Malformations vasculaires au cours du syndrome de Williams-Beuren: à propos de trois nouvelles observations

Le syndrome de Williams-Beuren est une maladie génétique rare, il associe classiquement une dysmorphie  faciale assez spécifique, des malformations cardiovasculaires et un profil neuropsychologique particulier. Nous rapportons les observations de trois enfants atteints du syndrome de Williams-Beuren en insistant surtout sur les anomalies vasculaires observées sur l'angio-scanner et angio-IRM.Key words: Williams Beuren, sténose supra aortique, angioscanner, angio IR

Epilepsy as a Presentation of a Neuroglial Cyst Associated with Dysgenesis of Corpus Callosum in a Child

Neuroglial (glioependymal) cyst is a rare congenital tumor of the central nervous system usually found in childhood. It can be isolated or associated with other brain malformations. Magnetic resonance imaging is the technique of choice for making the diagnosis. We report the case of a 10-year-old child who presented with epileptic seizures revealing a neuroglial cyst and dysgenesis of the corpus callosum

Multiseptate gallbladder in an asymptomatic child: Case report and review of the literature

Multiseptated gallbladder also known as “Honeycomb gallbladder” is a rare condition that was first described by Tandon in 1963. It is described as a congenital anomaly in most of the cases. It may be asymptomatic or may lead symptoms. We present the case of a multiseptate gallbladder in a 5 year old girl who was admitted for managament of acute appendicitis

Symptomatic congenital hemangiomatosis in a neonate: Imaging of a life-threatening presentation with multifocal liver involvement

Hemangiomas are the most common benign vascular neoplasms of infancy. Congenital hemangiomas proliferate in utero, and are fully formed at birth. They are usually solitary. Generalized forms are exceptional. The liver is the second most common site of hemangiomas after the skin. When >5 cutaneous hemangiomas are present, screening abdominal ultrasound is recommended. Based on the degree of liver parenchyma involvement, 3 hepatic hemangiomas’ subtypes are defined: focal, multifocal, and diffuse. Hepatic hemangiomas’ clinical presentation varies from asymptomatic to life-threatening. High output cardiac failure, consumptive coagulopathy, abdominal compartment syndrome, and liver dysfunction are possible complications. We report an unusual case of symptomatic congenital hemangiomatosis in a male infant born with innumerable generalized cutaneous hemangiomas whose screening abdominal ultrasound revealed multifocal hepatic hemangiomas with extensive mixed shunts. We aim to highlight this unique entity with severe associated complications and stress the role of imaging at initial presentation, for follow-up, and to guide therapeutic choices

Ollier disease: A case report and literature review

Ollier disease is an uncommon disease characterized by several enchondromas and an asymmetric distribution of cartilage lesions, which can vary significantly in size, location, age, and gender. The primary symptom of this condition is a nonossifying chondrocyte mass or hamartomatous chondrocyte growth in the metaphysis of a short or long bone. Specific cases can progress to chondrosarcoma or osteosarcoma. X-ray is the most fundamental diagnostic technique for skeletal illnesses. In this article, we present a case of Ollier disease from Mother and Child Hospital IBN SINA, Rabat, Morocco

Myelin insults differentials on MRI in children: In the light of an ADEM case

Acute disseminated encephalomyelitis (ADEM) is an acute and rapidly progressive auto-immune demyelinating disorder in the central nervous system. It is a rare disease but is more frequently observed in the pediatric population. We report a case of a monophasic postvaccination ADEM, which presented with paraparesis associated with fever. It showed a favorable evolution under corticosteroids, without recurrence after 3 years of follow-up. The diagnosis was established due to the postvaccination context and the MRI abnormalities characteristics. This case prompted a general discussion about the etiologies of myelin insults in children, especially demyelinating disorders, by shedding the light on their MRI features

Pituitary stalk interruption syndrome on MRI: Case report

Key clinical message Pituitary stalk interruption syndrome (PSIS) is an antenatal anatomical defect characterized by pituitary insufficiency with symptomatology depending on associated hormonal deficits. Diagnosis is often delayed because many clinical findings. The gold standard for detection is pituitary MRI showing absence of pituitary stalk, anterior pituitary hypoplasia, and postpituitary ectopy. The treatment remains polyhormonal substitution. Abstract Pituitary stalk interruption syndrome (PSIS) is an antenatal anatomical defect. It is characterized by pituitary insufficiency with symptomatology depending on associated hormonal deficits. Diagnosis of PSIS is often delayed probably because of various clinical characteristics findings. Pituitary imaging abnormality is a specific indicator of hypopituitarism. The symptomatological triad associates a very thin or interrupted pituitary stalk, an ectopic or absent pituitary gland and hypoplasia of the anterior pituitary gland. The gold standard for detection is pituitary MRI. Some genetic factors are associated with the disease. The treatment remains polyhormonal substitution depending on the associated deficits. We reported the case of a 14‐year‐old child with growth retardation in whom the biological work‐up and pituitary MRI concluded that the diagnosis was PSIS with growth hormone deficiency. The treatment implemented was a recombinant growth hormone treatment. The immediate outcome was marked by a regression of symptoms

Dyke-Davidoff-Masson syndrome in an 8-year-old child: Report of a case

Dyke-Davidoff-Masson syndrome (DDMS) is a rare entity. Few cases have been described in the literature. It can be symptomatic or asymptomatic. The clinical signs are very varied. Imaging is the key to diagnosis. Calvarial thickening, enlargement sinus, and cerebral hemiatrophy are suggestive signs. It is a cause of cerebral hemiatrophy and epilepsy. We report the clinical and radiological signs of this syndrome through a case of an 8-year-old male child treated for epilepsy. The importance of our article is to report a case diagnosed at an early age (8 years). Most studies report cases diagnosed in adults. MRI revealed pathognomonic signs of Dyke-Davidoff-Masson syndrome

Spinal metastasis of nephroblastoma: Yes it exists

Nephroblastoma or Wilms' tumor is the most common malignant tumor of the kidney in the pediatric population. Metastasis is caused by hematogenous spread. The most common localizations in decreasing order of frequency are lymph nodes, lungs, and liver. The bone is very rarely affected. According to the literature, bone metastases have been described in the iliac bone, skull, and mandible. The vertebral localization was described in 3 cases only, the first 1 in 2009, and the 2 others in 2015 . The goal of our work is to report a very rare case of metastatic vertebral localization of a Wilms' tumor in relapse after treatment; and thus to underline the potential for vertebral and intracanal involvement in nephroblastoma