Thoracic Musculoskeletal Deformities Following Surgical Treatment of Esophageal Atresia - Thoracoscopic Versus Open Approach: A Retrospective Two Centers Cohort Study.

INTRODUCTION: 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. METHODS: 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. RESULTS: 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. CONCLUSIONS: 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.

Benefits of the Erector Spinae Plane Block before Cryoanalgesia in Children Undergoing Surgery for Funnel Chest Deformity.

Thoracic surgery causes significant pain despite standard multimodal analgesia. Intraoperative cryoanalgesia may be a solution. The onset of the clinical effect of cryoanalgesia can take 12-36 h. The addition of a regional anaesthesia before the cryoanalgesia procedure can enable analgesic protection for the patient during this period. The main aim of the study was to evaluate the benefits of the erector spinae plane (ESP) block prior to Nuss surgery. The 'control' group consisted of 10 teenagers who underwent cryoablation together with intravenous multimodal analgesia according to the standard protocol. The 'intervention' group included 26 teenage patients who additionally received an erector spinae plane block before operation. Pain relief (p = 0.015), opioid use (p = 0.009), independent physical activity and rehabilitation (p = 0.020) were faster in the intervention group. No features of local anaesthetic drug toxicity or complications of the ESP block were observed. The bilateral ESP block together with intraoperative intercostal nerve cryoablation performed prior to Nuss correction of funnel chest were more effective in terms of pain control.

Laparoscopic ureteroneocystostomy in the treatment of urinary incontinence due to ectopy of the ureters in female dogs: A pilot study.

Ureteral ectopia is rare and requires surgical treatment after a thorough diagnostic workup. Open surgical techniques for repositioning ectopic ureters have been known for many years and are well described in the literature. However, to the best of our knowledge, no laparoscopic method of correcting this pathology has been described, which, in our opinion, would benefit the animal in terms of the healing process and overall clinical outcomes. This study aimed to evaluate the possibility of laparoscopic treatment of ureteral ectopia, which causes urinary incontinence in dogs. All of the operated ten dogs presented in this study were client-owned females with symptoms of urinary incontinence due to a unilateral intramural ectopic ureter. A three-trocar laparoscopic technique was used to perform the ureteroneocystostomy of the ectopic ureter. In this article, clinicopathological data, imaging features, procedural findings, complications, and short- and long-term outcomes are presented. The procedure was feasible in all cases. No major postoperative complications were observed. Among the minor complications, slight hematuria was observed in three dogs, which resolved spontaneously. In the period of at least one year after surgery, no negative impact of the procedure was observed. Seven of the ten operated dogs regained urinary continence. The remaining three dogs required additional surgery (urethral bulking) because of a lack of improvement after adjuvant pharmacological treatment. Overall, good-to-excellent long-term outcomes can be achieved; however, dogs that remain incontinent after laparoscopic ureteroneocystostomy may require additional treatment.

Epigenetic Findings in Twins with Esophageal Atresia.

Esophageal atresia (EA) is the most common malformation of the upper gastrointestinal tract. The estimated incidence of EA is 1 in 3500 births. EA is more frequently observed in boys and in twins. The exact cause of isolated EA remains unknown; a multifactorial etiology, including epigenetic gene expression modifications, is considered. The study included six pairs of twins (three pairs of monozygotic twins and three pairs of dizygotic twins) in which one child was born with EA as an isolated defect, while the other twin was healthy. DNA samples were obtained from the blood and esophageal tissue of the child with EA as well as from the blood of the healthy twin. The reduced representation bisulfite sequencing (RRBS) technique was employed for a whole-genome methylation analysis. The analyses focused on comparing the CpG island methylation profiles between patients with EA and their healthy siblings. Hypermethylation in the promoters of 219 genes and hypomethylation in the promoters of 78 genes were observed. A pathway enrichment analysis revealed the statistically significant differences in methylation profile of 10 hypermethylated genes in the Rho GTPase pathway, previously undescribed in the field of EA (ARHGAP36, ARHGAP4, ARHGAP6, ARHGEF6, ARHGEF9, FGD1, GDI1, MCF2, OCRL, and STARD8).

Reliability and Validity of the Polish Version of the Esophageal-Atresia-Quality-of-Life Questionnaires to Assess Condition-Specific Quality of Life in Children and Adolescents Born with Esophageal Atresia.

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.

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.

Ingrowing Liver as Atypical Recurrent Diaphragmatic Hernia Presentation-Diagnostic and Treatment Difficulties: A Case Report.

(1) Introduction: Recurrent diaphragmatic hernia is a relevant diagnostic and treatment dilemma. We have presented a patient with ingrowing liver as an atypical diaphragmatic hernia recurrence and discussed major aspects of diagnostic methods and the selection of an appropriate operative treatment. (2) Case description: We discuss a case of a patient with right-sided recurrent CDH (Congenital Diaphragmatic Hernia) who had primary thoracoscopic repair in newborn period. During infancy and early childhood, the patient presented recurrent upper and lower respiratory tract infections and bronchial hyperreactivity. The clinical picture was initially unclear. A CT scan was inconclusive to diagnose a recurrence. The patient was scheduled to have a re-thoracoscopy. A part of the liver was herniated into the pleural cavity. This fragment of 'ingrowing' liver was removed, and the diaphragmatic secondary defect was repaired. (3) Conclusions: This case proved that thoracoscopy can be a preferred technique in the diagnosis and treatment of CDH recurrence.

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.

Staged Thoracoscopic Repair of Long-Gap Esophageal Atresia Without Temporary Gastrostomy.

Introduction: Primary repair of long-gap esophageal atresia (LGEA) continues to present a surgical challenge. The number of treatment options, including the use of native esophagus or esophageal replacement, shows the difficulty in the treatment and lack of one superior option. Until recently all children with LGEA required temporary gastrostomy before esophageal reconstruction. Objective: The aim of the study is to present new therapeutic management in patients with LGEA, involving staged thoracoscopic procedure with internal traction without gastrostomy. Materials and Methods: Four neonates with LGEA were treated in two academic Departments of Pediatric Surgery in Poland from 2015 to 2018. The intervention was staged thoracoscopic approach, consisting in internal traction, followed by delayed esophageal anastomosis in neonatal period. The outcome measures were successful anastomosis, short time anastomosis complications, and timing of gastric/oral feeding. Results: Internal traction was placed between 2 and 6 days of life. A successful repair of native esophagus by the second approach in neonatal period without gastrostomy was achieved in 3 out of 4 neonates, 5-8 days after internal traction placement. No anastomotic leakage was observed, two anastomoses developed stricture managed with three and four sessions of repeated dilatation. Nasogastric tube feeding started between 5 and 7 days with full oral feeding achieved between 10 and 35 days after anastomosis. Follow-up was after 1-34 months. Conclusions: Our technique should be considered an alternative first-line strategy for the repair of LGEA without the use of a gastrostomy.

Neonatal gastric outlet obstruction by isolated pyloric atresia, an often forgotten diagnosis.

BACKGROUND: Pyloric atresia (PA) is a rare condition, and may be misdiagnosed and especially confused for duodenal atresia pre-operatively. We looked for clues to avoiding pre-operative misdiagnosis and hence allow the best neonatal medical and surgical management. METHODS: A retrospective case-note review was carried out of the five patients managed in four centres with the diagnosis of isolated PA. We focused on antenatal ultrasound findings, postnatal clinical and radiological features, operative findings, surgical procedures and outcomes. RESULTS: Four patients had polyhydramnios and one double bubble sign on antenatal ultrasound. After birth, non-bilious vomiting and upper abdominal distension were the main symptoms. Gastric decompression showed non-bilious gastric fluid. Radiological findings were a large gastric air bubble with no gas beyond in all cases. The diagnosis of duodenal atresia was postulated at first in all cases. The diagnosis of PA was established peroperatively. One patient referred late, died 13-day post-operatively of cardiopulmonary failure secondary to a severe pneumonia that may be related to aspiration syndrome. Outcomes were otherwise satisfactory. CONCLUSIONS: Even though it is a rare diagnosis, PA has a specific clinical and radiological presentation underlined here that should be kept in mind when managing a neonate with a gastric outlet obstruction.

Thyroidectomy in children: changing trends and surgical strategies.

BACKGROUND: Surgical thyroid pathology is an uncommon problem in children and due to environmental factors may be locally-specific. Until the mid-90's, Lower Silesia had been regarded as a region of endemic goiter but since then a better system of iodine prophylaxis has been introduced. OBJECTIVES: To assess changing trends in the epidemiology of surgical thyroid diseases in children and to examine whether they have influenced potential alterations of operative strategies during the study period. MATERIAL AND METHODS: The medical records of all children operated on for thyroid disease between 1993 and 2010 in the university pediatric surgical center were retrospectively reviewed. The data regarding the indications for thyroidectomy, details of surgical management and outcome were collected and analyzed in three periods of time 1993-1998, 1999-2004 and 2005-2010. RESULTS: There were 46, 63 and 41 children operated on in the analyzed periods of time, respectively, with no significant differences regarding their age and gender. Preoperative thyroid ultrasound scan showed nodular lesions within the thyroid gland in 145 children (94.7%). The frequency of unilateral and bilateral nodular lesions was basically similar throughout the whole study. There has been a significant decrease of subtotal resections from around 63% in the first period to less than 16% in the other two periods of the study. The reverse trend can be clearly seen with regard to unilateral total lobectomy. Since 1999, total lobectomy or total thyroidectomy has been performed in more than 80% of the children. Nodular goiter was the most common indication for surgical operation followed by follicular adenoma. Malignant disease was recorded in 7 children (4.6%). CONCLUSIONS: The gradual decrease of the number of children with surgical pathology of the thyroid gland seems to reflect an effective iodine prophylaxis. Depending on the extent of thyroid disease, unilateral lobectomy, either alone or coupled with partial or total resection of the contralateral lobe should be a standard surgical procedure in children.

Preduodenal portal vein, malrotation, and high jejunal atresia: a case report.

Preduodenal portal vein (PDPV) is a rare congenital anomaly. In most patients, it is associated with other congenital defects including situs inversus, malrotation, and biliary atresia or occurs as part of the heterotaxia syndrome or polysplenia syndrome. We describe a newborn affected by high jejunal atresia, malrotation, and a complex cardiac anomaly, in whom PDPV was diagnosed at early relaparotomy because of stenosis of the jejunal anastomosis. Occurrence of PDPV with intestinal atresia has not been previously reported in the literature.

Pyelonephritis xanthogranulomatosa in a 7-year-old girl.

Xanthogranulomatous pyelonephritis (XGPN) is rare form of chronic, usually unilateral, renal infection that involves damage to the renal glomeruli and periglomerular tissue and destruction of the renal parenchyma. Nephrectomy is usually necessary and the prognosis is good if XGPN is unilateral and treated early. XGPN typically affects middle-aged females and is extremely rare in children. We report one case of XGPN in a seven-year-old girl who was operated on at our institution be-cause of lower urinary tract dysfunction and renal insufficiency - the left kidney was removed and the bladder was augmented by means of the remaining ureter. The histopathological evaluation of the removed kidney showed chronic XGPN.