The congenital H-type rectovestibular fistula, a fistula between the anorectum and genital tract besides a normal anus is a rare variant of anorectal deformities. This disease needs proper treatment but there are no standard of diagnosis and treatment. The purpose of this report is to review a 13-year experience of approach and management for H-type rectovestibular fistula at a single institution.
From February 2002 to August 2015, we cared for 11 patients who had congenital H-type rectovestibular fistula and reviewed their clinical presentation, accompanied anomalies, diagnostic modalities, operative technique, and postoperative progress.
Most patients with H-type rectovestibular fistula presented with symptoms including vestibular defecation and major labial abscess. We could find the fistula tract in most of patients by fistulography using contrast dye. All of the patients had been operated. There were 2 recurrences after surgical treatment who had inflammation and infection associated with the fistula. All other patients recovered without complications.
We think the operation including fistulectomy and repair of perineal body through a transanal approach can be a feasible option to the congenital H-type rectovestibular fistula. Also, combined inflammation and infection should be treated prior to surgery to reduce postoperative complications.
Hirschsprung’s disease (HD) is a congenital intestinal disorder with absence of ganglion cells in the intestinal muscle and submucosa. Diagnosis is based on histopathological study such as H&E, and acetylcholinesterase (AchE) immunohistochemistry. Calretinin immunohistochemistry was introduced as a new diagnostic method against limitations of other staining. The aim of this study is to investigate the usefulness of calretinin immunohistochemistry for the diagnosis of HD compared to H&E and AchE.
Ten patients with HD and 22 non-HD patients were included in the study. H&E staining, AchE and calretinin immunohistochemistry were performed in all 32 patients. All slides were evaluated by same single pathologist and the diagnostic value was calculated for each H&E stain, AchE immunohistochemical staining, and calretinin immunohistochemical staining.
Calretinin method had sensitivity of 100% and specificity of 100% for diagnosis of HD. Its diagnostic accuracy was 100%. AchE staining showed 100% of specificity and 80% of sensitivity. Diagnostic accuracy of H&E staining was 56.3%.
We concluded that calretinin immunohistochemistry is a very useful and valuable method to diagnosis HD patient.
Varicose veins requiring operative treatment have been more common recently, but there are not enough studies among children dermographics because most patients belong to the adult population. We concentrated on varicose vein of under 18 years old, and here intend to report cases of our clinical experiences.
From January 2003 until December 2014, there were 6 children under 18 years old who required varicose vein management. Data was collected by the investigation of medical records retrospectively, including preoperative symptoms, diagnostic tools, treatment methods, results of treatment, and complications.
The mean age was 11 years (range, 3-17 years), and gender ratio was 1:2 (2 male, 4 female). The involved legs were on the right in 3 cases, on the left in 2 cases, and on both in 1 case. The most common symptoms were venous bulging and tortuosity in 6 cases, and other symptoms were Klippel-Trenaunay-Weber syndrome in 2 cases, pain and fatigue in 1 case, port-wine stain in 1 case, and telangiectasia in 1 case. Duplex sonography was performed to confirm venous reflux in all cases. The additional venography was performed to check for anatomical variation in 1 case, and three-dimensional CT in 2 cases. Treatments were high ligation and stripping in 3 cases, and endovenous laser therapy in 3 cases. Additionally, remnant varicosities after first operations were treated by endovenous laser therapy in all cases. During the mean postoperative follow-up period of 60 months, complications included edema of the foot and petechia in 2 cases each, and were not severe.
Operative treatments including high ligation and stripping, and endovenous laser therapy are very effective for the management of varicose vein in the pediatric population, with improvements in quality of life including relief of symptoms and management of the cosmetic aspect.
The use of CT as a diagnostic tool in pediatric acute appendicitis is increasing because of its high sensitivity and specificity. However, due to both the serious concerns about radiation of CT and the convenience and reasonable cost of ultrasound (US) examination, US has value on the initial diagnosis of acute appendicitis despite of the lower sensitivity in children. The purpose of this study was to examine the factors that affect the rate of false negative diagnosis of the ultrasound from the patients who received laparoscopic appendectomy.
The pediatric appendectomy cases from 2002 to 2013 in Yeouido St. Mary's Hospital have been reviewed through the medical records. We included patients who underwent an initial screening by ultrasound examination.
Among 181 patients, 156 patients were the sono-positive group and 25 patients were sono-negative group. There is no significant difference in ages, genders, physical examination findings and white blood cell count between the two groups. But, the degree of inflammation of appendicitis (simple, 58.3% vs. 32.0%; complicated, 41.7% vs. 68.0%) and the appendix position (antececal, 85.0% vs. 12.0%; retrocecal, 13.7% vs. 44.0%; pelvic, 1.3% vs. 44.0%) were significantly different between the two groups (sono-positive group vs. sono-negative group; p<0.05).
The position of the appendix may act as a factor that causes an error in the diagnostic ultrasound, especially, in the retrocecal type and the pelvic type with the higher risk of necrosis or perforation.
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.
Number of pediatric cholecystectomy has been recently showing a gradually increasing trend. The purpose of this study was to investigate the clinical features of patients who underwent pediatric cholecystectomy, and the latest trend in cholecystectomy.
In the present study, we conducted a retrospective chart review on 47 patients who had undergone cholecystectomy at a single center. The entire patient population was divided into two groups, according to the time of cholecystectomy (early group, January 1999 to December 2006; late group, January 2007 to August 2014).
The comparison between the early and late groups showed that the number of cholecystectomy increased from 13 to 34 cases representing a 2.6-fold increase. The mean patient age also increased from 5.94±4.08 years to 10.51±5.57 years (p=0.01). Meanwhile, laparoscopic surgery also increased from 15.4% to 79.4%, respectively (p<0.001). However, sex, mean body mass index, comorbidities, indications of cholecystectomy, and previous total parenteral nutrition were not statistically significant.
The results of this study showed that pediatric cholecystectomy cases are increasing, particularly in the 10 to 19 years age group and laparoscopic cholecystectomies are also being performed at an increasing rate. When the patients were compared according to the time of cholecystectomy, there were no differences in other risk factors or indications for cholecystectomy.
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.
Farber disease (FD) is a rare lysosomal storage disorder that shows autosomal recessive inheritance. We report the case of a 58-month-old girl with FD, who was misdiagnosed with epithelioid hemangioendothelioma. The patient had undergone five surgeries for sacrococcygeal masses and three surgeries for scalp masses owing to misdiagnosis. Here, we describe this rare case of FD.
Lymph node enlargement is a common finding in children suggesting normal or benign. Palpable nodes which are large, hard or fixed must be examined carefully to rule out malignant diseases. In this case, a 15-year-old boy presented to our hospital to inspect the palpable mass at his post-auricular area being found 2 months ago. It was diagnosed as nodal marginal zone lymphoma (NMZL) through excisional biopsy and immunohistochemistry. NMZL is very rare, especially in children and young adults, but occurs locally in most cases with a good prognosis compared to adults. We described a rare case of NMZL diagnosed in adolescent.