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Objectives: The appropriate time to perform feminizing genitoplasty in patients with congenital adrenal hyperplasia (CAH) with the 46, XX genotype is still debatable. The aim of our study was to evaluate the relationship between age at surgery and long-term surgical outcomes of patients who underwent feminizing genitoplasty., Methods: We retrospectively analyzed 14 patients with CAH and 46, XX genotypes with feminizing genitoplasty (clitoroplasty + vaginoplasty) between 2005 and 2022. The patients were divided into two groups. Group 1 consisted of seven girls (n = 7/14) who have been operated before the age of 2 years. Group 2 consisted of seven girls (n = 7/14) who have been operated after the age of 2 years. The two groups are compared regarding anatomical assessments, overall cosmetic results, need for additional intervention using Creighton's criteria. Additionally, the cosmetical satisfaction of the patients/parents is questioned., Results: The mean age of the girls was 32.42 months (10-96 months) during operation time. The mean age of Group 1 patients (n = 7/14) who have been operated before the age of 2 years was 11.71 months (10-19 months). The mean age of Group 2 patients (n = 7/14) who have been operated after the age of 2 years was 53.14 months (36-96 months). The mean follow-up time was 10.57 years (3-18 years). There was no statistically significant difference between those operated on before and after two years in terms of anatomical assessments, overall cosmetic results and patient/parent satisfaction except the need for additional intervention (p = 0.049). In Group1 (operation age < 2 years old), five out of seven (71.42%) patients needed additional major surgery (four urogenital sinus re-mobilization, one redo-clitoroplasty). Those who received additional major surgery were the ones who were not satisfied. In Group 2 (operation age > 2 years old), two patients out of seven patients (28.57%) received major surgery (two redo-urethroplasties) and those patients were not satisfied. When patient/parent satisfaction was compared with additional surgical intervention, as expected, patients'/parents' satisfaction increased as major surgical intervention decreased. This was statistically significant (p = 0.007). The main source of dissatisfaction was repeated surgery among the parents., Conclusions: The possibility of this additional surgical intervention increases, and patient/parent satisfaction decreases in patients below the age of 2 years. The corrective surgeries can be differed until the gender identity of the patient matures and the patient autonomy in deciding whether this surgery should occur., (© 2023. The Author(s), under exclusive licence to Springer-Verlag GmbH Germany, part of Springer Nature.)

Purpose: The purpose of the study was to evaluate our experience with enteric duplication cysts in 40 children during the past 26 years, while assessing the variability of their presentations and to propose an algorithm for surgical management., Methods: We retrospectively analysed sex, age, clinical presentations, duplication site, surgical treatment, presence of ectopic tissue, complications, associated anomalies, and prognosis of 40 patients with gastrointestinal tract duplications who were surgically treated in our clinic., Results: Overall, there was a predominance of boys (28 males, 70 %; 12 females, 30 %). The presenting symptom was vomiting in 23 patients, rectal bleeding in 11 patients, abdominal mass in 10 patients, abdominal pain in 9 patients, constipation in 6 patients, cough in 2 patients, and respiratory distress in 2 patients. In 30 patients, a complete excision of the cyst with additional segmental intestinal resection and anastomosis was performed. Cystectomy was performed in seven patients, while complete excision of the cyst with additional wedge resection was performed in two. A Wrenn procedure (mucosectomy) was performed in one patient., Conclusion: Due to the variability in the site of enteric duplications, a wide range of presenting symptoms can exist, which is challenging for diagnosis. In children with a diagnosis of acute abdomen, enteric duplication cysts should be considered, and these children should be further investigated for additional skeletal, urogenital, and gastrointestinal system pathologies. Surgical treatment depends on the site and type of the cyst.

Introduction: We sought to retrospectively assess the operative findings and clinical outcomes of 148 girls who underwent laparoscopic inguinal hernia repair with the percutaneous internal ring suturing (PIRS) technique., Methods: Between 2010 and 2014, girls with inguinal hernia underwent surgery using the laparoscopic PIRS technique described by Patkowski. Demographic and perioperative findings, complications, and recurrences were evaluated., Results: A total of 205 inguinal hernia repairs were performed in 148 children with a mean age of 5.83 years (1 month-16 years). In 57 girls (38.5 %), the hernias were bilaterally repaired, while in 91 girls (61.5 %) hernias were unilaterally repaired. The mean follow-up time was 3.6 years (range 2.5-6.1 years). No serious complications or recurrence were noted. Granuloma occurred in one patient., Conclusion: The PIRS technique is a safe, simple and effective procedure for girls. Excellent cosmetic results and reduced recurrence rates are associated with this method. This procedure is particularly suitable for girls because they lack a spermatic cord and vascular structures that can cause complications with this technique in boys. Based on our experience and others in the literature, we suggest that the PIRS procedure might be considered a gold standard for inguinal hernia operations in girls.

Purpose: We aimed to compare laparoscopic hernia repair and open hernia repair in recurrent cases after first open repair according to the length of time taken to perform the procedure., Methods: Between November 2009 and December 2011, the medical records of 26 male paediatric patients who were treated with laparoscopic surgery (with Schier's intracorporal "N" suture closure) and open surgery (with high ligation technique) in our institution for recurrent inguinal hernia were reviewed for the length of the operative time and post-operative complications retrospectively. Thirteen cases operated with laparoscopic repair were regarded as Group 1 and other 13 cases operated with the open high ligation repair were regarded as Group 2. All recurrent hernia cases had been performed in other hospitals with the open high ligation technique previously., Results: Thirteen internal inguinal ring closures in Group 1 were performed laparoscopically. In Group 2, 13 cases underwent open high ligation repair. Comparing the laparoscopic and open-repair techniques in the recurrent cases (Group 1 vs. Group 2; 32.36 vs. 61.07 min, respectively) showed that the length of the operation time was much shorter in laparoscopic repair group (Group 1) than open repair group (Group 2). A statistically significant difference was also observed (p = 0.001). No post-operative testicular atrophy or recurrence was seen until present time in all groups., Conclusion: We propose that laparoscopic repair in recurrent childhood inguinal hernia cases, developed after open repair, avoids entering a fibrotic inguinal canal, making the procedure easier and shorter. Laparoscopic herniorrhaphy is a good alternative option in recurrent childhood hernia.

Diaphragmatic rupture may be traumatic or may occur spontaneously. Spontaneous diaphragmatic rupture is a rare entity seen almost entirely in adults. We report here a case with spontaneous rupture of a congenital diaphragmatic evantration in a child.

We report here a fetus, who was delivered via cesarean section in 32th gestational week from a 37-year-old mother and sustained multiple gunshots. Post-natal evaluation revealed eight entry-exit holes and the baby was transferred to our clinic.Upon admission, peripheral pulses were nonpalpable, appropriate fluid administration and blood transfusion were conducted and further examinations revealed bladder perforation. Bladder was repaired over a suprapubic catheter and bullet holes were primarily sutured. Left foot drop was observed on follow up and the patient was discharged with no further complication. Although the maternal and the fetal morbidity and mortality rates are high in intrauterine gunshot wounds, appropriate management may provide survival as seen in our case.

Aim: Progressive familial intrahepatic cholestasis (PFIC) is a hereditary disease with severe cholestasis progressing to cirrhosis and chronic renal failure usually during the first decade. An alternative approach is partial diversion of bile. The aim of this study is to describe four patients with PFIC who underwent partial internal biliary diversion (PIBD)., Methods: Review of three patients, their clinical, laboratory and histologic workups to evaluate the short-term effects of PFIC, a 1-year follow-up. For PIBD, a conduit is performed between the terminolateral side of the gall bladder and distal colon using a segment of jejunum, to divert the biliary flow from the enterohepatic cycle without any external stoma., Results: All four patients were presented with jaundice, pruritus, hepatomegaly, sleep disturbance. They fulfilled the criteria for PFIC. The surgery was uneventful. At follow-up, biochemical parameters improved significantly, growth was regained, relief in pruritus, sleeping pattern was normalized., Conclusions: Partial internal biliary diversion had a dramatic effect on cholestasis, growth, sleeping and biochemical parameters. It also avoids the disadvantages of a permanent stoma. We believe that it is one of the best surgical procedures ever described for PFIC. Since long-term results of partial external biliary diversion on liver histopathology are successful, we hope that our long-term results will also be similar.