Spinal kord lipomları, pediatrik beyin cerrahisi alanının en zorlayıcı lezyonları arasındadır. Spinal lipomların doğal seyirleri ve tedavileri tartışmalıdır. Bu çalışmada spinal kord lipomu nedeni ile kliniğimizde opere edilen hastaların klinik, demografik verileri ve tedavi sonuçlarının değerlendirilmesi amaçlanmıştır. Elli dokuz hastanın verileri retrospektif olarak değerlendirilmiştir. Hastaların yaş ortalaması 10±5,8 (1-25) ay, en sık başvuru şikayeti bel bölgesindeki şişlik idi. Hastalarımızın başvuru anında 36’sında nörolojik defisit varken 23’ünde nörolojik defisit yoktu. Olgularda postopertif dönemde nörolojik kötüleşme izlenmedi. Hastalar ortalama 39,9± 32,9 (1-132) ay takip edildi. 13 (%22) olgu takiplerinde nörolojik kötüleşme olması nedeni ile yeniden opere edildi. Asemptomatik olsalar dahi uygun zamanda uygulanan cerrahi müdahale, bu lezyonlara sahip çocuklarda gelişebilecek nörolojik bozulmayı önleyebilir. Çok merkezli prospektif çalışmalar bu konu hakkında daha rasyonel bilgilere ulaşmamızı sağlayacaktır.

A bstract: Ventriculoperitoneal shunt surgery is the primary treatment for hydrocephalus; however, while common complications include infection and dysfunction, shunt-related acute abdomen syndrome due to catheter knotting is exceedingly rare, with fewer than 30 cases reported in the literature. We present a 9-year-old male with congenital hydrocephalus and a history of neonatal surgery for esophageal atresia and Nissen fundoplication who, despite having a stable neurological course for 8 years, suffered from multiple hospitalizations due to nonspecific gastrointestinal symptoms. He eventually presented with acute nausea, vomiting, and abdominal distension, where radiography revealed distal shunt knotting and acute ileus. Emergency laparotomy confirmed a small bowel volvulus caused by the knotted catheter, and following externalization, the patient was successfully treated with a ventriculoatrial shunt. Based on our clinical observations and literature review, we identified that knotting occurs at intestinal “corner points” where chronic peristaltic movements force redundant catheter lengths to rotate and eventually self-entangle. To overcome the limitations of 2D imaging and improve diagnostic accuracy, we emphasize the potential of developing three-dimensional computed tomography reconstruction techniques, similar to those used in aneurysm surgery, to better visualize the spatial orientation of the catheter. We conclude that managing distal catheter length and maintaining a high clinical suspicion regarding the “neurologically silent phase” are the cornerstones of preventing such life-threatening complications, particularly in patients with complex abdominal histories.

Background: Hutchinson–Gilford progeria syndrome (HGPS) is an exceedingly rare genetic disorder characterized by accelerated aging and profound vascular fragility. While cerebrovascular accidents are common in this population, traumatic epidural hematomas (EDH) are clinical rarities, with only two pediatric cases previously documented. Currently, it is estimated that fewer than 200 children with HGPS are living worldwide. The primary objective of this report is to present a comprehensive multidisciplinary management strategy for traumatic EDH in a 15-year-old HGPS patient, highlighting the critical interplay between progerin-induced vascular stiffening, reduced intracranial compliance, and the formidable anesthetic challenges encountered in the oldest reported survivor of this pathology. Case presentation: A 15-year-old female with HGPS presented with a right frontoparietal EDH following a ground-level fall. Initial Glasgow Coma Scale (GCS) was 14 but declined to 12 within 4 h. Cranial computed tomography (CT) revealed a large EDH measuring 22 mm at maximum thickness, causing a 6 mm midline shift. Due to severe glottic sclerosis and anatomical distortion, multiple orotracheal and fiberoptic (2.8 mm) intubation attempts failed. An emergency percutaneous tracheostomy was performed to secure the airway. Intraoperative findings revealed active bleeding from a coronal suture diastasis, which was managed with bone wax and dural tenting sutures. The patient achieved full neurological recovery and was discharged on postoperative day 10. Conclusion: Managing HGPS patients requires a high index of clinical suspicion for intracranial injury even after minor trauma, as progerin-induced vasculopathy accelerates hematoma expansion. Standard airway techniques can fail due to progressive laryngeal changes, necessitating early consideration of surgical airways. Prompt multidisciplinary intervention and meticulous neurosurgical technique are essential to mitigate risks associated with extreme vascular fragility and complex anatomy in this high-risk population.

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