ABSTRACT
The aim of this study was to compare the virtually planned position to the postoperative position of the maxilla, having performed the maxilla-first sequence or mandible-first sequence orthognathic surgery. An audit of 64 patients who underwent bimaxillary surgery between 2017 and 2020 was performed. Thirty patients had maxilla-first surgery and 34 had mandible-first surgery. The planned and post-surgical positions were analyzed using specific skeletal landmarks. Differences were calculated and the two-sample t-test was used to compare the groups. Measured differences between the planned and postoperative results differed significantly between the mandible-first and maxillary-first surgery groups (P < 0.001). The maxillary central incisors were under-advanced in the anterior-posterior direction in both groups. Most data points showed deviation from the surgical plan ≤ 2 mm and ≤ 4°. Secondarily, maxillary under-advancement in the mandible-first cohort was evaluated; these patients were subdivided into rigid and non-rigid fixation groups. The non-rigid fixation group showed less accuracy compared to the rigid fixation group, which was statistically significant (P = 0.014). The findings of this study demonstrate that virtual surgical planning can be less accurate in predicting the maxillary incisor position when performing mandible-first surgery, but this inaccuracy is within the acceptable range and can be mitigated by rigid fixation of the mandible.
ABSTRACT
OBJECTIVE: We report a novel presentation of childhood cerebral X-linked adrenoleukodystrophy: status epilepticus followed by abrupt and catastrophic neurologic deterioration. METHODS: A description of the clinical presentation, laboratory evaluation, and imaging findings leading to a diagnosis of X-linked adrenoleukodystrophy. RESULTS: A 3-year-old male with prior history of autism presented with fever, diarrhea, and status epilepticus requiring a pentobarbital coma. Admission labs were notable only for a glucose level of 22 mg/dL, which stabilized after correction. The child never returned to his prior neurologic baseline, with complete loss of gross motor, fine motor, and speech skills. Serial brain magnetic resonance imaging (MRI)/magnetic resonance spectroscopy (MRS) was notable for progressive diffuse cortical signal changes with swelling, diffusion restriction, and ultimately laminar necrosis. Nine months after presentation, CSF (cerebrospinal fluid) protein and MRS lactate were persistently elevated, concerning for a neurodegenerative disorder. This led to testing for mitochondrial disease, followed by lysosomal and peroxisomal disorders. Very long-chain fatty acids were elevated. Identification of a pathogenic ABCD1 mutation confirmed the diagnosis of X-linked adrenoleukodystrophy. CONCLUSIONS: Boys with childhood cerebral X-linked adrenoleukodystrophy typically present with gradual behavioral changes. Rare reports of boys presenting with transient altered mental status or status epilepticus describe a recovery to their pre-presentation baseline. To our knowledge this is the first X-ALD patient to present with status epilepticus with abrupt and catastrophic loss of neurologic function. X-linked adrenoleukodystrophy should be suspected in young males presenting with seizures, acute decline in neurologic function, with persistently elevated CSF protein and MRS lactate.
ABSTRACT
We treated 34 chemotherapy-naive patients with stage IIIb or IV non-small cell lung cancer with trimetrexate 150-200 mg/m2 intravenously over 30 minutes every two weeks. Six of 31 evaluable patients (19%) achieved a partial response. The major toxic effects from this regimen were myelosuppression, nausea/vomiting, and skin rash. We conclude that this well-tolerated schedule of trimetrexate has significant activity as a single agent against non-small cell lung cancer.