broad areas which include 1) preoperative diagnosis and management, 2) surgical technic, 3) long gap, 4) postoperative management, and 5) complications and courses. A total of 148 cases was returned by 28 members working at 23 institutions and 27 members returped questionnaires. We obtained the following results by analysis of the 148 cases of tracheoesophageal anomalies. The incidence of tracheoesophageal anomaly was about 1/10,000-11,000 in 1994, which is one third of that of anorectal malformations in Korea and the distribution of the patients was almost proportionate to the size of each province. Both sexes are about equally affected. Majority of the members make diagnosis of tracheoesophageal anomaly by taking a simple infantogram with a radiopaque tube in upper pouch and a little under half(46%) prefers to perform echocardiography as a part of preoperative management to identify congenital heart disease and lateralize the aorta. Esophageal atresia with distal TEF(87.50/0) was by far the most common and threre were pure esopahgeal atresia(5.6%), H-type TEF(2.l%), and so on. About half(49%) of the patients had one or more associated anomalies in addition to tracheoesophageal anomalies. Congenital heart disease was associated in 46 cases(31 %), anorectral malfomations in 19 cases(13%), musculoskeletal anomalies in 15 cases(10%), genitourinary anomalies in 10 cases(7%) and gastrointestinal anomalies in 7 cases(5%). Postoperatively, parenteral nutrition and assisted ventilation were given in 66% and 52% of patients respectively. Ninety three(74%) of 126 cases who underwent surgical procedure, experienced one or more complications such as respiratory complication (65%), leak(22%), stricture(21%) and so on. The survival rates related to the Waterston risk categories were 90.2% in grpup A, 71.4-75% in B₁, B₂, and C groups, and 28% in group C₂ and the overall survival rate was 71.4%. Thirty six(28.6%) of 126 cases died of pneumonia/sepsis(12 cases), respiratory failure(l2 cases), and congenital heart disease(4 cases). With short term follow-up, 69% of patients have been excellent whereas remainders of the cases have suffered from some sort of morbidity related to gastroesophageal reflux, recurrent respiratory infection, and esophageal stricure.
Aorta ; Diagnosis ; Echocardiography ; Esophageal Atresia ; Follow-Up Studies ; Gastroesophageal Reflux ; Heart ; Heart Defects, Congenital ; Humans ; Incidence ; Korea ; Parenteral Nutrition ; Surgeons ; Survival Rate ; Tracheoesophageal Fistula ; Ventilation

The survey on branchial anomalies was conducted by Korean Association of Pediatric Surgeons. A total of 173 cases were reported, which were managed by 36 members and cooperators during the three years from January 1, 1993 through December 31, 1995. The following results were obtained by retrospective analysis of the 173 cases of branchial anomalies. The presenting symptoms were cervical mass in 101 cases, pit with or without discharge in 71, cervical abscess in 47 and respiratory difficulty in 3. The average age of the patients with cervical abscess was 52 months. Seventy(79%) of 89 patients with branchial anomalies and a cystic mass had their first clinical manifestations by 1 year of age, while 40(51%) of 78 patients with only a branchial cyst had their first clinical manifestation in first year of life. Radiologic studies were carried out in 77 patients(43%). The preferred diagnostic modalities were ultrasonography(47 patients), simple neck radiogram(l9) and CT scan(17). Preoperative diagnosis was correctly made in 156(91%) of 173 patients. Seventeen patients were incorrectly diagnosed as thyroglossal duct cyst in 5 patients, cystic hygroma in 4, dermoid cyst in 3, and lymphadenopathy in 3. There were no remarkable difference in sex and laterality of presentation but bilateral lesions were found in 9(5%) patients and unusual locations of the anomalies were the manubrium, left subclavicular area, median cervial area, preauricular and parotid area. There were 78(45%) patients with cyst, 52(30%) patients with sinus, 35(20%) patients with fistula and 8(5%) patient with skin tag. Embryological classification was possible in only 64(37%) patients. The 2nd branchial anomaly was present in 50 (78%), the 1st branchial anomaly in 10(18%), and the 3rd or 4th branchial anomaly in 4(6%). Histopathological study of the lining epithelium(N=134) is recorded that 45% were lined with squamous epithelium, 17% with respiratory epithelium, 6% with. squamous and respiratory epithelium, 14% with inflammatory change. Lymphoid tissue was common(62%) in the wall of the lesions. Twelve(7%) of 158 patients had postoperative complications including wound complication, recurrence and facial nerve palsy.
Abscess ; Branchioma ; Child ; Classification ; Dermoid Cyst ; Diagnosis ; Epithelium ; Facial Nerve ; Fistula ; Humans ; Korea ; Lymphangioma, Cystic ; Lymphatic Diseases ; Lymphoid Tissue ; Manubrium ; Neck ; Paralysis ; Postoperative Complications ; Recurrence ; Respiratory Mucosa ; Retrospective Studies ; Skin ; Surgeons ; Thyroglossal Cyst ; Wounds and Injuries

Introduction: To report the indications and early treatment outcomes of pre-operative halo-pelvic traction in patients with neurofibromatosis associated with severe proximal thoracic (PT) spinal deformity. Materials and methods: We reviewed four patients with neurofibromatosis with severe PT spinal deformity. Case 1, a 16-year-old male presented with severe PT kyphoscoliosis (scoliosis: 89°, kyphosis: 124°) and thoracic myelopathy. Case 2 was a 14-year-old, skeletally immature male who presented with a PT lordoscoliosis (scoliosis: 85°). Case 3, a 13-year-old male, presented with severe PT kyphoscoliosis (scoliosis: 100°, kyphosis: 95°). Case 4, a 35-year-old gentleman, presented with severe PT kyphoscoliosis (scoliosis: 113°, kyphosis: 103°) and thoracic myelopathy. All patients underwent pre-operative halo-pelvic traction. After a period of traction, all patients underwent posterior spinal fusion (PSF) with autologous bone grafts (local and fibula bone grafts) and recombinant human bone morphogenetic protein-2 (rhBMP-2). Results: Both patients with thoracic myelopathy regained near normal neurological status after halo-pelvic traction. Following traction, the scoliosis correction rate (CR) ranged from 18.0% to 38.9%, while the kyphosis CR ranged from 14.6% to 37.1%. Following PSF, the scoliosis CR ranged from 24.0% to 58.8%, while the kyphosis CR ranged from 29.1% to 47.4%. The total distraction ranged from 50-70mm. Duration of distraction ranged from 26-95 days. The most common complication encountered during halo-pelvic traction was pin-related e.g. pin tract infection, pin loosening and migration, osteomyelitis, and halo-pelvic strut breakage. No patients had cranial nerve palsies or neurological worsening. Conclusion: Pre-operative correction of severe PT spinal deformities could be performed safely and effectively with the halo-pelvic device prior to definitive surgery.

Intraosseous lesions at phalanges are rare. They frequently present with pain and swelling. Fortunately, the majority of the lesions are benign. However, some lesions are destructive and early interventions are required. We report two cases of similar presentations of swelling and discomfort at the little finger for six months. The lytic lesions involved the whole middle phalanx with cortical breach sparing the joints. A provisional diagnosis of osteomyelitis was made, although unlikely. Bone biopsy was performed early to plan for definitive treatment and surgery. Patient 1 was diagnosed for intraosseous gout whereas Patient 2 for epithelioid hemangioma. Both were benign destructive bone lesions. Thus, we counselled the patients for curettage of lesion, bone grafting and spanning external fixation in view of extensive lesion. Patient 1 defaulted treatment. Patient 2 had an uneventful surgery. She regained her grip strength. In two years follow up, there was no evidence of infection, recurrence or malignant transformation.