Neuropathic arthropathy of the shoulder as a presenting feature of Chiari malformation with syringomyelia: a case report with a systematic literature review.

BACKGROUND: Neuropathic arthropathy (NA) of the shoulder is a chronic progressive process characterized by joint destruction in the presence of a neurosensory deficit. Syringomyelia, a spinal cord disease, is the leading cause of NA in the upper extremity. OBJECTIVE: We present a systematic review of NA with syringomyelia cases alongside a case report of an adult with NA of the shoulder that occurs a few 4 years after a revelation and surgical management of a Chiari malformation with syringomyelia. METHODS: A systematic review was conducted following PRISMA guidelines. A PubMed, Scopus, Isiknowledge, and manual search through references of relevant publications were used to identify all published case reports of NA. Data were collected from each case report on patient characteristics. RESULTS: The systematic review identified 56 publications and 85 patients (including ours): nearly the same number of males (n = 41) and females (n = 44). The mean age was 50,69. Presentations included reduction of mobility (n = 66), swelling (n = 61) and sensory disorder (n = 63). The pain was absent in 41 cases. In the majority of reported cases 56 (65.1%), syringomyelia was revealed by neuropathic arthropathy, and eleven patients (12.9%) had a history of syringomyelia. Treatment was categorized into non-operative management (37[43.5%]), operative management (27[31.7%]). Following-up was non-reported in 31 (36%) cases. Improvement was reported more with patients who underwent a surgical approach than medical one 28.5% versus 8.1%. CONCLUSION: Physicians need to be more aware of this destructive joint disease, rare, and often misdiagnosed. Also, it is imperative to integrate clinical, pathological, and imaging findings for accurate diagnosis and for delivering appropriate therapy.

Mucocèles nasosinusiennes post-radiques à propos de trois cas.

Nasosinusal mucoceles, although benign, are aggressive and sometimes destructive lesions. They may occur as a result of trauma, infection, sinus surgery and head and neck radiation. We describe three patients presenting mucocele, with the history of nasopharyngeal and temporal fossa cancers treated by radiotherapy.

[Septic cavernous sinus thrombosis following ethmoiditis: a case report]. / Thrombose du sinus caverneux compliquant une ethmoïdite aiguë

Acute ethmoiditis is a rare infection of ethmoidal cells. The pathognomonic sign is an edema of the internal corner of the eye. Imaging may be necessary to verify the absence of orbital or endocranial complications. Thrombophlebitis of the cavernous sinus is a serious complication of this infectious process. We report the case of an 11-year-old boy who presented with ethmoiditis complicated with thrombophlebitis of the cavernous sinus, with right hemiplegia and left Bell palsy sequelae. Early diagnosis of this disorder and urgent therapy are essential. Treatment is based on the antibiotic therapy.

Prise en charge des paralysies faciales compliquant un traumatisme de l'os temporal

Introduction : Les paralysies faciales peripheriques (PFP) compliquant les traumatismes de l'os temporal sont devenues de plus en plus frequentes; du fait de developpement de 'activite humaine. Le but de ce travail est de rapporter nos resultats en matiere de prise en charge de ces PFP; et a travers une revue de la litterature; de preciser les caracteristiques cliniques; paracliniques et de proposer une conduite a tenir adequate devant cette pathologie. Materiel et methodes : Il s'agit d'une etude retrospective a propos de 16 patients; pris en charge dans notre service sur une periode de 12 ans (2001-2012). Tous les patients ont beneficie d'un interrogatoire; d'un examen ORL complet. Une tomodensitometrie des rochers a ete realisee dans tous les cas. Les explorations du nerf facial ont ete basees surtout sur l'EMG de detection. Le traitement a ete medical ou medico-chirurgical. Le suivi a ete clinique avec un recul moyen de 26 mois. Resultats : Il s'agissait de 14 hommes et de 2 femmes. L'age moyen etait de 27 ans (6-73 ans). La PF etait d'installation immediate apres le traumatisme chez 10 patients et tardive dans 6 cas. La TDM des rochers a montre une fracture extra-labyrinthique dans 7 cas (43;7); trans-labyrinthique dans 5 cas (31;2) et mixte dans 4 cas (25). L'atteinte du canal facial a ete notee chez 12 patients. L'EMG de detection a ete pratiquee dans 4 cas. Tous nos patients ont beneficie d'un traitement medical; consistant en une corticotherapie par voie generale; des vasodilatateurs et une reeducation motrice. Sept patients ont beneficie d'une decompression du nerf facial. L'indication a ete retenue devant l'installation d'une PF immediate et complete. En per-operatoire; nous avons decouvert un trait de fracture du canal facial dans 5 cas; mais la continuite du nerf etait conservee dans tous les cas. Nous avons realise une decompression de la 2e portion; du coude et de la 3e portion. La decompression etait etendue au ganglion genicule dans 2 cas. Sur les 7 patients operes; 4 ont ete ameliores. Un des 3 patients non ameliores a garde un grade V et a beneficie d'une anastomose VII-XII. Pour les 9 patients non operees; une amelioration a ete notee dans 5 cas. Au total; le pourcentage d'amelioration de la PF etait de 55;5 apres traitement medical et de 71;4 apres traitement medico-chirurgical. Conclusion : Les paralysies faciales constituent une complication assez frequente des traumatismes de l'os temporal. L'imagerie est indispensable pour le diagnostic topographique des lesions. La conduite a tenir therapeutique depend aussi bien de la severite et du delai de l'installation de la paralysie que des donnees electrophysiologiques et evolutives

Carcinome verruqueux du larynx : Diagnostic et traitement

Introduction : Les carcinomes verruqueux du larynx sont des tumeurs rares. Ils posent des problemes diagnostics et therapeutiques. Le but de notre travail est de detailler les difficultes diagnostiques et therapeutiques du carcinome verruqueux du larynx. Patients et methodes : Treize malades ont ete traite d'un carcinomes verruqueux du larynx entre 1992et 2007. Resultats : Une chirurgie a ete pratiquee pour dix patients dont 7 ont eu un curage ganglionnaire. Deux patients etaient traites par radiotherapie exclusive et un autre par radio chimiotherapie concomitante. L'evolution etait bonne sans recidive ni metastases chez tous les patients qui ont ete traites chirurgicalement avec un recul moyen de 37 mois. Conclusion : Le carcinome verruqueux du larynx est une forme rare; de bas grade et bien differenciee du carcinome epidermoide. Il s'agit d'une tumeur a croissance lente et localement agressive dont le traitement de choix est l'exerese chirurgicale en marges saines

[Bronchoscopy for foreign body inhalation in the pediatric population: lessons learned from 223 cases]. / Bronchoscopie pour inhalation de corps étrangers chez l'enfant : à propos de 223cas.

INTRODUCTION: Inhalation of foreign body in children is a serious accident that may compromise the vital prognosis of the child. The diagnostic was difficult in the absence of a recognizable penetration syndrome. Bronchoscopy is still recommended as the appropriate diagnostic and treatment of foreign bodies. The purpose of this study was to analyze the diagnostic and the treatment result of bronchoscopy and discuss its indications. MATERIAL AND METHODS: [corrected] A retrospective study analyzing data related to 223 children undergoing bronchoscopy due to suspicion of foreign body aspiration over a period of 10 years (2000-2009). The average age of the children was 29 months (range: one month-13 years). Approximately, two thirds of these patients were boys. The penetration syndrome was reported in 79.8% of cases. RESULTS: During bronchoscopy, the foreign body was confirmed only in 57.4%. Foreign bodies were found in the bronchus in 79.7% of cases. Among the foreign bodies, 78.1% were of vegetal origin. The average time of stay of the foreign body was of 16.1 days. Penetration syndrome and abnormal physical exam were the most sensitive parameters (79.7% and 82.8%, respectively) but with low specificity (24.2% and 35.8%, respectively). The combination of clinical and radiological signs suggestive of foreign body was the most specific sign (74.7%). Similarly, we found a statistically significant correlation between positive bronchoscopy and simultaneous suggestive clinical and radiological signs (P=0.03). The multivariate study showed that predictors factors of positivity of the bronchoscopy were: abnormal physical exam (P=0.016), abnormal radiological exam (P=0.003) and type of indication (P=0.005). DISCUSSION: The diagnosis of laryngotracheobronchial foreign body recures an array of arguments. It is suspected on the clinical interview specially penetration syndrome and on the clinical and radiological presentation. Any suspicion should lead to a bronchoscopy.

[Multiple cerebral cavernomatosis in a 1-year-old child]. / Cavernomatose cérébrale chez une fille de 1 an.

Cavernous malformations are vascular lesions of the central nervous system. They are very rare in childhood. We report the case of sporadic cavernous cerebral angioma in a 1-year-old girl without pathologic antecedents revealed by a partial seizure. With this case and a review of the literature, we show the clinical and therapeutic characteristics of cerebral cavernomatosis in childhood.

[Use of recombinant factor VII in Glanzmann thrombasthenia: a case report]. / Intérêt du facteur VII recombinant dans la thrombasthénie de Glanzmann: à propos d'une observation.

Glanzmann thrombasthenia (TG) is a congenital platelet function disorder characterized by frequent and occasionally severe bleeding events. Treatment is based on platelet transfusion at the time of bleeding. We report a case of GT revealed in the neonatal period, a severe hemorrhagic syndrome refractory to transfusions, treated at the age of 6 years. Activated recombinant factor VII (Novoseven) injections were necessary. The advantages of recombinant activated factor VII in GT patients with platelet antibodies and/or platelet transfusions refractoriness are discussed.

Osteite frontale post-sinusitique : etude retrospective a propos de 31 cas

Objectif : L'osteite frontale post-sinusitique est definie par l'extension de l'infection du sinus frontal aux structures osseuses adjacentes. Elle constitue une urgence diagnostique et therapeutique. Le but de ce travail est d'analyser les particularites epidemiologiques; cliniques et paracliniques; et de passer en revue les differentes attitudes therapeutiques devant cette pathologie. Materiel et methodes : Il s'agit d'une etude retrospective portant sur 31 patients diagnostiques et traites entre 1996 et 2010. Tous les patients ont beneficie d'un examen ORL et neurologique complet; d'un bilan biologique et d'une tomodensitometrie du massif facial et cerebrale. L'antibiotherapie a ete administree par voie intraveineuse. Le drainage chirurgical du sinus frontal se faisait soit par trepanation; soit par confection d'un volet frontal; soit par mise en place d'unclou de Lemoyne. Le drainage par voie endonasale comportait une meatotomie moyenne avec ethmoidectomie anterieure. La cranialisation du sinus frontal se faisait par voie coronale. L'evolution a ete evaluee sur des criteres cliniques; biologiques et radiologiques. Resultats : L'age moyen etait de 24;4 ans (8-62 ans) et le sex-ratio etait de 4;16. Les cephalees frontales (10 cas) et les signes d'hypertension intracranienne (8 cas) etaient les signes fonctionnels les plus frequents. Une tumefaction frontale etait notee chez 9 patients; associee dans 5 cas a une tumefaction orbitaire au niveau de l'angle interne de l'oeil. Les signes rhinologiques etaient domines par la rhinorrhee purulente (9 cas) et l'obstruction nasale (5 cas). L'endoscopie nasale trouvait du pus au niveau du meat moyen dans 12 cas. La TDM a revele un aspect typique d'osteite frontale sous forme d'un foyer d'osteolyse. Un abces sous-perioste etait associe chez 7 patients. Une extension intracranienne a ete notee dans 15 cas et orbitaire dans 7 cas. L'examen bacteriologique a mis en evidence un germe dans 8 cas. Les germes les plus frequents etaient le streptocoque et le staphylocoque aureus (3 cas pour chacun). Le traitement antibiotique a ete instaure d'emblee chez tous les patients. Pour le traitement chirurgical initial; 9 patients ont beneficie d'un drainage frontal et 2 autres d'un drainage orbitaire. Par ailleurs; 4 patients ont eu un drainage d'abces sous perioste seul. Celui-ci a ete associe a une cranialisation d'emblee dans un autre cas. Pour les patients ayant des complications endocraniennes; un drainage d'empyeme a ete realise dans 14 cas; associe chez 6 d'entre eux a une cranialisation d'emblee. Un drainage d'abces cerebral a ete pratique chez un autre patient. L'evolution clinique et radiologique a ete favorable chez 24 patients (77;4). Les sept autres patients ont necessite une reprise chirurgicale devant la persistance ou l'aggravation de la symptomatologie clinique. L'evolution ulterieure a ete favorable. Conclusion : L'osteite frontale post-sinusitique est une affection rare et grave. Son diagnostic; qui repose sur la clinique et l'imagerie; doit etre precoce. Un traitement adequat doit etre instaure afin de prevenir des complications qui peuvent mettre en jeu le pronostic vital

[Devic's neuromyelitis optica in children: a case report and review of the literature]. / Neuromyélite optique de Devic chez l'enfant: à propos de 1 cas, avec revue de la littérature.

UNLABELLED: Devic neuromyelitis optica (NMO) or Devic's syndrome is an uncommon clinical syndrome associating unilateral or bilateral optic neuritis and transverse myelitis. Usually reported in adults, childhood cases constitute a distinctive clinical entity. CASE REPORT: We report a case of NMO occurring in a 9-year-old girl, admitted for paraplegia, sphincter troubles as acute installation bladder retention and of a sudden decline of the visual acuity. Magnetic resonance imaging (MRI) revealed abnormalities of spinal cord signal with hypo intensity in T1-weighted images and hyper intensity in T2-weighted images along the spinal cord. However, the cerebral region was normal. Visual evoked potentials were consistent with retrobulbar optic neuropathy. Our patient received corticosteroids (methyl prednisolone) during 5 days followed by oral prednisone. At week three, an immunosuppressant (azathioprine) was added. Clinical outcome was favourable with disappearance of sphincter troubles, a correction of the visual acuity and a progressive disappearance of motor troubles. CONCLUSION: Pediatric Devic's NMO is rare. It is a different clinical entity with an excellent visual and neurological prognosis. Review of the literature shows that recurrence is rare in children and seems to be without long-term sequelae with corticosteroids and immunosuppressant therapy.

[Imaging of rhinocerebral mucormycosis]. / L'imagerie dans la mucormycose rhinocérébrale.

PURPOSE: Rhinocerebal mucormycosis is a rare life threatening fungal infection observed in immunocompromised patients. We report six cases of patients with rhinocerebral mucormycosis confirmed histologically. Our study confirms the necessity of early diagnosis when clinical and CT findings are suggestive. MATERIALS AND METHODS: This is a retrospective study including 6 diabetic patients (3 women and 3 men) aged from 28 and 63 years. Five patients had ethmoiditis evolving for a few days (3 to 5 days), and one patient was in an ketoacidotic coma and had a severe infectious syndrome with purulent rhinorrhea evolving for 4 days. All of our patients underwent computed tomography (CT) scan of the paranasal sinuses. MRI was performed in two patients with neurological findings. RESULTS: Unilateral ethmoido-maxillary sinusitis was noted in 5 cases. Only one case of pansinusitis was found. All patients presented orbital involvement. Cerebral involvement was noted in 4 cases (cerebral venous thrombosis: 2 cases; abscess: 2 cases; cerebral ischemia: 2 cases). The diagnosis of mucormycosis was based on endonasal biopsy. When available, MRI allowed a more precise evaluation of the orbital and cerebral extension. CONCLUSION: Mucormycosis is an opportunist mycosis due to mucoralis fungus. It is very invasive with a highly aggressive potential in diabetic or immunocompromised patients. Imaging study particularly CT scan, plays an important role in diagnosis especially to evaluate cerebral extension.

[Nasal angiocentric T cell-natural killer cell lymphoma with pancreatic localisation in a child]. / Lymphome N.K./T. nasal angiocentrique avec localisation pancréatique chez un enfant.

The nasal angiocentric T-cell-natural killer cell lymphoma is an aggressive tumor, exceptional in Tunisia, which is observed especially in Asia, Mexico and South America. We report the case of an 11-year-old boy, presenting with an exuberant and ulcerative tumor of the right nasal pit. Radiological investigation evidenced a right ethmoïdo-frontal tumor. Evolution was characterized by the installation of abdominal pain and fever, the evidenciation of a mass within the head of the pancreas that compressed the extra hepatic biliary duct and Wirsung's canal and was associated to hyperamylasemia. Biopsy under ultrasound revealed a histiocytic nature. Nasal biopsy found histiocytic and lymphocytic cells and led to a diagnosis of rhinoscleroma. The absence of improvement under antibiotic treatment and the development of fever with leuconeutropenia oriented towards the diagnosis of a non-Hodgkinian malignant lymphoma. Medullary biopsy and immunohistochimic study confirmed a T/NK cells lymphoma, with medullary invasion. The child died from acute respiratory distress syndrome. The authors insist on the rarity of this bipolar location, in particular in children, emphasize the difficulty of the diagnosis and review the literature.

Association of severe autosomal recessive osteopetrosis and Dandy-Walker syndrome with agenesis of the corpus callosum.

A severe form of autosomal recessive osteopetrosis associated with Dandy-Walker syndrome and agenesis of the corpus callosum is reported in a full-term boy born to consanguineous parents. The diagnosis was made shortly after birth. Clinical features were cranio-facial dysmorphy, macrocephaly, hepatosplenomegaly, severe anemia and thrombocytopenia. Skeletal radiographs revealed generalized increase in bone density and abnormal metaphyseal remodeling. Cranial ultrasonogram and computed tomography scan showed Dandy-Walker syndrome, agenesis of corpus callosum and hydrocephalus. The patient rapidly developed severe medullary deficiency and a severe pulmonary infection. He died at the age of 2 months. This association seems extremely rare and was not previously reported in the literature.

[Amniotic band syndrome. Case report]. / La maladie des brides amniotiques. A propos d'une observation.

Amniotic band syndrome is a rare fetal malformation involving limb ring strictions, cranio-facial and trunck abnormalities. The authors report a case of 5 months old infant with also facial cleft. The plastic reconstructive surgery leads to a better functional result. This affection represents a sporadic disease without genetic causes. The pregnancy ultrasonography visualise fetal abnormalities.

[Fistula of the 4th endobranchial pouch: a case report]. / Les fistules de la 4ème fente endobranchiale: à propos d'une observation.

The congenital fistula of the 4th branchial pouch is rare. Clinical and therapeutic difficulties are often found. The authors describe a new case of fistula of the fourth endobronchial pouch and precise the embryogenesis, the clinical diagnosis and the surgical treatment.