Melnick Needles Syndrome: Computed Imaging and Management Difficulties

Melnick-Needles syndrome is an osteo-dysplasia caused by a mutation of a gene, FLNA, coding for filamine A. It is at the origin of a set of complex congenital malformations, mainly of interest to the members, but also to the cranio-facial region. Melnick-Needles syndrome was first described in 1966 by two Americans John Melnick, a radiologist, and Carl F Needles, a pediatrician. They described cases of families of several generations who had a characteristic severe congenital bone disorder. They thought that human-to-human transmission had occurred in one case. We report a case of Melnick-Needles syndrome which is a pathology with very high mortality especially in a male subject. This was an observational study of a case received in consultation with the pediatric service of the Souro Sanou University Hospital Center in Bobo Dioulasso in Burkina Faso. It was a 3-year-old boy born in a low-term pregnancy without any prenatal consultation, imaging test and prenatal biology performed. The exact pathogenesis of this condition is not established but is linked to a mutation of the Filamine A gene linked to sexual chromosomes X. Pathology is rare, around a hundred cases have been reported worldwide. Its incidence, according to the literature is around 1/100,000. The sex ratio is at 7. The malformations of this acquired embryo-fetopathy are multiple, polymorphic and asymmetrical. The diagnosis of this pathology is suspected by the clinic and established by molecular biology by sequencing the genes responsible for the mutation. However, imagery, in particular the scanner has a major role by its protocol with multi-planar reconstructions and its analysis in double windowing which allow a better description of the malformations of this syndrome. Its management is complex, and multidisciplinary, and the unfortunate prognosis in our context is because of the precarious technical platform. In our case despite the obvious malformations presented by the patient, the parents consulted late in a health center because of the automation and especially the socio-cultural constraints which incriminate this type of polymalformation as being a curse. The patient underwent abdominal surgery and even the operating procedures were simple, he died four months after leaving the hospital, due to an unspecified cause.

Isolated Rupture of Corpus Spongiosum Associated a Partial Rupture of the Urethra during a False Step of the Coitus: A Case Reported at the University Hospital Sanou Souro

Penile fracture is a rare urological emergency. It occurs mainly in young adults during sexual intercourse. In many instances, one of the corpora carvanosus is involved though bilateral injuries with or without corpus spongiosus involvement is not uncommon. However, isolated injury to corpus spongiosus is extremely rare. We report a case of isolated rupture of corpus spongiosum secondary to penile injury during coitus in a 43-year-old man that presented to University Hospital Souro Sanou of Bobo Dioulasso. He presented with history of persistence bleeding per urethra following penile injury during sexual intercourse. The Retrograde urethrography (RUG) showed a partial rupture of urethra, Moore type 3. Complementary penile ultrasound revealed extensive contusion of the urethra with circumferential hematoma and rupture of the distal 1/3 of the corpus spongiosus. He had gentle per urethral catheterization which was left for one month. Penile ultrasound sound done after removal of catheter showed residual injury and narrowing of the urethra.

Imaging Findings of Children Stroke in Burkina Faso and Etiological Approach

Introduction: Stroke is defined as a sudden onset focal neurological deficit of presumed vascular origin. Although it is essentially adult and elderly condition, stroke can occur in children. Their diagnosis essentially radiological is based on two main imaging modalities namely Computed Tomography (CT) and/or Magnetic Resonance Imaging (MRI). Our study aimed to study radiological features of stroke in children with an etiologic approach in three health facilities of Ouagadougou. Material and Method: We conducted a retrospective study with descriptive focus during 11-year period from January 1st, 2006 to September 1st, 2017 among children aged between 02 months and 16 years who underwent brain CT scan and/or MRI;in whom diagnosis of stroke had been established in radiology department of Yalgado Ouedraogo Teaching Hospital (CHU-YO), Schiphra Methodist Medical Center (CM Schipphra) and Saint Camille Hospital of Ouagadougou (HOSCO). Results: 40 cases of pediatric stroke were collected including 23 at HOSCO, 15 at CHU-YO and 03 at CM Schipphra. Mean age was 6.45 years, with extremes of 2 months and 192 months. Unilateral contralateral motor weakness was the most common clinical presentation (32.5%) followed by seizures (17.5%). Ischemic stroke dominated in 82.5% of cases. Sino-venous thrombosis was rare and suspected in only one case. Sylvian artery was mostly affected in ischemic stroke for 56.25% of patients. The main etiologic factors found were hematologic (67.50%) then infectious factors (25%). Sickle cell disease was always incriminated in hematologic factor. Homozygous SS patients were the most numerous (82%). Conclusion: Stroke diagnosis in children is based on etiologic investigation, biology with a major role of medical imaging. Sickle cell disease accounts a lot in occurrence of stroke in children in our context.