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Albert J. Fenoy, Arnold H. Menezes, Kathleen A. Donovan and Stephen F. Kralik

Object

Calcium pyrophosphate dihydrate (CPPD) deposition is a rare cause of retroodontoid mass lesions in elderly individuals. However, this condition may be severely underdiagnosed if sufficient attention is not paid to imaging characteristics and clinical presentation. The authors sought to evaluate the decision-making process in both the diagnosis and surgical treatment of CPPD.

Methods

A retrospective review of University of Iowa medical records and radiographs accumulated between 1977 and 2006 was performed. The inclusion criterion was histopathological findings consistent with pseudogout at the craniovertebral junction (CVJ). Twenty-one patients with a mean age of 70.3 years and a mean symptom duration prior to presentation of 17.5 months were identified and included in this study.

Results

The patients presented most frequently with occipital and neck pain (85%) and numbness or paresthesias (61%). Lower cranial nerve deficits were seen in 29%. Calcification of the mass or transverse ligament was seen on computed tomography in all included patients. Gross-total resection was achieved in all patients: 19 of 21 patients underwent transoral–transpalatopharyngeal resection, with only 16 requiring concomitant dorsal occipital–cervical fusion. The mean follow-up duration was 15 months. Eighteen patients (86%) had improvement or resolution of symptoms after treatment, and 3 were lost to follow-up.

Conclusions

Although rare, CPPD deposition at the CVJ should be suspected on finding calcification of and around the transverse ligament on neuroimaging. Transoral–transpalatopharyngeal resection is preferred to halt the progression of neurological deterioration; dorsal fusion is not always mandatory as concomitant ligamentous calcification and atlantoaxial joint ankylosis may provide added stability.

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Aditya Vedantam, Katie M. Stormes, Nisha Gadgil, Stephen F. Kralik, Guillermo Aldave and Sandi K. Lam

OBJECTIVE

Resection of posterior fossa tumors in children may be associated with persistent neurological deficits. It is unclear if these neurological deficits are associated with persistent structural damage to the cerebellar pathways. The purpose of this research was to define longitudinal changes in diffusion tensor imaging (DTI) metrics in white matter cerebellar tracts and the clinical correlates of these metrics in children undergoing resection of posterior fossa tumors.

METHODS

Longitudinal brain DTI was performed in a cohort of pediatric patients who underwent resection of posterior fossa tumors. Fractional anisotropy (FA) of the superior cerebellar peduncles (SCPs) and middle cerebellar peduncles (MCPs) was measured on preoperative, postoperative, and follow-up DTI. Early postoperative (< 48 hours) and longer-term follow-up neurological deficits (mutism, ataxia, and extraocular movement dysfunction) were documented. Statistical analysis was performed to determine differences in FA values based on presence or absence of neurological deficits. Statistical significance was set at p < 0.05.

RESULTS

Twenty children (mean age 6.1 ± 4.1 years [SD], 12 males and 8 females) were included in this study. Follow-up DTI was performed at a median duration of 14.3 months after surgery, and the median duration of follow-up was 19.7 months. FA of the left SCP was significantly reduced on postoperative DTI in comparison with preoperative DTI (0.44 ± 0.07 vs 0.53 ± 0.1, p = 0.003). Presence of ataxia at follow-up was associated with a persistent reduction in the left SCP FA on follow-up DTI (0.43 ± 0.1 vs 0.55 ± 0.1, p = 0.016). Patients with early postoperative mutism who did not recover at follow-up had significantly decreased FA of the left SCP on early postoperative DTI in comparison with those who recovered (0.38 ± 0.05 vs 0.48 ± 0.06, p = 0.04).

CONCLUSIONS

DTI after resection of posterior fossa tumors in children shows that persistent reduction of SCP FA is associated with ataxia at follow-up.

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Vijay M. Ravindra, Stephen F. Kralik, Julius Griauzde, Nisha Gadgil, Melissa A. LoPresti and Sandi Lam

OBJECTIVE

Moyamoya disease is a progressive occlusive arteriopathy for which surgical revascularization is indicated. In this retrospective study, the authors investigated the use of preoperative CT perfusion with the aim of establishing pathological data references.

METHODS

The authors reviewed the medical records of children with moyamoya disease treated surgically at one institution between 2016 and 2019. Preoperative CT perfusion studies were used to quantify mean transit time (MTT), cerebral blood volume (CBV), cerebral blood flow (CBF), and time to peak (TTP) for the anterior, middle, and posterior cerebral artery vascular territories for each patient. CT perfusion parameter ratios (diseased/healthy hemispheres) and absolute differences were compared between diseased and normal vascular territories (defined by catheter angiography studies). Sensitivity, specificity, and positive (PPV) and negative (NPV) predictive values for CT perfusion parameters for severe angiographic moyamoya were calculated.

RESULTS

Nine children (89% female) had preoperative CT perfusion data; 5 of them had evidence of unilateral hemispheric disease and 4 had bilateral disease. The mean age at revascularization was 77 months (range 40–144 months). The etiology of disease was neurofibromatosis type 1 (3 patients), Down syndrome (2), primary moyamoya disease (2), cerebral proliferative angiopathy (1), and sickle cell disease (1). Five patients had undergone unilateral revascularization. Among these patients, pathological vascular territories demonstrated increased MTT in 66% of samples, increased TTP in 66%, decreased CBF in 47%, and increased CBV in 87%. Severe moyamoya (Suzuki stage ≥ 4) had diseased/healthy ratios ≥ 1 for MTT in 78% of cases, for TTP in 89%, for CBF in 67%, and for CBV in 89%. The MTT and TTP region of interest ratio ≥ 1 demonstrated 89% sensitivity, 67% specificity, 80% PPV, and 80% NPV for the prediction of severe angiographic moyamoya disease.

CONCLUSIONS

Pathological hemispheres in these children with moyamoya disease demonstrated increased MTT, TTP, and CBV and decreased CBF. The authors’ results suggest that preoperative CT perfusion may, with high sensitivity, be useful in deciphering perfusion mismatch in brain tissue in children with moyamoya disease. More severe angiographic disease displays a more distinct correlation, allowing surgeons to recognize when to intervene in these patients.