Cervical vascular and upper airway asymmetry in Velo-cardio-facial syndrome: correlation of nasopharyngoscopy with MRA.

PURPOSE: Velo-cardio-facial syndrome (VCFS), the most common genetic syndrome causing cleft palate, is associated with internal carotid and vertebral artery anomalies, as well as upper airway asymmetry. Medially displaced internal carotid arteries, often immediately submucosal, present a risk of vascular injury during pharyngeal flap surgery for velopharyngeal insufficiency (VPI). We evaluate the frequency and spectrum of cervical vascular anomalies in a large cohort of VCFS patients correlating MRA with nasopharyngolaryngoscopy in detecting at risk carotid arteries. Furthermore, we assess the relationship with respect to laterality between cervical vascular patterns and the asymmetric abnormalities of these subjects' upper airways. METHODS: Cervical MRAs of 86 subjects with VCFS and 50 control subjects were independently reviewed by three neuroradiologists. The course of the internal carotid and vertebral arteries was identified within the pharyngeal soft tissues. Medial deviation, level of bifurcation, dominance, anomalous origin, and vessel tortuosity were recorded. Nasopharyngoscopy examinations were available for retrospective review in 43 patients and were assessed for palatal and posterior pharyngeal wall symmetry, true vocal cord motion and size, and for the presence or absence of carotid pulsations. The endoscopic findings were compared with MRA results. RESULTS: Of the 86 subjects, 80 (93%) had one or more vascular anomalies. 42 subjects (49%) were found to have medial deviation of at least one internal carotid artery. In 24 subjects (28%) the anomalous internal carotid artery was directly submucosal; four of these were bilateral (5% of the total sample, 17% of those with a submucosal internal carotid). Other carotid anomalies included low carotid bifurcation (44 subjects or 51%), anomalous origin of the right common carotid (32 cases, or 37%), and two cases of internal carotid agenesis/hypoplasia. Vertebral artery anomalies included vessel tortuosity (34 cases, or 40%), hypoplasia (10 cases, or 12%), looping (4 cases, or 5%), and one case of a double left vertebral artery. Though patients in our study showed an asymmetric distribution of vascular anomalies, no association was found between the laterality of palatal motion, pharyngeal fullness, or laryngeal movement and structure with ipsilateral vertebral or carotid artery anomalies. Of the 33 pulsatile carotid arteries visualized at nasopharyngoscopy, only nine were found to be submucosal on MRA. In contrast, 11 submucosal carotid arteries confirmed at MRA demonstrated no visible pulsations. Positive and negative predictive values of pulsative arteries seen endoscopically for MRA confirmation of a submucosal carotid course was 27% and 79% respectively. CONCLUSIONS: Carotid and vertebral artery anomalies are common in VCFS including marked medial deviation of the internal carotid artery in close proximity to the donor site for pharyngeal flap surgery. Lack of correlation between laterality of vascular anomalies and upper airway structural asymmetry in VCFS does not support the hypothesis that palatal, pharyngeal, and laryngeal anomalies are due to secondary developmental sequences caused by in utero vascular insufficiency. The presence or absence of carotid pulsations seen by nasopharyngoscopy does not correlate with the carotid arterial depth identified on MRA. Furthermore, identification of the relative medial-lateral retropharyngeal position of a submucosal carotid affords the opportunity to modify the surgical approach. These findings further support the routine use of pre-operative neck MRA in VCFS patients in surgical planning.

Radiologic diagnosis of cerebral venous thrombosis: pictorial review.

OBJECTIVE: Cerebral venous thrombosis is often associated with nonspecific clinical complaints. In addition, the imaging findings are often subtle. Underdiagnosis or misdiagnosis of cerebral venous thrombosis can lead to severe consequences, including hemorrhagic infarction and death. CONCLUSION: This article reviews the radiologic findings and diagnostic pitfalls of cerebral venous thrombosis. After completing this article, the readers should have an improved ability to diagnose cerebral venous thrombosis accurately, using the optimal imaging tools to achieve this goal.

Predicting deep neck space abscess using computed tomography.

PURPOSE: To investigate objective measures that could increase the positive predictive value of computed tomography (CT) in diagnosing deep neck space infections (DNSIs). METHODS: A retrospective analysis of patients surgically treated at a tertiary care hospital for DNSIs for more than 2 years were reviewed. Patients who had had CT with contrast scanning suggestive of deep neck space abscess within 24 hours before surgery were included. The average Hounsfield units for each abscess were calculated. Based on the intraoperative finding of pus, the patients were divided into groups. Student t tests compared the average Hounsfield units, white blood cell count, and maximum temperature between the groups. Outcomes were measured by comparing overall length of hospital stay, length of postoperative stay, and complications. RESULTS: Of the 32 patients surgically drained, 24 (75%) had discreet collections of pus, whereas 12 (25%) did not. Hounsfield unit measurement was not reliable in distinguishing abscess from phlegmon. None of the other clinical variables studied to distinguish abscess from phlegmon were statistically different either. A statistical difference between the 2 groups was not identified. CONCLUSION: Although CT with contrast plays an important role in the diagnosis and management of DNSIs, the decision for surgical drainage of an abscess should be made clinically. A negative exploration rate of nearly 25% despite careful selection criteria should be expected.

Pharyngeal flap and the internal carotid in velocardiofacial syndrome.

Internal carotid artery anomalies have been documented as a common clinical feature in velocardiofacial syndrome. There has been some controversy over the need for preoperative imaging procedures, such as magnetic resonance angiography, when planning pharyngeal surgery for correcting velopharyngeal insufficiency. The purpose of this article is to describe 20 patients with velocardiofacial syndrome who received comprehensive evaluation and underwent pharyngeal flap surgery within a 2-year period and to report the technique used for dissecting the flap and the surgical outcomes. Anomalies of the major neck vessels were present in all cases, but 5 of these 20 cases had particularly severe anomalies of the internal carotid arteries that placed the vessels directly deep within the donor site for the pharyngeal flap. Surgery was carried out successfully in all 20 cases using a modified approach after radiographic imaging was performed to locate the arteries. In the 5 cases with severe malpositioning of the internal carotid arteries, it was clear that the vessels could have been injured had their location not been identified and the surgical approach modified to avoid them.