The journal focuses on neuroimmunology and neuroinflammation, and the coverage extends to other basic and clinical studies related to neuroscience including molecular biology, psychology, pathology, physiology, endocrinology, pharmacology, oncology, etc.

(1) Background: The aim of the present study was to evaluate our institutional outcome in tuberculum sellae meningioma (TSM) patients treated microsurgically using multiple skull base approaches, including a transcranial approach and an extended endonasal transsphenoidal approach. (2) Materials and Methods: This is a retrospective study that includes 34 patients with TSM. The study aimed to observe the efficacy of the different common approaches used by a single neurosurgeon. All the patients were evaluated preoperatively and during follow-up with campimetry, head CT scan, and post-contrast MRI. (3) Results: After a transcranial approach, visual acuity improved in 86.20%, was stable in 10.34%, and deteriorated in 3.45%. Through transsphenoidal surgery, vision improved in 80%, was static in 20%, and deteriorated in 0%. Transcranial approaches included pterional, mini-bifrontal basal, and supraciliary keyhole microscopic craniotomies. Gross total removal was performed in 58.82%, near total in 10.34%, and partial removal in 3.45%. The transcranial/supraciliary keyhole endoscopic-assisted approach showed a gross total removal rate of 80%, and near total in 20%. The transsphenoidal approach showed a gross total removal rate of 60%, near total in 20%, and partial removal in 20%. (4) Conclusion: Endoscopic-assisted keyhole supraciliary mini craniotomy for resection of tuberculum sellae meningioma offers low morbidity and good visual outcome. The endonasal route is preferred for the removal of TSM when they are small and midline placed. The major limitation of this approach is a narrow surgical corridor and the restriction on midline-placed lesions. Gross total removal was better achieved with mini-bifrontal basal and pterional craniotomies.

Frontonasal dysplasia type-2(FND2), a rare phenotypically variable and heterogeneous developmental anomaly resulting from mutation of the ALX4 gene, is primarily characterized by malformation of the skull and facial skeleton. This study was designed to showcase a clinical, imaging, and genetic analysis of FND2 in a consanguineous family of Bangladeshi origin. Clinical imaging and whole genome sequencing of mother, father and patient was done by using Nextera DNA flex library preparation kit (Illumina, USA) using Novaseq 6000 next generation sequencer to find out ALX4 mutation which causes FND2 in patient. We report the clinical as well as molecular findings in an 8-year-old girl with FND2. The child presented with various characteristic features of skull and facial anomalies associated with FND 2 along with numerous other features many of which have not been described in previous literature. The parents also showed some key clinical, radiological, and genetic features of FND 2. The whole genome sequencing (WGS) revealed homozygosity for a 793C-T transition in the ALX4 gene, which resulted in premature termination at codon 265 (p.Arg265Ter). Both of her parents were heterozygous carriers of ALX4 mutation. This is the first report that associates clinical, imaging, and genomics analyses in a Bangladeshi patient for better understanding the disease FND2. These results will facilitate diagnosis and genetic counseling of the future FND2 patients.

Background and Objectives: Juvenile nasopharyngeal angiofibroma (JNA) is an angiomatous hamartoma of the nasal cavity. It is a benign but locally aggressive vascular tumor of the nasopharynx affecting adolescent males. Many surgical procedures are in practice, but the extended endonasal endoscopic (EEE) approach for JNAs is a suitable and effective technique. Materials and Methods: Fifteen adolescent patients having JNA who underwent extended endonasal endoscopic (EEE) surgery from January 2010 to January 2022 were studied retrospectively. Patients having residual and recurrent JNAs and those who underwent surgery other than EEE were excluded. Results: The average age of the patients was 18.3 years of age. A total of six patients (40%) each had stage V and IV while three patients (20%) had stage III JNAs. Gross total removal was achieved in eight (53.3%) patients and seven (43.7%) had partial removal. There was no per or postoperative mortality. All the patients had at least 3 years of postoperative follow-up and during follow-ups, seven patients were found to have residual tumors, and two had recurrences. Discussion: During the last decades, the endoscopic approach for the resection of JNAs has gained increasing popularity due to its obvious advantages over transfacial approaches. The magnified and angled field of view “behind the corner” helping in a more complete inspection for the resection and shorter hospitalization time makes it a better choice than the other approaches. Conclusions: Endoscopy is an excellent approach for primary JNA. It allows well visualization and precise removal of the angiofibroma. An endoscopic multiangle, multicorridor skull base approach including Denker’s anteromedial maxillotomy is suitable and preferable for the resection of extensive JNAs.

DOI: http://dx.doi.org/10.3329/bmrcb.v39i1.15810Bangladesh Med Res Counc Bull 2013; 39: 45-46