Suppressive role of vascular endothelial growth factor on intestinal apoptosis in induced necrotizing enterocolitis in rats

Purpose@#Necrotizing enterocolitis (NEC) is a devastating disease that can cause mortality in preterm babies. NEC may develop through an apoptotic pathway that is known to be inhibited by vascular endothelial growth factor (VEGF). This study determined whether VEGF exerted a protective effect against the development of NEC and apoptosis in rats. @*Methods@#To determine the effect of VEGF in NEC rats, neonatal rats were randomized into 4 groups: the control group, the NEC group, the NEC + intraperitoneal VEGF (50 ng/kg) group (NEC + VEGF IP group), and the NEC + oral VEGF (50 ng/ kg) group (NEC + VEGF OR group). NEC was induced by lipopolysaccharide/hypoxia and cold stress. The animals were sacrificed 72 hours later. After laparotomy, we obtained a region of the proximal small bowel from the ileocecal valve about 18 cm in length. @*Results@#The NEC histological grade, apoptosis histological score, and caspase-3 activity were lower in the NEC + VEGF IP and OR groups than in the NEC group. In the NEC + VEGF IP and OR groups, the messenger RNA expression of apoptotic and inflammatory genes, such as Bax, NF-κB, p53, Fas, FasL, and PAF-R, but not that of Bcl-2, was decreased, as was the Bax/Bcl-2 protein ratio. Histological analysis revealed that the apoptosis-blocking effect of VEGF was more effective in the NEC + VEGF IP group than in the NEC + VEGF OR group. @*Conclusion@#We identified apoptotic and inflammatory genes to confirm the preventive effect of VEGF pretreatment on NEC in rats. This study presents a novel approach to prevent apoptosis via VEGF pretreatment in rats with lipopolysaccharide/ hypoxia-induced NEC.

Perinatal Prognostic Factors for Congenital Diaphragmatic Hernia: A Korean Single-Center Study

Purpose@#This study aimed to identify prognostic factors based on treatment outcomes for congenital diaphragmatic hernia (CDH) at a single-center and to identify factors that may improve these outcomes. @*Methods@#Thirty-five neonates diagnosed with CDH between January 2011 and December 2021 were retrospectively analyzed. Pre- and postnatal factors were correlated and analyzed with postnatal clinical outcomes to determine the prognostic factors. Highest oxygenation index (OI) within 24 hours of birth was also calculated. Treatment strategy and outcome analysis of published literatures were also performed. @*Results@#Overall survival rate of this cohort was 60%. Four patients were unable to undergo anesthesia and/or surgery. Three patients who commenced extracorporeal membrane oxygenation (ECMO) post-surgery were non-survivors. Compared to the survivor group, the non-survivor group had a significantly higher occurrence of pneumothorax on the first day, need for high-frequency ventilator and inhaled nitric oxide use, and high OI within the first 24 hours. The non-survivor group showed an early trend towards the surgery timing and a greater number of patch closures. Area under the receiver operating characteristic curve was 0.878 with a sensitivity of 76.2% and specificity of 92.9% at an OI cutoff value of 7.75. @*Conclusion@#OI within 24 hours is a valuable predictor of survival. It is expected that the application of ECMO based on OI monitoring may help improve the opportunity for surgical repair, as well as the prognosis of CDH patients.

Assessment of defecation function beyond infantile period for transanal single-stage endorectal pull-through in Hirschsprung disease

Purpose@#Transanal single-stage endorectal pull-through (TERPT) procedure for patients with Hirschsprung disease (HD) has favorable outcomes, with a lower complication rate. Nevertheless, various degrees of bowel dysfunction and fecal incontinence can persist for a long time in some patients. The aim of this study was to assess the mid- and long-term outcomes of TERPT performed during the infantile period after the completion of toilet training. @*Methods@#We retrospectively reviewed 82 patients aged ≥4 years who underwent TERPT during the infantile period after the pathological diagnosis of HD between 2001 and 2013. Functional outcomes were investigated according to the answers of the Bowel Function Score (BFS) questionnaire, a previously validated 7-item questionnaire about bowel habits. Normal values were obtained in a previous study on BFS for children in Western countries, and a one-sample t-test was used for statistical analysis. @*Results@#Overall, BFS was similar in all investigated age groups. On comparing fecal soiling and social problems between the HD and normal populations, a lower score at an early age in patients with HD was noted; however, the scores became similar when the patients were 7 years of age. Stool frequency decreased continuously but was not significantly different between the 2 groups. @*Conclusion@#The functional outcomes of TERPT performed during the infantile period, after completing toilet training, were similar to that of the normal population. In most cases, uncomfortable symptoms were diminished and functions improved with age.

Successful Laparoscopic Removal of a Huge Trichobeozar in Cases of Rapunzel Syndrome in Children

Rapunzel syndrome is a very rare condition. The trichobezoar, in cases of Rapunzel syndrome, extend from the stomach into the duodenum and small bowel. Trichobezoars are usually encountered in young women with psychiatric problems, such as trichotillomania, trichophagia, or mental retardation and pica. Large trichobezoars, which are associated with Rapunzel syndrome, are removed during open surgery which requires large incisions. This Case Report describes 2 girls who had Rapunzel syndrome where the trichobezoars reached the jejunum and laparoscopic surgery was successful in the removal of the trichobezoars. Laparoscopic removal of a trichobezoar can be considered as a treatment option for children with Rapunzel syndrome.

Successful Laparoscopic Removal of a Huge Trichobeozar in Cases of Rapunzel Syndrome in Children

Rapunzel syndrome is a very rare condition. The trichobezoar, in cases of Rapunzel syndrome, extend from the stomach into the duodenum and small bowel. Trichobezoars are usually encountered in young women with psychiatric problems, such as trichotillomania, trichophagia, or mental retardation and pica. Large trichobezoars, which are associated with Rapunzel syndrome, are removed during open surgery which requires large incisions. This Case Report describes 2 girls who had Rapunzel syndrome where the trichobezoars reached the jejunum and laparoscopic surgery was successful in the removal of the trichobezoars. Laparoscopic removal of a trichobezoar can be considered as a treatment option for children with Rapunzel syndrome.

Changes in the prevalence of index diseases in pediatric surgery: case analysis of single national children’s hospital

Pusan National University Children’s Hospital (PNUCH) opened in November 2008 as the second National Children’s Hospital in Korea and celebrated its 10th anniversary in 2018. In the last decade, birth rates and pediatric populations have been declining sharply and has been more serious in Busan, Ulsan, and Gyeongsangnam-do where the PNUCH is located, affecting the prevalence of index diseases. The number of the index diseases was investigated by year according to changes in the population. Patients who underwent surgery at PNUCH for 10 years were included. Changes in pediatric population and the number of live births were analyzed using the data from Statistics Korea. The number of the index diseases was analyzed retrospectively through the review of electronic medical records. Statistical analysis was performed through Poisson regression. The number of live births and pediatric population decreased from 69,528 to 48,600 (30.1%) and 1,880,284 to 1,444,961 (23.2%), respectively. Operations of pediatric surgery decreased by 32.3% compared to that during the peak season, but the prevalence rate ratio of the pediatric population increased to 1.03 (P<0.001). The number of emergency operation increased due to the largest surge in acute appendicitis operations. Both esophageal atresia and Hirschsprung disease decreased, while hypertrophic pyloric stenosis significantly increased. The absolute number of pediatric surgery cases decreased. However, considering the changes in pediatric population and the number of live births, the relative number of pediatric surgery cases does not changed. The decrease in the number of each disease would be related to the decrease in pediatric population and the number of live births. We should consider the increase of emergency operations such as acute appendicitis.

Two cases of splenic infarction due to torsion of wandering spleen requiring laparoscopic splenectomy in adolescent girls

Wandering spleen is a rare condition in which the spleen is not found in its usual location or presentation. Its vascular pedicle can be twisted easily, and compromised vasculature of spleen. Consequently, it may result in splenic infarction requiring diagnosis and treatment. Here we present 2 cases of splenic infarction due to torsion of wandering spleen in 2 adolescent girls who visited the emergency department presenting with abdominal pain. Prompt computed tomography scan led early diagnosis and successful laparoscopic splenectomy. We also reviewed the literature on this entity briefly.

Alimentary Tract Duplication in Pediatric Patients: Its Distinct Clinical Features and Managements / 대한소아소화기영양학회지

Purpose@#Alimentary tract duplication (ATD) is a rare congenital condition that may occur throughout the intestinal tract. Clinical symptoms are generally related to the involved site, size of duplication, or associated ectopic mucosa. This study aimed to identify clinical implications by anatomical locations and age group and then suggest a relevant management according to its distinct features. @*Methods@#We retrospectively reviewed the clinical data of pediatric patients who received a surgical management due to ATD. Furthermore, data including patients' demographics, anatomical distribution of the duplication, clinical features according to anatomical variants, and outcomes were compared. @*Results@#A total of 25 patients were included in this study. ATD developed most commonly in the midgut, especially at the ileocecal region. The most common clinical presentation was abdominal pain, a sign resulting from intestinal obstruction, gastrointestinal bleeding, and intussusception. The non-communicating cystic type was the most common pathological feature in all age groups. Clinically, prenatal detection was relatively low; however, it usually manifested before the infantile period. A laparoscopic procedure was performed in most cases (18/25, 72.0%), significantly in the midgut lesion (p=0.012). @*Conclusion@#ATD occurs most commonly at the ileocecal region, and a symptomatic one may usually be detected before the early childhood period. Surgical management should be considered whether symptom or not regarding its symptomatic progression, and a minimal invasive procedure is the preferred method, especially for the midgut lesion.

Perforated Choledochal Cyst: Its Clinical Implications in Pediatric Patient / 대한소아소화기영양학회지

Purpose@#Perforation of choledochal cyst (CC) is a relatively rare clinical presentation in pediatric populations and difficult to predict preoperatively. We assess the clinical implications by comparing clinical parameters based on a single-center experience between perforated and nonperforated CC to facilitate the appropriate management for future interventions. @*Methods@#A total of 92 cases of CC in pediatric patients (aged <18 years) who received surgical management between January 2003 and December 2018 at a Pusan National University Children's Hospital were reviewed. After screening the clinical features of perforated cases, we compared the demographic findings, clinical characteristics, and some laboratory results between the perforated and nonperforated groups. @*Results@#Perforated CC was identified in 8 patients (8.7%), and nonperforated CC in 84 patients (91.3%). Perforation can be classified into three categories: free perforation of cyst (3 cases), pinpoint perforation of cyst (2 cases), and necrotic change of cyst (3 cases). CC perforation occurred significantly more commonly in patients aged <24 months. Clinically, the perforated group showed significantly higher frequency of fever and higher C-reactive protein (CRP) level during the initial visit. @*Conclusion@#Perforation is more likely to be suspected in patients aged <24 months presenting together with fever and high CRP level in the initial visit. It is also necessary to keep in mind that it indicates not only a possibility of complicated disease status regardless of its association with stones but also a difficulty of applying a minimal invasive procedure and relatively increased length of hospital stay.

Congenital Esophageal Stenosis in Children: From Etiology to Prognosis

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.

Esophageal Reconstruction with Gastric Pull-up in a Premature Infant with Type B Esophageal Atresia

Esophageal atresia (EA) with proximal tracheoesophageal fistula (TEF; gross type B) is a rare defect. Although most patients have long-gap EA, there are still no established surgical guidelines. A premature male infant with symmetric intrauterine growth retardation (birth weight, 1,616 g) was born at 35 weeks and 5 days of gestation. The initial diagnosis was pure EA (gross type A) based on failure to pass an orogastric tube and the absence of stomach gas. A “feed and grow” approach was implemented, with gastrostomy performed on postnatal day 2. A fistula was detected during bronchoscopy for recurrent pneumonia; thus, we confirmed type B EA and performed TEF excision and cervical end esophagostomy. As the infant's stomach volume was insufficient for bolus feeding after reaching a body weight of 2.5 kg, continuous tube feeding was provided through a gastrojejunal tube. On the basis of these findings, esophageal reconstruction with gastric pull-up was performed on postnatal day 141 (infant weight, 4.7 kg), and he was discharged 21 days postoperatively. At 12 months after birth, there was no catch-up growth; however, he is currently receiving a baby food diet without any complications. In patients with EA, bronchoscopy is useful for confirming TEF, whereas for those with long-gap EA with a small stomach volume, esophageal reconstruction with gastric pull-up after continuous feeding through a gastrojejunal tube is worth considering.

Internal Hernia through a Defect in the Broad Ligament of Uterus: Laparoscopic Management Using a Self-Anchoring Barbed Suture

The occurrence of internal hernia through a defect in the broad ligament is a very rare condition, which may cause small bowel obstruction. This is a case of a 50-year-old woman who developed intestinal obstruction induced by internal hernia and who had undergone laparoscopic myomectomy 7 years prior to visiting our emergency room. Abdominopelvic computed tomography showed luminal narrowing of the ileum and dilatation of the pelvic loop of the small bowel at the left side of the uterus. We detected internal hernia through the defect in the broad ligament and managed it successfully by performing a laparoscopic procedure using a barbed suture, V-Loc (Covidien, Mansfield, MA).

Congenital Esophageal Stenosis in Children: From Etiology to Prognosis / 소아외과

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.

Clinical Implication of Surgically treated Abdominoperineal Soild Tumor in the Newborn : A Single-Center Experience

PURPOSE: Abdominoperineal solid tumors presenting in neonates often require surgical intervention during the neonatal period. Although we report our single-center experience, this study would be meaningful to understand the clinical implications of these neoplasms. METHODS: We retrospectively reviewed and analyzed the clinical data and characteristics of 22 patients (≤28 days old) diagnosed with histopathologically confirmed abdominoperineal solid neoplasms (benign or malignant) after surgical resection. RESULTS: The mean gestational age and postnatal age at the time of operation were 38.3±1.8 weeks and 13.5±8.3 days, respectively. Most patients (18/22, 81.8%) were diagnosed during antenatal care visits; however, 4 (18.2%) were identified after birth. The mean tumor size was 6.4×5.3 cm (3.5–17.0 cm), and tumors occurred most frequently within the sacrococcygeal region (8/22, 36.4%). Histopathologically, 14 patients (63.6%) demonstrated benign tumors and 8 (36.4%) demonstrated malignant tumors. Germ cell tumors and hepatoblastomas were the most commonly observed tumors. Fortunately, all patients showed a localized pattern of tumor involvement without distant metastasis. No recurrence or mortality was observed during the follow-up period (mean 66.4±44.2 months). CONCLUSION: Abdominoperineal solid tumors occurring in neonates show variable clinical patterns during the antenatal and postnatal monitoring/screening periods. We conclude that aggressive and multidisciplinary approaches could achieve good clinical results in these patients.

Clinical implication of spontaneous gastrointestinal perforation in pediatric patients: its difference according to age group

PURPOSE: Spontaneous gastrointestinal perforations (SGIPs; not associated with injury or disease) occur rarely in pediatric patients. This study aimed to define age-specific features associated with SGIPs in pediatric patients. METHODS: Retrospectively reviewed the clinical data of children (before adolescence) who received surgery due to a SGIP at a single institution. Thirty-nine patients were enrolled. Characteristics were compared between the 2 age groups: neonates (group A) and beyond neonates (group B). RESULTS: Group A included 24 patients (61.5%) an group B included 15 patients (38.5%). Thirteen perforations occurred in the stomach (33.3%), 12 in the small intestine (30.8%), and 14 in the large intestine (35.9%). A significantly higher proportion of perforations occurred in the stomach and small intestine in group A, while more perforations occurred in the large intestine in group B (P = 0.01). Several associated conditions during the preoperative period were identified in both groups. The overall mortality rate was 15.4% (6 of 39). Mortality was relatively high in group A (5 of 24, 20.8%) and for perforations of stomach (3 of 13, 23.1%) and small intestine (3 of 12, 25.0%); however, there were no significant differences with regard to age or perforation site (P = 0.244, P = 0.122, respectively). CONCLUSION: SGIPs in pediatric patients had diverse clinical features and different perforation patterns according to age group. However, no significant group differences in mortality were found. Thus, favorable results regardless of age can be expected with prompt recognition, medical resuscitation, and adequate surgical management.

Clinical Study of Congenital Esophageal Stenosis: Comparison according to Association of Esophageal Atresia and Tracheoesophageal Fistula / 대한소아소화기영양학회지

PURPOSE: Congenital esophageal atresia (CES) is a rare congenital disease. The severity of symptoms is variable; thus, diagnosis is difficult and tends to be delayed. CES is frequently accompanied by esophageal atresia (EA) with/without tracheoesophageal fistula (TEF). We investigated the characteristics of CES by reviewing our experience with CES patients and researched the differences between CES with EA-TEF and isolated CES. METHODS: A total of 31 patients underwent operations for CES were reviewed retrospectively. The patients were divided into two groups according to the association with EA-TEF, and compared the differences. RESULTS: Sixteen boys and 15 girls were included. The mean age at symptom onset was 8 months old, and the mean age at diagnosis was 21 months old. Nine patients with EA-TEF were included group A, whereas the other 22 patients were assigned to group B. There were no differences in sex, gestational age, associated anomalies and pathologic results between the groups. In group A, the age at diagnosis and age at surgery were younger than in group B despite the age at symptom occurrence being similar. Postoperative complications occurred only in group A. CONCLUSION: In this study, symptoms occurred during the weaning period, and vomiting was the most frequent symptom. CES patients with EA-TEF tended to be diagnosed and treated earlier despite the age at symptom occurrence being similar. CES patients with EA-TEF had more postoperative complications; therefore, greater attention should be paid during the postoperative period.

Anterior Anorectocolonic Tubular Duplication Presenting as Rectovestibular Fistula in an Infant / 소아외과

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 in Children / 소아외과

PURPOSE: 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. METHODS: 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. RESULTS: 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. CONCLUSION: 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.

A Rare Case of Life-threatening Sudden-onset Spontaneous Gastric Perforation in a 13-Year-Old Boy

Spontaneous gastric perforation in a healthy child is extremely rare; however, its outcome can be life-threatening. Gastric perforation may be caused by inflammation, mechanical injury, ingestion, and vascular compromise. We report a rare case of gastric perforation with unfavorable results in a 13-year-old, previously healthy, boy. We conclude that acute gastric volvulus is a possible cause of gastric perforation in this case.

Spontaneous Perforation of Colon in Previously Healthy Infants and Children: Its Clinical Implication / 대한소아소화기영양학회지

PURPOSE: Spontaneous colon perforations are usually encountered as necrotizing enterocolitis in the neonatal period, but occur rarely in infants and children without pathological conditions. This study was conducted to describe its clinical implication beyond the neonatal period. METHODS: Cases of spontaneous colon perforation confirmed after the operation were reviewed retrospectively and the clinicopathological characteristics were analyzed. Clinical data were compared according to the presence of pneumoperitoneum as initial findings. RESULTS: Eleven patients were included in the study period and showed a history of hospitalization before transfer due to management for fever, respiratory or gastrointestinal problems. Six patients showed a sudden onset of abdominal distention and only seven patients showed a pneumoperitoneum as initial radiologic findings, however there were no significant clinicopathological differences. Perforation was found evenly in all segments of the colon, most commonly at the sigmoid colon in four cases. There were no specific pathologic or serologic causes of perforation. CONCLUSION: When previously healthy infants and children manifest a sustained fever with a sudden onset of abdominal distention during management for fever associated with respiratory or gastrointestinal problems, there is a great likelihood of colon perforation with no pathological condition. Prompt surgical management as timely decision-making is necessary in order to achieve a good progress.