Francesca Ormitti

Diagnostic Imaging of Diffuse Infiltrative Disease of the Lung

Plain chest radiography remains the first diagnostic approach to diffuse infiltrative lung disease but has limited diagnostic sensitivity and specificity. Many diseases remain occult or are not correctly assessed using chest X-ray, appearing as a nonspecific ‘reticulonodular pattern’. High-resolution CT (HRCT) is actually the recommended imaging technique in the diagnosis, assessment, and follow-up of these diseases, allowing also the evaluation of the effectiveness of the medical therapy and the selection of the type and the location of the biopsy when required. Appropriate techniques must be used to acquire high-quality HRCT scans, with the thin collimation and high spatial reconstruction algorithm being the most important factors. A nodular pattern, linear and reticular opacities, cystic lesions, ground-glass opacities and consolidations are the most common HRCT patterns of diffuse infiltrative lung disease. This article reviews the role of chest radiography and HRCT in the diagnosis and assessment of these diseases, the technical aspects of HRCT, its clinical indications and the radiological pattern of the most common types of chronic diffuse infiltrative lung disease.

Cochlear implantation in children with cerebral palsy. A preliminary report

OBJECTIVES The aim of this study is to assess the post-implantation speech perception and intelligibility of speech produced by five profoundly deaf children with cerebral palsy. METHODS This study is derived by a review of a prospectively maintained data collection on all patients entering the cochlear implant program. Five children with cerebral palsy who underwent cochlear implantation participated in this study. Functional outcome was assessed using the Speech Perception Categories and the Speech Intelligibility Rating scale. The follow-up of the series ranged from 12 to 45 months. RESULTS At the last follow-up, two children who were placed into speech perception category 1 (detection of a speech signal) preoperatively progressed to category 6 (open-set word recognition with familiar words) postoperatively. Two children moved from preoperative category 2 (pattern perception) to postoperative category 6. One child placed into category 0 (no detection of speech) preoperatively progressed to category 4 (word identification) postoperatively. Before implantation, three children had connected speech unintelligible, and two subjects had connected speech intelligible to a listener who concentrates and lip-reads. At the last follow-up, one child had connected speech unintelligible, two children had connected speech intelligible to a listener who concentrate and lip-reads, one child had connected speech intelligible to a listener who has little experience of a deaf persons speech, and one child had connected speech intelligible to all listeners. CONCLUSIONS Cochlear implantation allowed these patients to dramatically improve their quality of life, increasing their self-confidence, independence and social integration.

Cochlear implantation in children with cochlear nerve deficiency

OBJECTIVE The aim of this study was to report on auditory performance after cochlear implantation in children with cochlear nerve deficiency. METHODS A retrospective case review was performed. Five patients with pre-lingual profound sensorineural hearing loss implanted in an ear with cochlear nerve deficiency participated in the study. Postoperative auditory and speech performance was assessed using warble tone average threshold with cochlear implant, speech perception categories, and speech intelligibility ratings. All patients underwent high resolution computed tomography and magnetic resonance imaging. RESULTS According to Govaerts classification, three children had a type IIb and two a type IIa cochlear nerve deficiency. Preoperatively, four patients were placed into speech perception category 1 and one into category 2. All patients had an improvement in hearing threshold with the cochlear implant. Despite this, at the last follow-up (range 18-81 months, average 45 months), only one girl benefited from cochlear implantation; she moved from speech perception category 2 to 6 and developed spoken language. Another child developed closed set speech perception and had connected speech that was unintelligible. The other 3 children showed little benefit from the cochlear implant and obtained only an improved access to environmental sounds and improved lipreading skills. None of these 4 children developed a spoken language, but they were all full-time users of their implants. CONCLUSIONS The outcomes of cochlear implantation in these five children with cochlear nerve deficiency are extremely variable, ranging from sporadic cases in which open set speech perception and acquisition of a spoken language are achieved, to most cases in which only an improved access to environmental sound develops. Regardless of these limited outcomes, all patients in our series use their device on a daily basis and derive benefits in everyday life. In our opinion, cochlear implantation can be a viable option in children with cochlear nerve deficiency, but careful counseling to the family on possible restricted benefit is needed.

Bilateral cerebellopontine angle lipomas

Cerebellopontine angle (CPA) lipomas are extremely rare lesions and usually unilateral. We describe a case of a 36-year-old man with bilateral aural fullness that was discovered to have bilateral CPA lipomas associated with an abnormal hindbrain segmentation appearance. The patient was evaluated with 3.0T magnetic resonance imaging (MRI) system. MRI demonstrated the presence of bilateral CPA masses partially extending into the internal auditory canals. These lesions were hyperintense on both T1- and T2 weighted images. Facial and vestibulo-cochlear nerves together with tortuous vascular structures and fibro-connective septa were identified on T2-weighted 3D Fast Imaging Employing Steady-state Acquisition (FIESTA) sequences as areas of lower intensity coursing through the masses. Gadolinium administration yielded no enhancement. Conservative management was adopted.

Goldenhar syndrome associated with contralateral agenesis of the internal carotid artery.

Congenital absence of the internal carotid artery (ICA) is an extremely rare vascular anomaly. Aplasia and displacement of the horizontal portion of the petrous carotid artery have been described in a patient with mandibulofacial dysostosis. To the best of our knowledge, the association between Goldenhar syndrome and ipsilateral ICA agenesis has emerged only in one case documented in the medical literature to date. We describe here a case that illustrates the association of Goldenhar syndrome with contralateral agenesis of the ICA incidentally detected on brain magnetic resonance imaging and subsequently confirmed on magnetic resonance angiography and high resolution computed tomography.

Desmoplastic Fibroma of the Mandible: Usefulness of CT and MR Imaging in Diagnosis and Treatment A Case Report

Desmoplastic fibroma (DF) is a rare non-metastasizing benign neoplasm of the bone characterized by aggressive local infiltration, also known as desmoid tumour. Desmoplastic fibroma of the mandible may recur locally when incompletely excised. In particular it can occur adjacent to and display contiguous extension along the mandibular nerve. This case report describes the imaging findings of a histopathologically proven desmoplastic fibroma involving the right mandibular region in a three-year-old Italian girl. We also discuss the usefulness of different imaging modalities (CT and MRI) in DF diagnosis and treatment.

A painful stiff neck following an ear, nose, and throat surgical procedure: case report.

Grisel syndrome is a rare, nontraumatic atlantoaxial subluxation, typical of developmental ages and characterized by head flexion/rotation and painful fixation. Neurological symptoms may occur. It is secondary to head/neck infections and ear, nose, and throat surgery (adenoidectomy, tonsillectomy, and mastoidectomy). Here, we report the case of a child who presented a painful stiff neck following an adenotonsillectomy, with imaging evidencing an atlantoaxial subluxation. The child showed improvement in his condition following a conservative treatment with antibiotics, anti-inflammatory, and analgesic therapy and cervical collar. We believe it is of great significance for clinicians taking into account this peculiar condition in the differential diagnosis of a stiff neck in pediatric patients, thus avoiding misdiagnosis and delays. Indeed, its diagnosis is mainly based on a focused anamnesis associated with the detection of the typical neuroradiological findings.

Unilateral ectopic parotid gland in CHARGE syndrome

Unilateral absence of a parotid gland at the expected location is an extremely rare condition with only a few cases reported in the medical literature and, to our knowledge, never previously described in association with CHARGE syndrome (Coloboma of the eye, Heart defects, Atresia of the choanae, Retardation of growth and/or development, Genital and/or urinary abnormalities, and Ear abnormalities and deafness). Although this entity is usually associated with a complex constellation of anomalies, additional findings have been described, including cranial nerve dysfunction (VII, VIII, IX and X). We present a case that illustrates the association of CHARGE syndrome with absence of parotid gland at normal location with ectopic parotid tissue lateral to masseter muscle, incidentally detected on brain MRI and subsequently confirmed on neck MRI.

Influenza A H1N1 Related Acute Necrotizing Encephalopathy: Radiological Findings in Adulthood.

In 2009 a novel swine-origin Influenza A H1N1 virus was identified in Mexico and Southern California. Since it was first recognized, neurological complications including acute necrotizing encephalopathy (ANE) have been globally documented in association with this viral infection. ANE is mostly known to occur in the paediatric population. We describe a fatal case of ANE in a previously healthy 40-year-old man infected with influenza A H1N1 virus presenting with severe neurologic decline. Computed tomography (CT) scan and magnetic resonance imaging (MRI) findings were consistent with ANE. CT and MR findings typically documented in paediatric cases of ANE - including bilateral thalamic necrosis with petechial hemorrhage - have been seldom described in adulthood.

Basilar Dependence on a Persistent Hypoglossal Artery Visualized at CT Angiography. A Case Report.

The persistent hypoglossal artery is a rare perseverance of an embryonic vessel connecting the anterior and posterior circulations and is generally considered an incidental finding. This report describes a patient with a basilar dependence on a persistent hypoglossal artery visualized at CT angiography. The pertinent findings and clinical implications of this anomalous vessel are discussed.