Introduction/Aim: The aim of the study was to determine the frequency of various histopathological findings in biopsies of children with intestinal hypomotility and the incidence and characteristics of Hirschsprung disease (HD). Methods: Biopsies of colon and rectum taken due to intestinal hypomotility and chronic constipation at the Department of Pediatric Surgery of the University Children's Hospital in Belgrade over the 10-year period (from 2009 to 2018) were reviewed using pathology reports from the archive of the Institute of pathology, Faculty of Medicine, University of Belgrade. Results: A total of 287 patients with intestinal motility disorder were identified, with 554 biopsy samples. Of the total number of patients, 56% (161/287) were without any morphological changes in enteric nervous system (ENS). The most common histopathological findings were HD (69/287; 24%) and immaturity of ganglion cells (29/287; 10%). Isolated hypoganglionosis of ENS was found in 5 (2%) cases. Heterotopia of ganglion cells was the only finding in 8 (3%) cases. Rare causes of intestinal dysmotility were: eosinophilic proctitis/colitis (EPC) (4/287), neuronal intestinal dysplasia B (2/287), unclassified disganglionoses (3/287). Rectosigmoid variant of HD was the most frequent HD variant (80.3%). Acetylcholinesterase method and immunohistochemical staining were used in 19.5% cases. Conclusions: HD and immaturity of ganglion cells were the most common pathological findings in ENS of constipated children. It is important to differentiate EPC from other lesions of enteric plexuses due to different natural history and therapy.

Introduction. Empyema thoracis, defined as the accumulation of pus in the pleural space, is rare in the neonatal population. Limited data are reported in the medical literature, and still, no treatment guidelines are available for this age. Case report. We present a term 12-day-old neonate (born healthy) who developed sepsis caused by methicillin-resistant Staphylococcus aureus (MRSA) and pneumonia associated with advanced-stage empyema. The child was admitted to our hospital with a few-hours history of difficulty breathing and lethargy. On admission, the child was cyanotic with desaturation and in severe respiratory distress; therefore, the child was intubated, and mechanical ventilation was started. Imaging tests were performed in an emergency, hence chest computed tomography (CT) scan was done without contrast. Suspected congenital pulmonary airway malformation with trapped air collections, significant mediastinal shift on CT scan, and deterioration of the patient?s condition indicated urgent surgery. Intraoperatively, the diagnosis of stage II empyema was established, and decortication of thickened parietal and visceral pleura was performed. Afterward, the baby showed quick and progressive clinical improvement. Conclusion. The diagnosis and management of empyema in neonates may be challenging, especially in the case of unremarkable history, fulminant progression of the disease, and incomplete imaging tests.

Background/Objectives: Long-gap esophageal atresia (LGEA) is most commonly associated with Gross types A and B, which are usually diagnosed preoperatively, but may also be encountered in Gross type C, where the long gap is recognized intraoperatively, during fistula ligation. This study aimed to describe and explore outcomes associated with two native esophagus-preserving procedures, delayed primary anastomosis (DPA) and the Foker procedure (FP), and to assess their respective roles in the management of LGEA. Methods: We analyzed a population-representative cohort of 38 patients with LGEA treated at two tertiary centers in Belgrade between 2003 and 2023. Patients treated with DPA (n = 20) or FP (n = 18) were evaluated regarding Gross type, gap length, gastrostomy, treatment duration, complications, major adverse outcomes (including esophageal replacement, redo surgery, or death), and hospital stay. Results: Gross types A and B were analyzed together and were more frequently treated with DPA, whereas FP predominated in type C (p = 0.001). Sex, gestational age, birth weight, and gap length did not differ between treatment groups. Gastrostomy was performed in all DPA cases and in 50% of FP cases. No statistically significant differences in complication rates or major adverse outcomes were observed between the DPA and FP groups. Hospital stay was significantly longer in the DPA group (p < 0.001), although this may be partially influenced by temporal bias related to the later introduction of FP. Conclusions: FP may offer practical advantages in type C LGEA, where thoracic access is already required for fistula ligation, whereas DPA may be a suitable approach in types A/B, avoiding an additional thoracic procedure. These findings suggest that anatomical subtype may be an important consideration in treatment selection in LGEA, although validation in larger multicenter prospective studies is warranted.

Introduction. Waugh syndrome (WS) is a rare condition characterized by association of intussusception and gut malrotation. Due to non-operativne management for intussusception in certain number of patients, it still remains undiagnosed. So far, only less than hundred casses have been reported in literature, most of them treated surgicaly, with good outcome and no complication if surgery is done timely after symptoms appeared. Case presentation. We present a five month old infant which was admited due to vomiting, blood and mucus in stool. Clinical findings revealed abdomen which was painful on palpation, without distension and abdominal mass. Blood and mucus were noted on rectal examination. After native X ray, abdominal ultrasound was done and confirmed intussusception. After unsuccessful attempt of hydrostatic reduction, ultrasound was repeated, the target sign persisted, this time accompanied by obvious “whirlpool sign” and inverse ratio of superior mesentery artery and vein. Infant underwent emergency surgery, and bowel resection with ileo-colic T-T anastomosis. Recovery was complete after two weeks. Conclusion. Waugh syndrome should be considered whenever hydrostatic reduction fails in a patient with previously diagnosed intussusception, and ultrasound verification of the superior mesenteric artery and vein relationship should be insisted upon to confirm the diagnosis off associated malrotation. In proven Waugh syndrome, timely surgical intervention is indicated to prevent bowel resection.

Introduction. Biliary atresia (BA) is an idiopathic, progressive obliterative cholangiopathy of unknown etiology. The incidence of BA is 5?10 cases per 100,000 live births. Two clinical phenotypes of BA have been described: syndromic and non-syndromic form. From 1959, Kasai procedure is a standard therapeutic procedure. However, patients in whom there is an association of BA with other structural anomalies may have a worse outcome after the procedure. The goal was to present our patient with unusual form of BA associated with intestinal malrotation. Case report. We present a two-month-old female infant hospitalized because BA was suspected. On the echosonographic examination of the abdomen the gallbladder was not visible. Intraoperative diagnosis of BA was confirmed, and the Kasai procedure was performed. During the operation, intestinal malrotation with Ladd?s bands was identified. In this case, after the complete Ladd procedure, we decided to trace the Roux coil through the mesoduodenum and then behind the duodenum towards the portal plate, where a portoenterostomy was then created in a standard way. During the ?follow-up? the infant was free of complaints, the stools were normally discolored, and the values of liver function tests had a downward trend. Conclusion. Any doubt about the diagnosis of BA obliges us to determine the existence of all other possible anatomical abnormalities and associated anomalies, due to their potential importance in changing treatment plans and surgical approach, but also the impact on the outcome of treatment.

Diagnosing Hirschsprung disease (HD) and related disorders can be complex and demands a deep understanding of the mechanisms governing intestinal motility, which involves the enteric nervous system (ENS), interstitial cells of Cajal (ICCs), and the muscle layers of the intestine. The London classification identifies three groups of gastrointestinal neuromuscular disorders: neuropathies, myopathies, and ICC abnormalities. Hirschsprung disease, characterized by the absence of ganglion cells, is the most common intestinal neuropathy and it results from the impaired migration of neural crest cells during development. It affects about 1 in 5,000 live births and involves several genetic factors, notably the RET gene. HD typically affects the rectum and a part of the colon, with varying extents of aganglionosis. The diagnosis is based on the histopathological analysis of suction biopsies, the absence of ganglion cells, and the presence of thick submucosal nerves on a standard hematoxylin and eosin stain, supplemented by enzyme histochemistry (acetylcholinesterase method) or immunohistochemical methods (calretinin and other antibodies) staining. The treatment for HD involves surgical resection of affected bowel segments. Accurate intraoperative assessment of tissue margins is critical to preventing postoperative complications related to pseudoobstruction. Communication between surgeons and pathologists is essential to ensure successful treatment outcomes. Other intestinal neuropathies include intestinal hypoganglionosis, hyperganglionosis, delayed maturation of ganglion cells, and gliopathies. Enteric myopathies are exceptionally rare conditions, with typical morphological changes such as atrophy of the muscularis propria, intracellular vacuolization of smooth muscle cells, and interstitial fibrosis. Disruption in ICC network and arrangement forms the morphological basis of slow transit constipation. Each of aforementioned disorders has unique characteristics and diagnostic challenges. Understanding and diagnosing these conditions often require a combination of histological, histochemical, immunohistochemical, and sometimes genetic analyses. The integration of these techniques is vital for accurate diagnosis and effective treatment planning. In summary, the complexity of intestinal dysmotility disorders necessitates a thorough understanding of intestinal motility mechanisms and the utilization of advanced diagnostic methods to provide accurate diagnoses and effective treatments.