[Prenatal diagnosis of abdominal wall defects]. / Diagnostic anténatal des anomalies de la paroi abdominale antérieure.

Anterior abdominal wall defects (AAWD) correspond to a wide spectrum of congenital defects affecting 6.3/10,000 pregnancies. They have in common a closure defect of the anterior abdominal wall and can be fatal or expose the fetus and the neonate (NN) to many complications. This study was based on a retrospective study of 22 cases of AAWD collected between May 2009 and December 2014. Its purpose was to specify the importance of prenatal ultrasonography in the diagnosis and prognosis assessment of these defects. These 22 AAWDs consisted in 13 cases of omphalocele (including four cases of Beckwith-Wiedemann syndrome), four of gastroschisis, one of pentalogy of Cantrell, three of vesical exstrophy and one of cloacal exstrophy. Prenatal ultrasonography provided the diagnosis of 14 of these defects with a changing sensitivity with the gestational age varying from 17% in the first trimester to 71.4% and 77.8% in the second and third trimesters, respectively. The relevance of this examination was improved when performed by an imaging specialist. The prenatal diagnosis of these defects indicated an amniocentesis in eight cases, allowing the diagnosis of two cases of trisomy 18. It also motivated a therapeutic termination of the pregnancy (TTP) in ten cases. Prenatal ultrasonography allowed better prenatal follow-up and planning of the delivery of the continued pregnancies. It indicated an emergency C-section in only one case by showing intestinal complications of gastroschisis. Four NNs died (two cases of omphalocele and two of gastroschisis), three of which postoperatively and the prenatal diagnosis did not improve survival. Prenatal ultrasonographic diagnosis provided a precise morphological study determining the type of the AAWD, a complete malformation assessment, and the prognosis factors. This resulted in adequate multidisciplinary pre and postnatal care, including a rigorous ultrasound follow-up, a TTP in case of associated defects, and emergency delivery once the complications of poor diagnosis are detected.

[Congenital cholesteatoma in children]. / Cholestéatome congéntial.

OBJECTIVES: To report on two cases of congenital cholesteatoma in patients aged 1 month. To review the clinical and radiological features of congenital cholesteatoma and to clarify the contribution of the CT scan in the diagnosis and the preoperative workup. PATIENTS AND METHODS: The authors report two observations of congenital cholesteatoma. These children were investigated using high-resolution CT. The treatment was surgical in both cases. RESULTS: The children were 1 month old, presenting with facial paralysis. In the otoscopic exam, the tympanic membrane was normal in both cases, with a whitish retrotympanic soft mass. The diagnosis of congenital cholesteatoma was made based on the CT data. CONCLUSION: Congenital cholesteatoma is rare. Otoscopic examination must be undertaken during the physical exam of a child at a young age to establish an early diagnosis. CT scan allows positive diagnosis and a precise preoperative workup.

Primary hydatid disease of the thigh. A rare location.

Primary muscle hydatidosis is very rare, accounting for less than 1% of hydatid cyst locations. Clinical symptoms are insidious and non-specific causing a frequent delay in diagnosis. Intramuscular hydatid disease can cause a variety of diagnostic problems, especially in the absence of typical radiologic findings. We report the observation of an 82-year-old man consulting for inguinal tumefaction with radiological exploration suggestive of hydatid cyst of the adductors muscles. Magnetic resonance imaging (MRI) is helpful in diagnosis, since it reveals a very suggestive aspect and demonstrates the relationship between cysts and adjacent structures. Treatment of muscle echinococcosis is based on surgery, which is curative and incurs a low risk of local relapse.

[Isolated hypophyseal tuberculoma: often mistaken]. / Tuberculome hypophysaire isolé: un diagnostic souvent oublié.

Hypophyseal tuberculoma is extremely rare and difficult to diagnose without a clearly suggestive context. Its radiologic features are not specific and are better recognized on MRI with a mass of a variable signal related to the percentage of caseous necrosis. A frequently associated thickening of the pituitary stalk suggests tuberculosis, requiring the search for another tuberculosis location. This paper reports an original case of hypophyseal tuberculoma since it occurred with no other tubercular infection, which delayed the diagnosis despite thickening of the pituitary stalk on MRI.

[Osteogenesis imperfecta and hearing loss in childhood]. / L'ostéogenèse imparfaite, cause rare de surdité chez l'enfant.

We report a case of bilateral hearing loss in a child, caused by osteogenesis imperfecta and we evaluate CT scan findings. A 12-year-old child consulted for bilateral hearing loss. A computed tomography scan of the petrous temporal bone showed bilateral extensive unmineralized bone involving the cochleae, vestibules, and semicircular canals extending to the internal auditory canals. Osteogenesis imperfecta of the temporal bone is a genetic connective tissue disorder with increased bone fragility, low bone mass, and other extraskeletal manifestations. Hearing loss is rare in the first 2 decades of life, but it is one of the frequent features of this disorder in adult patients. Thin-section CT scans of the temporal bone show a remarkable proliferation of unmineralized bone involving the otic capsule. This demineralization is similar to that observed in the cochlear form of otospongiosis.

[Maxillo-facial location of hydatic cyst]. / Kyste hydatique du massif facial.

OBJECTIVE: A maxillofacial location of a hydatid cyst is rare, accounting 2% of cases. The purpose of this study was to review clinical and radiological features by emphasizing diagnostic difficulties. MATERIALS AND METHODS: Two observations of hydatid cyst with a maxillofacial location are presented. Two cases were investigated by panoramic dental radiography, an ultrasound and CT scan. MRI was done in one case. RESULTS: The first observation reports a 23-year-old woman who consulted for jugal tumefaction with pharyngeal extension evolving in spurts. The second observation was a 16-year-old girl, which consulted for a genian and masseter tumefaction with a tight trismus. In both cases, the imaging studies suggested the diagnosis of the hydatid cyst confirmed by the anatomopathologic exam. CONCLUSION: The hydatid cyst is an endemic parasitic pathology involving most often the lung and the liver. The maxillofacial location is exceptional. In cases of a cyst mass in an endemic zone, the diagnosis of the hydatid cyst must be entertained. CT scan and MRI provided a complete lesion workup. Treatment is surgical.

[Osteoid osteoma of the talus and simple bone cyst of the homolateral calcaneus. A rare association]. / Ostéome ostéoïde du talus et kyste osseux essentiel du calcanéus homolatéral. Une association rare.

Osteoid osteoma and simple bone cyst are readily observed in long bones, but are much less common in the short bones of the foot. The association of these two tumors in the same foot is exceptional. We report the case of a 15-year-old girl who presented an osteoid osteoma of the talus and a simple bone cyst of the calcaneus of the left foot. The patient complained of pain in the rear foot for four months which worsened at night and was of the inflammatory type. The physical examination was normal. Standard x-rays revealed a simple bone cyst in the calcaneus which could not explain the nighttime inflammatory pain. Scintigraphy and computed tomography of the left foot revealed an osteoid oseoma of the talus. Biopsy total resection of the osteoid osteoma with curettage and filling with cancellous bone of the simple bone cyst were performed. The patient has been free of recurrence at three years follow-up.

[Retrovesical hydatid cyst in children: report of 3 cases]. / Le kyste hydatique rétrovésical chez l'enfant: à propos de trois cas.

Retrovesical hydatid cyst is rare, even in endemic regions. From a presentation of three cases, the authors will review the clinical findings and illustrate the imaging features of this pathology and relate diagnostic and therapeutic difficulties.