Neurodevelopmental evaluation of children who were operated due to congenital anomaly with the 'Ages and Stages Questionnaire (ASQ)' and 'ASQ: Social-Emotional'.

BACKGROUND: The purpose of surgeries performed for congenital anomalies in children is to increase the survival rates and provide a developmental comparison to that of their peers. AIM: The objective of this study was to investigate the development of children following surgery for congenital anomalies and the risk factors affecting their development. METHODS: Our study included 33 children who underwent surgery for gastrointestinal anomalies in our clinic between 2011 and 2016, and did not have any syndrome, chromosomal abnormality, or additional abnormality. Developmental levels were evaluated using the Ages and Stages Questionnaire (ASQ) and the ASQ: Social-Emotional (ASQ: SE) scales adapted for the use on Turkish children. Data on patient history were obtained retrospectively from patient files. RESULTS: The study included 33 patients, including 11 with esophageal atresia, 6 with intestinal atresia, 11 with anorectal malformation, and 5 with Hirschsprung's disease. Developmental delay was found in the ASQ of 72.7% of the patients and the ASQ: SE tool was 27% of the patients. The rate of patients with scores below the threshold from each parameter of ASQ was higher than that of the normal population (p < 0.05). Development delay was detected using the ASQ scale in 100% of those with microcephaly at birth, in 91% of premature infants born between 1500 and 2500 g, and in 83.3% of those with low birth weight to gestational age. CONCLUSIONS: In children who underwent surgery due to congenital anomalies, an evaluation through developmental tests, a post-surgical follow-up process, and a referral to the relevant disciplines when necessary may increase the success of surgery as well as increase the life quality of the patient.

An algorithm for retrieval tools in foreign body ingestion and food impaction in children.

The aim of this study is to review the experience on managing foreign body ingestion in children with special emphasis on the endoscopic techniques and specific retrieval devices used for foreign body (FB) extraction. The charts of 341 children were reviewed retrospectively. Demographic data, ingested material, removal technique and tool, level of FB, complications, and outcomes were recorded. A total of 364 FBs were removed from 341 children. Among these, 56.5% (n: 206) were entrapped in esophagus, 39% (n: 142) were in stomach, and 4.5% (n: 16) in duodenum and intestine. The most frequently ingested items were coin (42.5%), button batteries (20.6%), and safety pins (12%). Optical forceps (37.9%) were the most commonly used tool and they were used during retrieval of esophageal FB by rigid endoscopy. Retrieval net (20.7%) was the second most common tool and the most common one during flexible endoscopy. Depending on our experience, we strongly advocate rigid endoscopy for esophageal FBs and food impaction in children because it allows both to use optical forceps with a strong grasping ability for blunt FBs and to position sharp and pointed objects inside the rigid endoscope. We recommend retrieval net as the first tool for the extraction of blunt objects and rat tooth retrieval forceps is the best tool for sharp and pointed FBs in stomach.

Determination of pentraxin 3 levels in diagnosis of appendicitis in children.

BACKGROUND: The aim of this study is to determine whether pentraxin 3 (PTX3) levels can be used in the diagnosis of appendicitis in children. METHODS: Fifteen children under 18 years old, who gave blood samples for reasons other than inflammatory conditions, and 40 children who were proved to have appendicitis (non-perforated or perforated) between August 2017 and January 2018, were enrolled in the study. Patients were classified into subgroups: group 1 (healthy children without any sign of inflammation, n = 15), group 2 (non-perforated appendicitis, n = 25), and group 3 (perforated appendicitis, n = 15). RESULTS: The median PTX3 value was 1.01 ng/mL (minimum value: 0.82, maximum: 1.28) in the control group. The median PTX3 values prior to surgery were 20.68 ng/mL (minimum: 1.02, maximum: 28.471) and 1.46 (minimum: 1.05, maximum: 23.421) ng/mL for non-perforated appendicitis and perforated appendicitis respectively. The PTX3 levels were significantly higher in groups 2 and 3 than in group 1 (P < 0.01). According to receiver operating characteristics analysis the cut-off value of PTX3 levels at admission for appendicitis was 1.30 ng/mL with a sensitivity of 75% and a specificity of 100% (area under the curve, 0.939; P = 0.000). CONCLUSIONS: Pentraxin 3 levels were significantly higher in children with appendicitis than in healthy children. Children with high PTX3 levels complaining of right lower quadrant pain may support a diagnosis of appendicitis. Future studies should be conducted to evaluate changes in PTX3 levels by comparing children with appendicitis versus acute abdominal pain in larger populations to further determine the value of PTX3 in the diagnosis of appendicitis in children.

Laparoscopic assisted anterior transabdominal wall closure using loop suture removing technique in Morgagni hernia: safe and easy method.

PURPOSE: The treatment of MH entails surgical repair either by open abdominal or thoracic approaches or by minimal invasive surgery. The aim of this study is to evaluate the surgical and clinical outcomes of children who underwent laparoscopic assisted transabdominal wall closure and subcutaneous knot placement technique. METHODS: This retrospective study includes pediatric patients who underwent surgery for MH between January 2015 and February 2019. The patients' demographic data, symptoms, operative findings and technique and postoperative outcomes were recorded. RESULTS: A total of 17 children with MH were treated. There were 13 boys (76.5%) and four (23.5%) girls. Six patients had trisomy 21 (37%). The mean operation time was 40 min (25-90 min). The hernia sac was removed in all patients and there was no need for prosthetic patch in any of the children. Mean time to start feeding was 10 h (6-24 h). The mean hospitalization time was 2 days (1-5 days). There were no complications intraoperatively or postoperatively. The mean follow-up time was 24 months (6-40). No recurrence occurred during the follow-up time. CONCLUSION: Laparoscopic assisted transabdominal wall closure and subcutaneous knot placement technique is a preferable method with excellent outcomes and short hospitalization and feeding time postoperatively. This technique may be performed easily and safely without requiring any additional experience other than basic laparoscopy skills.

Allogeneic hematopoietic stem cell and liver transplantation in a young girl with dedicator of cytokinesis 8 protein deficiency.

DOCK8 deficiency is a rare inherited combined immunodeficiency, caused by mutations in the DOCK8 gene. We describe a case with DOCK8 deficiency associated with severe CLD in whom orthotopic LT was performed successfully after allogeneic HSCT. A 5 year-old girl with DOCK8 deficiency presented with mild direct hyperbilirubinemia and abnormal GGT level and without a previous history of jaundice. She had severe growth retardation, hepatosplenomegaly and generalized eczema. Progressive worsening of CLD was observed within 4 months. Investigations for etiology of liver disease were negative. Liver biopsy showed bridging necrosis, cholestasis and, cirrhosis. Recurrent immune hemolytic crisis and several viral infections developed in follow-up. She underwent whole cadaveric LT for end-stage liver disease (ESLD) 1 year after allogenic HSCT from a full matched related donor. The postoperative course was uneventful. The patient is alive with normal liver function and moderate skin graft versus host disease for 36 months after LT. In conclusion DOCK8 deficiency can be associated with severe CLD. Successful LT following HSCT is possible in patients with ESLD in DOCK8 deficiency. The timing of LT is challenging in patients requiring both HSCT and LT since conditioning regimens for HSCT can be highly hepatotoxic and the patients with suboptimal liver function can become decompensated during HSCT.

The Effects of Local and Systemic Growth Hormone Treatment on Germ Cell Population and Fertility in an Experimental Unilateral Testicular Torsion and Orchiectomy Model.

PURPOSE: We evaluated the effects of local and systemic growth hormone on the germ cell population of the contralateral testes of pubertal rats subjected to unilateral testicular torsion and orchiectomy 24 hours later. MATERIALS AND METHODS: A total of 40 male Wistar-Albino rats at age 3 weeks were divided into 5 groups. In the sham operated group the right testis was sutured and orchiectomy was performed 24 hours later. In groups 2 to 5 orchiectomy was performed 24 hours later following testicular torsion. In groups 3 and 4 unloaded and growth hormone loaded gelatin films, respectively, were sutured on the contralateral testes. In group 5 systemic growth hormone was administered for 7 days. Five weeks later each rat was cohabited with 2 female rats and the left testes were removed for evaluation. Mean seminiferous tubular diameter, mean testicular biopsy score and the mean haploid cell percentage were calculated. Mating studies were performed and fertility parameters were assayed. RESULTS: Mean seminiferous tubular diameter, mean testicular biopsy score and the mean haploid cell percentage of the contralateral testes were significantly decreased in the control and gelatin groups compared with the other groups. There was no difference between the local and systemic growth hormone groups regarding the haploid cell percentage. There were no differences between the groups in mean fetus numbers, mating or fertility and fecundity indexes except in the gelatin group, in which the mean fetus number was significantly lower. CONCLUSIONS: Fertility is not affected in rats after 24 hours of testicular torsion and orchiectomy, although there is germ cell injury and a decrease in the percent of haploid cells. Growth hormone administration resulted in the restoration of germ cell histology and an increase in the haploid cell percentage of the contralateral testes. Growth hormone may improve fertility after unilateral testicular torsion and orchiectomy.

LOCAL-IGF-1 and GH application IMPROVES germ cell histology, spermatogenesis and fertility after experimental testicular torsion and detorsion.

OBJECTIVE: To evaluate the impact of insulin like growth factor-1(IGF-1) and growth hormone (GH) on testis histology, spermatogenesis, and fertility in prepubertal rats exposed to 6 h of testicular torsion (TT) and detorsion. MATERIAL-METHOD: Forty-eight male Wistar-albino rats weighing 30-70g and at 3-week age were allocated into six groups involving eight rats in each group as follows: Group 1:Sham, Group 2:Control, Group 3:Gelatin, Group 4:Local-IGF-1, 5: Local-GH, Group 6: Systemic-GH. Right testis was only exposed and sutured in the sham group, and right testes were rotated clockwise, 720°, fixed, and 6 h later, detorsion on the testis was done in groups 2-6. Unloaded gelatin, 5 µg local-IGF-1 loaded, and 2IU rhGH loaded gelatin were sutured to the right testis after detorsion in groups 3-5. In Group 6, 0.3IU/100gr/d rhGH was given for seven days via subcuticular route after detorsion. Each of the rats cohabited with two female rats five weeks later. Afterward, both right and left testes were removed. Mean diameter of seminiferous tubules (STD), mean biopsy score count of the testis (TBSC), mean percentage of haploid cells (HCP) were assessed, and fertility parameters were evaluated. RESULTS: STD and TBSC of the ipsilateral testes were significantly reduced in control and gelatin groups when compared to sham, local-IGF-1, and local-GH groups. STD and TBSC of the ipsilateral testes of the systemic-GH group were decreased compared to the sham group. HCP of the ipsilateral testes of control, gelatin, and systemic-GH groups were significantly lower than the sham, local-IGF-1, and local-GH groups. STD, TBSC, and HCP of the contralateral testes were significantly reduced in control and gelatin groups when compared separately to sham, local-IGF-1, systemic- GH, and local-GH groups. The difference between groups regarding potency, fertility, fecundity indexes, and mean fetus numbers were not significant. CONCLUSION: Even though there was significant and permanent histologic germ cell damage and reduced HCP in both ipsilateral and contralateral testes, experimental 6 h TT and detorsion in prepubertal rats did not have a negative impact on future fertility. Local-IGF-1and rhGH treatment improved germ cell histology and spermatogenesis in both ipsilateral and contralateral testes of prepubertal rats, subjected to 6 h of TT and detorsion.

Effects of intraperitoneal bupivacaine injection in laparoscopic appendectomy in children on post-operative pain: A controlled randomized double-blinded study.

BACKGROUND: The aim of this study is to determine the effects of local anesthetics administered intraperitoneally in laparoscopic appendectomy. METHODS: Patients who underwent laparoscopic appendectomy due to acute appendicitis were enrolled in the study. The children were divided into two groups. Intraperitoneal bupivacaine injection to appendectomy site and subdiaphragmatic area was performed after resection of appendix and aspirastion of intraperitoneal reactive fluid in Group 1 while Group 2 did not receive this therapy. The children were questioned by a nurse at postoperative 1st, 6th, 12th, and 24th h. Pain scores (PS) (abdominal), abdominal wall incisional pain (IP), shoulder pain (SP), and first need for analgesics were recorded. RESULTS: One hundred and twenty children were enrolled to the study. There was no significant difference in PS values and IP values between the two groups (p>0.05). SP values at 12th and 24th h were significantly lower in Group 1 (p<0.05). There was a statistically significant reduce in analgesic need in Group 1 (p=0.007). CONCLUSION: Intraperitoneal bupivacaine instillation to surgery site and subdiaphragmatic area seems to reduce the SP post-opera-tive and also reduce post-operative analgesic need. More meaningful results can be obtained with an increase in the number of patients.

Outcomes of Surgical Strategies for Living Donor Liver Transplantation in Patients With Portal Vein Thrombosis: A Cohort Study.

BACKGROUND: Adequate portal flow to the liver graft is the requirement of a successful liver transplant (LT). Historically, portal vein thrombosis (PVT) was a contraindication for LT, especially for living donor LT (LDLT), demanding technically more difficult operations and advanced technique. In this study, the outcomes of patients with and without PVT after LDLT were compared. METHODS: Adult LDLTs performed by 2 centers (n = 335) between 2013 and 2020 were included into this large cohort study. PVT was classified based on Yerdel classification grade 1 to 4. RESULTS: Sixty-two patients with PVT constituted 19% of the study cohort of 335 recipients. While mean platelet count was found to be lower (P = .011) in the PVT group, patient age (P = .035), operation duration (P = .001), and amount of intraoperative blood transfusion (P = .010) were found to be higher. Incidence of PVT was higher in female patients than males (22.7% vs 16.1%, P = .037). There was no significant difference in survival between patients with and without PVT on 30-day (P = .285), 90-day (P = .565), 1-year (P = .777), and overall survival (P = .917). Early thrombosis did not show a better survival rate than Grades 2, 3, or 4 PVT. Thrombosis limited to portal vein was not found to bring a survival advantage compared with Grade 3 and 4 thromboses. Eversion thrombectomy was the most common procedure (66%) to overcome PVT intraoperatively. CONCLUSION: Although technically more challenging, PVT is not a contraindication of LDLT. Similar outcomes can be achieved in LDLT in patients with PVT after proper restoration of portal flow, which eliminates the default survival disadvantage of patients with PVT.

Hypospadias and the Use of the Ages and Stages Questionnaire to Evaluate Neurodevelopmental Status of Boys with Hypospadias.

INTRODUCTION: Placental insufficiency is one of the reasons for the reduction of hormone production. Thus, if one of the suspected causes of hypospadias is placental insufficiency, then the neurodevelopmental status of boys with hypospadias may be impaired. The aim of this study was to evaluate the neurodevelopmental status of boys with hypospadias and guide the parents of those who need support to related departments for early intervention. MATERIALS AND METHODS: Boys were divided into two groups, those with hypospadias (group H) and healthy children undergoing traditional circumcision (group C). The parents of the boys completed the Ages and Stages Questionnaire (ASQ) and ASQ-Social Emotional (SE), both of which are screening instruments for the early identification of developmental and social-emotional problems, respectively. RESULTS: Seventy-eight boys had hypospadias and 59 were admitted for traditional circumcision. The group H had statistically significant more impaired scores than group C in communication, gross motor, and personal-social skill sections. The multivariate logistic regression analysis revealed that hypospadias was the independent predictive factor for communication and personal-social skills. CONCLUSION: Hypospadias and neurologic impairment may share common etiologic factors. Accordingly, physicians should keep in mind that if a boy presents with hypospadias, the possibility of having neurologic impairment is higher than normal population and early intervention has crucial importance. Every boy with hypospadias should be evaluated for neurodevelopmental status.

3D-reconstruction and heterotopic implantation of reduced size monosegment or left lateral segment grafts in small infants: A new technique in pediatric living donor liver transplantation to overcome large-for-size syndrome.

BACKGROUND: Monosegmental grafts and reduced left lateral segment grafts have been introduced to overcome the problems of large-for-size grafts in pediatric living donor liver transplantation. Here, we introduce a new method of reduced size monosegment or left lateral segment grafts transplanted in the right diaphragmatic fossa heterotopically in small infants. METHODS: There were 4 infants who underwent living donor liver transplantation with heterotopically implanted reduced monosegmental or left lateral segment grafts at our center. The demographic, operative, postoperative, and follow-up data of these infants were collected from our prospectively designed database and reviewed. Technical details of the donor and recipient operation are shared and a supplemental provided. RESULTS: The mean recipient age was 7.5 ± 0.9 months (range: 5-10 months), and body weight was 5.9 ± 0.7 kg (range: 4.6-7.8). Primary diagnoses of the recipients were biliary atresia (n:3) and progressive familial intrahepatic cholestasis (n:1). Mean graft-recipient weight ratio was 3.3 ± 0.2. Reduced monosegment III grafts were used in 2 cases, and reduced left lateral segment grafts were used in the other 2 patients. Bile duct reconstruction was done by Roux-en-Y hepaticojejunostomy in 3 patients and duct-to-duct anastomosis in the remaining patient. All patients recovered from the liver transplantation operation and are doing well at a mean follow-up of 8 months. CONCLUSION: Living donor liver transplantation with heterotopically implanted reduced monosegmental or left lateral segment seems feasible for the treatment of neonates and extremely small infants. Further accumulation of cases and long-term follow-up are necessary to collect data for the establishment of this treatment modality.

An indirect effect of COVID-19 pandemic: Increased pediatric perforated appendicitis rate due to delayed admission.

Objectives: Appendicitis is a common surgical emergency among children. The coronavirus pandemic affected the system of hospitals more than any other field, and great amount of people were concerned about visiting the hospitals for any reason. In this study, it was aimed to evaluate the profile of appendicitis by emphasizing perforated and acute appendicitis in the pandemic period and to compare the rates with previous three years. Material and Methods: Charts of the children who underwent laparoscopic appendectomy due to appendicitis between March 11-September 30 between 2017-2020 were retrospectively analyzed in terms of demographic data, duration of symptoms, duration between hospital admission and surgery, radiologic imaging and perioperative outcomes. Results: This study includes 467 children who underwent laparoscopic appendectomy. There were 97 procedures in 2020, 111 in 2019, 146 in 2018 and 113 in 2017. Multiple comparison tests revealed that age did not show difference; but onset of symptoms in admission (p= 0.004), hospitalization time before surgery (p <0.001), total hospitalization time (p <0.001) showed statistically significant difference between years. Pairwise comparisons showed that these parameters were increased in 2020 compared to other years. Perforated appendicitis rate was significantly increased in 2020 when compared to previous years. Conclusion: Although there is no direct relation between appendicitis and COVID-19 infection in the current knowledge, perforated appendicitis was found to be increased in children during the COVID pandemic. Reason of the higher rate of perforated appendicitis may be multifactorial; however, the pandemic appears to have a role in increased morbidity in children with appendicitis indirectly due to delay of hospital admissions.

A Safe and Minimally Invasive Method for Thoracoscopic Lung Biopsy in Interstitial Lung Disease.

Background: Interstitial lung disease (ILD) represents a spectrum of lung diseases that may contain different levels of fibrosis and inflammation. Sometimes, the clinical picture and imaging studies are insufficient to determine the diagnosis and lung biopsy becomes crucial, which may be performed in a thoracoscopic or open manner. The aim of this study was to present a new minimally invasive biopsy method that seems to be technically easy, safe, and inexpensive. Herein, we present a minimally invasive method for thoracoscopic lung biopsy using a Vicryl loop. Methods: The charts of patients with suspected ILD who underwent thoracoscopic lung biopsy using a Vicryl loop between 2007 and 2017 were analyzed retrospectively. Results: Nine patients were identified, four were boys and five were girls. The median age was 76 months. The mean weight of the patients was 10.9 kg. The mean chest tube removal time was 3.8 days. The mean surgical time was 37 minutes. There was an air leakage for 13 days in one child postoperatively. Conclusions: Thoracoscopic lung biopsy with a knot and scissors is an easily applicable and minimally invasive method that may be performed in children with suspected ILD.

Successful living-related liver transplantation in a child with familial yellow nail syndrome and fulminant hepatic failure: report of a case.

An 11-yr-old boy with familial YNS and FHF and who underwent LRLT is presented. LRLT was performed from his father with YNS. The findings of hepatic failure resolved immediately after LRLT, but severe respiratory complications and chylous ascites were observed during the follow-up. At 12 months after successful LT, the patient has good graft function, but findings of YNS including chronic cough, lymphedema and yellow nails are still present. To the best of our knowledge, this is the first case of YNS who underwent LRLT for FHF.

Crohn's disease of the vulva in a 10-year-old girl.

Crohn's disease may involve all parts of the gastrointestinal tract and may often involve other organs as well. These non-intestinal affections are termed extraintestinal manifestations. Vulval involvement is an uncommon extraintestinal manifestation of Crohn's disease, and it is very rare in children. Patients with vulval CD typically present with erythema and edema of the labia majora, which progresses to extensive ulcer formation. Vulval Crohn's disease can appear before or after intestinal problems or it may occur simultaneously. We present a 10-year-old girl with intestinal Crohn's disease complicated with perianal skin tags and asymptomatic unilateral labial hypertrophy. The course of her lesion was independent of the intestinal disease and responded significantly to medical treatment including azathioprine and topical steroid. We emphasize that although vulval involvement in childhood is uncommon, Crohn's disease must be considered in the differential diagnosis of nontender, red, edematous lesions of the genital area.

Stuck tunneled central venous catheters in children: Four cases removed by angiography assistance.

Ates U, Tastekin NY, Mammadov F, Ergün E, Göllü G, Can ÖS, Uçar T, Bingöl-Kologlu M, Yagmurlu A, Aktug T. Stuck tunneled central venous catheters in children: Four cases removed by angiography assistance. Turk J Pediatr 2018; 60: 221-224. Adherent tunneled catheters can usually be removed by a surgical cut down, but in some cases the line can become stuck to the wall of the central veins. In such cases, forceful traction can cause vascular injury, or fracture of the catheter. We present four cases of fixated cuffed tunneled catheters. Three children had acute lymphoblastic leukemia and one had an immunodeficiency syndrome. All catheters were made from polyurethane. Indwelling times were 12-24 months. All patients` catheters were removed with great difficulty by trans-femoral access. The angiography-assisted technique is safe and easily-applied for the removal of stuck catheters in pediatric patients. These cases raise important questions concerning the maximum indwelling time and the choice of catheter material when implanting permanent central venous catheters (CVCs) in children.

Endoscopic drainage and cystoduodedonstomy in a child with pancreatic pseudocyst.

An 11-year-old morbidly obese boy was diagnosed with pancreatic pseudocyst. Following fine needle aspiration, the cyst recurred in 1-month follow-up. Therefore, endoscopic drainage and cystoduodenostomy was performed following endosonography. Control ultrasonography (USG) revealed a completely shrunken cyst. During the 3 years of follow-up, the patient was asymptomatic with no evidence of cyst on computerized tomography scans. Endoscopic drainage and cystoduodenostomy is a minimally invasive, effective, and safe approach in the management of pancreatic pseudocysts in children.

An exceptional complication following appendectomy: acute inguinal and scrotal suppuration.

Inguinal and scrotal suppuration following appendectomy is a rare event, occurring mostly when a patent processus vaginalis is present. Herein we report a small series of children operated for appendicitis and presented with acute inguinal and scrotal symptoms postoperatively. Although acute scrotum is commonly attributed to torsion of testis or its appendage, epididymo-orchitis and incarcerated hernia, following appendicitis scrotal and/or inguinal abscess should be considered.