Abdominal wall muscle weakness outcomes after split abdominal flap repair of large congenital diaphragmatic hernias in newborn.

PURPOSE: Split abdominal wall muscle flap (SAWMF) is a technique to repair large defects in congenital diaphragmatic hernia (CDH). A possible objection to this intervention could be any associated abdominal muscle weakness. Our aim is to analyze the evolution of this abdominal muscle wall weakness. METHODS: Retrospective review of CDH repair by SAWMF (internal oblique muscle and transverse) from 2004 to 2023 focusing on the evolution of muscle wall weakness. RESULTS: Eighteen neonates of 148 CDH patients (12,1%) were repaired using SAWMF. Mean gestational age and birth weight were 35.7 ± 3.5 weeks and 2587 ± 816 g. Mean lung-to-head ratio was 1.49 ± 0.28 and 78% liver-up. Seven patients (38%) were prenatally treated by tracheal occlusion. Ninety-four percent of the flaps were used for primary repair and one to repair a recurrence. One patient (5.6%) experienced recurrence. Abdominal muscle wall weakness was present in the form of a bulge. Resolution of weakness at 1, 2 and 3 years was 67%, 89% and 94%, respectively. No patient required treatment for weakness or died. CONCLUSIONS: Abdominal muscular weakness after a split abdominal wall muscle flap repair is not a limitation for its realization since it is asymptomatic and presents a prompt spontaneous resolution. LEVEL OF EVIDENCE: IV.

Are Low Weight or Cardiopathy Contraindications for Thoracoscopic Repair of Esophageal Atresia with Tracheoesophageal Fistula?

BACKGROUND: Thoracoscopic repair of esophageal atresia (EA) with tracheoesophageal fistula (TEF) is becoming an increasingly widespread technique; there is still controversy about its indication in certain patients. Our objective is to analyze if potential risk factors such as major congenital heart disease (CHD) or low birth weight (LBW) are a limitation to this approach. METHODS: Retrospective study (2017-2021) of patients with EA and distal TEF who underwent thoracoscopic repair were included. Patients with LBW less than 2,000 g or major CHD were compared with the rest. RESULTS: Twenty-five patients underwent thoracoscopic surgery. Nine patients (36%) had major CHD. Five of them (20%) were LBW less than 2,000 g, and only 8% (2/25) presented both risk factors. There were no differences in terms of operative time, conversion rate, tolerance evaluated with gasometric parameters (pO2, pCO2, pH) or complications (anastomotic leak and stricture, both early or during follow-up) in patients with major CHD and LBW (1,473 ± 319 vs. 2,664 ± 402 g). One conversion to thoracotomy was performed in a neonate weighing 1,050 g due to anesthetic intolerance. There was no recurrence of TEF. One patient died at the age of 9 months, due to major uncorrectable heart disease. CONCLUSION: Thoracoscopic repair of EA/TEF is feasible technique in patients with CHD or LBW, with similar results to other patients. The complexity of this technique warrants individualizing the indication in each case. LEVEL OF EVIDENCE: IV.

Anastomosis near to the ileocecal valve in neonates with focal intestinal perforation, is it safe.

BACKGROUND: Anastomosis near the ileocecal valve (ICV) are controversial due to the increased pressure on the suture; in this situation, the valve could be removed at a first stage or at the moment of stoma closure. However, preservation of the ICV has proved important benefits in the long term. The aim of this study is to evaluate its feasibility in neonates with focal intestinal perforation (FIP). METHODS: Retrospective study (2010-2019) of neonates with FIP who underwent intestinal resection and primary anastomosis. Patients were divided into group A (anastomosis less than 5 cm from ICV) and group B (more than 5 cm). RESULTS: Forty patients were treated. Patients ostomized or with resection of ICV were excluded. Finally, 24 patients (birth weight 1043 ± 594 g (520-3000), age 8.8 ± 7.8 days (2-39)) were included for analysis. Patent ductus arteriosus was present in 75%. There were 6 patients in group A (25%) and 18 in group B (75%). Groups were comparable in terms of gestational age, birth weight, and age at the time of surgery (p > .05). There were no cases of dehiscence nor stenosis of the anastomosis. There were no differences in reoperation rate, infectious complications, time to enteral feeding, days of parenteral nutrition, hospital stay nor survival (p > .05). CONCLUSION: Ileo-ileal anastomosis closer to the ileocecal junction, in neonates with focal intestinal perforation, is an effective and safe option which also allows the preservation of the ICV avoiding the complications derived from its absence in a group of patients with high morbidity.

Results of Ovarian Sparing Surgery in Pediatric Patients: Is There a Place for Laparoscopy?

Background: Laparoscopy is widely accepted for the surgical treatment of benign ovarian lesions in adult women, with results similar to an open approach when spillage is avoided by different means such as endobag retrieval. This approach is controversial in children. Materials and Methods: Retrospective study (2009-2018) of all pediatric ovarian tumors with histological confirmation treated in a tertiary center. Data regarding preoperative selection criteria, surgical technique, and follow-up were registered. Laparoscopy was used only in tumors without malignant features and whose size allowed retrieving them through a small incision without morcellating. Emphasis was set in ovarian sparing surgery (OSS), comparing the results of open (OP) and laparoscopic (LPC) approach. Results: During the study period, 48 suspicious lesions in 45 patients were operated. Thirty cases (32 tumors) showed benign features (10 cystadenomas, 14 mature teratomas, 6 nontumor lesions); treatment consisted in oophorectomy in 13 (OP 6, LPC 7) and OSS in 17 (OP 5, LPC 12). There was one conversion in the LPC group. Mean follow-up was 3.44 years (range 0.03-8.84), based on annual ultrasound (US) and tumor markers during at least 5 years, recommending a lifetime follow-up by a gynecologist. There was only one relapse in a laparoscopically enucleated cystadenoma, and 2 patients developed contralateral metacronous teratomas. No procedure-related complications happened. Conclusions: Our series, although small and with a limited follow-up, suggest that laparoscopy could be an option of treatment in a subset of highly selected patients, although laparotomy remains the gold standard approach if malignancy is suspected.

Outcomes after Split Abdominal Wall Muscle Flap Repair for Large Congenital Diaphragmatic Hernias.

INTRODUCTION: Repair of large congenital diaphragmatic hernias (CDHs) is challenging. As primary repair is not always feasible, patches are commonly used. An alternative treatment is split abdominal wall muscle flap repair, which uses vascularized autologous tissue. The aim of this study was to analyze the long-term outcome of large CDH defects undergoing split abdominal wall muscle repair. MATERIALS AND METHODS: This is a retrospective review (2003-2016) of large CDH treated by split abdominal wall muscle flap repair. RESULTS: In a total of 107 CDH patients, the abdominal muscle flap technique was used in 10 (9.3%); 7 had been prenatally treated with tracheal occlusion. Two patients experienced recurrence at 2 months and 6 years, respectively. Only one patient required abdominoplasty due to abdominal wall muscle weakness. Two patients developed progressive scoliosis; one of them required orthopaedic treatment. Minor chest wall deformities were detected in seven, but only one required orthopaedic treatment. The lung-to-head ratio was 0.79 in patients developing musculoskeletal deformities, and 1.5 in those without this complication (p < 0.05). Median follow-up was 11.2 years (3.5-14.2), and all patients were alive at the time of writing this article. CONCLUSION: The split abdominal wall muscle flap technique is a valid option for repair of large CDH. Associated musculoskeletal deformities seem to be influenced not only by the repair technique used but also by the degree of pulmonary hypoplasia and inherent pathophysiological changes.

Management of neonates with right-sided aortic arch and esophageal atresia: International survey on IPEG AND ESPES members´ experience.

AIM: The optimum surgical approach of neonates with right-sided aortic arch (RAA) and esophageal atresia (EA)/tracheoesophageal fistula (TEF) is still an unsolved question. In order to propose an operative algorithm in the era of endoscopic surgery, we performed an international survey to know the current practice between pediatric endoscopic surgeons. Two of the most important societies in endoscopic pediatric surgery were queried: the International Pediatric Endosurgery Group (IPEG) and the European Society of Paediatric Endoscopic Surgeons (ESPES). MATERIALS AND METHODS: During December 2016, an anonymous online-based survey was sent to all IPEG and ESPES members, collecting data regarding perioperative management and surgical repair of EA/TEF with RAA. RESULTS: 144 surgeons from 23 countries completed the questionnaire. 69.2% of respondents were IPEG members, 30.8% were ESPES members. 71.5% of members who answered the survey had more than 10years of surgical experience. A preoperative echocardiography was almost uniformly performed (93.1%). 31.9% of the surveyed surgeons had never treated an EA/TEF with RAA. The remaining 98 surveyed surgeons had managed 279 cases of EA/TEF with RAA. Thoracotomy was considered the preferred approach for 54.2% of the surgeons, and 51.9% chose a right-sided approach. When RAA was an intraoperative finding, 76% would perform a contralateral thoracotomy if difficulties arose. Thoracoscopy was preferred by 45.8% of surgeons. If RAA was suspected preoperatively, 63.1% preferred to attempt a left-sided thoracoscopy and only 24.2% would change their approach to a thoracotomy. If RAA was an intraoperative finding and a safe surgical repair could not be achieved through right-sided thoracoscopy, 51.5% of them chose to perform a left sided thoracoscopy, rather than convert to thoracotomy. CONCLUSIONS: Preoperative echocardiography performed by experienced examiners helps in surgical planning. Preoperative diagnosis of RAA should not discourage thoracoscopic repair, which is increasingly becoming more popular for the correction of EA/TEF. In case of an unexpected intraoperative diagnosis of RAA or operative difficulties when approaching through the right side, thoracoscopy offers a less aggressive approach. LEVEL OF EVIDENCE: V.

Bladder neck closure in children: long-term results and consequences.

AIM: Only few studies have gathered information on the long-term outcomes of children undergoing bladder neck closure (BNC). In the present study, we analyze the long-term results and consequences in this population. PATIENTS AND METHODS: The medical records of 20 patients (12 males and 8 females), who underwent BNC after several failed procedures to improve urinary incontinence were revised. Exstrophy complex was the underlying diagnose in all the cases: bladder exstrophy in 15 and cloacal exstrophy in 5. The median age of the patients at the time of surgery was 11.5 years (range 4-19 years). Previous surgeries were bladder neck reconstruction in 14 patients, bladder neck injection in 4 patients, and previous BNC in 1 patient. Overall 17 patients had concomitant bladder augmentation. The catheterizable stoma was made with appendix in 14, bowel in 3, and ureter in 3. The nine male patients aged>18 years at the time of this study received a questionnaire with specific questions on erections, orgasm, and details of ejaculation and were also asked to provide a semen sample. RESULTS: The median follow-up was 10 years (range 2-17 years) and median patient age was 21 years (range 13-32 years). Urinary continence was achieved initially in 16 patients (80%); 4 had a bladder neck fistula, 3 of them underwent surgical revision and achieved dryness. Long-term complications were: bladder stones (eight patients); stomal problems (four stenosis and three leaks); bladder perforation (two patients), and orchitis (one patient). All patients above the age of 18 years reported erections and orgasms although two had medium erectile dysfunction. The ejaculate volume was reported as normal in five, scarce in three, and absent in one, with slow ejection in seven. Only four supplied semen samples and only two showed normal values. CONCLUSIONS: BNC is an effective approach to incontinence when other procedures have failed. In the long term, the most frequent complications are those related with catheterizable stoma and stones. The high incidence report of a low fertility index and erectile dysfunction meant further study in a larger cohort.

Early endoscopic dilation and mitomycin application in the treatment of acquired tracheal stenosis.

INTRODUCTION: Acquired airway stenosis is a common complication in children after periods of tracheal intubation. We reviewed our experience in the endoscopic treatment of these lesions. PATIENTS AND METHODS: We performed a retrospective review of patients who presented acquired tracheal-subglottic stenosis (SGS) treated at our center from 2005 to 2012. We reviewed the etiology, age, clinical presentation, methods of diagnosis, number of bronchoscopies, angioplasty balloon dilations performed, and long-term results. RESULTS: A total of 18 patients (13 M, 5 F) were treated at our institution between 2005 and 2012. Median age at treatment was 3.5 months (range, 1-96 months). Of the 18 children, 16 children had SGS (all cases were postintubation), and 2 children presented tracheal stenosis (1 postintubation, 1 after tracheal surgery). Median intubation time was 30 days (range, 3-120 days). Extubation failure and stridor were the main clinical features. SGS were diagnosed as grade I in three patients, grade II in nine patients, and grade III in six patients. Bronchoscopy allowed diagnostic in all cases, and was followed by angioplasty balloon dilation, with a median of 2.5 (range, 1-5) sessions. In SGS grade I, the relation patient/number of dilations was 1; in SGS grade II 2.6, and in SGS grade III 3.5. Mitomycin was applied in 15 patients. No patients presented intraoperative complications or required reoperation. Median follow-up time was 36 months (range, 5-72 months) and no recurrence was noticed. CONCLUSIONS: Early endoscopic dilation with balloon shows as an effective and safe treatment in acquired tracheal and SGS.

Ganglioneuroma: to operate or not to operate.

INTRODUCTION: Ganglioneuroma (GN) is a benign, differentiated variety of neurogenic tumor. It is often asymptomatic and may be diagnosed by serendipity. Surgical removal is the treatment of choice. However, it has been suggested that postoperative complications and sequelae might outweigh the benefits of this approach. The purpose of the present study was to examine these issues in a large experience of neural tumors. METHODS: Patients treated between 1992 and 2012 were retrospectively reviewed. Modern imaging, measurement of catecholamine metabolite excretion and metaiodobenzylguanidine were used for workup. Surgical treatment aimed at complete resection. Complications and sequelae were recorded. Literature was searched for regrowth or malignant transformation of GN. RESULTS: Of 227 patients with neural tumors, 24 were GN patients (12 abdominal, 11 thoracic and 1 cervical with 8 dumbbell extensions). Six children were symptomatic (three with abdominal pain and mass, one with stridor or dysphonia, and one each with anisocoria and opsomyoclonus). However, 18 (75%) were asymptomatic and the diagnosis was incidental. Several tumors were large and involved more than one body space. There were no neurologic symptoms in eight cases with dumbbell extension. Complete resection was achieved in 20 children (83%) whereas gross residual was left in four. Postoperative complications were: Horner syndrome (3 patients), mild scoliosis (1 patient), adhesive bowel obstruction (1 patient) and acute urinary retention (1 patient). There was no evidence of either regrowth or malignant behavior in residual masses left in place after follow-up of 84 (1-194) months. CONCLUSIONS: There were a limited number of general minor complications in this series that did not include cases of regrowth or malignant transformation. However, these unfavorable events were occasionally reported in the literature. Since diagnosis of GN cannot be ascertained before removal of the mass, this should remain the aim of the treatment, although limiting the chances of complications to a minimum even if incomplete resection is the price to pay. Nonoperative attitudes should not be recommended in all cases, but they are certainly justified in some.

Failed Nissen fundoplication in children: causes and management.

INTRODUCTION: Nissen fundoplication (NF) is the gold standard procedure for the treatment of gastroesophageal reflux (GER) in children. However, it fails in a significant proportion of patients. The purpose of this study was to identify preoperative predictors of failure and to examine the results of reoperations. PATIENTS AND METHODS: The charts of patients who underwent NF at our institution between 1992 and 2011 were retrospectively reviewed. Surgery was indicated in patients with symptomatic GER in whom medical treatment failed, particularly, in cases of esophageal atresia (EA), congenital diaphragmatic hernia (CDH), and neurologic impairment (NI). Chi-square comparisons and logistic regression were used to test comorbidities, previous abdominal surgery, surgical technique, gastrostomy, pyloromyotomy or pyloroplasty, age, weight, and surgical complications as possible predictors of NF failure. RESULTS: A total of 360 children (217 male and 143 female) underwent NF. Comorbidities were NI (n = 100, 27.8%), EA (n = 50, 13.9%), CDH (n = 22, 6.1%), and abdominal wall defects (AWD) (n = 6, 1.7%). A total of 35 patients (9.7%) had esophageal stenosis. Age at surgery was 3.06 years (0.04-20.7 years) and weight was 12 kg (2-77 kg). NF was open in 196 patients (54.4%) and laparoscopic in 164 patients (45.6%) (with 9 conversions). Follow-up was 6.7 years (0.01-18.7 years). A total of 42 patients (11, 7%) had postoperative complications (10 wound infection, 9 dumping syndrome, 8 gastrostomy related complications, 7 intestinal obstruction, 5 evisceration, 2 chylothorax, and 1 pneumothorax). Reflux recurred in 42 patients (11.7%) and 35 patients (9.7%) underwent redo NF 1.01 years (0.02-8.4 years) after the initial surgery. A total of nine patients (2.5%) required further interventions (five another redo NF, three esophageal replacements, and one esophago-gastric disconnection). A total of 29 patients (8.1%) died during the follow-up (25 because of their baseline disease, 3 in the postoperative period, and 1 because of pulmonary aspiration 3 years after surgery). EA (31.6% failure) and CDH (46.7% failure) were the only comorbidities predictive of NF failure (p < 0.05). CONCLUSIONS: Failure of NF is particularly frequent in patients previously operated upon for EA or CDH and can be predicted preoperatively. However, the benefits of the operation may outweigh this risk. Redo NF is indicated if symptoms of GER recur, but the proportion of failure is even higher. In subsequent failures, other options like esophageal replacement or esophagogastric dissociation should also be considered.

Pyloroduodenal duplication cysts: treatment of 11 cases.

INTRODUCTION: Abdominal enteric duplications are found in 1 out of 4,500 autopsies, and only 4 to 5% of them are located in the duodenum, where they may be connected with the biliary or pancreatic ducts. The aim of this study was to describe the clinical features, management, and outcome of a large series of duodenal duplication cysts. MATERIALS AND METHODS: The charts of all patients treated at our institution between 1985 and 2011 were reviewed retrospectively with particular attention to imaging, surgical technique, pathology, and outcome. RESULTS: During that period, 11 cases (81.8% females) were treated. Out of the 11 patients, 8 were symptomatic (vomiting in 3, recurrent acute pancreatitis in 2, and abdominal pain in 3) and 3 were tentatively diagnosed prenatally as choledochal cysts. Median age at surgery was 2.3 years (0 to 13.7) and preoperative diagnosis was correct in five cases. Five cysts were developed intraluminally and three communicated with the biliary duct (one), pancreatic duct (one), or both (one). Surgical treatment consisted of complete resection (four cases, including one pancreaticoduodenectomy), partial removal including all mucosa (four cases), and internal marsupialization (three cases). In all cases, the ductal communications were divided and opened into the duodenal lumen. In six cases, ectopic gastric mucosa was found. All patients recovered uneventfully. CONCLUSION: Duodenal duplication cysts are rare and may have bizarre anatomical patterns due to biliopancreatic involvement. Optimal treatment is complete surgical removal, and, if this is not possible, partial removal including the mucosa or marsupialization are also good alternatives. In cases with biliary and pancreatic tract connections, these have to be taken down carefully and drained into the duodenum.