Postnatal Intracranial Findings Following Fetal Repair of Spinal Dysraphisms.

OBJECTIVE: Our objective is to document the imaging appearance in the intracranial compartment at the time of the infants' first postnatal brain MR imaging after fetal repair for spinal dysraphisms. METHODS: Twenty-nine patients were evaluated on fetal and postnatal magnetic resonance imaging for a series of features of Chiari II malformation. RESULTS: Of the 29 infants, 55% had resolution of tonsillar ectopia, and 62% showed a dorsal outpouching of the near the foramen magnum on postnatal magnetic resonance imaging. The majority had persistence of Chiari II features including: prominent massa intermedia (93%), tectal beaking (93%), towering cerebellum (55%), flattening of the fourth ventricle (90%), hypoplastic tentorium (97%), and tonsillar hypoplasia (59%). CONCLUSIONS: Normally positioned or minimally descended, oftentimes hypoplastic tonsils in the presence of a posterior fossa configuration typical of Chiari II, was the most common presentation. An additional documented feature was an outpouching of the dorsal thecal sac between the opisthion and the posterior arch of C1.

Immune checkpoint inhibitor related hypophysitis: diagnostic criteria and recovery patterns.

Data on the diagnosis, natural course and management of immune checkpoint inhibitor (ICI)-related hypophysitis (irH) are limited. We propose this study to validate the diagnostic criteria, describe characteristics and hormonal recovery and investigate factors associated with the occurrence and recovery of irH. A retrospective study including patients with suspected irH at the University of Texas MD Anderson Cancer Center from 5/2003 to 8/2017 was conducted. IrH was defined as: (1) ACTH or TSH deficiency plus MRI changes or (2) ACTH and TSH deficiencies plus headache/fatigue in the absence of MRI findings. We found that of 83 patients followed for a median of 1.75 years (range 0.6-3), the proposed criteria used at initial evaluation accurately identified 61/62 (98%) irH cases. In the irH group (n = 62), the most common presentation was headache (60%), fatigue (66%), central hypothyroidism (94%), central adrenal insufficiency (69%) and MRI changes (77%). Compared with non-ipilimumab (ipi) regimens, ipi has a stronger association with irH occurrence (P = 0.004) and a shorter time to irH development (P < 0.01). Thyroid, gonadal and adrenal axis recovery occurred in 24, 58 and 0% patients, respectively. High-dose steroids (HDS) or ICI discontinuation was not associated with hormonal recovery. In the non-irH group (n = 19), one patient had isolated central hypothyroidism and six had isolated central adrenal insufficiency. All remained on hormone therapy at the last follow-up. We propose a strict definition of irH that identifies the vast majority of patients. HDS and ICI discontinuation is not always beneficial. Long-term follow-up to assess recovery is needed.