Visceral and mucosal involvement in neonatal haemangiomatosis.

BACKGROUND: Two types of neonatal haemangiomatosis (NH) are distinguished: diffuse which is associated with a high rate of mortality linked to mucosal/visceral involvement, and benign. OBJECTIVES: First, this study aimed to examine the frequency of mucosal and visceral (especially hepatic) involvement in NH, according to skin extension, and second, it aimed to examine clinical, pathological (with glucose transporter 1 (GLUT-1) immunostaining), and imaging features of NH, including follow-up data. METHODS: This was a descriptive retrospective study carried out in the University Hospital Center of Tours, France. RESULTS: The study included 19 patients with cutaneous NH (number of skin haemangiomas ranging from 5 to >100). Mucosal involvement was observed in 32% of all cases (100% and 19% in diffuse and other cutaneous cases respectively) and hepatic involvement in 42% (67% and 38% respectively). The number of hepatic haemangiomas ranged from 1 to >10. Half of the hepatic haemangiomas cases exhibited increased hepatic arterial blood flow. CONCLUSIONS: Mucosal and hepatic involvement was frequent in cases with a high number of cutaneous haemangiomas (>100), but only frequency of mucosal involvement was statistically significant (P = 0.021).

[Imaging of anorectal malformations in the neonatal period]. / Imagerie des malformations anorectales en période néonatale.

PURPOSE: The classification of anorectal malformations (ARM) as high or low is based on clinical and anatomical characteristics. It has an impact on the surgical management but also on the functional prognosis. In the absence of consensus, our goal was to determine the value of imaging in the neonatal period for diagnosis and management of infants with ARM. MATERIALS AND METHODS: Retrospective study of 43 infants imaged over a 9 year period. The different imaging studies (abdominal and pelvic ultrasound, radiographs, percutaneous opacifications and fistulograms) performed for each infant were collected and analyzed then correlated to clinical and surgical findings. RESULTS: Clinical evaluation could classify 30 ARM as low and 4 ARM as high while 9 ARM without fistula remained indeterminate. Imaging findings were mixed: on ultrasound, the rectal cul-de-sac to perineum distance did not appear to be determinant, contrary to published data. Pelvimetry showed limitations, irrespective of the technique. Morphological evaluation provided the following data: presence of fistula, sphincter anatomy, rectal cul-de-sac to perineum distance. CONCLUSION: Classification relies on clinical evaluation in most cases. Opacification techniques and ultrasound remain useful in some cases. MRI could become the preferred imaging modality.

[Madelung deformity due to abnormal radiolunate ligament: report of 4 cases]. / Déformation de Madelung de l'enfant liée à la présence d'un ligament radio-lunaire anormal: à propos de 4 cas.

PURPOSE: To define the value of CT and MR in the demonstration of an abnormal radiolunate ligament contributing to a Madelung deformity and emphasize the features on wrist radiographs suggesting the presence of such ligament. OBSERVATIONS AND RESULTS: Four cases of adolescents presenting with uni- or bilateral wrist pain are reviewed. Standard radiographs demonstrated the present of isolated Madelung deformity with increased inferior radioulnar curvature with triangularization of epiphyses and decreased carpal angle. Each patient underwent either noncontrast CT, CT arthrography or MRI. All cross-sectional imaging studies demonstrated the presence of an abnormal radiolunate ligament with radial insertion within a bony gutter. Retrospectively, the bony gutter could be identified on radiographs. In two cases, excision of the ligament provided partial correction of the deformity and symptoms, without need for classical osteotomy. CONCLUSION: In patients with Madelung deformity, CT or better yet non-ionizing and noncontrast MR may demonstrate a abnormal radiolunate ligament within a bony gutter. Detection of this gutter on radiographs suggests the presence an abnormal ligament. Early release of the abnormal ligament appears to decrease wrist deformity, improve range of motion and reduce symptoms.

A Rare Complication of Central Venous Catheter Extravasation in a Preterm Neonate: Hemidiaphragmatic Paralysis.

We report a case of a preterm neonate born at 26 weeks' of gestation diagnosed with unilateral diaphragmatic paralysis. This paralysis was a consequence of a phrenic nerve injury due to extravasation of hyperosmolar parenteral nutrition fluid in the upper thorax. Chest X-rays and ultrasonography confirmed the diagnosis. The neonate was treated with prolonged respiratory support and did not require surgical treatment. This report describes a case of hemidiaphragmatic paralysis as a complication of central venous catheter insertion. In neonates, spontaneous recovery of diaphragmatic paralysis is possible. This study concludes that recovery of extravasation injury-induced phrenic nerve palsy in the context of conservative management is possible.

A single enhanced phase is sufficient for the initial computed tomography evaluation of retroperitoneal tumors in children.

PURPOSE: The goal of this study was to evaluate the incremental value of unenhanced phase in the initial evaluation of retroperitoneal tumors in children by comparison with an enhanced phase alone using computed tomography (CT). MATERIALS AND METHODS: A total of 53 patients (26 girls, 27 boys) with a total of 53 tumors who had CT examination of the abdomen and pelvis for the initial assessment of retroperitoneal tumor were retrospectively included. All CT examinations were obtained with an unenhanced set of CT images and a set of CT images obtained after intravenous administration of iodinated contrast material. One junior and one senior radiologist independently evaluated the two sets in two separate reading sessions. CT images were analyzed for tumor calcifications, tumor location, vascular encasement, local invasion and tumor content. RESULTS: Calcifications were present in 24/53 tumors (45%). On the enhanced set, the senior radiologist was able to detect calcifications in 22/24 tumors (92%) and the junior radiologist in 20/24 tumors (83%), yielding sensitivities of 92% and 83%, and specificities of 96.5% and 100%, respectively. Inter-observer agreement was excellent (Kappa=0.89). Tumor location was correctly determined by the senior radiologist in 53/53 tumors (100%) and 37/53 tumors (70%) by the junior radiologist. Using the unenhanced set, the senior radiologist was able to assess vascular encasement in 26/53 tumors (49%) against 21/53 (39%) for the junior radiologist. For tumor content, agreement between the enhanced and combined unenhanced and enhanced CT was 77% for both radiologists. CONCLUSION: Enhanced CT performs as well as unenhanced CT for evidencing calcifications and is therefore sufficient for the initial assessment of retroperitoneal tumor in children.

[Fetal MRI practices in a university prenatal center]. / Pratique de l'IRM fœtale dans un centre hospitalo-universitaire de diagnostic prénatal.

OBJECTIVES: Fetal MRI is a third intention examination to prenatal diagnosis. If its diagnostic value is well known in many pathologies, its place in the management of pregnancies remains unclear. METHODS: We collected retrospectively demographical, radiological (fetal MRI indications, fetal anatomical region and diagnostic information provided by fetal MRI) and obstetrical data of pregnant patients in university prenatal center during a 5 years' period. RESULTS: Among 2439 patients of the prenatal center, 196 (8%) patients with fetal MRI were included. The main anatomical regions studied were the brain (n=132, 67%), the thorax (n=31, 16%) and the abdomen (n=25, 13%). No cardiac fetal MRI was performed. Ninety-five percent of fetal MRI was consecutively of an ultrasound sign. Fetal brain MRI was abnormal in 65% of cases, the thoracic and abdominopelvic MRI in 81.5%. The ultrasound diagnosis was unchanged in 42%, completed in 50% and redirected in 8% of cases. A termination of pregnancy was deemed admissible in 31% of patients with MRI versus 21% in patients without MRI (P=0.001). CONCLUSION: Fetal MRI requires selective indications and provides additional diagnostic information with important implications for the future of the pregnancy, particularly in case of severe and incurable pathologies. Our results could be useful as a reference basis for the comparison with others prenatal center practices.

[The role of aortic morphology in the occurrence of hypertension in patients undergoing surgery for coarctation of the aorta before the age of 1 year]. / Rôle de la morphologie aortique dans la survenue d'une hypertension artérielle chez des malades opérés d'une coarctation de l'aorte avant l'âge de 1 an.

OBJECTIVE: Hypertension on effort can be observed following surgical treatment of coarctation of the aorta even if the operation has been performed early and has been judged to be satisfactory. The pathophysiology of this hypertension has not been completely elucidated. The aim of our study was to look for a residual morphological anomaly of the aortic arch which might be responsible. METHODS: This was a retrospective study of patients aged over 10 years who had undergone surgery for coarctation of the aorta before the age of 1 year, consecutively between 1979 and 1993, and free from re-coarctation clinically or with Doppler. All of them had a blood pressure effort test on a cycling machine, echocardiography and an MRI of the thoracic aorta. RESULTS: 61 patients were included. Their median age was 15 years, 59% were male, 15% were hypertensive at rest and 56% on effort. With univariate analysis, only the average blood pressure (p=0.03) and the resting pulse pressure (p=0.001) were associated with hypertension on effort. With multivariate analysis the only factors that correlated independently with maximum arterial pressure on effort were the height of the patient (p=0.02) and the pulse pressure (p<0.0001). The surgical technique and the age at intervention were not associated with hypertension on effort (p=0.96 and 0.69 respectively). The diameter of the horizontal aorta and the aorta at the site of repair, measured on MRI, added to the diameter of the descending aorta at the diaphragm were not lower in patients with hypertension on effort (p=0.77 and 0.38). There were proportionally more Roman type aortic arches than Gothic types in patients with hypertension on effort, but this difference was not significant (p=0.18). CONCLUSION: In our study, the patients with hypertension on effort following correction of coarctation of the aorta did not display any residual obstruction at the level of the aortic cross on MRI. However, they had a resting pulse pressure that was significantly higher than the non hypertensive patients, reflecting an increase in the aortic wall rigidity.

[Sonographic cerebral sulcal pattern in normal fetuses]. / Repères échographiques de gyration cérébrale foetale normale.

PURPOSE: Define normal sulcation patterns and their chronological order of appearance on transabdominal ultrasound by comparing them with brain maturation references available in fetopathological studies and MRI findings. PATIENTS AND METHODS: By means of a prospective study, 158 normal fetal brains aged 21 to 34 gestational weeks have been analyzed with standardized data by transabdominal ultrasound in eleven different views using axial, coronal and sagittal orientation. RESULTS: The sequential development of cerebral sulci has been described according to the gestational age. This chronology was consistent with anatomo-pathologic references presenting a mean late period of one week and with MRI but without any late period. This study is available on the following website: CONCLUSION: This ultrasound study provides accurate landmarks and imaging features of normal fetal brain sulcation. The analysis and the knowledge of this sulcation provide better understanding of the brain cortex maturation and may be helpful in diagnosing brain diseases.

[Pericochlear hypodensity on CT: normal variant in childhood]. / Hypodensité tomodensitométrique péricochléaire: variante de la normale chez l'enfant.

PURPOSE: Evaluation of the prevalence of a pericochlear hypodensity on CT in children. Materials and methods. This study correlates the findings on temporal bone CT to the indications for CT (headache, trauma). Helical CT acquisitions using 0,5 mm, 0,6 mm or 0,75 mm slice thickness according to the material available, with multiplanar reconstructions. Only patients with significant pericochlear hypodensity, larger or equal to 0,4 mm, were taken into account, and the curvilinear or nodular nature of the lesion on axial and coronal sections was recorded. RESULTS: A pericochlear hypodensity was identified in nearly 40% of cases. There was no population predominence according to the indication for the CT. The curvilinear type was more frequent on coronal images, with unilateral and bilateral involvement being equally frequent. There was no significant difference according to the sex. Patients of all ages showed lesions, from newborns to adolescents, with increased frequency in childhood. CONCLUSION: This study confirms a high prevalence of pericochlear hypodensity in a paediatric population, without clinical correlation, which requires prudence when interpreting the significance of this CT sign commonly described in association with pericochlear otosclerosis and the pericochlear form of osteogenesis imperfecta.

[Cortical malformations and epilepsy: Role of MR imaging]. / Malformations corticales et épilepsie: apport de l'IRM.

Malformations of cortical development are increasingly recognized as important causes of epilepsy, developmental delay and other neurological disorders. Our purpose is to present the relevance of the MRI in these pathologies with the clinical, genetic and therapeutic aspects. This classification is based on the three fundamental events of cortical formation: proliferation of neurons and glie in the periventricular zone, migration of postmitotic neurons to the periphery, subsequent cortical organization. MR analysis evaluates particularly the cortical thickness, sulcal and cortical morphology, gray-white matter junction, and looks for gray matter in abnormal location. These data coupled with the familial history, the seizure characteristics and genetic findings should allow an appropriate classification of the lesions. MR imaging allows the detection and classification of cortical malformations. MR imaging findings are primordial to consider surgery when the epilepsy becomes refractory to the anti-epileptic drugs. An adequate classification of these malformations should help to provide to the family an appropriate counseling both in terms of genetics and outcome.

[Spasmodic torticollis caused by metoclopramide: a rare etiology of C1-C2 rotatory pseudoluxation in children]. / Un torticolis spasmodique lié à la prise de métoclopramide: une cause rare de pseudoluxation rotatoire C1-C2 chez l'enfant.

Torticollis in children must always instigate a search for trauma. Many other etiologies can be found. The authors report a case of postmedicinal atlantoaxial rotatory pseudoluxation (AARP) occurring in a child. This child had fallen down in the morning with head trauma followed by headache. Clinically, a stiff neck with an irreducible right rotation of his neck, and an osteotendinous hyperreflexia were noted. There was a C1-C2 rotatory dislocation with no traumatic lesion on the cervical CT scan. After a few hours, the torticollis spontaneously reduced and then reappeared on the left side. This clinical fluctuation and the absorption of metoclopramide (Primpéran) started in the morning for acute gastroenteritis provided the diagnosis of AARP. This entity was confirmed by the good clinical and radiological follow-up and was caused by the substantial ligament laxity of the craniovertebral junction encountered in children. The analysis of medical imaging and the systematic search for a medicinal cause helped make the right diagnosis.

[Neurofibromatosis type 1 complications in the pediatric age: follow-up of a hundred cases]. / Complications de la neurofibromatose de type 1 chez l'enfant: à propos d'une série de 100 cas.

UNLABELLED: Neurofibromatosis 1 (NF1) is a frequent genetic disease. Diagnostic criterias were established in 1988. The patients can exhibit various and unpredictable complications. OBJECTIVES: To check the efficiency of a coordinated follow-up in specialized multidisciplinary centers providing a higher quality of management and to have a better knowledge of the complications including their true frequencies. POPULATION AND METHODS: We report a serie of 100 NF1 children who were followed-up during 4 years in a specialized center at the Tours University Hospital. Three hospital check-up at 2-5, 6-7, 14-15 years of age were performed as well as an annual physical examination. RESULTS: In our serie, the mean age was 7.8 years old with a sex ratio of 1. The mean age at diagnosis was 3.8 years old and the main diagnosis criteria were the café-au-lait spots and the family history for 80% of the patients. The optic nerve glioma has a low frequency of 5%. Learning disabilities clearly represent the most frequent complication (46% of the patients). CONCLUSION: An early detection of these difficulties is a priority for the appropriate management of these children.

[Limb emergencies in children]. / Les urgences des membres de l'enfant.

The three main categories of limb emergencies are trauma, infection and the painful hip. Conventional radiography is used as the initial screening imaging modality that can sometimes be complemented with sonography. Computed tomography and MRI are rarely needed. Familiarity with conditions specific to the pediatric population is essential to the diagnosis and management of these patients. Traumatic injuries in children involve growing bones with large cartilaginous component and biomechanical resistance different from that in adults. Lim infections in children are affected by the characteristic vascular supply to the metaphyseal-epiphyseal region, the intra-articular location of the metaphysis, and loose periosteal attachment. Limping (transient synovitis, Legg-Calvé-Perthes disease and slipped capital femoral epiphysis) is frequent cause of consultation and diagnosis relies on the patient's age and plain film findings.

[Aortic diameter measurements on MRI follow-up of coarctation]. / Mesure des diamètres aortiques dans le suivi IRM des coarctations.

OBJECTIVE: During aortic coarctation follow-up, accurate diameter measurements using MRI are required. The purpose of this study was to compare the reproducibility of aortic measurements on axial and sagittal views using black blood (Fast-Spin-Echo) and three-dimensional Gadolinium-enhanced MR angiography. SUBJECTS AND METHODS: We studied 34 patients who underwent evaluation after surgical treatment in the first year of life. The aortic measurements were made at six thoracic levels. We calculated the mean difference between the two sequences, and used Bland and Altman method and the comparison to zero. RESULTS: With black blood, either on axial or sagittal views, aortic measurements were in agreement. Between two sequences, we considered as acceptable a mean difference of 0,5 mm with limits of agreement of +/- 3 mm. Difficulties were found for proximal ascending aorta measurements on all sequences. Using MR angiography, the highest agreement at the site of repair was on sagittal view, while the highest agreement at other sites was on axial views. CONCLUSION: Black blood sequence provides a better reproducibility for aortic measurements than native slices of MR angiography.

MR assessment of the brain maturation during the perinatal period: quantitative T2 MR study in premature newborns.

The purpose of our study is to trace in vivo and during the perinatal period, the brain maturation process with exhaustive measures of the T2 relaxation time values. We also compared regional myelination progress with variations of the relaxation time values and of brain signal. T2 relaxation times were measured in 7 healthy premature newborns at the post-conceptional age of 37 weeks, using a Carr-Purcell-Meiboom-Gill sequence (echo time 60 to 150 ms), on a 2.35 Tesla Spectro-Imaging MR system. A total of 62 measures were defined for each subject within the brain stem, the basal ganglia and the hemispheric gray and white matter. The mean and standard deviation of the T2 values were calculated for each location. Regional T2 values changes and brain signal variations were studied. In comparison to the adult ones, the T2 relaxation time values of both gray and white matter were highly prolonged and a reversed ratio between gray and white matter was found. The maturational phenomena might be regionally correlated with a T2 value shortening. Significant T2 variations in the brainstem (p < 0.02), the mesencephalon (p < 0.05), the thalami (p < 0.01), the lentiform nuclei (p < 0.01) and the caudate nuclei (p < 0.02) were observed at an earlier time than they were visible on T2-weighted images. In the cerebral hemispheres, T2 values increased from the occipital white matter to parietal, temporal and frontal white matter (p < 0.05) and in the frontal and occipital areas from periventricular to subcortical white matter (p < 0.01). Maturational progress was earlier and better displayed with T2 measurements and T2 mapping. During the perinatal period, the measurements and analysis of T2 values revealed brain regional differences not discernible with T2-weighted images. It might be a more sensitive indicator for assessment of brain maturation.

[Videoscopic resection of a thrombosed aneurysm of a patent ductus arteriosus in a neonate]. / Résection par vidéo-thoracoscopie d'un anévrisme thrombosé du canal artériel du nouveau-né.

Aneurysmal dilatation of a patent ductus arteriosus is a rare condition. One hundred and fifty three cases have been reported, of which 59 (39%) were operated, with an operative mortality of 16%. The authors report a case detected in a premature neonate on chest X-ray showing a mass in the mid-mediastinal region. Surgical resection was undertaken by video-thoracoscopy. Peroperative analysis of the resected specimen suggested a vascular structure and this was confirmed by histological study, which showed spontaneous aneurysm of a thrombosed patent ductus arteriosus or cystic residue of a patent ductus arteriousus. The authors underline the importance of peroperative puncture of structures of uncertain origin and the possibility of conversion of such a procedure to that of an extreme emergency.

[Hemangiomas and superficial vascular malformations of the head and neck. Classification, diagnosis, treatment]. / Hémangiomes et malformations vasculaires superficielles de la tête et du cou. Classification, diagnostic, traitement.

Each kind of superficial vascular malformation (or "angioma") has been included in a widespread classification that has been elaborated by means of a multidisciplinary approach. At present, therapeutic management of these vascular malformations often needs one or several procedures of interventional radiology. The purpose of this paper in to illustrate embolization techniques in each indication and to describe advantages and drawbacks of these different techniques.

[Comparison of three fat suppression sequences for the detection of vertebral detection. Turbo STIR, phase contrast gradient-echo, and MISTEC-Chopper after gadolinium injection]. / Comparaison de trois séquences de suppression de graisse pour la détection des métastases vertébrales: Turbo STIR, écho de gradient en opposition de phase et MISSTEC-Chopper après injection de Gadolinium.

OBJECTIVES: Assess three fat suppression sequences used to search for spinal metastases: TurboSTIR, phase contrast gradient-echo, and MISTEC-Chopper after gadolinium injection. MATERIAL AND METHODS: A prospective study was conducted in 10 patients with primary neoplasia. MIR sequences acquired (1 Tesla) were TurboSTIR, T1 spin-echo with and without gadolinium injection, phase contrast gradient-echo and M-Chop after gadolinium injection. Signal intensity in normal bone marrow, metastatic tissue, and subcutaneous fat as well as background noise was measured. Signal-to-noise (S/N) ratio was determined. Lesion borders, artefacts, and extent of detected lesions were determined quantitatively. Bone marrow signal intensity was also recorded. RESULTS: S/N ratio was best with gradient-echo which identified well the borders of lesions within the hemopoietic marrow. For lesions located in high-fat marrow (as in post-radiation marrow), the high intensity signal of the lesion confounded with the fat signal. TurboSTIR gave effective fat signal suppression and was particularly useful for yellow marrow, less so for red marrow. This technique confounded cell proliferation with perilesional edema (enlarging lesion extention). In one case, this sequence did not detect a small lesion visible with the two other sequences. This sequence was sensitive to artefacts (especially vascular artefacts) which can produce false nodular images. M-Chop gave good suppression of vertebral fat tissue (better for yellow marrow) but subjective detection of lesions was more difficult. CONCLUSION: The phase contrast gradient-echo sequence after gadlinium injection appeared to be the best sequence excepting cases of post-trauma (radiotherapy or chemotherapy) fat transformation of the marrow where the TurboSTIR sequence could be preferred.

[Reduction of acute intestinal intussusception: when and how?]. / Invagination intestinale aiguë: la désinvagination, quand et comment?

After sonographic diagnosis, non-surgical therapy for intussusception in children uses pneumatic or hydrostatic (barium or water soluble) reduction. The purpose of this paper, in the light of our experience, is to answer the questions raised by general radiologists in emergency activity about the technique, indications, sedation and risks.