Congenital hernia of the cord is a different type of ventral abdominal wall defect in which the bowel usually herniates into the base of normally inserted umbilical cord through a patent umbilical ring. It is rare congenital anomaly with incidence of 1 in 5000. Although it was described as a distinct entity since 1920s it is often misdiagnosed as a small omphalocele. We present an unusal case of term male newborn with umbilical cord hernia associated with patent omphalomesenteric duct. The diagnose was made after birth despite antenatal ultrasound scans and it is managed successfully with uneventful recovery. If this is missdiagnosed, it could cause iatrogenic atresia of the ileum by clamping the umbilical cord after birth.
Background/Aim. Hirschsprung?s disease is the most common identifiable developmental disorder of the enteric nervous system, characterized by a failure of its formation in a variable segment of distal bowel. Currently available surgical therapies for Hirschsprung?s disease, although lifesaving, are associated with numerous complications. The aim of our study was to evaluate the effectiveness of different surgical methods and the incidence of serious complications after radical surgery of rectosigmoid Hirschsprung?s disease. Methods. A retrospective analysis, from June 1997 until May 2012 was carried out on 84 patients operated for Hirschsprung?s disease of rectosigmoid colon. Transanal endorectal pull-through was performed in 30 (35.7%) patients (group I), while 54 (64.3%) patients were operated by other (Soave, Duhamel or Swenson) procedures (group II). The age at operation, the incidence and severity of postoperative complications, the need for previous colostomy and the number of reoperations are countered in order to evaluate the efficacy of surgical procedures. Results. In the group I, the mean age at operation was 9.41 ? 6.37 months and in the group II the mean age at operation was 16.8 ? 13.9 months which was significantly higher (p < 0.01). In the group I there were only 3 (10%) patients with complications, one (3%) of them was prone to only one redo procedure (1.00 ? 0.00) and there was no need for previous colostomy in all patients (100%). In the group II there were 16 (30%) patients with significantly frequent complications (p < 0.05), about 2 reoperations on the average (1.94 ? 1.84) in 4 of them (25%) and 22 (41%) redo procedures, which was, in total, significantly higher than in the group I (p < 0.01). Only Soave?s procedure was performed without previous colostomy in 20 (37%) patients. Conclusion. Transanal endorectal pull-through in surgical treatment of patients with Hirschsprung?s disease is more effective than other procedures concerning earlier onset, low incidence and less severe complications, which would require further operations, and no scars.
Thoracoschisis is a rare condition. A female newborn presented with right-sided thoracoschisis, associated with diaphragmatic hernia and protrusion of an accessory liver lobe through the chest wall defect along with deformity of the right forearm and hand duplication. Diagnosed as part of the limb-body wall complex (LBWC), management included resection of the exteriorized liver lobe followed by right hemidiaphragm and thoracic wall reconstruction.
Background and Aim: Atresia is the most common congenital anomaly of the esophagus, with an increased risk of complications after surgical correction. The aim of our study was to evaluate the risk factors associated with early and late postoperative complications in neonatal patients with esophageal atresia. Methods: The study sample comprised 109 neonatal patients aged between 0 and 27 days of life who were prenatally diagnosed with esophageal atresia or diagnosed at birth. For the purpose of this study, neonatal and perinatal factors and factors associated with the mother’s medical condition were analyzed. Complications after surgical intervention were classified as early and late. Results: Patients with early postoperative complications experienced significantly more frequent complications during delivery (p = 0.002), asphyxia (p = 0.038), and postoperative sepsis (p = 0.045) and were more likely to have received medicamentous therapy (p = 0.035). Patients with late postoperative complications had significantly more frequent complications during delivery (p = 0.025), respiratory distress (p = 0.043), and postoperative sepsis (p = 0.010), were more likely to have received preoperative mechanical ventilation (p = 0.014), and showed a significantly different frequency distribution among the different classes of the Spitz classification (p = 0.008). A risk factor for early postoperative complications in patients with atresia in the upper part was complications during delivery (OR-3.09; p = 0.007). The risk factors for late postoperative complications for patients with upper atresia were preoperative mechanical ventilation (OR: 2.77; p = 0.041), postoperative sepsis (OR: 2.60; p = 0.028), and belonging to relatively high- and high-risk groups according to the Spitz classification (OR: 3.50; p = 0.022). Conclusions: In neonates who have undergone surgical intervention for esophageal atresia, a risk factor for early postoperative complications is complications during delivery, while the risk factors for late postoperative complications are preoperative mechanical ventilation, postoperative sepsis, and belonging to relatively high- and high-risk groups according to the Spitz classification. Therefore, a multidisciplinary approach and continuous monitoring are essential to reduce morbidity and mortality, as well as to improve quality of life, in these patients.
Background: Recognized as one of the most serious musculoskeletal deformities, occurring in 1–2 per 1000 newborns, 80% of clubfeet are idiopathic while 20% present with associated malformations. The etiopathogenesis of clubfoot is described as multifactorial, including both genetic and environmental risk factors. The aim of this study was to analyze possible genetic causes of isolated and syndromic clubfoot in Serbian children, as well as to correlate clinical and genetic characteristics that would provide insight into clubfoot etiopathogenesis and possibly contribute to global knowledge about clinical features of different genetically defined disorders. Methods: We evaluated 50 randomly selected, eligible children with clubfoot aged 3 to 16 years that were initially hospitalized and treated at University Children’s Hospital between November 2006 and November 2022. The tested parameters were gender, age, dominant foot, affected foot, degree of deformity, treatment, neuromuscular disorders, positive family history, and maternal smoking. According to the presence of defined genetic mutation/s by whole exome sequencing (WES), patients were separated into two groups: positive (with genetic mutation/s) and negative (without genetic mutation/s). Results: Seven patients were found to be positive, i.e., with genetic mutation/s. A statistically significant difference between categorical variables was found for families with a history of clubfoot, where more than half (57.14%) of patients with confirmed genetic mutation/s also had a family history of genetic mutation/s (p = 0.023). Conclusions: The results from this study further expand the genetic epidemiology of clubfoot. This study contributes to the establishment of genetic diagnostic strategies in pediatric patients with this condition, which can lead to more efficient genetic diagnosis.