Iatrogenic meningoencephalocele after traumatic perforation of the cribriform plate during nasal intubation of a preterm infant.

Traumatic iatrogenic meningoencephaloceles infants are rare and there is no consensus on management in the literature. This article presents a case of a meningoencephalocele diagnosed 15 months after a traumatic perforation of the cribriform plate due to a difficult intubation of a preterm infant that was treated by an endoscopic endonasal surgery. A close collaboration between pediatricians and ENT surgeons appears essential for early diagnosis and management. Endoscopic endonasal approach for meningoencephalocele management has several advantages and is a safe procedure when performed by an experienced surgeon.

Imaging sphenoid diseases.

In this review we provide comprehensive analysis of the imaging features of diseases affecting the sphenoid sinus, including a large and heterogeneous spectrum of pathologies such as sinusitis, pseudotumours, bony pathologies, and tumours. Clinical symptomatology related to sphenoid pathologies is often non-specific and patient clinical examination and endoscopic investigations are not definitive; thus, radiological imaging is mandatory for diagnosis. Strengths and limitations of both morphological and functional imaging methods such as computed tomography (CT), magnetic resonance imaging (MRI), and combined positron-emission tomography/computed tomography (PET/CT) have been considered and integrated into a well-defined clinical context in order to recognise specific imaging features and to underline their clinical relevance for an early and accurate diagnosis. An overview of several sphenoid conditions is herein selected with a didactic objective including both common and less common diseases.

Conservative management of an atypical intra-sinusal ossifying fibroma associated to an aneurysmal bone cyst.

Ossifying fibroma (OF) is a benign fibro-osseous lesion mainly occurring in young adults and seems to originate from the periodontal ligament. Aneurysmal bone cyst (ABC) is a benign intraosseous lesion characterized by blood-filled spaces of various sizes. These two lesions can specifically affect the jaws and are commonly described in the literature. However, few cases describing an association of OF and ABC have been reported in the literature, especially in the maxillary sinus. We report the case of a 40-year-old male patient affected with an asymptomatic lesion with a dual component of OF and ABC laying in the maxillary sinus. Our aim is to discuss its clinical and morphological features as well as treatment results.

[Congenital lacrimonasal duct cyst: Do not forget this radiological and clinical entity]. / Kyste lacrymonasal congénital: une entité radioclinique à ne pas méconnaître.

Although obstruction of the lacrimonasal duct is a fairly common finding in newborns, development of a dacryocystocele (nasolacrimal duct cyst) is uncommon and is caused by stenosis in the proximal and distal area of the nasolacrimal duct leading to a cystic dilatation. Its diagnosis remains difficult for the pediatrician, the ENT specialist, the ophthalmologist, and the radiologist. The study of six cases of dacryocystocele and the review of the literature led the authors to describe the clinical and radiological features of this uncommon entity. The symptomatology includes nasal obstruction and, when bilateral, significant respiratory distress in the newborn (obligate nose-breather) and dilatation of the lacrimal duct with blue cystic swelling inferior to the medial canthus or with an inflammatory aspect of the lacrimal duct in case of infection. A careful endoscopic examination of the nasal cavities and CT or MRI imaging reveals a cystic tumor, which arises in the inferior meatus, inferolateral to the inferior turbinate, and can partly or completely obstruct the endonasal space, uni- or bilaterally. CT and MRI are equally sensitive in detecting dacryocystocele and are also useful for differential diagnosis for other cystic or tumoral nasal lesions such as meningoencephalocele, dermoid cyst, and glioma. To avoid the risk of potential complications (respiratory distress or even sudden infant death, infectious ophthalmologic complications), this radiological and clinical entity should not be forgotten. Endoscopic marsupialization leads to immediate and definitive healing recovery.

Value of MRI olfactory bulb evaluation in the assessment of olfactory dysfunction in Bardet-Biedl syndrome.

Olfactory bulb (OB) volume evaluation by magnetic resonance imaging (MRI) has been demonstrated to be related to olfactory dysfunction in many different diseases. Olfactory dysfunction is often overlooked in Bardet-Biedl syndrome (BBS) patients and is rarely objectively evaluated by MRI. We present a series of 20 BBS patients with olfactory dysfunction. The OB was evaluated separately and blindly by two radiologists (SR and SM) with 3 Tesla MRI imaging comparatively to 12 normal control subjects by global visual evaluation and by quantitative measurement of OB volume. In the 12 control cases OB visual evaluation was considered as normal in all cases for radiologist (SR) and in 10 cases for radiologist (SM). In the 20 BBS patients, OB visual evaluation was considered as abnormal in 18 cases for SR and in all cases for SM. OB volumetric evaluation for SR and SM in BBS patients was able to provide significant correlation between BBS and olfactory dysfunction. This study indicates that OB volume evaluation by MRI imaging like structural MRI scan for gray matter modifications demonstrates that olfactory dysfunction in BBS patients is a constant and cardinal symptom integrated in a genetical syndrome with peripheral and central olfactory structure alterations.

Middle ear cholesteatoma: Compared diagnostic performances of two incremental MRI protocols including non-echo planar diffusion-weighted imaging acquired on 3T and 1.5T scanners.

BACKGROUND AND PURPOSE: To compare diagnostic performances for cholesteatoma diagnosis of incremental MRI protocols including non-echo planar diffusion-weighted imaging (DWI) performed on 3T and 1.5T scanners. MATERIALS AND METHODS: Thirty-nine patients with suspected cholesteatoma underwent 3T and 1.5T non-echo planar DWI and additional unenhanced T1-, delayed gadolinium-enhanced T1- and high-resolution T2-weighted standard acquisitions. Patients either underwent surgical tympanoplasty (n=21) or close clinicoradiological follow-up (n=18). Four radiologists independently and prospectively interpreted two incremental MRI protocols, differing in the magnetic field strength of the diffusion-weighted acquisition and comprising the three standard sequences. At each step, diagnostic performances were expressed as sensitivity, specificity, positive predictive value, negative predictive value and accuracy. RESULTS: Forty middle ear lesions including 21 cholesteatomas were identified. Univariate and multivariate analysis did not demonstrate significant reader, sequence addition or DWI magnetic field effect on diagnostic performances. Concerning non-echo planar DWI alone, sensitivity, specificity, positive predictive value, negative predictive value and accuracy ranged between 90.5-100%, 68.4-100%, 76.9-100%, 90.0-100% and 82.5-95.0, respectively. CONCLUSION: Non-echo planar DWI for cholesteatoma diagnosis can be performed on 1.5T or 3T scanners indifferently. High sensitivity and negative predictive value and relatively lower specificity and positive predictive value are achieved by a single non-echo planar DWI protocol.

Olfaction evaluation and correlation with brain atrophy in Bardet-Biedl syndrome.

Bardet-Biedl syndrome (BBS) is a well-recognized ciliopathy characterized by cardinal features namely: early onset retinitis pigmentosa, polydactyly, obesity, hypogonadism, renal and cognitive impairment. Recently, disorders of olfaction (anosmia, hyposmia) have been also described in BBS patients. Moreover, morphological brain anomalies have been reported and prompt for further investigations to determine whether they are primary or secondary to peripheral organ involvement (i.e. visual or olfactory neuronal tissue). The objective of this article is to evaluate olfactory disorders in BBS patients and to investigate putative correlation with morphological cerebral anomalies. To this end, 20 BBS patients were recruited and evaluated for olfaction using the University of Pennsylvania Smell Identification Test (UPSIT). All of them underwent a structural magnetic resonance imaging (MRI) scan. We first investigated brain morphological differences between BBS subjects and 14 healthy volunteers. Then, we showed objective olfaction disorders in BBS patients and highlight correlation between gray matter volume reduction and olfaction dysfunction in several brain areas.

Pseudotumoral allergic fungal sinusitis with skull base involvement.

Here we report a case of pseudotumoral recurrence of allergic fungal sinusitis with involvement of the skull base that was successfully treated with systemic corticosteroids and itraconazole without surgery. This report discusses the sometimes misleading radiological and clinical features as well as the diagnostic and therapeutic challenges of a condition that should be recognized by ENT specialists, neurosurgeons, ophtalmologists and radiologists.

Interest of [18F]FDG PET/CT for treatment efficacy assessment in aggressive phenotype of sarcoidosis with special emphasis on sinonasal involvement.

AIM: The aim of this paper was to evaluate the clinical usefulness of [18F]FDG PET/CT for treatment efficacy assessment in patients with severe multisystemic phenotype of sarcoidosis with special emphasis on sinonasal involvement. METHODS: Thirteen patients with biopsy-proven sinonasal sarcoidosis (SNS) who underwent two [18F]FDG-PET/CT were selected. PET/CT results were correlated with nasal endoscopy, biology and conventional imaging techniques (CT, MRI). Four and nine patients underwent first PET/CT before beginning treatment and during CS therapy, respectively. On the other hand, ten and three patients underwent second PET/CT during CS and after treatment withdrawal, respectively. The mean duration of clinical and endoscopic follow-up after the second scintigraphic examination was 51 months. RESULTS: Eleven out of 13 selected patients presented with pathological nasal endoscopy at inclusion. Among them: 1) 5 showed persistent endoscopic abnormalities at follow-up evaluation. Radiological and PET/CT imaging was consistent with these results in 4 and 5 patients, respectively; 2) 2 showed a complete endoscopic, radiologic and PET/CT normalization after CS treatment; 3) 4 showed important alterations of the sinonasal structures preventing a definitive diagnosis by endoscopic and radiologic techniques. PET/CT suggested a residual inflammatory disease in two cases. No scintigraphic abnormalities were detected in the other 2 patients. Scintigraphic results were finally confirmed by a mean follow-up of 51 months. No pathologic sinonasal [18F]FDG uptake was observed in the remaining 2/13 patients who showed doubtful endoscopic and radiologic results during primary evaluation. The stability of endoscopic results without clinical and biological evolution was observed during 39 and 38 months of follow-up after the second PET/CT. CONCLUSION: [18F]FDG PET/CT seems to be a valuable non-invasive imaging technique able to evaluate the response to treatment in aggressive SNS, identifying persistent active disease even in those patients with destructive sinonasal aftereffects and/or with atypical therapeutic evolution. Finally, [18F]FDG PET/CT could be clinically useful to modulate CS treatment eventually integrating immunosuppressive drugs.

[Morpho-functional imaging in a patient with hyperparathyroidism and multifocal maxillary brown tumor]. / Imagerie morphologique et fonctionnelle des tumeurs brunes. À propos d'un cas de localisation maxillo-mandibulaire.

INTRODUCTION: Brown tumors are uncommon osteolytic lesions directly related to the increased osteoclastic activity due to hyperparathyroidism. CASE REPORT: A 37-year-old woman presented with hypercalcemia related to primary hyperparathyroidism. Multiple and bilateral maxillary osteolytic lesions showing intense fluorodesoxyglucose (FDG) uptake were noted in a positron emission tomography computed tomography (PET-CT). Diagnosis of maxillary brown tumors was discussed and confirmed by both orthopantomogram and magnetic resonance imaging. Left inferior parathyroid adenoma was detected by both cervical ultrasonography and parathyroid scintigraphy, and then surgically treated with consequent improvement of hyperparathyroidism. CONCLUSION: Our case emphasizes the necessity of a multidisciplinary diagnostic approach to optimize the interpretation of the available imaging, especially in unusual and unrecognized pathology as brown tumors.

[Intracranial complications from ENT infections]. / Complications méningo-encéphaliques des infections ORL.

Sinonasal and temporal bone infections may extend to the skull, skull base, meninges, pericerebral spaces, brain parenchyma, dural sinuses, deep cerebral or cortical veins, intracranial arteries and cranial nerves either via contiguous or hematogeneous spread. The site of infection dictates the sites of potential complications: orbital with ethmoid sinusitis, cavernous sinus thrombophlebitis and oculomotor palsies with sphenoid sinusitis, transverse sinus thrombophlebitis with mastoiditis and superior sagittal sinus thrombophlebitis with frontal sinusitis. All may result in brain abscess. Congenital and acquired defects of the skull and meninges, with or without associated meningocele or meningoencephalocele, perilymphatic fistulas, and some anomalies of the inner ear may predispose to the intracranial extension of ENT infections.

[Sarcoidosis of the upper respiratory tract]. / Sarcoïdose des voies aérodigestives supérieures.

INTRODUCTION: Sarcoidosis is a non-caseating granulomatous disease of unknown origin, principally affecting the respiratory tract. BACKGROUND: Sarcoidosis of the upper respiratory tract (SURT) includes sino-nasal sarcoidosis (SNS) and pharyngo-laryngeal sarcoidosis (PLS). SURT may be isolated or, more often, part of multisystemic sarcoidosis. Its clinical symptomatology is protean and non specific. The natural history, course and prognosis are poorly understood and unpredictable. The treatment has not yet been standardised and the long-term therapeutic results are often disappointing. VIEWPOINT: In this work, we try to make a synthesis of our experience and publications, and the data in the existing international literature, to improve the diagnosis and therapeutic management of SURT. The usefulness of both morphological and functional imaging techniques, in particular 18F-fluorodeoxyglucose positron emission tomography (18F-FDG PET/CT), is evaluated for use in the management of the severe phenotypes of sarcoidosis such as SURT. CONCLUSIONS: Even if guided biopsy remains necessary for confirmation of SURT, medical imaging plays an important role in the management of this disease: CT imaging allows the description of SNS and classification into two stages that correlate well with the severity, reversibility and course of the sino-nasal involvement, 18F-FDG PET/CT, providing a complete morpho-functional mapping of active inflammatory lesions, could be a useful technique in patients with biopsy-proven SURT, for both diagnosis and follow up of medical treatment.

Interest of diffusion-weighted and gadolinium-enhanced dynamic MR sequences for the diagnosis of parotid gland tumors.

PURPOSE: This study aimed to evaluate the value of diffusion-weighted imaging (DWI) and gadolinium-enhanced dynamic magnetic resonance imaging (MRI) for differentiating benign and malignant parotid gland tumors, and for characterizing the various histological types (pleomorphic adenoma, and Warthin's and malignant tumors). PATIENTS AND METHODS: This retrospective study involved 60 patients with suspected parotid gland tumors (mean age: 59.4 years), and was carried out from April 2005 to February 2008. All had undergone pathological examination. All MRI examinations were performed using the Siemens Magnetom Avanto 1.5T MRI system. Non-enhanced T1-weighted (T1W), gadolinium-enhanced fat-suppressed T1W and T2-weighted (T2W) images were obtained for all 60 patients, with diffusion-weighted echoplanar imaging (DW-EPI) and apparent diffusion coefficient (ADC) evaluation in 59 patients, and gadolinium-enhanced dynamic MRI sequences in 51 patients. Interpretation was carried out by two experienced radiologists (the first evaluation used T1W, gadolinium-enhanced fat-suppressed T1W and T2W images; the second evaluation used T1W, T2W, DWI and dynamic MRI) and, for each case, the benign/malignant nature of the tumor and its histological type were determined. RESULTS: After the second reading, increases were noted in sensitivity, specificity, malignant positive predictive value (PPV) and negative predictive value (NPV), as well as in accuracy (90-100% for the first observer, and 90-97% for the second observer). Interobserver reliability also showed a significant increase from the first to the second reading (kappa=0.63 to 0.87, respectively). CONCLUSION: Gadolinium-enhanced dynamic MRI and DW-EPI with ADC evaluation improved the performance of MRI in distinguishing between benign and malignant parotid gland tumors, and characterizing the different histological types of benign tumors (pleomorphic adenoma and Warthin's), thus leading to greater consensus in interpretation of the images.

Wegener's granulomatosis of the temporal bone and skull base that mimicked an inflammatory myofibroblastic tumour: a case report.

OBJECTIVE: To compare two potential diagnoses of a temporal bone pseudotumour: an inflammatory myofibroblastic tumour and Wegener's granulomatosis. METHODOLOGY: A case of Wegener's granulomatosis that mimicked an inflammatory myofibroblastic tumour is reported. The clinical presentation, staging of the disease, histology, and follow-up are analysed. RESULTS: Histopathology of the temporal bone failed to provide an accurate diagnosis, even after immunocytochemical analyses. The diagnosis of Wegener's granulomatosis was suspected after biopsy of a pulmonary mass and was confirmed by the presence of anti-neutrophil cytoplasmic antibodies in blood samples. CONCLUSION: Irrespective of the aetiology, a pseudotumour of the temporal bone should always be investigated by biology and radiology. Radiological investigations will allow staging of the disease and specific localisation for biopsies.

Imaging of the facial nerve.

The facial nerve is responsible for the motor innervation of the face. It has a visceral motor function (lacrimal, submandibular, sublingual glands and secretion of the nose); it conveys a great part of the taste fibers, participates to the general sensory of the auricle (skin of the concha) and the wall of the external auditory meatus. The facial mimic, production of tears, nasal flow and salivation all depend on the facial nerve. In order to image the facial nerve it is mandatory to be knowledgeable about its normal anatomy including the course of its efferent and afferent fibers and about relevant technical considerations regarding CT and MR to be able to achieve high-resolution images of the nerve.

Middle ear adenomatous tumor: a not so rare glomus tympanicum-mimicking lesion.

BACKGROUND AND PURPOSE: Middle ear adenomatous tumors (MEAT) are rare tumors which can be begin or malignant and can present a neuroendocrine differentiation. Their radiological aspect is very similar to glomus tympanicum (GT) which are the most common tumoral lesions of the middle ear. We present several radiological and clinical findings that could help radiologists to accurately identify MEAT. MATERIAL AND METHODS: We retrospectively reviewed the radiological and clinical findings of three patients with MEAT and of eight patients with GT. Diagnostic was obtained after surgical resection in all cases. All patients had high resolution CT and MR of the middle ear associated with a subtracted digital carotid angiography. Tumor location, size, extension, signal intensity, and enhancement were analysed. From the medical records of the patients, clinical manifestations (hearing loss, tinnitus), evolution length and recurrences were noted. RESULTS: MEAT and GT appeared as tissular lesion with significant enhancement on CT and MR. A vascular blush was present on angiography in all cases of GT and absent from all cases of MEAT. A close relationship between the tumor and the Jacobson's nerve or its branches was identified in all cases of GT. Pulsatile tinnitus was present in all patients with GT and absent in all patients with MEAT. CONCLUSION: A middle ear tissular lesion clearly separated from the Jacobson nerve or its branches, showing significant enhancement after contrast medium injection but with a normal angiography, should make one suspicious for MEAT.

Imaging in sinonasal sarcoidosis: CT, MRI, 67Gallium scintigraphy and 18F-FDG PET/CT features.

OBJECTIVES: Attempt to describe and analyse the radiological and nuclear medicine patterns of sinonasal sarcoidosis (SNS) still poorly reported in the literature. MATERIAL AND METHODS: Retrospective single institution study of 22 consecutive patients with symptomatic biopsy-proven SNS to evaluate the interest of CT, MRI, (67)Ga scintigraphy and (18)F-FDG PET/CT for diagnosis and therapeutic follow-up. RESULTS: Nodules of the septum and turbinates are the most suggestive CT and MRI features. Other CT features such as sinusal filling, mucosal thickening, osteosclerosis or destructive sinonasal lesions are not specific and depend on clinical context and evolutive stage of SNS. (18)F-FDG PET/CT provides complete morphofunctional mapping of active inflammatory sites related to sarcoidosis with a better diagnostic sensitivity (100%) compared to (67)Gallium scintigraphy (75%). The changes in (18)F-FDG uptake intensity could reflect the efficacy of treatment. CONCLUSION: SNS is an uncommon and probably underdiagnosed phenotype of sarcoidosis. Even if guided biopsy remains necessary for SNS confirmation, medical imaging plays an important role in diagnosis and therapeutic follow-up. CT features with nodules of the septum and/or turbinates are suggestive of SNS contrary to other nonspecific CT findings. CT imaging is directly related severity, reversibility and course of SNS and provide an original radiological staging system in order to predict patient clinical outcome. PET/CT may be used for diagnosis assessement but also to monitor treatment response in a given clinical context, in a patient with histopathologically-proven SNS. Prospective and long term studies are necessary to validate these preliminary results.