Novel Left-Sided Thoracoscopic Approach to Recurrent Tracheoesophageal Fistula and Post-Fistula Tracheal Diverticula.

BACKGROUND: Recurrent tracheoesophageal fistula (RTEF) is usually a consequence of leakage or other complications after esophageal atresia repair performed through right-sided access. This results in extensive intrapleural adhesions, and open redo surgery poses a challenge. Alternatively, endoscopic endotracheal fistula obliteration usually requires repetitive procedures, and its success rate varies significantly between centers. We present a novel approach to recurrent fistulas. The innovation is in reaching the fistula through the virgin field via left-sided three-port thoracoscopy instead of classical right-sided thoracotomy. METHODS: This is a presentation of a new operative technique based on a retrospective case series of patients operated on at our department between 2016 and 2023. RESULTS: Eight patients after esophageal atresia repair (six with RTEF and two with post-fistula tracheal diverticula) were successfully treated with left-sided thoracoscopy. There were no conversions. One patient required rethoracoscopy for chylothorax. Another one, after RTEF closure, underwent multiple endoscopic obliterations of subsequent tracheal diverticulum. No other major complications nor re-recurrences were noted. CONCLUSIONS: Left-sided thoracoscopy in redo esophageal atresia has the advantage of a "virgin" operative field and grants feasible access to the RTEF or tracheal diverticulum. We believe that this approach is worth further exploration because it combines minimal invasiveness with high effectiveness without all the consequences of a thoracotomy.

Biomechanics of esophageal elongation with traction sutures on experimental animal model.

Esophageal elongation is one of the methods of long gap esophageal atresia treatment. The aim of the study was to determine the best type of traction suture for esophageal lengthening on an animal model. White Pekin Duck's esophagi were used as a model (fresh-frozen and thawed). The esophagus was cut in half, then both ends were sutured together and extended on a tensiometer. Tested sutures involved simple suture, suture aided by a single or double clip, and suture aided by pledget (10 samples each). Constant and 2 methods of intermittent traction were also compared. The histological study showed similarities between duck's and newborn's esophagus. The highest maximal force was achieved with pledget suture (F = 8.59 N ± 1.45 N), then with double clip (F = 5.74 N ± 1.29 N) and the lowest with single suture (F = 3.80 N ± 0.54 N) (p < 0.001). Pledget suture also allowed for the greatest elongation (p < 0.01). Intermittent traction results in better elongation at the same breaking strength as constant traction (p < 0.05) if traction is maintained during breaks. Reinforced sutures (pledget or double clip) should be taken into consideration in internal traction. When performing traction sutures, it is worth step by step carefully tightening the sliding knot in short periods before its final binding.

Occurrence of Esophageal Atresia With Tracheoesophageal Fistula in Siblings From Three-Generation Family Affected by Variable Expressivity MYCN Mutation: A Case Report.

The MYCN oncogene encodes a transcription factor belonging to the MYC family. It is primarily expressed in normal developing embryos and is thought to be critical in brain and other neural development. Loss-of-function variants resulting in haploinsufficiency of MYCN, which encodes a protein with a basic helix-loop-helix domain causes Feingold syndrome (OMIM 164280, ORPHA 391641). We present an occurrence of esophageal atresia (EA) with tracheoesophageal fistula in siblings from a three-generation family affected by variable expressivity of MYCN mutation p.(Ser90GlnfsTer176) as a diagnostic effect of searching the cause of familial esophageal atresia using NGS-based whole-exome sequencing (WES). All of our affected patients showed microcephaly and toe syndactyly, which were frequently reported in the literature. Just one patient exhibited clinodactyly. None of the patients exhibited brachymesophalangy or hypoplastic thumbs. The latest report noted that patients with EA and Feingold syndrome were also those with the more complex and severe phenotype. However, following a thorough review of the present literature, the same association was not found, which is also confirmed by the case we described. The variable phenotypic expression of the patients we described and the data from the literature guide a careful differential diagnosis of Feingold syndrome even in cases of poorly expressed and non-specific symptoms.

Thoracoscopic repair of congenital isolated H-type tracheoesophageal fistula.

BACKGROUND/PURPOSE: The available literature on congenital isolated H-type tracheoesophageal fistula (TEF) is limited, and preferred approach varies among centers (cervicotomy, thoracotomy, thoracoscopy). We aimed to present one of the biggest case series of thoracoscopic approach for congenital isolated TEF and to assess the method's feasibility and outcomes. METHODS: Retrospective review of thoracoscopic TEF repair experiences at 2 European university pediatric surgery centers. RESULTS: 9 boys and 3 girls were involved in the study (age 5 days-4 years), 8 of them were newborns (mean birth weight 3013 g, mean gestational age 39 weeks). The most common presenting symptoms were desaturations on feeding in neonates and recurrent respiratory tract infections in older children. The diagnoses were established on contrast study and confirmed with rigid bronchoscopy. The fistulas were located at or below the thoracic inlet; the fistulas were 2 cm above the carina to half the height of the trachea. All patients underwent successful thoracoscopic TEF repair. There were no conversions. The postoperative course was uneventful in all but one who had rethoracoscopy for prolonged postoperative chylothorax. All patients had satisfactory vocal cord function. One patient required reoperation for fistula recurrence 8 months after primary surgery. CONCLUSION: Thoracoscopic approach for isolated H-type TEF seems to be possible as a procedure of choice with satisfactory results and all benefits of minimally invasive procedure. LEVEL OF EVIDENCE: IV (case series).

Current management of pediatric appendicitis: A Central European survey.

BACKGROUND: Appendicitis is one of the most common diagnoses in pediatric populations. Although new recommendations for the treatment of pediatric appendicitis were published, management varies among different institutions. OBJECTIVES: To determine current practices in 4 (n = 4) representative pediatric surgical departments in Central Europe. MATERIAL AND METHODS: One department from each of the 4 countries was surveyed using an online questionnaire. Questions focused on preoperative, operative and postoperative practices in 2018, particularly those related to antibiotic (ATB) therapy and laparoscopy. RESULTS: A total of 519 appendectomies were performed, among which 413 (79.6%) were laparoscopic appendectomies (LAs), with a conversion rate of 5.1%. Appendectomy, as an elective procedure, was performed in 43 (8.3%) patients. One-quarter (129 patients) had complex appendicitis and 72.3% of these were operated laparoscopically. In 3 departments, ATB prophylaxis was administered, based on the decisions of the operating surgeon. One department used standard ATB prophylaxis (metronidazole). Whenever phlegmonous appendicitis was detected, ATB were administered therapeutically in 2 departments. Two other departments administered ATB based on surgeon decision. The choice of ATB was not standardized. If complex appendicitis was detected, all sites administered ATB therapeutically. The type of ATB treatment was standardized in complex cases in 2 departments. Thirty-four complications (6.6%) at surgical sites were recorded - 4.1% (16/390) after uncomplicated and 14% (18/129) after complex appendicitis. Thirty-two occurred after acute surgeries and 26 of these followed laparoscopic procedures. Postoperatively, intra-abdominal abscesses occurred in 3.5% of laparoscopic and in 2.9% of open appendectomy (OA) cases. CONCLUSIONS: This questionnaire study showed that treatment outcomes for appendicitis in children in Central Europe are comparable with data reported in the literature. Laparoscopic appendectomy is the predominant surgical method, but there is a little consensus for ATB treatment in the management of appendicitis at our 4 pediatric surgical departments.

Laparoscopic versus open pyeloplasty in children: experience of 226 cases at one centre.

INTRODUCTION: The aim of the study was to compare the efficacy of laparoscopic versus open dismembered pyeloplasty in children. MATERIAL AND METHODS: Two hundred and twenty-six Anderson-Hynes pyeloplasties were performed, out of which 131 by open access (OP) and 95 by laparoscopic access (LP). Retrospective analysis of data was performed. The median follow-up was 3 years for LP patients and 6 years for OP patients (p < 0.05). RESULTS: Success was achieved in 87 (91.57%) patients who had LP surgeries and in 121 (91.7%) patients who had OP (p > 0.05). Eight patients in the LP group and nine in the OP group required another surgery because of recurrent UPJO, and one patient in the OP group required a nephrectomy. The median operating time was 125 min (range: 70-225) for LP surgeries and 90 (40-200) for OP surgeries (p < 0.05). In the last 30 LP procedures, operation time decreased to a median of 95 min. Improvement in ultrasound analysis of the kidney was achieved in 89.06% of patients who had LP and 82.35% of patients who had OP. A stable or better function of the kidney in diuretic renography was achieved in 87.5% of patients in the LP group and 96.15% of patients in the OP group. CONCLUSIONS: Laparoscopic and open pyeloplasty is a highly efficient procedure employed to treat UPJO in children with comparable success rates in both groups. In experienced hands, it is possible to reduce the LP operation time to that comparable to the OP group.

Rare Course of Bilateral Congenital Diaphragmatic Hernia Treated Thoracoscopically-Case Report.

We present a rare case of metachronous bilateral congenital diaphragmatic hernia (CDH) in a newborn with additional malformations: macroglossia, pectus excavatum, and confirmed Simpson-Golabi-Behmel syndrome. We performed a successful thoracoscopic subsequent repair with a patch of the bilateral type C CDH. Despite using insufflation that should cause bilateral pneumothorax at first procedure there was no clinical signs of such a one. Contralateral CDH diagnosis was possible only on the basis of the symptoms that occurred after the first operation. After 13 months the patient is without recurrence. In selected patients, staged thoracoscopy may be a safe and feasible method of bilateral CDH treatment.

Detection rate of crossing vessels in pediatric hydronephrosis: Transperitoneal laparoscopy versus open lumbotomy.

BACKGROUND: A bundle of crossing vessels (CV) supplying the lower pole of the kidney and causing mechanical obstruction of the ureteropelvic junction (UPJ) has been the subject of many discussions. During pyeloplasty, it is possible to overlook the CV. This may result in recurrent dilatation of the kidney and the need for re-surgery. OBJECTIVES: To compare the detection rate of CV in UPJ obstruction (UPJO) depending on the operational access applied (transperitoneal laparoscopy (LAP) vs open lumbotomy (OPEN)). Assessment of features that could indicate the presence of CV. MATERIAL AND METHODS: Two hundred and forty-six pediatric pyeloplasties were performed between January 2006 and July 2017 in the Department of Pediatric Surgery and Urology at the Wroclaw Medical University, Poland - 111 out of them by LAP and 135 by OPEN, on 98 girls and 148 boys. A retrospective analysis of the patient records for the detection of CV and characteristics of the CV before surgery was performed. RESULTS: Intraoperative CV causing obstruction of the UPJ in the LAP group were recognized in 34.2% (n = 38) of the patients, and within the OPEN group in 12.5% (n = 17) (p < 0.0001); 90% (n = 27) of patients with the diagnosed CV did not show congenital hydronephrosis. In 68% (n = 21) of the patients there were cases of recurrent renal colic. The presence of CV was suspected in 7.2% of kidney ultrasounds and in 12.5% in computed tomography (CT) urograms. CONCLUSIONS: The detection rate of CV in UPJO is statistically higher in LAP access than in open retroperitoneal lumbotomy. The distinguishing features of patients with CV are the lack of prenatal diagnosis for hydronephrosis and the presence of pain in the lumbar region.

Hydronephrosis in Children Caused by Lower Pole Crossing Vessels-How to Choose the Proper Method of Treatment?

Objectives: Assessment of the efficacy of intraoperative diagnosis between extrinsic and intrinsic UPJO in children. Assessment of the efficacy of laparoscopic vascular-hitch procedure in UPJO caused by lower pole crossing vessels (CV). Materials and Methods: Between 2008 and 2017, 47 laparoscopic procedures were performed with the CV discovered intraoperatively. CV were translocated cephalad, and the UPJ was carefully inspected. The Chapman's vascular hitch procedure was accomplished in the case of decreasing sizes of the pelvis and clear, visible peristalsis of the UPJ (31 patients). In the other cases, Anderson-Hynes (A-H) pyeloplasty with posterior translocation of the CV was performed (16 patients). Results: The median age at operation was 6 years (range 1-16) in VH and 6 years (range 2-17) in A-H (p = 0.4635). Prenatal dilatation of kidney was diagnosed in 18.7% of VH and 10% of A-H cases (p = 0.5474). Success was achieved in 16 (100%) patients in the A-H and in 29 (93.54%) in the VH groups. Two patients (6.5%) in VH required repeated surgery because of a misdiagnosed intrinsic obstruction. Median operation time in VH was 80 min (range 40-105) and was 105 (range 70-225) in A-H (p < 0.05). Conclusions: The intraoperative selection based on intraoperative pelvis and UPJ appearance after vessel transposition is sufficient in majority of cases. Laparoscopic vascular hitch seems to be effective and safe procedure, but can only be performed on carefully selected patients. In case of misdiagnosis, reoperation is possible with the same laparoscopic access.