신생아 및 영아기의 허쉬슈프렁병 진단

김대연,김성철,김경모,김애란,김기수,김정선,구현우,윤종현,김진천,피수영,김인구,Kim, Dae-Yeon,Kim, Seong-Chul,Kim, Kyung-Mo,Kim, Ellen Ai-Rhan,Kim, Ki-Soo,Kim, Jung-Sun,Goo, Hyun-Woo,Yoon, Chong-Hyun,Kim, Jin-Cheon,Pil, Soo-Young,Kim, I 대한소아외과학회 2002 소아외과 Vol.8 No.1

Diagnosing Hirschsprung's disease is a clinical challenge. Hirschsprung's disease should be considered in any child who has a history of constipation dating back to the newborn period. We examined diagnostic methods and their results retrospectively in 37 neonates and infants who underwent both barium enema and anorectal manometry for the diagnosis of Hirschsprungs disease at Asan Medical Center between January 1999 and April 2001. Two radiologists and a surgeon repeatedly reviewed both of the diagnostic results. In anorectal manometry, thirty-four studies were in agreement with the definitive diagnosis, giving an overall diagnostic accuracy of 91.9 % (neonate; 100 %, infant; 85.7 %). The accuracy and specificity of barium enema was lower than those of anorectal manometry, but sensitivity was higher. There was no significant difference between the two methods. Both studies showed findings consistent with the final diagnosis. However, discordant results needed further evaluation or close observation to diagnose accurately. We conclude that Hirschsprungs disease should not be diagnosed by only one diagnostic method.

미세아의 외과적 문제점들

김대연,김성철,김애란,김기수,피수영,김인구,Kim, Dae-Yeon,Kim, Seong-Chul,Kim, Ellen Ai-Rhan,Kim, Ki-Soo,Pi, Soo-Young,Kim, In-Koo 대한소아외과학회 2006 소아외과 Vol.12 No.1

Recent advances in neonatal management have resulted in a dramatic increase in survival of very low birth weight infants. These critically ill infants, however, continue to pose significant challenges in management and ethics. There is little information on the outcome of the micropremie (birth weight less than 800 g) that require surgery. The records of 171 micropremies treated over a 15 year period (beginning in 1989) at Asan Medical Center was reviewed retrospectively. Forty-one (24.0 %) infants required surgical interventions by pediatric surgeons. There were 90 boys and 81 girls. The smallest infant, weighed 396g at birth, had esophageal atresia and died before surgery. The smallest survivor, birth weight 645 g, received anenterostomy for necrotizing enterocolitis at the weight of 590 g. The gestational age of the group rangedfrom 21 to 36 weeks. The most common surgical problem was inguinal hernia. There were 20 inguinal hernias, and repairs were performed on17 infants. Excluding 2 cases, hernia repair was performed at the time of discharge. There was only one recurrence of adirect inguinal hernia. Necrotizing enterocolitis developed in 17 patients, 11 were operated upon, two had peritoneal drainages, and 9 had enterostomies. Five of 11 surgical infants died after operation and three of the nonsurgical infants died of various complications. Although micropremies have potentially high risks of serious complications and death, the outcome can improve with careful surgical observation and judgment.

소아 비천공 충수염에 대한 복강경 충수절제술

김대연,김성철,김인구,Kim, Dae-Yeon,Kim, Seong-Chul,Kim, In-Koo 대한소아외과학회 2003 소아외과 Vol.9 No.1

Appendicitis is the most common surgical emergency in childhood. Open appendectomy has been the standard treatment. The minimally invasive techniques have been adopted in children after experiences in various surgical conditions in adults has accumulated. It is debatable whether laparoscopic appendectomy (LA) is superior to open appendectomy (OA) in children. The goal of this study is to review the results of laparoscopy and laparotomy in the nonperforated appendicitis. The records of 22 patients under 15 years of age who were operated upon for nonperforated appendicitis at Asan Medical Center were analyzed between December 2002 and April 2003. Age, type and length of intervention, frequency of analgesic use, complication, length of hospitalization, and cost for each treatment groups (N=11) were compared. Laparoscopy patients were older (13.0 vs. 10.1 years; p > 0.05), and operative time was longer (55.0 vs. 35.0 minutes; p < 0.05). There was no conversion (OA to LA). The median length of hospital stay was significantly shorter in laparoscopy (3.0 vs. 2.0 days; p < 0.05). The median cost for LA was more expensive (W833, 836 vs. W751,398; p < 0.05). Even though there were higher costs and longer operative times with laparoscopic procedures, the shorter hospital stay was an advantage.

WDHA증후군을 동반한 신경절모세포종

김대연,김기홍,김상범,정성은,이성철,박귀원,김우기,Kim, Dae-Yeon,Kim, Ki-Hong,Kim, Sang-Beom,Jung, Sung-Eun,Lee, Seong-Cheol,Park, Kwi-Won,Kim, Woo-Ki 대한소아외과학회 2000 소아외과 Vol.6 No.1

The WDHA syndrome characterized by watery diarrhea, hypokalemia, and achlorhydria (WDHA syndrome) is rare, and with neurogenic tumors. A 20-month-old girl presenting with symptoms of WDHA syndrome was transferred to our hospital. She had a ganglioneuroblastoma of the adrenal gland. The serum level of VIP was elevated. After complete excision of the tumor, all symptoms related to the WDHA syndrome were relieved and serum VIP level dropped to normal. The postoperative course was uneventful. The patient was treated with postoperative chemotherapy and radiation therapy. There was no evidence of disease 33 months after operation.

소아외과에서의 Henoch-Sch$\ddot{o}$nlein 자반증

김대연,김성철,김인구,Kim, Dae-Yeon,Kim, Seong-Chul,Kim, In-Koo 대한소아외과학회 2000 소아외과 Vol.6 No.1

Henoch-Sch$\ddot{o}$nlein purpura is a systemic vasculitis of unknown etiology that is probably related to an autoimmune phenomenon. Henoch-Sch$\ddot{o}$nlein purpura is characterized by a purpuric rash, arthralgia, nephritis, and gastrointestinal manifestations. We reviewed 169 children hospitalized with a diagnosis of Henoch-Sch$\ddot{o}$nlein purpura between 1989 and 1998. One-hundred thirty-nine (82.2 %) had gastrointestinal findings including abdominal pain, nausea, vomiting and gastrointestinal bleeding. Surgical consultations were obtained for ten children, and laparotomy was performed in five. Three children suspected of having appendicitis underwent appendectomy. None had appendicitis. One child thought to have been intestinal strangulation was found to have hemorrhagic edema of the proximal jejunum and of the distal ileum. Another child underwent resection for an hemorrhagic infarct of the distal ileum. A high index of suspicion of this disease entity in the differential diagnosis of abdominal pain in children can avoid unnecessary laparotomy in most cases. However, life-threatening gastrointestinal complications may occur in low percentage of cases. Prompt recognition and adequate radiologic evaluation of the abdominal manifestation of this entity is necessary for early surgical intervention.

만삭아에 발생한 괴사성 장염

김대연,김성철,김경모,김애란,김기수,피수영,김인구,Kim, Dae-Yeon,Kim, Seong-Chul,Kim, Kyung-Mo,Kim, Ellen Ai-Rhan,Kim, Ki-Soo,Pi, Soo-Young,Kim, In-Koo 대한소아외과학회 2003 소아외과 Vol.9 No.1

Necrotizing Enterocolitis (NEC) is usually a disease of premature infants, but occasionally it affects the term neonate. Twenty-five infants with NEC were treated at Asan Medical Center between January 2000 and December 2002, and 13 of them were term infants. In each case, the diagnosis of NEC was established by a clinical illness fulfilling the Bell's stage II or III NEC as modified by Walsh or by surgical findings. There were six males and seven females. The birth weight was from 1,960 to 3,700 g. The age at diagnosis was from 1 to 40 days. Four patients had congenital heart disease: one of who had hypothyroidism and cleft palate. Abdominal distension was present in all, and bloody stools in four. One patient had history of hypoglycemia, three had Rota viral infection. Eight patients had leucopoenia (<$5.0{\times}10^9/L$), seven had thrombocytopenia (<$100{\times}10^9/L$), and three severe thrombocytopenia (<$50{\times}10^9/L$). Laparotomy was required in 10 of the 13 patients. Indications for operation in the acute phase were failure to respond to aggressive medical therapy in five, and perforation in three patients. There were two late phase operations for intestinal stricture and fistula. There were no operative complications. Ten of thirteen patients survived (76.9%). Two patients died of septic complication. There was a delayed death due to heart failure. There was a significant difference in survival according to platelet count ($50{\times}10^9/L$) (p<0.05). Congenital heart disease and Rota viral infection are associated with NEC in term infants and thrombocytopenia and leucopoenia may be surgical indications.

소아의 위장관 중복증

김대연,김성철,김인구,Kim, Dae-Yeon,Kim, Seong-Chul,Kim, In-Koo 대한소아외과학회 2001 소아외과 Vol.7 No.1

Gastrointestinal duplications are rare congenital malformation that may require surgical intervention in the neonate, infant, and occasionally the older child. Symptoms produced by duplications vary according to their location, size, type and histology. We report the clinical characteristics and the surgical results of 9 cases of the gastrointestinal duplications treated at at Asan Medical Center between 1989 and 2000. Five patients were boys and four were girls; age of patients ranged from 5 days to 10 years. Eight duplications were cystic and one was tubular. One involved the stomach; five were in the ileum, and two in the cecum. The most common presentation was intestinal obstruction. There was associated anomaly in one patient, pulmonary sequestration and double ureter. Ectopic gastric mucosa was found in two. All patients underwent surgical resection. There was no perioperative mortality or morbidity. Although gastrointestinal duplication is a rare entity. consideration of associated anomalies and being familiar with the anatomy and clinical features are required for adequate management. In cystic form. complete excision is recommended but planned surgery is required for long segment tubular lesion.

극소 저출생체중아에서의 소장천공

김대연,김성철,김애란,김기수,피수영,김인구,Kim, Dae-Yeon,Kim, Seong-Chul,Kim, Ai-Rhan,Kim, Ki-Soo,Pi, Soo-Young,Kim, In-Koo 대한소아외과학회 2001 소아외과 Vol.7 No.2

With the advances in neonatal intensive care, pediatric surgeons experience very low birth weight infants, weighing <1,500 g, more frequently. We report our 14 cases of very low birth weight infants with intestinal perforations without congenital causes, at the Asan Medical Center during the 11-year period from 1989 to 2000. The average birth weight was 919 g(563-1,490), and average gestational age was 206 days(161-286). There were nine males and five females, Operation was performed at an average age of 14.0 days(3-38). Ten neonates with symptomatic PDA were given indomethacin in an attempt to close the ductus. Bowel perforation involved the jejunum in two and ileum in twelve. At laparotomy, there were seven focal intestinal perforations, five typical NEC, one intussusception, and an unknown cause, Four neonates underwent resection and anastomosis of the bowel, and nine underwent exteriorization. One underwent resection and anastomosis after peritoneal drainage. Four patients had postoperative complications; two leakage of anastomosis, one stoma necrosis, and one internal herniation. Seven of fourteen patients survived(50.0 %). Seven patients died of septic complication. There was a significant difference in the birth weight and gestational age in survivors compared with those who died(p<0.05). There was an increased risk of bowel perforation in indomethacin treatment for PDA. Careful clinical observation and keen judgment are essential for this particular group of infants.

선천성 무섬유소원혈증 환자에 발생한 비장 파열의 치료

김대연,김성철,김인구,Kim, Dae-Yeon,Kim, Seong-Chul,Kim, In-Koo 대한소아외과학회 1999 소아외과 Vol.5 No.2

Congenital afibrinogenemia is a rare disorder characterized by a congenital lack of fibrinogen, a key component of the hemostatic system. Bleeding manifestations of congenital afibrinogenemia vary in severity from mild to catastrophic. This is a case report of splenic rupture occurred in an eight-year-old boy with congenital afibrinogenemia. Nonoperative treatment with cryoprecipitate and virally inactivated, purified fibrinogen concentrates successfully avoided splenectomy.

신경모세포종 -15년간 한 병원에서의 임상적 경험-

김대연,김성철,김인구,Kim, Dae-Yeon,Kim, Seong-Chul,Kim, In-Koo 대한소아외과학회 2005 소아외과 Vol.11 No.2

Neuroblastoma treatment remains challenging, but treatment has become more effective due to the establishment of clinical and biological variables that determine prognostic risks. Initially, stage and age were the prime determinants of survival used in clinical practice. Risk-based therapy currently is the hallmark of neuroblastoma treatment. This study reviews one center's experience with the management of neuroblastoma. Sixty-three patients with neuroblastoma were treated from 1989 to 2003. All patients were graded according to the International Neuroblastoma Staging System (INSS) at diagnosis. There were 37 boys and 26 girls. The median age was 2.14 years (range, 33 days-10.2years). The primary site was the adrenal gland in 47, dumbbell shape extending into spinal canal in 6, retroperitoneum in 5, mediastinum in 3, and other sites in 2. The probability of 5-year overall survival (OS) and event free survival (EFS) were 46.7 % and 44.2 % by Kaplan-Meier method. According to INSS, there were stage 1 in 2 cases, stage 2 in 5, stage 3 in 12, stage 4 in 42, and stage 4s in 2. There were statistically significant differences in the survival rates between patients with stage1, 2 and stage 3, 4(P<0.05). For the stage 3 and 4, the extents of surgical resection, determined from the operative records and pathologies, were complete resection in 17 cases, minimal residual in 15, and partial resection 11, and the 5-year OS rate was 57.8, 51.4, and 13.6 %, respectively. There is a trend toward higher OS with more complete resection (P<0.05). We conclude that age and stage at diagnosis are prognostic factors, and complete excision of the primary tumor can provide better prognosis for patients with stage 3 and 4 neuroblastoma.