Interstitial Cells of Cajal and P2X3 Receptors at Ureteropelvic Junction Obstruction and Their Relationship with Pain Response.

Introduction: Etiopathogenesis and the symptomatology of ureteropelvic junction obstruction (UPJO) in the pediatric population has not yet been definitely clarified, suggesting a multifactorial nature of the condition. The aim was to analyze the association between the number of Interstitial Cells of Cajal (ICCs), as well as P2X3 receptors in ureteropelvic junction (UPJ) and the pain response in pediatric patients with hydronephrosis. Methods: 50 patients with congenital hydronephrosis underwent open or laparoscopic pyeloplasty at one of two departments of pediatric surgery and urology in Poland. Patients were divided into two groups according to the pain symptoms before surgery. A total of 50 samples of UPJ were obtained intraoperatively and underwent histopathological and immunohistochemical (IHC) analysis. Quantitative assessment of ICCs was based on the number of CD117(+) cells of adequate morphology in the subepithelial layer and the muscularis propria. Expression of P2X3 receptors was evaluated as the intensity of IHC staining. Results: Patients with hydronephrosis and accompanying pain were on average 60 months older (77 vs. 17 months) than children with asymptomatic hydronephrosis (p = 0.017). Symptomatic children revealed higher numbers of ICCs in both the subepithelial layer and in the lamina muscularis propria. In particular, symptomatic patients aged 2 years or more exhibited significantly higher numbers of ICCs in the subepithelial layer. Significant differences in the distribution of ICCs between the subepithelial layer and the lamina muscularis propria were observed in both groups. Expression of P2X3 receptors was limited to the urothelium and the muscle layer and correlated between these structures. There was no relationship between pain response and the expression of P2X3 receptors. Conclusions: ICCs and P2X3 receptors may participate in the pathogenesis of UPJO and in the modulation of pain response to a dilatation of the pyelocaliceal system. Explanation of the role of ICCs and P2X3 receptors in propagation of ureteral peristaltic wave and the modulation of pain stimuli requires further studies.

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.

Thoracoscopic Stage Internal Traction Repair Reduces Time to Achieve Esophageal Continuity in Long Gap Esophageal Atresia.

OBJECTIVE: Management of long gap esophageal atresia (LGOA) is controversial. This study aims at comparing the management of LGOA between two high-volume centers. METHODS: We included patients with LGOA (type A and B) between 2008 and 2022. Demographics, surgical methods, and outcomes were collected and compared. RESULTS: The study population involved 28 patients in center A and 24 patients in center B. A surgical approach was thoracoscopic in center A, only for one patient was open for final procedure. In center B, 3 patients were treated only thoracoscopically, 2 converted to open, and 19 as open surgery. In center A primary esophageal anastomosis concerned 1 case, two-staged esophageal lengthening using external traction 1 patient, and 26 were treated with the multistaged internal traction technique. In 24 patients a full anastomosis was achieved: in 23 patients only the internal traction technique was used, while 1 patient required open Collis-Nissen procedure as final management. In center B primary anastomosis was performed in 7 patients, delayed esophageal anastomosis in 8 patients, esophageal lengthening using external traction in 1 case, and 9 infants required esophageal replacement with gastric tube. Analyzed postoperative complications included: early mortality, 2/28 due to accompanied malformations (center A) and 0/24 (center B); anastomotic leakage, 4/26 (center A) treated conservatively-all patients had a contrast study-and 0/24 (center B), 1 case of pleural effusion, but no routine contrast study; recurrent strictures, 13/26 (center A) and 7/15 (center B); and need for fundoplication, 5/26 (center A) and 2/15 (center B). Age at esophageal continuity was as a median of 31 days in center A and 110 days in center B. Median time between initial procedure and esophageal anastomosis was 11 days in center A and 92 days in center B. CONCLUSION: Thoracoscopic internal traction technique reduces time to achieve esophageal continuity and the need for esophageal substitution while maintaining a similar early complication rate.

Preserved Voluntary Micturition Control despite Early Urinary Diversion in Infancy-A Clue to a New Strategy.

Micturition is an involuntary process based on spinal arcs in infants and children until a defined age. The awareness and voluntary control of voiding depends on specific areas in the central nervous system, especially cortical regions. The cells and connections between these areas develop over time and regulate the voiding process. The ability to maintain continence and to adjust physiological needs to appropriate environmental conditions is considered to be acquired through systematic behavioral education, especially toilet training. The recommendations specify the age at which to start establishing the relevant habits. The purpose of these guidelines is to achieve proper micturition control development and to avoid functional lower urinary tract (LUT) disorders. We present a case of a patient who underwent complete urinary diversion in infancy and reconstruction of the urinary tract eleven years later. For eleven years, she had an empty bladder and no toilet training. After undiversion, she regained full continence in a short space of time. The presence of proper LUT function and a controlled micturition process raises the question of the standard toilet training recommendations' validity. The aim of our work focuses on the following question: Is toilet training the only way to achieve micturition skills and proper urinary tract function? The history of our patient and the literature reveal that voluntary micturition may develop without stimulating signals of filling from bladder receptors and independently of recommended behavioral education, so toilet training seems to not be necessary.

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.

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.

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.