Determining the Risk Factors for Anastomotic Stricture Development after Esophageal Atresia Repair: Results from the Turkish Esophageal Atresia Registry.

INTRODUCTION: Anastomotic stricture (AS) is the second most common complication after esophageal atresia (EA) repair. We aimed to evaluate the data in the Turkish Esophageal Atresia Registry to determine the risk factors for AS development after EA repair in a large national cohort of patients. METHODS: The data between 2015 and 2021 were evaluated. Patients were enrolled into two groups according to the occurrence of AS. Patients with AS (AS group) and without AS (non-AS group) were compared according to demographic and operative features, postoperative intubation status, and postoperative complications, such as anastomotic leaks, fistula recanalization, and the presence of gastroesophageal reflux (GER). A multivariable logistic regression analysis was performed to define the risk factors for the development of AS after EA repair. RESULTS: Among the 713 cases, 144 patients (20.19%) were enrolled into the AS group and 569 (79.81%) in the non-AS group. The multivariable logistic regression showed that, being a term baby (odds ratio [OR]: 1.706; p = 0.006), having a birth weight over 2,500 g (OR: 1.72; p = 0.006), presence of GER (OR: 5.267; p < 0.001), or having a recurrent tracheoesophageal fistula (TEF, OR: 4.363; p = 0.006) were the risk factors for the development of AS. CONCLUSIONS: The results of our national registry demonstrate that 20% of EA patients developed AS within their first year of life. In patients with early primary anastomosis, birth weight greater than 2,500 g and presence of GER were risk factors for developing AS. When patients with delayed anastomosis were included, in addition to the previous risk factors, being a term baby, and having recurrent TEF also became risk factors. LEVEL OF EVIDENCE: III.

An Unexpected Infection in Loss-of-Function Mutations in STAT3: Malignant Alveolar Echinococcosis in Liver.

Loss-of-function (LOF) mutations in signal transducer and activator of transcription 3 (STAT3) gene causes autosomal dominant hyper immunoglobulin E syndrome (AD-HIES or Job's Syndrome), a rare and complex primary immunodeficiency (PID) syndrome characterized by increased levels of IgE (>2000 IU/mL), eosinophilia, recurrent staphylococcal skin abscesses, eczema, recurrent pneumonia, skeletal and connective tissue abnormalities. Although bacterial and fungal infections are common in AD-HIES, susceptibility to parasitic infections has not been reported. Alveolar echinococcosis (AE), a zoonosis caused by the growth of the Echinococcus multilocularis (EM) metacestode, mimics slow-growing liver cancer. The mortality rate of AE is very high when it is diagnosed late or under-treated. Here, we report a 14-year-old boy with AE infections of the liver and the lung resulting in liver failure and diagnosed as STAT3-LOF. To our knowledge, the association between these two conditions has not been reported in the literature before.

Assessment of developmental and radiological long-term outcomeof children with surgically treated midgut volvulus.

BACKGROUND/AIM: The aim is to evaluate the long-term outcome of asymptomatic patients who underwent surgical correction for midgut volvulus. MATERIALS AND METHODS: Seven patients managed surgically for midgut volvulus in the last 3 years were included. Demographic features, symptoms at presentation, diagnoses, surgical procedures, and complications were recorded. Patients were then contacted for follow-up and evaluation of long-term outcomes. General physical conditions, anthropometric parameters, feeding habits, and defecation histories were evaluated. Laboratory examinations were performed. Color Doppler ultrasonography (CDUS) was performed to evaluate blood flow in the superior mesenteric artery (SMA). RESULTS: In total, seven patients were identified. The median age at admission was 3 days (0-90 days). Mean age at follow-up was 17 ± 2 months. Growth parameters were normal in all cases. Four patients had low levels of ferritin and transferrin saturation. One patient had microcytic anemia. Another patient had low serum zinc level. One patient who had ileal resection had a high level of steatorrhea in stool examination. In CDUS, SMA blood flow volume was low in all cases. Peak-systolic velocity and resistance index were low in all but one case. CONCLUSION: Despite uneventful postoperative courses, all patients operated on for midgut volvulus showed mild laboratory changes and decreased blood flow in the SMA in long-term follow-up.

Hormone therapy in hypospadias surgery: a survey on the current practice in Turkey.

BACKGROUND/AIM: Nowadays surgical intervention is possible in smaller phalluses and younger children with hypospadias disease. Different hormone treatments with different doses, modalities, indications, and treatment times come along with some disputes. The aim of this study is to evaluate the management approaches in hypospadias surgery of surgeons in regards to hormone preparations. MATERIALS AND METHODS: Questionnaires were sent via e-mail to 110 actively working pediatric surgeons and urologists. The answers of 99 surgeons were evaluated (90%). Two surgeons declared that they did not perform hypospadias surgery. RESULTS: When testosterone usage in penile surgery was questioned, 44.4% of participants (n = 44) answered positively. Small-short penis glans, narrow urethral plate, chordee, disorders of sexual development, buccal mucosa-graft operations, slight tissue, and defective ventral skin were the indications for usage. Forty of forty-four surgeons stated usage in proximal hypospadias, 18 of them in penile hypospadias, and 15 of them in distal hypospadias. The most common form was dihydrotestosterone (62%). According to the respondents, fistulas (83%), infections (78%), and wound dehiscence (77%) were reduced. Fifty-six percent of the surgeons stated that bleeding was increased and 39% stated easier dissection. CONCLUSION: As a result of this questionnaire we can understand that there is no standard usage of testosterone in Turkey. Optimal points of usage can be introduced by increasing prospective randomized trials and education programs can ensure similar effective usage.