[An intracranial haemorrhage in a febrile newborn]. / L'image du mois. Une hémorragie intracrânienne chez un nouveau-né fébrile.

A 15 days old newborn was admitted to the emergency room for a recent fever onset. In newborns, the priority is to rule out any bacterial infection and initiate broad spectrum antibiotics. The hemorrhagic appearance of the cerebrospinal fluid after lumbar puncture can sometimes be the only sign to suspect, other than obvious infectious aetiology, an intracranial haemorrhage.

: Un nouveau-né de 15 jours est admis en salle d'urgence pour de la fièvre. Au vu de l'immaturité immunitaire des nouveau-nés et selon les recommandations internationales, la priorité est d'exclure une infection d'origine bactérienne et d'administrer une antibiothérapie intraveineuse empirique. Lors de la réalisation du bilan étiologique, l'aspect hémorragique du liquide céphalo-rachidien peut être le témoin d'un syndrome méningé d'étiologie autre qu'infectieuse, et notamment d'une hémorragie intracérébrale.

[A rare cause of respiratory distress in the newborn]. / Une cause rare de détresse respiratoire du nouveau-né.

Respiratory distress in the newborn represents a real medical challenge from both the etiological and therapeutic points of view. The causes of respiratory distress are usually divided into two categories: upper and lower airway impairments, with the glottis as the reference level. Concerning the upper airway impairment in the newborn, nasal malformations are one of the main causes, choanal atresia in particular and more rarely congenital nasal pyriform aperture stenosis. The latter can be symptomatic in a variable degree, depending on the severity of the obstruction. Moreover, the potential syndromic character with the association of other malformations, such as single median incisor or intracranial midline anomalies, should systematically lead to the realisation of a cerebral MRI. Several lines of treatment are available depending on the clinical response ranging from conservative treatment to surgical intervention.

La détresse respiratoire du nouveau-né représente un véritable enjeu médical tant du point de vue étiologique que thérapeutique. On regroupe habituellement en deux catégories les causes de détresse respiratoire : les atteintes hautes et les atteintes basses avec, comme point de référence, le plan glottique. Concernant les atteintes hautes chez le nouveau-né, les malformations nasales sont une des principales causes, notamment l'atrésie des choanes et, plus rarement, la sténose congénitale des orifices piriformes. Cette dernière peut être symptomatique de manière variable en fonction de la gravité. De plus, le caractère syndromique potentiel avec l'association à d'autres malformations, comme l'incisive médiane unique ou des anomalies intracrâniennes de la ligne médiane, doivent systématiquement conduire à la réalisation d'une IRM cérébrale. Plusieurs lignes de traitement sont disponibles en fonction de la réponse clinique allant d'un traitement conservateur jusqu'à une intervention chirurgicale.

[From surgery for esophageal atresia in the infancy to late complications of ib fusion and scoliosis]. / L'image du mois. D'une chirurgie pour atrésie de l'oesophage dans l'enfance à des complications tardives de type fusion costale et scoliose.

Following surgey for esophageal atresia, orthopedic complications can sometimes appear late in childhood, hence the importance of regular monitoring. These complications include rib fusion secondary to thoracic surgery and possible infectious complications. Undiagnosed, this condition can lead to severe scoliosis in adolescence.

Après une chirurgie thoracique pour une atrésie de l'œsophage, les complications orthopédiques peuvent apparaître parfois tardivement durant l'enfance, d'où l'importance d'un suivi régulier. Parmi ces complications figure la fusion costale secondaire à l'intervention chirurgicale et aux éventuelles complications infectieuses. Non diagnostiquée et traitée, cette complication peut être responsable d'une scoliose sévère à l'adolescence.

[Congenital cystic adenomatoid malformation]. / L'image du mois. Malformation adénomatoïde kystique congénitale.

Cystic adenomatoid malformations are part of localized malformations of lung tissue. They are characterized by the presence of cysts. The diagnosis is typically made during the second trimester of pregnancy. Altough ultrasound of the third trimester can show normalization, it is essential to complete the medical investigation during the first months of life with a computed tomography of the chest. In asymptomatic forms, performing surgical resection is a matter of discussion and, when a conservative treatment is chosen, radiological monitoring should be performed during the entire life.

Les malformations adénomatoïdes kystiques font partie des malformations localisées du tissu pulmonaire. Elles se caractérisent par la présence de kystes. Le diagnostic est souvent posé précocement durant le 2ème trimestre de la grossesse. Bien que l'échographie du 3ème trimestre puisse se normaliser, il est indispensable de compléter le bilan durant les premiers mois de vie par une tomodensitométrie thoracique. Dans les formes asymptomatiques, la réalisation d'une exérèse chirurgicale est discutée, et lors du choix d'un traitement conservateur, une surveillance radiologique doit être réalisée à vie.

[Cytotoxic lesion of the corpus callosum (CLOCC) in a context of viral meningitis]. / Le cas clinique du mois. Lésion cytotoxique du corps calleux (CLOCC) dans un contexte de méningite virale.

We report the case of a young patient of 16 years admitted in the emergency department for headache, nausea and vomiting, of brutal installation. After clinico-biological confrontation, the diagnosis of viral meningitis (aseptic) was made. During the initial assessment several complementary examinations and various brain imaging exams (CT, MRI) were performed in the course of the treatment, showing a focal lesion of the splenium of the corpus callosum, with transient aspect and spontaneously resolving during iterative control. MRI is the modality that formally revealed this callosal lesion. In terms of signaling behavior, this lesion is characterized by an hyperintensity on FLAIR/T2 weighted sequence and a restriction of diffusion (cytotoxic edema). No other signaling abnormalities or malformative lesions are found. The iconographic diagnosis of «cytotoxic lesions of the corpus callosum¼ (CLOCC for «Cytotoxic lesion of the corpus callosum¼) was made.

Nous rapportons le cas d'une jeune patiente de 16 ans admise dans le service des Urgences pour céphalées, nausées et vomissements, d'installation brutale. Après confrontation clinico-biologique, le diagnostic de méningite virale (aseptique) est posé. Lors du bilan initial, plusieurs examens complémentaires et imageries cérébrales (TDM, IRM) ont été réalisées dans le décours de la prise en charge, démontrant une lésion focale du splénium du corps calleux, d'aspect transitoire et spontanément résolutive, lors de contrôles itératifs. L'IRM est la modalité qui a permis de mettre en évidence, de manière formelle, cette lésion calleuse. En termes de signal, cette lésion est caractérisée par une hyperintensité sur les séquences FLAIR/T2 et une restriction de la diffusion (œdème cytotoxique). Aucune autre anomalie de signal ou lésion malformative n'ont été mises en évidence. Le diagnostic iconographique de «lésion cytotoxique du corps calleux¼ (CLOCC pour «Cytotoxic lesion of the corpus callosum¼) a été posé.

[Hereditary hemorrhagic telangiectasia and pulmonary arteriovenous malformations in children]. / Le cas clinique du mois. Maladie de Rendu-Osler et malformations artério-veineuses pulmonaires chez l'enfant.

Hereditary hemorrhagic telangiectasia is a constitutional vascular dysplasia characterized by chronic epistaxis, mucocutaneous and visceral telangiectasias and arteriovenous malformations. Apart from family screenings, the disease is rarely diagnosed during the pediatric age given the late advent of typical clinical symptoms. Nevertheless, arteriovenous malformations are sometimes already present at a young age with significant morbidity risk. Therefore, it is important to establish an early diagnosis. We describe two pediatric cases of hereditary hemorrhagic telangiectasia and pulmonary arteriovenous malformations with divergent clinical presentation.

La maladie de Rendu-Osler, ou télangiectasies hémorragiques héréditaires, est une dysplasie vasculaire constitutionnelle. Elle se caractérise par des épistaxis spontanées et récidivantes, des télangiectasies cutanéo-muqueuses et viscérales et des malformations artério-veineuses. En dehors d'un dépistage familial, cette maladie est rarement diagnostiquée à l'âge pédiatrique étant donné l'apparition tardive des symptômes cliniques typiques. Cependant, les malformations artério-veineuses sont parfois présentes dès le plus jeune âge avec des risques importants de morbidité, d'où l'importance d'un diagnostic précoce. Nous décrivons deux cas pédiatriques de maladie de Rendu-Osler et de malformations artério- veineuses pulmonaires avec des présentations cliniques très différentes.

MR enterography in children with Crohn disease: results from the Belgian pediatric Crohn registry (Belcro).

INTRODUCTION: Magnetic Resonance enterography (MRE) is an imaging modality avoiding ionizing radiation and the discomfort associated with enteroclysis. The results of MRE at diagnosis in the patients of the Belgian pediatric Crohn registry (Belcro) are compared to endoscopical and histological results. METHODS: Results of MRE, endoscopy and histology were obtained from the medical charts and assigned to one of the following segments: jejunum, ileum, ascending colon, transverse colon, descending colon or rectosigmoid. MRE images were reviewed in a blinded way by 4 radiologists with specific interest in pediatric MRE. RESULTS: From the Belcro registry, twenty-two patients underwent a MRE during their work-up for Crohn disease. The results of endoscopy, histology and MRE were concordant (either all negative or positive) in the ileum in 16/18 patients and in the rectosigmoid, descending colon, transverse colon and ascending colon in resp 9, 8, 8 and 8/22 patients. In the non-concordant cases (MRE colon negative but endoscopy and/or histology positive), MRE could not reflect the subtle endoscopic or histologic lesions such as erosions that were described.In 4 cases where ileocaecal valve intubation was impossible ileal MRE findings were abnormal. MRE detected ileal stenosis, jejunal lesions and fistula in resp 4/22, 3/22 en 2/22 patients. The 100% and 75% interobserver agreement was resp 50-82% and 773-100% according to the different intestinal segments. CONCLUSIONS: MRE is a promising imaging modality avoiding radiation in Crohn disease. It should probably become the technique of first choice for the evaluation of extensive small bowel disease in children with Crohn disease.

Primary bone lymphoma in a 10-year-old boy.

Primary bone lymphoma has been defined as a solitary lesion in bone, without concomitant involvement of the extra osseous hematopoietic system, with no evidence of extra osseous disease within 6 months of the onset of symptoms. The vast majority of cases are of the large B-cell non-Hodgkin type. They are rare bone tumor. Distinguishing primary bone lymphoma from other bone tumors is important because the former has a better response to therapy and a better prognosis.

Retroperitoneal abscess from dropped appendicolith complicating laparoscopic appendectomy.

Abscesses can occur after appendectomy. With the increasing use of laparoscopy, this risk has increased in the same way as the incidence of abscesses related to dropped gallstones increased after laparoscopic cholecystectomy. However, this occurrence has been rarely reported. We describe here the case of a young patient who developed retroperitoneal abscess one year after laparoscopic appendectomy.

[Clinical case of the month. Nutcracker syndrome in association with a painful nephrologic disease]. / Le cas clinique du mois. L'anatomie au secours des cliniciens face à une douleur répétitive abdominale: le syndrome de la pince aorto-mésentérique.

Compression of the left renal vein between the aorta and the superior mesenteric artery causes a physiological condition, the so-called nutcracker phenomenon, but it can sometimes lead to left venous hypertension, or "nutcracker syndrome". Classical manifestations of which are an association of left flank pain, unilateral proteinuria and unilateral hematuria, without renal impairment. We report an atypical association of nutcracker syndrome with IgM nephropathy.

[Spondylocostal dysostosis: a rare genetic disease]. / Dysostose spondylo-costale: une maladie génétique rare.

Spondylocostal dysostoses represent a group of very rare genetic disorders, characterised by vertebral and costal segmentation defects, sometimes accompanied by visceral malformations. The major gene involved is DLL3, on chromosome 19. A mutation may lead to a somitogenesis defect, with segmentation defect of axial skeleton and deformations. Depending on the nature of the mutation of DLL3, spondylocostal dysostosis is transmitted as an autosomal dominant (less severe) or autosomal recessive trait (often more severe, but non lethal). Spondylocostal dysostoses must not to be confused with the Jarcho-Levin spondylothoracic dysostosis, a severe, autosomal recessive syndrome. Its most typical aspect is the crab-like appearance of the rib cage leading to major respiratory disorders. Death, due to respiratory insufficiency, usually occurs before the age of two, most often during the first few months. At this time, guidelines for treatment do not exist. We report a case of spondylocostal dysosotosis in a patient born to consanguineous turkish parents, and review the clinical and genetic data on that group of skeletal disorders.

Primary intraventricular brain abscess.

We report a primary intraventricular brain abscess in a 13-year-old boy. We discuss possible explanations for this rare occurrence.

[Retrocaval ureter in a 10-year-old boy]. / Uretère rétrocave chez un enfant de dix ans.

Retrocaval ureter is an uncommon abnormality of the inferior vena cava, which is rarely detected in the child due to its non-specific symptomatology. Despite a varying state of severity, chronic ureteral obstruction generally leads to the deterioration of renal function during adulthood. Treatment depends on the symptomatology in question, and surgical management should be as conservative as possible.

[Diagnosis and follow-up of a large intrahepatic portocaval fistula in a newborn]. / Diagnostic et suivi d'une large fistule porto-cave intrahépatique chez un nouveau-né.

Portocaval fistulas are rare and only exceptionally discovered in newborns. We report the case of a large portocaval fistula associated with portal hypoperfusion detected at Doppler US imaging in an otherwise asymptomatic 5 week old infant. The patient remained asymptomatic over the following two years. At that time, preoperative angiogram showed a normal portal venous system and the fistula was surgically closed. Postoperative US showed a normal and patent portal system, without evidence of portal hypertension.

Obstruction of unusual origin in a small preterm baby-girl.

Intussusception is a rare cause of obstruction in the neonate. The authors report the case of a preterm babygirl operated at D8 for total rupture of the distal ileum proximal to intussusception. They suggest that the great prematurity might be responsible for this lesion similar to small bowel atresia secondary to antenatal intussusception.

A scoring system in predicting the risk of intestinal stricture in necrotizing enterocolitis.

Of 46 infants with a diagnosis of necrotizing enterocolitis (NEC) admitted to the neonatal intensive care unit over the period 1981-1985, 40 have been followed from 2 to 6 years after the acute episode. A contrast enema (CE) to look for intestinal strictures (IS) was performed either during the first months in surgically managed patients, or between 2 and 6 years in asymptomatic patients. Clinical, laboratory and radiology parameters collected during the 7 days following NEC were used to establish a score which was correlated with radiological data obtained after CE. Of the 40 infants, 17 developed symptomatic or asymptomatic IS and 16 of these 17 infants has a score greater than or equal to 7. Nineteen of the 23 patients without IS had a score less than 7. We conclude that the proposed score established on day 8 after onset of NEC helps to identify infants at higher risk of developing IS and for whom closer follow up appears necessary.