The Effect of the Ganglionic Segment Inflammatory Response to Postoperative Enterocolitis in Hirschsprung Disease.

INTRODUCTION: We examined the relationship between proinflammatory cytokines that occur in the inflammatory reaction in the intestine in Hirschsprung disease (HD) and Hirschsprung-associated enterocolitis (HAEC). METHODS: Thirty cases (M:27, F:3) operated on due to HD. The cases were divided into three groups: group 1 with pre and post operative EC, group 2 with post-operative, and group 3 with pre-operative EC. The intestinal segments were evaluated by immunohistochemistry for interleukin 1 beta (IL-1ß), tumor necrosis factor-alpha (TNF-α), and interleukin 6 (IL-6). RESULTS: IL-1ß staining was significantly higher in the ganglionic zone of groups with enterocolitis compared to the control group (p = 0.012). TNF-α staining in the transitional zone of Group 3 and IL-1ß staining in the ganglionic zone of Group 1 was significantly higher than the control group (p = 0.030, p = 0.020). CONCLUSION: In our study, older age at diagnosis and more than 20% IL-1ß staining in the ganglionic segment were found to be risk factors for HAEC. It is noteworthy that the increase in IL-1ß can be associated with HAEC.

Anorectal Malformations and Late-Term Problems.

Anorectal malformation is a disease with different subtypes and anatomical and functional multisystemic involvement that requires a unique approach in each age group. Anomalies associated with vertebral defects, anal atresia, cardiac defects, tracheo-esophageal fistula, renal anomalies, and limb abnormalities (VACTERL) association require detailed investigation and management. Beginning from the neonatal period, treatment is carried out with different surgical procedures. The clinical course of these patients may be associated with medical problems, accompanying congenital anomalies, perioperative management, or late sequelae. Constipation and fecal-urinary incontinence are the most common problems encountered in long-term follow-up. Renal failure is the most important cause of long-term mortality. In addition, these patients need to be under control until adulthood due to cardiological, spinal, genital, gynecological, and endocrine problems. In this follow-up, many pediatric disciplines such as neonatal intensive care, cardiology, nephrology, gastroenterology, and endocrinology cooperate with pediatric surgeons and pediatric urologists.

Intralesional bleomycin injection treatment of intra-abdominal lymphangiomas presenting with acute abdomen in children.

BACKGROUND: We evaluated the results of urgent intralesional bleomycin injection (IBI) treatment of intra-abdominal lymphan-giomas (IAL) presenting with acute abdomen in children. METHODS: The records of patients who underwent urgent IBI due to acutely presenting IAL between January 2013 and January 2020 were reviewed retrospectively in terms of age, presenting symptoms, cyst type, number of injections, pre- and post-treatment cyst volume, clinical response, complications, and follow-up. RESULTS: Six patients with a mean age of 4.3 years (2-13 years) were treated. Presenting symptoms were acute abdominal pain (n=4), abdominal distention (n=1), hypoproteinemia and chylous ascites (n=1). Lesions were of macrocystic type in four and macro and micro cystic in two patients. The median number of injections performed was 2 (1-11). Mean cyst volume reduced dramatically from 567 cm3 (range 117-1656) to 3.4 cm3 (range 0-13.8) after treatment (p=0.028). Treatment response was excellent in four patients with complete resolution of the cysts, while good in the remaining two. No early or late complications or recurrence was observed in a mean follow-up period of 40 months (16-56 months). CONCLUSION: IBI is a safe, fast, and easily applicable method with satisfactory results in the treatment of acutely presenting IAL. It may be recommended in primary as well as recurrent lesions.

Endoscopic septum division of tubular esophageal duplication in two children and systematic review.

Tubular esophageal duplication is a rare congenital malformation The surgical treatment of this lesion can be challenging. We aimed to present our experience in two cases with tubular esophageal duplication. Both cases were endoscopically treated by a dual knife. The first patient underwent a single, while the second patient required three sessions of endoscopic septum division using an electrosurgical knife. Both showed satisfactory clinical, radiological and endoscopic response to treatment. Moreover, a systematic literature review has been performed. To identify all available studies, a detailed search on tubular esophageal duplication was performed according to the PRISMA (Preferred Reporting Items for Systematic reviews and Meta-Analyses) guidelines. Tubular esophageal duplication was reported in 16 pediatric cases in 14 studies in the literature. Surgery was applied to 10 of these cases and endoscopic treatment was applied to two of them. In the follow-up, nine patients who underwent surgical treatment and two patients who underwent endoscopic treatment were uneventful. Tubular esophageal duplications can be successfully treated endoscopically. Endoscopic septum division is a minimally invasive procedure that allows full anatomical recovery and satisfactory therapeutic response.

Endoscopic pilonidal sinus treatment (EPSiT) in the pediatric age group: Short-term results.

BACKGROUND: This study aims to evaluate the short term outcomes of the Endoscopic pilonidal sinus treatment (EPSiT) in the pediatric age group. METHODS: In this study, between June 2018 and July 2019, pediatric patients with pilonidal sinus (PS) who were treated with the EPSiT method were reviewed retrospectively. RESULTS: Of the twenty-nine patients (20 males, nine females), the average age was 15.5±2.8 years, and the average body mass index (BMI) was 25.8±4.2. Eight patients (28%) presented with a history of recurrence following the previous surgery. The average number of fistulas present in cases was 1.17 (1-2). The localization of the fistula was midline in twenty-four and lateral in five of the patients. The average time of the EPSiT procedure was 57±13.9 minutes, and the average time of hospital stay was 11.4±7.2 hours. The pain score average was 0.86 (range of 0-3) and the duration of analgesic use was 37 hr (12-72 hr). The mean post-operative time of total wound healing was 18.71 days (7-60 days) for primary presenting cases. Early wound healing was seen in twenty-five patients (average of 14 days), while late wound healing was observed in four patients (average of 60 days). The mean time of follow-up was 8.3±3.34 months. The average time of return to full daily activity was 2.1 days (0-30 days), while it was the same day for sixteen (53%) patients. In post-operative follow-up, early (bleeding: 1) and late (formation of granulation tissue: 1, recurrence: 8) complications were seen in nine patients. Of the eight patients (27.5%) whose recurrence was detected, seven were primary and one was secondary presenting patients. The average time of presentation for recurrence was 5.8 mo (1-10 mo). Re-EPSiT was applied in two of the eight patients with recurrence and is planned for five, while one of the patients lost to follow-up. CONCLUSION: EPSiT is an easily applicable, pain-free minimal invasive procedure with a short period of hospital stay and a fast return to routine daily activity. It provides comfortable and repeatable intervention in cases with recurrences after the EPSiT procedure and other methods for PS treatment.

The effect of azygos vein preservation on postoperative complications after esophageal atresia repair: Results from the Turkish Esophageal Atresia Registry.

AIM: Preservation of the azygos vein (AV) maintains normal venous drainage of the mediastinum and decreases postoperative congestion. The modification of esophageal atresia (EA) repair by preserving AV may prevent postoperative complications and may lead to better outcomes. The data from the Turkish Esophageal Atresia Registry (TEAR) were evaluated to define the effect of AV preservation on postoperative complications of patients with EA. METHODS: Data from TEAR for a period of five years were evaluated. Patients were enrolled into two groups according to the preservation of AV. Patients with divided (DAV) and preserved AV (PAV) were evaluated for demographic and operative features and postoperative complications for the first year of life. The DAV and PAV groups were compared according to the postoperative complications, such as fistula recanalization, symptomatic strictures, anastomotic leaks, total number of esophageal dilatations, and anti-reflux surgery. In addition, respiratory problems, which required treatment, were compared between groups. RESULTS: Among 502 registered patients; the data from 315 patients with the information of AV ligation were included. The male female ratio of DAV (n = 271) and PAV (n = 44) groups were 150:121 and 21:23, respectively (p > 0.05). The mean body weight, height, gestational age, and associated anomalies were similar in both groups (p > 0.05). The esophageal repair with thoracotomy was significantly higher in DAV group, when compared to the PAV group (p < 0.05). The rates of primary anastomosis and tensioned anastomosis were similar in both groups (p > 0.05). There was no difference between DAV and PAV groups for anastomotic leaks, symptomatic anastomotic strictures, fistula recanalization, and the requirement for anti-reflux surgery (p > 0.05). The rate of respiratory problems, which required treatment, was significantly higher in the DAV group (p < 0.05) CONCLUSION: The data in the TEAR demonstrated that preserving the AV during EA repair led to no significant advantage on postoperative complications, with exception of respiratory problems. AV should be preserved as much as possible to maintain a normal mediastinal anatomy and to avoid respiratory complications.

Outcome of Very Low and Low Birth Weight Infants with Esophageal Atresia: Results of the Turkish Esophageal Atresia Registry.

INTRODUCTION: The data of the Turkish Esophageal Atresia Registry (TEAR) was evaluated to define the outcome of very low birth weight (VLBW) and low BW (LWB) infants with esophageal atresia (EA). MATERIALS AND METHODS: The data registered by 24 centers between 2014 and 2018 were evaluated for demographic features, prenatal findings, associated anomalies, surgical treatment, and outcome. Patients were enrolled in three groups according to their BWs (VLBW <1,500 g), LWB = 1,500-2,500 g), and normal BW (NBW; >2,500 g). RESULTS: Among the 389 cases, there were 37 patients (9.5%) in the VLBW group, 165 patients (42.4%) in the LBW group, and 187 patients (48.1%) in the NBW group. Prenatal diagnosis rates were similar among the three groups (29.7, 34.5, and 24.6%, respectively). The standard primary anastomosis was achieved at a significantly higher rate in NWB cases than in the other groups (p < 0.05). In patients with tracheoesophageal fistula (TEF), patients of the NBW group had significantly higher rates of full oral feedings, when compared with VLBW and LBW cases (p < 0.05). At the end of the first year, when we evaluate all patients, the number of cases with fistula recanalization and esophageal anastomotic strictures (AS) requiring esophageal dilatation was similar among the groups. The weight and height measurements at 6 months and 1 year of age of the survivors were similar in all the groups. The overall mortality rate was significantly higher in the VLBW and LBW groups, when compared with the NBW patients, even in patients with tension-free anastomosis (p < 0.05). The incidence of the associated anomalies was 90.6% in cases with mortality, which was significantly higher than in survivors (59.6%; p < 0.05). According to Spitz's classification, the survival rate was 87.1% in class I, 55.3% in class II, and 16.7% in class III. The most common causes of mortality were associated with cardiovascular diseases, pneumonia, and sepsis. CONCLUSION: The national data of TEAR demonstrates that the developmental and feeding parameters are better in NBW patients. Although VLBW patients have higher risk of developing fistula canalization than the LBW and NBW groups, long-term complications, such as anastomotic strictures, weight, and height values, after 1 year are similar in both groups. According to our results, associated anomalies and LBWs are still significant risk factors for mortality in cases with EA.

Adrenal masses in children: Imaging, surgical treatment and outcome.

BACKGROUND/OBJECTIVE: This study aims to evaluate the current surgical approach to adrenal masses in the pediatric age group. METHODS: We retrospectively analyzed cases that underwent surgery for adrenal masses between 2007 and 2017. Patients were assessed regarding age, sex, primary diagnosis, image defined risk factors (IDRF), surgical treatment method, complications, duration of hospital stay, and follow-up. RESULTS: We examined 50 patients who underwent surgery for adrenal mass (mean age: 4.8 years; range: 5 days-14 years). For IDRF assessment, Ultrasonography was used in 42, Computed Tomography in 36, and Magnetic Resonance Imaging in 36 patients. Lesions were present on the right in 25, left in 21, and bilateral in 4 patients. Histopathological findings were neuroblastoma (n = 29), ganglioneuroma (n = 6), adrenal cortex tumor (n = 5), ganglioneuroblastoma (n = 4), pheochromocytoma (n = 3), cyst (n = 1), and adrenal hematoma (n = 2). Laparotomy was performed on 37 patients, and laparoscopy on 13 patients. None of the cases had any operative complications. CONCLUSION: The crucial factors determining the surgical approach to adrenal masses in pediatric cases are the histopathology of the mass, volume, and IDRF. Minimally invasive procedures could be reliably performed in appropriate cases.

Congenital generalized lipodystrophy: The evaluation of clinical follow-up findings in a series of five patients with type 1 and two patients with type 4.

Congenital generalized lipodystrophy (CGL) is a rare disorder characterized by lipoatrophy affecting the face, limbs and trunk, acromegaloid features, hepatomegaly, hypertriglyceridemia, and insulin resistance. The aim of this study is to evaluate the long-term follow-up findings including gastrointestinal and cardiac manifestations of the patients with CGL1 and CGL4, caused by mutations in the AGPAT2 and CAVIN1 genes, respectively. Two patients aged 2 and 9 years with the same biallelic CAVIN1 mutation and five patients aged between 6 months and 11 years 4 months with AGPAT2 mutations have been followed up for 3-9 years. The patients were between 7 and 20 years of age at their last examination. One of the two patients with CGL4 had congenital pyloric stenosis. The other patient with CGL4 have developed recurrent duodenal perforations which have not been reported in CGL patients previously. The pathological examination of duodenal specimens revealed increased subserosal fibrous tissue and absent submucosal adipose tissue. None of the five CGL1 patients had gastrointestinal problems. Two patients with CGL4 developed hypertrophic cardiomyopathy (HCMP) and severe cardiac arrhythmia, only one patient with CGL1 had HCMP. Hyperinsulinemia was detected in one patient with CGL4 and three patients with CGL1, these three CGL1 patients also had acanthosis nigricans. Hepatic steatosis was detected in one patient with CGL4 and two patients with CGL1 by ultrasonography. In conclusion, these findings suggest that CGL4 patients should also be carefully followed up for gastrointestinal and cardiac manifestations.

A rare case of paratesticular leiomyoma in a child.

Leiomyomas are benign, slow-growing, smooth muscle tumors, which can occur at many locations in the body. The male genitourinary tract is seldom affected and scrotal leiomyomas are extremely rare. Most of the scrotal leiomyomas are localized in the testis, epididymis, spermatic cord, subcutaneous tissue, tunica albuginea, and scrotal skin and only a few of them are reported in the origin of isolated tissue without paratesticular structures in the paratesticular region. We are presenting a case of solitary paratesticular leiomyoma in a child, which is very rare in terms of lesion location and patient age, and describing the imaging features of this lesion.

Which treatment modality for pediatric pilonidal sinus: Primary repair or secondary healing?

OBJECTIVE: To evaluate the outcome and characteristics of patients who were operated for sacrococcygeal pilonidal sinus (SPS) treatment using primary repair or secondary healing technique. METHODS: Forty-seven patients (female: 25, male: 22) diagnosed with pilonidal sinus operated between 2009 and 2015 were retrospectively analyzed. The cases were evaluated for age, gender, body mass index (BMI), surgical technique, hospital stay, recovery time and recurrence. Mainly two types of surgical techniques were applied: primary repair and secondary healing. RESULTS: Mean age of the patients was 15.6 (±1.2) years. Excision and primary repair was performed in 36, excision and secondary healing was in 11. There was no statistically significant difference between the groups regarding length of hospital stay and duration of postoperative pain (p > 0.05). There was a statistically significant difference between groups regarding recovery time (p < 0.05). Recurrence was detected in a total of 11 (23.4%) patients. CONCLUSION: We did not find any significant difference concerning length of hospitalization, duration of postoperative pain and recurrence rate. However we determined that the primary repair group recovery time is significantly shorter compared to our secondary healing group. Therefore we recommended primary repair in the treatment of primary repair PS in children.

Immune Thrombocytopenia in a Child with Neuroblastoma.

Thrombocytopenia is a frequent finding in patients with solid tumors. It is usually caused by bone marrow infiltration or by myelosuppression due to anticancer therapy; however immune thrombocytopenia (ITP) associated with solid tumors is rare. Neuroblastoma is the most common extracranial solid tumor in children. Here we report the case of a two-year-nine-month-old patient with adrenal neuroblastoma who presented with ITP. Paraneoplastic ITP was considered in the differential diagnosis. Bone marrow infiltration and other causes of thrombocytopenia were excluded and the patient was treated with intravenous immunoglobulin and tumor resection. Platelet count increased rapidly after surgery and complete remission of ITP was achieved.

A rare cause of vomiting: annular pancreas.

Annular pancreas is a rare congenital anomaly that consists of a ring of pancreatic tissue partially or completely encircling the second part of the duodenum. It can affect anyone from neonates to adults, and is difficult to diagnose because it can present in a wide range of clinical conditions. Although cases have also been reported in adults, symptomatic cases are often referred in infancy or early childhood. A 17-year-old female patient who was diagnosed as having annular pancreas is reported. The patient had had non-bilious vomiting accompanied by abdominal pain, especially 5-10 minutes after meals, for seven years. Annular pancreas, which may be seen at any age, should be considered in the differential diagnosis of patients with non-bilious vomiting, particularly after meals, over a long period.

A rare cause of abdominal pain: Ectopic ovary and intestinal malrotation.

Altinay-Kirli E, Özcan R, Öncül M, Özmen E, Eliçevik M, Büyükünal C, Emir H, Topuzlu-Tekant G. A rare cause of abdominal pain: Ectopic ovary and intestinal malrotation. Turk J Pediatr 2017; 59: 699-703. Ectopic ovary is a rare anomaly that can be associated with unicornuate uterus and renal anomalies. Intestinal rotational anomalies are failure of normal rotation and this arrest in development can predispose to develop a malfixated midgut that is a risk factor for volvulus and significant morbidity and mortality especially in early childhood. Cyclic abdominal pain is a common symptom for both of two distinct pathologies in adolescent ages. Here, we report a case of unicornuate uterus together with right ectopic ovary and intestinal malrotation.

A review of intussusception cases involving failed pneumatic reduction and re-intussusception.

BACKGROUND: The aim of the present study was to evaluate cases in which intussusception was unsuccessfully treated with pneumatic reduction (PR), and intussusception recurred following PR. METHODS: The medical records of 401 patients who presented with intussusception between 2003 and 2014 were retrospectively analyzed. Included were 61 patients, 20 of whom underwent unsuccessful PR (Group 1), and 41 of whom experienced intussusception recurrence following PR (Group 2). Treatment and outcome were summarized. RESULTS: In Group 1 (mean age: 14.2 months; range: 2.5 months-6 years) surgery was indicated due to PR failure in 15 patients, and perforation occurred during PR in 5. In these 5 patients, age was under 1 year (range 6-9 months) and mean time to presentation was 3 days (range 2-4). During laparotomy, pathologies were noted: mesenteric lymphadenopathy (LAP) and/or Peyer's patch hyperplasia was observed in 15 cases, Meckel's diverticulum in 5 cases. In Group 2 (mean age: 20 months; range: 3 months-6 years), intussusception developed after successful PR in 41 patients, most frequently within the first 24 hours (21.51%). Of the 41 patients, recurrent intussusception (RI) was treated with PR in 36, and laparotomy in 5. Operative findings were mesenteric LAP in 4 and polyp in 1. CONCLUSION: PR is effective for the treatment of intussusception and recurrences. Delayed presentation reduces the success rate. In the event of failure, a lead point is usually encountered at laparotomy.

[Duodenum perforations in children: case series]. / Çocuklarda duodenum perforasyonlari: Olgu serisi.

To analyse patients those admitted to our clinic due to perforation in duodenum. Fourteen patients those have been admitted to our center with perforation in duodenum between 1990 - 2014 analysed retrospectively. Ten patients (8M, 6F) those have been admitted to our clinic between 1990 - 2014 have a mean age of 6.2 years (25 days - 16 years). Two of cases admitted directly to our clinic and the rest referred from another hospitals. Mean time for appliance to our clinic was 3.2 days (1day - 1 week). Ulcer in duodenum was the cause of perforation in 10 cases while in 3 the cause was trauma and in 1 case was surgical complication of infantly persistant hyperinsulinism (IPHH). The case with hyperbilluribinemia after near total pancreatectomy due to IPHH had been explored and perforation in deuedenum diagnosed. Resection in first two parts of duodenum and pylor, choledochojejunostomy, gastrojejunostomy and jejunojejunostomy was performed. Primary repair was performed in the remaining patients. In one case with primary repair gastrostomy was performed, while in 9 cases omentoplasty were performed. In the follow-up 12 cases has no problems and doing well. Two patients died. Perforation in duedenum is a rare entity that pediatric surgeons should encounter and keep in mind. Primary repair (duodenoraphy ± omentoplasty) is safe and reliable surgical treatment modality.

Comparison of the efficiencies of esophageal manometry, vector volume analysis and esophagus pH monitoring in the diagnosis of gastroesophageal reflux.

AIM: In this study, we aimed to compare the superiorities of esophageal manometry, vector volume analysis and 24-hour pH meter studies in showing gastroesophageal reflux disease. MATERIAL AND METHODS: The files of the patients who presented to pediatric surgery and pediatric gastroenterology outpatient clinics of our hospital with suspicious gastroesophageal reflux disease between 2011 and 2012 and who were investigated were examined and 21 patients whose investigations had been completed were included in the study. The patients were evaluated by treatment method and were divided into three groups as Group 1 who were followed up with medical treatment, Group 2 in whom surgical intervention was performed and Group 3 who were not treated. Chi-square test was used in evaluation of the categorical variables, Kruskal Wallis test was used in comparison of the mean values between the groups and Dunn test was used in subgroup analyses when Kruskal Wallis test was found to be significant. A p value of <0.05 was considered statistically significant. RESULTS: Thirteen of 21 patients included in the study were female and eight were male. The mean age of the patients was 5.71 years (one-16 years). In the 24-hour pH monitoring study, the mean reflux index was found to be 48.7% in Group 1, 42.4% in Group 2 and 28.3% in Group 3. In esophageal manometry studies, the pressure difference at lower esophageal sphincter (LES) was found to be 13,4 cm H2O in Group 1, 31.8 cm H2O in Group 2 and 4.3 cmH2O in Group 3. In vector volume analyses, the mean vector volume was calculated to be 96.01 cm(3) in Group 1, 2 398.9 cm(3) in Group 2 and 196.3 cm(3) in Group 3. In the 24-hour pH monitoring study, a statistically significant difference (p<0.05) was found in terms of showing reflux, whereas statistical significance could not be shown in terms of need for surgical treatment or need for medical treatment in any other method (p>0.05). CONCLUSIONS: Twenty-four-hour pH monitoring was found to be efficient in making a diagnosis of gastroesophageal reflux disease, whereas esophageal manometry and vector volume analyses were not found to be efficient.