Prevalence and course of disease after lung resection in primary ciliary dyskinesia: a cohort & nested case-control study.

BACKGROUND: Lung resection is a controversial and understudied therapeutic modality in Primary Ciliary Dyskinesia (PCD). We assessed the prevalence of lung resection in PCD across countries and compared disease course in lobectomised and non-lobectomised patients. METHODS: In the international iPCD cohort, we identified lobectomised and non-lobectomised age and sex-matched PCD patients and compared their characteristics, lung function and BMI cross-sectionally and longitudinally. RESULTS: Among 2896 patients in the iPCD cohort, 163 from 20 centers (15 countries) underwent lung resection (5.6%). Among adult patients, prevalence of lung resection was 8.9%, demonstrating wide variation among countries. Compared to the rest of the iPCD cohort, lobectomised patients were more often females, older at diagnosis, and more often had situs solitus. In about half of the cases (45.6%) lung resection was performed before presentation to specialized PCD centers for diagnostic work-up. Compared to controls (n = 197), lobectomised patients had lower FVC z-scores (- 2.41 vs - 1.35, p = 0.0001) and FEV1 z-scores (- 2.79 vs - 1.99, p = 0.003) at their first post-lung resection assessment. After surgery, lung function continued to decline at a faster rate in lobectomised patients compared to controls (FVC z-score slope: - 0.037/year Vs - 0.009/year, p = 0.047 and FEV1 z-score slope: - 0.052/year Vs - 0.033/year, p = 0.235), although difference did not reach statistical significance for FEV1. Within cases, females and patients with multiple lobe resections had lower lung function. CONCLUSIONS: Prevalence of lung resection in PCD varies widely between countries, is often performed before PCD diagnosis and overall is more frequent in patients with delayed diagnosis. After lung resection, compared to controls most lobectomised patients have poorer and continuing decline of lung function despite lung resection. Further studies benefiting from prospective data collection are needed to confirm these findings.

Decompressive craniectomy in cerebral venous sinus thrombosis during pregnancy: a case report.

Pregnancy and puerperium are risk factors for cerebral venous sinus thrombosis (CVST), a condition which nowadays is treated non-operatively. Decompressive craniectomy is reserved only for emergency settings. We present a 22-year-old pregnant lady, who was transferred at the emergency department with a reduced level of consciousness, headache, and nuchal rigidity. Her MRI study showed CVST, causing hemorrhagic infarct and midline shift. She underwent decompressive craniectomy with partial removal of the hemorrhagic parenchyma. Remarkably, she recovered without any neurological deficits regardless of the substantial temporal lobe damage, while the thrombus nearly resolved using anticoagulation. Decompressive craniectomy can be life-saving in selected CVST patients, followed by anticoagulantion to augment the recanalization process.

When age is not an obstacle: A case series of endoscopic transsphenoidal resection of pituitary macroadenomas in older patients.

Due to the increase in life expectancy, the number of elderly patients suffering from a pituitary macroadenoma is expected to increase in the future. The endoscopic endonasal transsphenoidal (EET) approach tends to be the first choice for the treatment of pituitary macroadenomas in the general population. Notwithstanding, in the geriatric population, the goals of management for this condition remain unclear. The present study retrospectively evaluated and describes the cases of 6 patients >70 years of age with a pituitary macroadenoma who were treated by a skull base team, composed of one ENT surgeon and one neurosurgeon. All the patients experienced a notable improvement in their neurological deficit, while their hormonal status also improved or at least did not deteriorate after the surgery. The EET approach appears to be a safe and effective approach for the treatment of pituitary macroadenomas in the geriatric population.

Altered Motor Excitability in Patients With Diffuse Gliomas Involving Motor Eloquent Areas: The Impact of Tumor Grading.

BACKGROUND: Diffuse gliomas have an increased biological aggressiveness across the World Health Organization (WHO) grading system. The implications of glioma grading on the primary motor cortex (M1)-corticospinal tract (CST) excitability is unknown. OBJECTIVE: To assess the excitability of the motor pathway with navigated transcranial magnetic stimulation (nTMS). METHODS: Retrospective cohort study of patients admitted for surgery with diffuse gliomas within motor eloquent areas. Demographic, clinical, and nTMS-related variables were collected. The Cortical Excitability Score (CES 0 to 2 according to the number of abnormal interhemispheric resting motor threshold (RMT) ratios) was calculated for patients where bilateral upper and lower limb mapping was performed. RESULTS: A total of 45 patients were included: 9 patients had a low-grade glioma and 36 patients had a high-grade glioma. The unadjusted analysis revealed an increase in the latency of the motor evoked potential of the lower limb with an increase of the WHO grade (P = .038). The adjusted analysis confirmed this finding (P = .013) and showed a relation between the increase in the WHO and a decreased RMT (P = .037) of the motor evoked responses in the lower limb. When CES was calculated, an increase in the score was related with an increase in the WHO grade (unadjusted analysis-P = .0001; adjusted analysis-P = .001) and in isocitrate dehydrogenase (IDH) wild-type tumors (unadjusted analysis-P = .020). CONCLUSION: An increase in the WHO grading system and IDH wild-type tumors are associated with an abnormal excitability of the motor eloquent areas in patients with diffuse gliomas.