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.
Single-port laparoscopy-assisted surgery is being performed for various operations in pediatric patients recently. The aims of this study were to prove the safety and find well-matched indications of small bowel resection using single-site umbilical laparoscopic surgery (SSULS).
From 2011 to 2016, 29 pediatric patients underwent SSULS. Medical records were retrospectively reviewed.
A total of 29 patients were included and 30 SSULS were performed in this study. The mean age at operation was 5.7 years, and the mean weight was 21.9 kg. Meckel's diverticulum was the most common indication for SSULS, followed by small bowel intussusception due to leading point mass, small bowel duplication, and Crohn's disease. In most cases, estimated blood loss was negligible except in Crohn's disease with severe inflammation. While answering post-discharge questions about scars, most parents responded that they were satisfied with the postoperative wound.
SSULS is a useful operation to try even for surgeons who do not have advanced laparoscopic skills. Complication rates of single-port operations do not differ from those of conventional laparoscopic operations. Most lesions of the small bowel could be indications of SSULS. Careful attention is required when performing SSULS in patients with Crohn's disease.
Total proctocolectomy with ileal pouch-anal anastomosis (T-IPAA) in childhood is a surgical procedure mainly applied to familial adenomatous polyposis (FAP) or ulcerative colitis (UC), but it can be applied to non-FAP/non-UC disease (NFNU). Studies regarding the role of T-IPAA who underwent the operation in childhood, especially in terms of long-term gastrointestinal function, complications, and quality of life (QOL) are limited. The aim of this study was to evaluate the characteristics of patients receiving T-IPAA and to compare their bowel function outcomes and QOL.
Patients aged ≤18 years at the time of T-IPAA were included. Their medical records were retrospectively reviewed. Krickenbeck classification, Cleveland Clinic Incontinence (CCI) score, 36-item Short-form Health Survey Questionnaire, and Gastrointestinal Quality of Life Index were used for the evaluation of bowel function and QOL. The median follow-up period was 9.8 years.
Of the 25 patients, 9 had FAP, 9 had UC, and 7 had NFNU. NFNU include 3 of Hirschsprung disease, 2 of intestinal neuronal dysplasia, and 2 of imperforate anus. The median age at T-IPAA was 17.8, 14.2, and 9.3 years for FAP, UC, and NFNU, respectively (p=0.001). Bowel function was satisfactory in terms of voluntary bowel movement (VBM), soiling, and constipation. VBM and constipation were not different between the groups, but soiling was most in NFNU (100%, p=0.047). However, QOL was best in the NFNU group in surveys (p=0.034 and 0.004, respectively).
T-IPAA could be safely applied not only for FAP and UC but also for other diseases in selective cases, with caution.
Congenital esophageal stenosis (CES) is a rare disease that has been reported to occur once in every 25,000 to 50,000 births. According to its etiology, CES is divided into 3 subtypes, tracheobronchial remnants (TBR), fibromuscular hypertrophy (FMH) and membranous diaphragm (MD). Symptoms begin at the weaning period and the introduction of solid food around 6 months with dysphagia and vomiting. Esophagography is first screening test and endoscopic ultrasonography plays important roles to diagnose subtypes deciding therapeutic plan. TBRs were generally treated with surgical resection and end-to-end anasotomosis, whereas FMH and MD had good response rate to endoscopic or radiologic guided dilatation. This article reviews the literature on the etiology, clinical course, diagnosis and management of CES including recent opinion.
Anorectal duplications account for only 5% of gastrointestinal duplications, and cases with involvement of the anal canal are much rarer. Nearly all anorectal duplications are posterior to the rectum; duplications located anterior to the normal rectum are highly unusual, and only a few cases have been reported. We report the case of an anterior anorectocolonic duplication presenting as a rectovaginal fistula in a 2-month-old infant. After diagnosis, the duplication was excised completely without further intestinal complications.
Soft tissue hemangioendothelioma (STHE) is a rare vascular tumor, which has a similar prognosis to borderline malignancy. The disease is poorly understood in pediatric cases because of its low incidence; therefore, we investigated treatment strategies for STHE in children.
We retrospectively analyzed 8 patients with STHE, who were pathologically confirmed between January 1995 and June 2015. The median duration of follow-up was 72 months.
Five were male and the median age at the time of surgery was 1.2 years. Six patients presented with a palpable mass, and 2 patients experienced facial paralysis. The median tumor size was 4.0 cm. The following tumor locations were observed head (2 patients), neck (2 patients), chest wall (1 patient), sacrococcyx (1 patient), upper limb (1 patient), and lower limb (1 patient). The patients underwent either microscopic complete resection (R0) (3 patients), macroscopic complete resection (R1) (2 patients), or macroscopic incomplete resection (R2) (3 patients). After histopathological examination, 6 patients were diagnosed with kaposiform hemangioendothelioma (HE), one with retiform HE, and one with epithelioid HE. Postoperative sequelae occurred as gait disturbance, hearing impairment, and vocal cord palsy. Tumor recurrence or regrowth occurred in 4 patients. These patients underwent reoperation and IFN therapy; however, in the patient with epithelioid HE, metastasis to the scalp occurred after these therapies. The patient with the head tumor who underwent R2 resection, underwent resection three more times, but died 11 months after the first surgery.
When treating STHE in children, R0 resection should be first considered, but recurrence and metastasis should be monitored depending on the size, pathology, and location of the lesion. When major sequelae are expected, function-preserving surgery could be considered, depending on tumor location, size, and nearby organs.
Gastric teratoma is an extremely rare tumor that accounts for less than 1% of all teratomas. Gastric teratoma is mostly presented as a palpable abdominal mass, and is rarely accompanied with gastrointestinal bleeding such as melena or hematemesis. A 5-month-old male infant was brought with a history of pale facial color and dark-colored stool. The hemoglobin level was at 6.1 g/dL, with melena having begun 1 month previous. Upper gastrointestinal endoscopy revealed a polypoid mass with bleeding at the upper body and lesser curvature of the stomach. Wedge resection of the stomach was performed and histopathological analysis confirmed the mass to be a mature cystic teratoma. There was no recurrence after the operation during follow-up.
The duplication of gastrointestinal tract has been known to be a rare condition and two different forms, cystic and tubular type. This study was conducted to examine its clinical characteristics, especially cystic enteric duplication which was detected antenatally or postnatally.
There were 13 patients, who confirmed as cystic enteric duplication after operation between July 1996 and June 2015. Clinical data, including a gender, age at operation, presenting symptoms, diagnostic modalities, locations of lesion, and results of surgical treatment, were reviewed retrospectively according to cases detected antenatally and postnatally.
Five cases were included in antenatal diagnosis group and 8 cases in postnatal diagnosis group. Both groups show slightly common in female and the lesion most common in ileum. Antenatal diagnosis group shows 2 males and 3 females and the mean age at operation was 12±52 days (range, 5 to 90 days). They received operation regardless of symptom. Postnatal group shows 3 males and 5 females and the mean age at operation was 462.5±777.0 days (range, 4 days to 6 years). Moreover, 6 patients (75.0%) were age before 2 years. They usually presented abdominal pain with vomiting.
Cystic enteric duplication could present symptoms at any time during childhood, mainly before 2 years old, and so a proper management should be considered when suspect it. Although it is uncommon, surgical management including a minimal invasive procedure could be attempted despite the neonatal period.
Nodular hidradenoma was diagnosed in a 29-month-old girl on her axilla. Hidradenoma, sometimes designated as acrospiroma, is a benign sweat gland neoplasm, which mostly occurs in adults. Very few cases of hidradenoma have been documented in children in their first decade of life. This case demonstrates that when a child develops a skin nodule, nodular hidradenoma can be a diagnostic option.
Neonatal neuroblastoma (NBL) is the most common malignant tumor in neonates, but there have been few studies about it. The purpose of this study was to investigate the clinical features of NBL and to compare prenatal and postnatal diagnosed groups.
Nineteen patients who were diagnosed with NBL prenatally or within 28 days after birth from February 1986 to February 2013 in Seoul National University Hospital were enrolled in the study. The patients were categorized according to the International Neuroblastoma Staging System (INSS) and Children's Oncology Group (COG). Retrospective medical-record reviews were performed on these patients. The operative date, complication, pathological stage, and overall survival of the prenatally diagnosed group and the postpartum diagnosed group were compared.
Tumor was detected via prenatal ultrasonography in 8 patients (42.1%), and 11 patients (57.9%) were diagnosed within 28 days after birth. Based on INSS, the patients were divided into the stage I (n=8), stage II (n=1), stage III (n=3), stage IV (n=4), and stage IVs (n=3) groups, respectively. Based on COG, on the other hand, the patients were divided into the low-risk (n=8), intermediate-risk (n=8), and high-risk (n=3) groups. The postoperative complication rate was 29%. One patient died from complications from chemotherapy. The other 18 patients' mean follow-up period was 77.7 months. The differences between the postoperative complication rate, proportion of early-stage tumor, and overall survival of the prenatal and postnatal groups were not statistically significant (p=0.446, p=0.607, p=0.414).
NBL showed favorable outcomes but relatively higher postoperative complications. There seem to be no significant statistical differences in the postoperative complications, proportion of early-stage tumor, and overall survival between the prenatally diagnosed group and the postpartum diagnosed group.
Immature ganglion cell (IGC) is known for its relationship with intestinal motility and its impact on postoperative functional outcomes of Hirschsprung's disease (HD). There are few studies on the relationship between intestinal dysmotility and IGC in HD patients. 67 patients pathologically diagnosed with HD and who received definitive operation in Seoul National University Children's Hospital from 2010 to 2011 were included. 10 patients were excluded due to inadequate immunohistochemical staining results. The proximal end of resected ganglionic segment was evaluated with immunohistochemistry examination with MAP-2, a marker of ganglionic cells and bcl-2, a marker of IGCs The median age at operation was 155 (15-4678) day-old. 55 (96.5%) patients positive for bcl-2, were regarded as having IGC, and 2 (3.5%) patients positive for MAP-2 but negative for bcl-2, were regarded as having only mature ganglion cells. In the bcl-2 positive group, there were 7 patients (12.7%) with constipation, 15 patients (27.3%) with soiling, 3 patients (5.5%) with perianal excoriation and 6 patients (10.9%) with medication use. In bcl-2 negative group, intestinal dysmotility was not seen. There was no statistical significance in the two groups. Considering that HD is diagnosed at a young age, the rate of IGC present is very high and it might be inappropriate to relate IGC to functional outcome at young ages.
First
Prev
