The Korean Association of Pediatric Surgeons (KAPS) performed a nationwide survey on sacrococcygeal teratoma in 2018.
The authors reviewed and analyzed the clinical data of patients who had been treated for sacrococcygeal teratoma by KAPS members from 2008 to 2017.
A total of 189 patients from 18 institutes were registered for the study, which was the first national survey of this disease dealing with a large number of patients in Korea. The results were discussed at the 34th annual meeting of KAPS, which was held in Jeonju on June 21–22, 2018.
We believe that this study could be utilized as a guideline for the treatment of sacrococcygeal teratoma to diminish pediatric surgeons' difficulties in treating this disease and thus lead to better outcomes.
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The management of lymphatic malformation (LM) in pediatric patients is challenging. Complete excision of LM is difficult to achieve in some cases. We reviewed our experience how to manage LM.
We retrospectively reviewed the patients who were treated for LM between 2010 and 2017. Medical records were reviewed about age of diagnosis, age of treatment age, gender, symptom, location of tumor, treatment modality, response and complication.
Sixty-three patients (39 boys and 24 girls) were included. Mean age at diagnosis was 14.5±28.0 months (range, neonate-10 years). The involved lesion were head and neck in 27 patients (42.9%), abdominal cavity in 7 patients (11.1%), chest wall and abdominal wall in 11 patients (17.5%), buttock in 7 patients (11.1%), and extremities in 11 patients (17.5%). The treatment options were including surgical resection in 32 patients, sclerotherapy in 7 patients, surgical resection combined sclerotherapy in 19 patients, and close observation in 5 patients. We achieved complete remission in 39 patients. Fourteen patients showed partial remission and 6 showed recurrences.
Despite surgical difficulty, meticulous excision with supportive treatment, and adjuvant sclerotherapy could get a favorable outcome without fatal complication. Decision should be based on surgeon's experience, location of LM, related symptoms, and consultation with patient's parents.
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Long time total parenteral nutrition (TPN) can induce irreversible liver damage. In this study, we investigated the associated factors of parenteral nutrition associated cholestasis (PNAC) in neonates.
We retrospectively reviewed 227 neonates (male:female=110:117) those who had received TPN over 2 weeks from March 2010 to February 2014. PNAC was defined as direct bilirubin was higher than 2.0 mg/dL without any cause except TPN.
Overall incidence was 28.6%. PNAC was frequently developed in younger gestational age with lower birth weight. Episodes of sepsis, underlying bronchopulmonary dysplasia, history of necrotizing enterocolitis, and experience of gastrointestinal surgery increase the incidence of PNAC. PNAC was directly associated the duration of TPN and long period to full enteral feeding, reaching 60 and 150 mL/kg/day. Overall mortality rate was 9.7%. It was higher in PNAC group despite PNAC was not the primary cause of death. All survivors were recovered from cholestasis with encourage of enteral nutrition.
PNAC in neonate was associated with younger gestational ages and lower birth weights, duration of TPN, or who experienced sepsis, necrotizing enterocolitis, gastrointestinal surgery or bronchopulmonary dysplasia.
Congenital duodenal obstruction is a one of the emergent surgical conditions in neonates. Almost of them were diagnosed with double-bubble sign in prenatal ultrasonography. However, partial obstruction caused from duodenal web could be overlooked. We reported a duodenal web in early childhood. A three-year-old girl visited at our pediatric clinic for constipation. She had been showed non-bilious vomiting after weaning meal since 6 months old of her age, but her weight was relevant for 50-75 percentile of growth curve. Barium enema was initially checked, but any abnormal finding was not found. We noticed the severely distended stomach and 1st portion of duodenum. Upper gastrointestinal series revealed partial obstruction in 2nd portion of duodenum. After laparotomy, we found the transitional zone of duodenum and identified a duodenal web via duodenotomy. We performed duodeno-duodenostomy without any injury of ampulla of Vater. She was recovered uneventfully. During 6 months after operation, she does well without any gastrointestinal symptoms or signs, such as vomiting or constipation.
Palpable inguinal mass in children should be differentiated from inguinal hernia, hydrocele, lymph node, and tumor. Though using ultrasonography, fatty tumor would be misdiagnosed as incarcerated inguinal hernia containing fatty component. We experienced the huge inguinal lipoblastoma in 5-year-old girl mimicking recurrent incarcerated hernia. Laparoscopic exploration revealed it was not incarcerated hernia but well demarcated bulging mass from abdominal wall. Mass was about 10×4×3 cm and extended from internal inguinal ring to saphenous opening. It was near total excised because of right external iliac vein injury. Pathologically, it was proven as lipoblastoma containing mature adipocyte with lipoblast and fibrous septa. Postoperatively, we noticed a segmental thrombotic occlusion of external iliac vein. After 1 year, she has no symptom related to occluded vessel. The remained lipoblastoma showed no interval change. Even lipoblastoma has a good prognosis with low recurrence rate, we need careful follow-up.
Tracheoesophageal fistula without esophageal atresia (H-type TEF) is a congenital anomaly that is characterized by a fistula between the posterior wall of the trachea and the anterior wall of the esophagus, not accompanied by esophageal atresia. The purpose of this study is to investigate the clinical characteristics, diagnostic time, the side of cervical approach and short term result after surgery by searching medical records of patients treated for H-type TEF. The search was done at University of Ulsan, Department of Pediatric Surgery of Asan Medical Center, and the total number of patients from May 1989 to December 2010 was 9 with M:F ratio of 1:2. The median gestational age was 39(+6) (32(+6)~41(+0)) wks. Seven out of nine patients were born at term and the other two were born premature. The clinical presentation was aspiration pneumonia, difficulty in feeding, chronic cough, vomiting, abdominal distension and growth retardation. The symptoms presented right after birth. The diagnosis was made with esophagography and the median time of diagnosis was 52 days of life. The majority of surgical corrections were performed within two weeks of diagnosis (median; 15d, range; 1d - 6m). Six patients had associated anomalies, and cardiac anomalies were most common. The cervical approach was utilized in all cases (right 2, left 7). Transient vocal cord palsy and minor esophageal leakage complicated two cases. Although the diagnosis of H-type TEF was difficult and often delayed, we had a good short term result. The left cervical approach was preferred.
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Although the incidence of esophageal atresia (EA) is higher in twins than in singletons by two to three times, EA usually affects only one member of twins. We report one pair of twins concordant for EA. A 31-year-old healthy woman bore monozygotic female twins at 36 weeks of gestation. They weighed 2,216 and 2,480g, respectively. They had EA with distal tracheoesophageal fistula and underwent primary esophageal anastomosis on the birth day and the 2nd day of life, respectively. Twin A also had suspicious antral obstruction and pyloroplasty was done simultaneously with esophageal repair. She needed antral web excision for continued gastric stasis one month after 1st operation and three balloon dilatations of the esophagus. Twin B recovered uneventfully.
Esophageal atresia with double tracheoesophageal fistula is a very rare anomaly and is difficulty to diagnose preoperatively. We treated a full term baby with esophageal atresia with double tracheoesophageal fistula. At the first operation, only the distal tracheoesophageal fistula was identified and ligated. When the upper esophageal pouch was opened, intermittent air leakages in sequence with positive bagging were noticed. However, intraoperative bronchoscopy did not identify a fistula in the proximal pouch, and the operation was completed with end to end anastomosis of the esophagus. On the 7th postoperative day, esophagography showed another tracheoesophageal fistula proximal to the esophageal anastomosis. A wire was placed in the fistula preoperatively under bronchoscopy. At the 2nd operation through the same thoracotomy incision the proximal fistula was identified and ligated. On the 12th postoperative day, esophagography showed neither stricture nor leakage.
Prophylactic contralateral exploration in unilateral inguinal hernia repair is still controversial. The purpose of this study is to analyze the contralateral incidence of hernia and to verify the necessity of the simultaneous contralateral exploration. Infants and children operated on for inguinal hernia or hydrocele at the Department of Pediatric Surgery of Asan Medical Center from January 1996 to December 2005 were analyzed retrospectively. A total of 383 patients (9.8 %) out of 3,925 patients underwent a simultaneous bilateral operation. A total of 222 patients (6.2 %) out of 3,542 patients underwent a secondary metachronous contralateral operation after primary unilateral inguinal hernia or hydrocele repair. Because simultaneous bilateral operation cases included true bilateral inguinal hernia or hydrocele, and unilateral hernia and simultaneous contralateral exploration, bilateral incidence of inguinal hernia and hydrocele could be maximally considered as 15.4% (605 patients). Therefore, the prophylactic contralateral exploration in unilateral inguinal hernia or hydrocele should be determined carefully in considering history and physical examination of the patients, and postoperative complications.
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Recently, the survival rates of extremely low-birth-weight (ELBW) infants have improved with the development of neonatal intensive care. However, these infants were susceptible to intestinal perforation due to prematurity, fluid restriction, and injection of indomethacin, etc. Because of the risks of transportation, anesthesia and surgery itself, peritoneal drainage has been compared with laparotomy. Through our experience, we investigate the usefulness of peritoneal drainage retrospectively. From 1997 to 2007, six ELBW (M:F=5:1) underwent primary peritoneal drainage for intestinal perforation. Their median birth weight was 685 g (405~870) and gestational age was 25+1 weeks (24+3~27+0). We noticed the intestinal perforation at median 10.5 days (8~18) after birth, and placed Penrose drain or Jackson-Pratt drain through right lower quadrant incision under local anesthesia. The cause of intestinal perforation was necrotizing enterocolitis in one patient, but that of the others was not clear. Three patients who showed normal platelet count and stable vital signs recovered uneventfully. Two patients (birth weight less than 500 g) who showed unstable vital signs and low platelet count (12,000 / mm3 to 30,000 / mm3) expired despite aggressive resuscitation. One patient required laparotomy due to persistent intestinal obstruction after drain removal and survived. Our experience shows that peritoneal drainage was an acceptable treatment for ELBW infants and the prognosis was related to vital sign and platelet count at the time of intestinal perforation, and birth weight.
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There is significant morbidity and mortality associated with the combination of esophageal atresia (EA) and duodenal atresia (DA). Nevertheless, the management protocol for the combined anomalies is not well defined. The aim of this study is to review our experience with the combined anomalies of EA and DA. From May 1989 to August 2006, seven neonates were diagnosed as EA with DA at Asan Medical Center. In all cases, the type of EA was proximal EA and distal tracheoesophageal fistula (TEF). The diagnosis of DA was made in theprenatal period in 1, at birth in 4, 4 days after birth in 1 (2 days after EA repair) and at postmortem autopsy in 1. Except the one case where DA was missed initially, primary simultaneous repair was attempted. DA repair with gastrostomy followed by EA repair in 2, EA repair followed by DA repair without gastrostomy in 2, and TEF ligation followed by DA repair with gastrostomy in 1. There were two deaths. One baby had a large posterolateral diaphragmatic hernia, and operative repair was not attempted. The other infant who had a TEF ligation and DA repair with gastrostomy expired from cardiac failure due to a large patent ductus arteriosus. Simultaneous repair of EA and DA appears to be an acceptable management approach for the combined anomalies, but more experience would be required for the selection of the primary repair of both anomalies.
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Lipoblastoma is a rare benign soft tissue tumor occurring in infancy and early childhood. It is characterized by fat lobules with varying degrees of maturity, multivaculoated lipoblasts, fibrocapillary networks and myxoid stroma. Lipoblastoma has a good prognosis with no metastases despite its potential for local invasion. From Jan, 1990 through April, 2007, 12 children underwent the operation for lipoblastoma, 7 boys and 5 girls, diagnosed at median 22 months (5~43 months). Median follow up was 6 year 7 months. Primary sites included back (n=5), intraabdominal (n=2) and one in each of buttock, chest wall, neck, nose and scalp. Tumors presented with a growing mass in 9 patients, abdominal distension in 2, and an incidental finding on chest radiography in one. Complete excisions were done in all patients. There was one recurrence in a patient with a scalp mass. After reoperation, he has been doing well without evidence of recurrence. Lipoblastoma has a favorable prognosis, but recurrence can occur even with complete excision. Regular follow up is necessary to detect recurrences.
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Teratomas arising from totipotential primitive germ cell are composed of 2 or 3 germ cell layers. We reviewed the records of 166 children who underwent the operation for teratoma from Jan, 1990 through April, 2007. There were 40 boys and 126 girls (average age 6.93±5.83 years). Primary sites were ovary (n=88), sacrococcygeum (n=24), testis (n=16), retroperitoneum (n=16), mediastinum (n=8), brain (n=4), thymus (n=3) and a single tumor involved the adrenal gland, neck, middle ear, oropharynx, stomach, pelvis, omentum, chest wall and scalp. Teratomas were mature in 141 patients, and immature in 25. Six patients who had ovarian immature teratomas grade 2 or 3 with peritoneal gliomatosis underwent adjuvant chemotherapy. During follow up period, 6 mature teratomas recurred at sacrococcygeal area (n=1) and contralateral ovary (n=5). Five patients of them underwent reoperation and diagnosed as a mature teratoma, but one who had underwent a left salpingooophrectomy with right ovary cystectomy at initial operation was observed carefully. Teratomas were dominant in female patients and developed at various organs. Complete excision was needed for good prognosis. In case of immature teratoma, complete excision and appropriate chemotherapy according to grading can contribute to favorable results.
Meckel's diverticulum is the most common congenital anomaly of gastrointestinal tract in children. The incidence of complicated Meckel's diverticulum is about 4%. The major complications of Meckel's diverticulum are bleeding, intussusception, obstruction and perforation. The aim of this study was to investigate the clinical manifestations and the role of laparoscopic surgery in complicated Meckel's diverticulum in children. We retrospectively reviewed the medical records of 19 patients with complicated Meckel's diverticulum who underwent operation at Asan Medical Center between Jan. 1990 and Apr. 2007. Male to female ratio was 11:8, and median age was 1 year (1 day-13 years). The most frequent symptom was hematochezia (68%), followed by irritability or abdominal pain (16%), vomiting (11%), and abdominal distension (5%). Two operative procedures were performed; small bowel resection with anastomosis (68%) and diverticulectomy (32%). The operation proven complications of the Meckel's diverticulum were bleeding (68%), intussusception (16%), perforation (11%) and obstruction (5%). Ectopic tissues found by postoperative pathologic examination were gastric (84%) and pancreatic (11%). Hospital stay after laparoscopic operation for bleeding Meckel's was 5 days (median) and average first postoperative feeding was 1.5 days. On the contrary, hospital stay for open surgery was 7 days and first feed was 3 days. In summary, the most common compliation of Meckel's diverticulum in children was bleeding and ectopic gastric tissues were present in 84%. Laparoscopic procedure seemed to be useful for diagnosis as well as for definitive treatment.
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Fundoplication is accepted as an effective treatment of gastroesophageal reflux disease. The recent results of laparoscopic fundoplication demonstrated safety and less morbidity, shorter hospital stay and less pulmonary complication compared to the open operation. Laparoscopic fundoplication has been our first choice of operation for gastroesophageal reflux disease since 2003. Among 29 cases, there were 2 conversion cases because of severe distension of transverse colon and hepatomegaly. We studied 27 consecutive patients operated upon from January 2003 through December 2004. There were 15 boys and 12 girls, ages from 1.5 months to 12 years (median 25.3 months). Body weight ranged from 2.9 kg to 37 kg (median 9.8 kg). Neurological abnormalities were present in 23 patients. Indications for surgery included medically refractory reflux associated with vomiting, pneumopathy, otorhinolaryngologic pathology, failure to thrive, esophagitis, apnea and bradycardia. We used 4-5 trocars of 5 mm or 12 mm with 30° telescope and performed the Nissen technique in all patients. In neurological impaired patients, gastrostomy tube was placed at the time of fundoplication. Median operative time was 130 minutes (70 - 300 minutes). There was no mortality nor intraoperative complication. Twenty-six patients were followed for median of 19 months (8 - 31 months). Four patients (15.4 %), who were all neurological impaired, developed recurrent symptoms of gastroesophageal reflux disease. Two of these patients had reoperation (1 laparoscopic approach, 1 open method). There were significant increases in body weight in 11 patients after fundoplication. Laparoscopic fundoplication is acceptable as a safe and effective method for gastroesophageal reflux disease.
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Chemoport is widely used in pediatric surgery field. But various complications can occur during the process of insertion or removal of chemoport. Surgeons must be familiar with the treatment of these complications. We had one catheter cuts off during chemoport removal, become a catheter embolism. Interventional radiologic removal was successful. Verifying the length of removed catheter and careful observation of the catheter tip during removal procedure is important to prevent the possibility of catheter embolus. Radiologic intervention was accessible to remove the retained catheter.
Laparoscopic cholecystectomy has been increasingly used because of several advantages, less pain, better expectation for cosmesis (requires small incisions), and more rapid recovery compared with open cholecystectomy. Oral intake is tolerated on the day of operation or on the next. In this study, we evaluated the effectiveness and safety of laparoscopic cholecystectomy in children. Nine cases of laparoscopic cholecystectomy for acute and chronic cholecystitis in children were performed at Asan Medical Center between April 2002 and April 2004. Laparoscopic cholecystectomy was performed on a total of 10 patients, but one of them was excluded because of the simultaneous splenectomy for sickle cell anemia. Clinical presentation, operative findings, operation time, length of hospital stay, and postoperative complications were analyzed. Mean age was 10.4 (4–15) years, and only 3 of patients were less than 10 years. One patient was female. In 8 the diagnosis was calculous cholecystitis. Mild adhesions were found in 3 cases and intraoperative bile leakage in 2. There was no conversion to open surgery and there were no vascular, bowel, or bile duct injuries. Mean operation time was 82.2 (20–160) minutes ; mean length of hospital stay was 2.1 (1–3) day. There was no postoperative complication. Laparoscopic cholecystectomy in children was remarkably free of side effects and complications and had a short recovery time. Laparoscopic cholecystectomy for cholecystitis is considered to be a standard procedure in children.
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There are considerable controversies in the management of congenital diaphragmatic hernia. By 1997, early operation, routine chest tube on the ipsilateral side and maintainingrespiratory alkalosis by hyperventilation were our principles (period I). With a transition period from 1998 to 1999, delayed operation with sufficient resuscitation, without routine chest tube, and permissive hypercapnia were adopted as our practice. High frequency oscillatory ventilation (HFOV) and nitric oxide (NO) were applied, if necessary, since year 2000(period II). Sixty-seven cases of neonatal Bochdalek hernia from 1989 to 2005 were reviewed retrospectively. There were 33 and 34 cases in period I and II, respectively. The neonatal survival rates were 60.6 % and 73.5 %, respectively, but the difference was not significant. In period I, prematurity, low birth weight, prenatal diagnosis, inborn, and associated anomalies were considered as the significant poor prognostic factors, all of which were converted to nonsignificant in period II. In summary, improved survival was not observed in later period. The factors considered to be significant for poor prognosis were converted to be nonsignificant after change of the management principle. Therefore, we recommend delayed operation after sufficient period of stabilization and the avoidance of the routine insertion of chest tube. The validity of NO and HFOV needs further investigation.
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VATER association is defined as a combination of 3 or more anomalies-vertebra(V), imperforate anus (A), esophageal atresia with or without tracheoesophageal fistula (TE), renal and radial anomaly(R). We reviewed our experiences in one center to determine etiology, prevalence, clinical manifestation, other associated anomaly and prognosis. Two hundred and twenty-three cases that underwent operations for imperforate anus or esophageal atresia were analyzed retrospectively through medical records at Department of Pediatric Surgery, Asan Medical Center from June, 1989 to July, 2005. The total number of neonates who had been admitted during period of study were 46,773 and VATER association was 9 (0.019 %, 1.92 persons per 10,000 neonates). Median gestational age and birth weight were 37+4wk (35+1 – 41+4) and 2,594 g (1,671–3,660), respectively and median age of mother was 32 years (23–38). There was no family history. Three patients were twins but their counterparts had no anomalies. Patients who have 3 anomalies were 6, 4 anomalies in two and 5 anomalies in one patient. Vertebra anomalies were detected in 7(77.7 %), imperforate anus in 8(88.9 %), esophageal atresia in 5 patients (55.6 %), renal anomaly in 6(66.7 %), and radial anomaly in 5(55.6 %), respectively. Four patients are alive, 2 patients were lost during follow up period. Three patients died due to neonatal sepsis, respiratory dysfunction and cardiac failure. VATER association did not appear to be a definite risk factor, but merely a randomized combination of 5 anomalies. The prognosis was dependent on the other associated anomalies, appropriateness of management and operation. Careful follow-up and aggressive treatmentare required for improving survival and quality of life.
One day surgery in children has been practiced for last 10 years in this institution. This study is to examine its safety and effectiveness for patients younger than 15 years old treated at the Department of Pediatric Surgery, Asan Medical Center, from September. 1996 to December, 2005. A total of 3,709 patients, 37 % of the total pediatric operations, are included in this retrospective study. The most prevalent ages were between 1 and 3 years olds (1,199 patients). Twenty patients were younger than 6 months, and they all had one day surgery safely. Operations were herniorrhaphy in 3,126 patients,followed by excisional biopsy, chemoport removal, and OK 432 injection. There were 12 cases (0.32 %) of unplanned admissions, 7 occurred within 6 months of one day surgery. Perioperative fever was the most common cause of admission in 4 cases. The related conditions of unplanned admission were bleeding in 2 cases and radical surgery in 2. One day surgery in this institution was easily accessible and safe. This is to the result of appropriate selection of patients, cooperation with anesthesiologists, adequate control of postoperative pain, and home care programs.
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