Almost 23 years have passed since the first thoracoscopic procedure was done. However, according to the recent study in USA, only 16% of newborns with oesophageal atresia and distal tracheooesophageal fistula are managed by thoracoscopic approach with a very high 53% conversion rate. The aim of presentation based on experience of one paediatric surgery centre is to prove that thoracoscopic approach for this malformation is a powerful tool with a potential to change the current results. It requires considerable experience that comes only from the high number of operated cases. The technique is one amongst the many others means which taken together may really help to improve the results. Thus, the question of centralisation for special rare congenital malformations comes back to mind. Is it rational to operate on only 2 or 3 cases a year in a single centre, or is it the time for centralisation and close co-operation, as has been done with biliary atresia treatment in the UK? LEVEL OF EVIDENCE: III.

Thoracic musculoskeletal deformities are significant complications following open correction of esophageal atresia (EA) during long-term follow-up. We aimed to compare the frequency and severity of thoracic musculoskeletal deformities after open and thoracoscopic repair of EA. We hypothesized that fewer deformities would occur following the less invasive thoracoscopic approach. This retrospective study analyzed patients treated at two pediatric surgery departments in Poland between 2005 and 2021. The patient groups differed in surgical approach, operative techniques, indications for multi-staged surgery, and postoperative complications. The study encompassed all types of EA/TEF. The first group comprised 68 patients who underwent thoracoscopic esophageal atresia repair (Wroclaw), while the second group involved 44 patients who underwent open repair (Warsaw). Clinical data were retrospectively reviewed, with results considered significant at p < 0.05. The median age at examination was 6 years in the thoracoscopy group and 5.5 years in the thoracotomy group. In the thoracoscopy group, 53 out of 68 patients (77.9%) and in the thoracotomy group - 35 out of 44 patients (79.5%) were treated in one stage. The incidence of thoracic musculoskeletal deformities was significantly lower in the thoracoscopy group (1.5%) compared to the thoracotomy group (34.1%, p < 0.001). Scoliosis occurred significantly more often after thoracotomy (13.6% vs 1.5%, p = 0.016). There was no rib fusion (0% vs 37.1%, p < 0.001) and no scoliosis of =>20° (0% vs 6.8%, p = 0.058) after thoracoscopy. The coincidence of rib fusion and scoliosis was significant (9.1%, p = 0.022) for the open approach. In the thoracotomy group, multi-staged surgery and more frequent reoperations due to major complications were significantly associated with an increased occurrence of deformities. None of the patients after thoracoscopic multi-stage or complicated EA/TEF repair developed scoliosis. The frequency and severity of thoracic musculoskeletal deformities were significantly lower after the thoracoscopic approach. Thoracoscopy may be a more advantageous and preferred surgical approach for the EA/TEF treatment, although further randomized, controlled studies are necessary. Post-thoracotomy scoliosis may progress to a severity requiring surgery.

Aim: This study reports the reliability and validity of the Polish version of the Esophageal Atresia Quality of Life (EA-QOL) questionnaires, which were originally developed in Sweden and Germany. Methods: A total of 50 families of children (23 aged 2 to 7, and 27 aged 8 to 17) with EA/TEF (esophageal atresia/tracheoesophageal fistula) participated in the study. The development and validation of the Polish version of the EA-QOL involved forward-backward translation of the survey items following the guidelines for cross-cultural translation, cognitive debriefing and evaluation of psychometric properties, including assessment of internal and retest reliability, linguistic validity, content validity, known-group validity and convergent validity. The medical records of patients and standardized questionnaires were used to obtain clinical data. The level of significance was p < 0.05. Results: The Polish versions of the EA-QOL questionnaires demonstrated strong linguistic and content validity, are slightly discriminative for esophageal and respiratory problems, but do not show convergent validity with the PedsQL 4.0 generic core scales. In terms of reliability, the internal consistency of the subscale and total scale of Polish versions as measured by Cronbach’s alpha is good, and retest reliability is excellent. Conclusions: The Polish versions of the EA-QOL questionnaires meet most psychometric criteria that confirm the EA-QOL questionnaires’ reliability and validity. This study enables application of these questionnaires in future research among children with EA in Poland and participation in international multicenter studies focusing on advancing knowledge of condition-specific QOL in this population. Future cross-cultural research using larger sample sizes is still needed to better address the relationship between condition-specific and generic QOL, as well as the discriminative ability of the EA-QOL questionnaires.

Background: This study aimed to analyze the results, feasibility and safety of the thoracoscopic approach for patients with esophageal atresia with tracheoesophageal fistula (EA/TEF) depending on the patient’s birth weight. Methods: The study involved only type C and D EA/TEF. Among the analyzed parameters were the patients’ characteristics, surgical treatment and post-operative complications: early mortality, anastomosis leakage, anastomosis strictures, chylothorax, TEF recurrence, and the need for fundoplication or gastrostomy. Results: 145 consecutive newborns underwent thoracoscopic EA with TEF repair. They were divided into three groups—A (N = 12 with a birth weight < 1500 g), B (N = 23 with a birth weight ≥ 1500 g but < 2000 g), and C—control group (N = 110 with a birth weight ≥ 2000 g). Primary one-stage anastomosis was performed in 11/12 (91.7%) patients—group A, 19/23 (82.6%)—group B and 110 (100%)—group C. Early mortality was 3/12 (25%)—group A, 2/23 (8.7%)—group B, and 2/110 (1.8%)—group C and was not directly related to the surgical repair. There were no significant differences in operative time and the following complications: anastomotic leakage, recurrent TEF, esophageal strictures, and chylothorax. There were no conversions to an open surgery. Fundoplication was required in 0%—group A, 4/21 (19.0%)—group B, and 2/108 (1.9%)—group C survivors. Gastrostomy was performed in 1/9 (11.1%)—group A, 3/21 (14.3%)—group B and 0%—group C. Conclusion: In an experienced surgeon’s hands, even in the smallest newborns, the thoracoscopic approach may be safe, feasible, and worthy of consideration. Birth weight seems to be not a direct contraindication to the thoracoscopic approach.

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. 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. 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. 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. 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.

Congenital chloride diarrhea (CCD) is caused by a recessive mutation in the SLC26A3 gene and characterized mainly by watery diarrhea, hypochloremia and metabolic alkalosis. Various different mutations in SLC26A3 are responsible for the disease. In the prenatal period, the symptoms of CCD may include polyhydramnios, preterm labor and abdominal distension. The main feature of CCD is chloride-rich diarrhea, which leads to excessive loss of fluid and salt immediately after birth and is followed by weight loss and dehydration. Hyponatremia and hypochloremia are soon accompanied by hypokalemia and metabolic alkalosis. Untreated CCD is fatal even in the first weeks of life. Diagnosis is made by high fecal chloride concentrations in patients with serum electrolytes corrected by salt substitution and confirmed using genetic testing of peripheral blood samples. Here, we detail prenatal and postnatal manifestations of a preterm infant, born via Caesarian section, who was suspected to suffer intrauterine bowel obstruction. Upper median laparotomy was performed and no intestinal abnormalities found. The course of the neonatal period was complicated by severe diarrhea with hypochloremia, hyponatremia and metabolic alkalosis. Based on the patient's clinical picture and stool examination, a diagnosis of CCD was established. Mutation of the SLC26A3 gene was confirmed using genetic testing.