Chiari type III malformation associated with Klippel-Feil syndrome, a case report with a narrative review of the literature.

BACKGROUND: Chiari malformation type III (CM III), a rare hindbrain anomaly, often presents with various concurrent anomalies. This paper reports a unique case of CM III associated with Klippel-Feil syndrome (KFS), a condition previously unreported in Saudi Arabia and documented in only one other case globally in Turkey. This study aims to share insights into the unusual association between CM III and KFS, considering their close embryological development and involvement in the craniocervical junction. METHODOLOGY: The study presents a case of a 2.5-year-old female diagnosed with CM III and KFS. Diagnostic tools such as ultrasound, CT scans, MRI, and physical examinations were used to confirm the patient's condition. Surgical interventions, including decompression and encephalocele repair, were performed. RESULTS: Successful surgical interventions, including encephalocele repair and duraplasty, were carried out. Follow-up visits indicated a stable condition, marked improvement in lower limb strength, and the patient's ability to walk with assistance. CT follow-up affirmed a satisfactory surgical outcome. CONCLUSION: This case study illustrates the potential for an optimistic prognosis in CM III, even when accompanied by complex conditions such as KFS, through early diagnosis and intervention. It underscores the significance of antenatal screening for effective care planning and calls for further research and publications due to the rarity of this association. These findings contribute to our understanding of CM III and its related conditions, emphasizing the need for open-minded consideration of potential embryological associations.

Non-traumatic bilateral epidural hematoma in a child with sickle cell anemia: A case report and a review of the literature.

OBJECTIVE: To report a rare case of spontaneous bilateral epidural hematoma (EDH) in a 10-year-old Nigerian child with sickle cell disease (SCD) and review the literature regarding this unusual complication. METHODS: We present a case of a pediatric patient with SCD who developed a spontaneous bilateral EDH and discuss the potential underlying mechanisms, management approaches, and outcomes. We also conducted a literature review of existing cases of spontaneous EDH in patients with SCD. RESULTS: Our patient initially presented with a subgaleal hematoma and underlying bilateral EDH, but she was sent home without any neurosurgical consultation. Two years later, she returned with altered consciousness and left-sided weakness, revealing an increased size of the EDH with a noticeable mass effect. She underwent a successful emergency bilateral craniotomy, with noticeable improvement in her level of consciousness and left-sided weakness post-operatively. In our literature review, we found 40 reported cases of spontaneous EDH in SCD patients, with a male predominance (82.5%). The average age of patients was 15.282 years. The most common hematoma location was bifrontal (20%) and the most reported symptom was headache (47.5%). Most patients (97.5%) were already known cases of SCD. Among those treated, 77.5% survived. CONCLUSION: Spontaneous bilateral EDH in SCD patients is an uncommon complication, with a variety of proposed pathophysiological mechanisms. Prompt recognition and appropriate management, either conservative or surgical, are crucial to improve outcomes. Our case and literature review underscore the importance of considering spontaneous EDH in SCD patients presenting with neurological symptoms, even in the absence of trauma. Further research is needed to elucidate the precise etiology, identify risk factors, and optimize management approaches for this rare complication in SCD patients.