Lymphatic malformations (LMs) are congenital malformations of the lymphatic system which can be effectively treated by sclerotherapy. This study aims to evaluate the efficacy of doxycycline in the treatment of LMs.

We retrospectively reviewed the medical records of all patients who were diagnosed as LMs and underwent doxycycline sclerotherapy in Asan Medical Center between March 2013 and February 2014. Thirty-five sclerotherapy procedures were performed on 21 patients. The procedures were performed under general anesthesia. After each treatment, the clinical and radiographic response was characterized as complete (≥80% decrease in lesion size), partial (<80% decrease of size), or no response (no decrease of size).

There were 11 male patients and 10 female patients. The median age of sclerotherapy was 21 months (range, 2–180 months). The most common location was cervicofacial (52.3%), followed by extremity (28.6%) and truncal (19.0%). The most common lesion type was macrocystic (71.4%), followed by microcystic (28.5%). There was one (2.8%) skin necrosis which was recovered by wound management. Thirty-eight percent of patients had a complete response, 47.6% of patients had a partial response and 14.3% of patients had no response. Median frequency of treatment was one (range, 1–5). No response group consisted of all microcystic type.

Sclerotherapy with Doxycycline is safe and effective for macrocystic LMs.

Barium enema is one of the diagnostic modalities for Hirschsprung'sdisease. The present study aimed to investigate the diagnostic accuracy of barium enema for Hirschsprung's disease, especially total colonic aganglionosis (TCA).

We retrospectively reviewed the medical records of all the patients who were diagnosed as having TCA and underwent a barium enema in Asan Medical Center Children's Hospital between January 1998 and December 2016. All the tests were performed and reviewed by pediatric radiologists.

Among the total 19 patients with TCA who underwent barium enema, 9 patients (47.4%) had accurate radiographic results. Eight of the 13 neonate patients (61.5%) showed typical TCA radiological findings. However, only one of the 6 patients aged >4 weeks (16.7%) had accurate radiological diagnosis.

Barium enema showed low accuracy for TCA, and its diagnostic performance was better in neonatal period than in those aged >4 weeks.

Spontaneous neonatal gastric perforation is a rare but fatal disease with unclear etiology. In this study, we reviewed its clinical manifestations, outcomes, and discussed the etiology and prognostic factors.

There were 12 neonates with gastric perforation in our hospital from 1989 to 2015. Their medical records were reviewed retrospectively including birth record, associated disease, site and size of perforation, type of surgical management, clinical outcome. Also, the prognostic factors were analyzed.

The median gestational age and birth weight was 32 weeks (range, 26-43 weeks; preterm birth rate, 66.7%) and 1,883 g (range, 470-4,400 g), respectively. Five patients had associated gastrointestinal anomalies including esophageal atresia and tracheoesophageal fistula (two patients), midgut volvulus, non-rotation and microcolon, and meconium plug syndrome. The median age at surgery was six days after birth (range, 2-13 days), and the median weight at surgery was 1,620 g (range, 510-3,240 g). Upper third part of stomach was the most frequently involved location of perforation. The size of perforation varied from pin point to involving the whole greater curvature. Primary repairs were done in seven cases, and in five cases, resections of necrotic portion were needed. Mortality rate was 33.3% (n=4), morbidity (re-operation) rate was 16.7% (n=2). The causes of death were sepsis (n=3), and heart failure from Ebstein anomaly (n=1). The median hospital stay was 92.5 days (range, 1-176 days). The factors mentioned as prognostic factors in previous studies showed no significant relations to the mortality and morbidity in our study.

There were improvements of outcomes in patients with large size perforation. As previous studies, we assume these improvements were possible due to the improvements of critical care medicine. Given that rare incidence, a multi-center study can help us get a better understanding of this disease, and a better outcome.

This study aimed to assess the long-term clinical outcome of laparoscopic Nissen fundoplication (LNF) in children according to their neurologic status.

The study retrospectively analyzed the data of 82 children (62 neurologically impaired and 20 neurologically normal children with primary gastroesophageal reflux disease) who had undergone LNF between 2003 and 2012. The main outcome measures were the occurrence of recurrence that required reoperation and post-procedure complications such as infections, pneumonia, and gastrointestinal complications including ileus, dysphagia, and delayed gastric emptying.

The median age at the time of the LNF was 25 months (range, 1-192 months), and the median of body weight was 10.0 kg (range, 2.8-37.0 kg). The average weight gain was 1.55±1.68 kg at 6 months, 3.32±2.30 kg at 1 year, and 5.63±4.22 kg at 2 years after surgery. Six (9.7%) of the 62 neurologically impaired patients and two (10.0%) of neurologically normal lost their body weight or had no weight changes. Eight (12.9%) of the 62 neurologically impaired children had required redo surgery because of gastroesophageal reflux disease recurrences, while 2 (10.0%) of the 20 neurologically normal children had experienced recurrences. In the neurologically impaired children, the postoperative complications included pneumonia (n=1), wound infection (n=1), urinary tract infection (n=1), dysphagia (n=1), delayed gastric emptying (n=1), and ileus (n=2). All of these complications were not found in the neurologically normal group, except for only one case of infectious colitis. However, there was no statistically significant difference between the two groups in postoperative complications.

The outcomes of laparoscopic fundoplication were similar in the neurologically impaired children and in the neurologically normal children.

The single stage transanal pull-through (SSPT) for Hirschsprung’s disease is becoming the most popular procedure. This single center study compared the result of single stage operation with two-stage operation for Hirschsprung’s disease in neonates.

We retrospectively reviewed medical records of all patients who were diagnosed as Hirschsprung’s disease and underwent SSPT or two-stage operation operation in Asan Medical Center between January 2003 and July 2014.

There were 17 SSPT and 28 two-stage operation. The mean age of SSPT group was 14.2±7.1 days, and the mean age of two-stage operation group was 15.4±8.6 days for stomy formation, and 188.6±36.3 days for Duhamel operation. The operation time of SSPT was shorter than Duhamel operation (145.0±37.0 minutes vs. 193.0±36.0 minutes, p<0.001). The mean follow-up period of SSPT and two-stage operation was 35.5±34.9 months (range, 2-132 months) and 56.6±35.5 months (range, 1-121 months), respectively. Defecation problem rate such as fecal soiling or fecal impaction showed no significant difference between the two groups (p=0.719). Two SSPT patients required botulinum toxin injection due to rectal stenosis. Three patients of SSPT group underwent re-do endorectal pull-through due to remnant aganglionic or hypoganglionic bowel.

The SSPT showed shorter hospital days. However, few patients experienced rectal stenosis, but were manageable with botulinum toxin injection. The SSPT requires experienced-pathologist, as well as surgeon, because intra-operation pathology reading is critical for appropriate SSPT. SSPT is a feasible and reasonable option to treat Hirschsprung’s disease.