[Comparison of accreditation procedures, ISO 9000 certification procedures and total quality management. Personal experiences and application of quality assurance in a department of radiology and medical imaging]. / Comparaison des procédures d'accréditation, de certification ISO 9000 et d'évaluation en qualité totale. Expérience personnelle et application à la gestion de la qualité d'un département de radiologie et imagerie médicale.

Management of quality assurance protocols in a radiology department can be done by using several tools or models. Some are specific like accreditation manuals issued by some organizations, others like the ISO 9000 certification and the Total Quality management are more general and already well known by manufacturers. In order to implement a process of quality improvement, we have reviewed three models of quality assurance: evaluation in total quality based on the European model "EFQM", accreditation based on booklets from French cancer centers and Canadian radiology centers and, finally, accreditation based on the ISO 9002 certification model. Based on results of our comparative study, these three tools of quality management are not contradictory and may be complementary. However, they can be compared in terms of constraints they impose, of their historical background, of the criteria evaluated as well as the role of different teams. In conclusion, we suggest that directors of radiology department interested in implementing a quality assurance program first evaluate their department using the Canadian accreditation model issued in 1993 which is useful to become familiar with this new concept of quality. In a second step, a self assessment using the EFQM has to be done in collaboration with all members of the administration board in order to integrate all parameters and to share this protocol with all decision makers. The last step is to consolidate the organization of the quality assurance protocols by means of the ISO 9002 certification.

Central nervous system malformations and early end-stage renal disease in oro-facio-digital syndrome type I: a review.

Intracerebral cysts and porencephaly or arachnoid cysts are rarely but are repeatedly reported in orofaciodigital (OFD) syndrome type 1. We report on 2 families in which OFD syndrome type 1 was observed with central nervous system (CNS) malformations and 3 sporadic cases of OFD with CNS defects, most likely representing fresh mutations for OFD 1. In one case, vermis hypoplasia was present; in another, periventricular heterotopiae were noted. We review the literature on CNS anomalies in OFD syndromes and stress the difficulties in genetic counseling and functional prognosis for children of OFD 1 female carriers prenatally diagnosed with a malformation of the brain. As for CNS malformations, renal cystic disease is an often overlooked complication specific to OFD 1. In 1 family, cystic medullary disease was noted in OFD 1 carriers, leading 1 patient to dialysis by age 35 years and the other to severe renal insufficiency by age 28 years. Longitudinal follow-up of OFD 1 carriers should be performed, and renal function should be assessed in those with cysts because the functional prognosis of this developmental anomaly may be worse than usually reported in the literature.

Prenatal detection of a congenital pancreatic cyst and Beckwith-Wiedemann syndrome.

We report a case of congenital pancreatic cyst detected prenatally by ultrasound in a fetus with evidence for a diagnosis of Beckwith-Wiedemann syndrome (BWS). Neonatal hypoglycaemia was prevented. The cyst was managed by internal drainage. This is the second reported case of BWS associated with pancreatic cystic dysplasia and the first time that this association has been detected prenatally. Differential diagnosis of cystic abdominal lesions occurring in utero should take pancreatic cysts into consideration. This case suggests that pancreatic cysts should be included in the BWS phenotype.

[Spontaneous abortion of male fetuses with incontinentia pigmenti (apropos of a family)]. / L'interruption spontanée des grossesses de foetus mâles atteints dans l'incontinentia pigmenti (a propos d'une famille).

We report a family with incontinentia pigmenti. One affected woman had seven pregnancies, seven miscarriages; a prenatal diagnosis by molecular biology was undertaken in the last four cases (two males, two females). In the last two males, a miscarriage occurred at the beginning of the second trimester with cystic hygroma in a case. In the first two males a miscarriage was observed also at the beginning of the second trimester after chorionic biopsy or amniocentesis. These two miscarriages would not be a complication of prenatal diagnosis but spontaneous abortion of an affected male. The date of the miscarriage of affected males (the beginning of the second trimester) and the role of a cystic hygroma for the diagnosis of incontinentia pigmenti in this mother of a fetus karyotyped 46,XY are discussed.

[Reflections on 10 years of medically induced abortions in Ille-et-Vilaine]. / Réflexions sur 10 ans d'interruptions médicales de grossesse (IMG) en Ille-et-Vilaine.

The medical files of 532 patients who underwent medically induced abortion over a 10-year period (1982-1991) in the French department of Ille-et-Vilaine were studied in order to evaluate the indications and outcomes. Among the patients, 358 resided in the department (67%). Comparatively with the number of births during the 10-year period, there was a relative increase in the number of medically induced abortions from 3.5/1000 to 5.5/1000. This parameter was taken into consideration for the interpretation of a parallel decrease in the perinatal mortality during the same period, from 5.9/1000 to 5.1/1000. There was a maternal indication in 91 cases which correspond to the former category of therapeutic induced abortions. There was a clear increase in 1991 corresponding to abortions induced because of extremely premature rupture of the membranes which were formerly allowed to continue to dead births. Foetal indications were frequent: 441 cases (83%). Exogenous causes were lower (15.6%), particularly due to the disappearance of indications resulting from maternal irradiation. For indications related to infection, the vaccination against rubella and improved prenatal diagnosis resulted in the disappearance of rubella as an indication during the last three years of the study and a clear decrease in the number of toxoplasmosis indications. There were few indications due to maternal infection by human immunodeficiency virus (4 cases). Chromosomal abnormalities were the main cause of medically induced abortion among the foetal indications (27.7%).(ABSTRACT TRUNCATED AT 250 WORDS)

[Indications for therapeutic interruption of pregnancy in Ille-et-Villaine from 1982 to 1986. Apropos of 222 cases]. / Les indications des interruptions thérapeutiques de grossesse en Ille-et-Vilaine de 1982 à 1986. A propos de 222 cas.

The authors have made the census of all the Medical Terminations of Pregnancy (MTP) which have been carried out in the Department of Ille et Vilaine from 1982 to 1986, i.e. 222 cases, in order to precise the different indications and the diagnosis tools which were used. 132 MTP concern women who live in the Department of Ille et Vilaine. By referring this figure to the total number of pregnancies in this area, one can see that the average incidence is of 1.9%; MTP account for 1% of the total number of Terminations of Pregnancies. Foetal indications are more frequent (188 cases; i.e. 84.7%) than maternal ones (34 cases, 15.3%); these figures remained stable over the 5-year period of study. Chromosomal aberrations and closing defects of the neural tubule are the main causes of MTP (22.9% of foetal indications). Among the 43 chromosomal aberrations, trisomies are the most frequent ones (34.9%) because all women aged 38 or more are proposed a detection. The diagnosis of trisomy was made in 24 cases after tests were programmed either because of the age of the mother or because of family antecedents (amniocentesis: 22 times, punction of foetal blood: once, biopsy of chorion villosities: once), in 6 cases after tests were carried out on the basis of suspect clinical signs amniocentesis: once, punction of foetal blood: 5 times), and in 13 cases after the echography had revealed a major syndrome. Closing defects of the central nervous system mainly concern anencephaly (17.6% of foetal indications) since the echography enables an easy diagnosis. All anencephaly have actually been detected during the reference period of pregnancy.(ABSTRACT TRUNCATED AT 250 WORDS)

Prenatal diagnosis of familial tuberous sclerosis following detection of cardiac rhabdomyoma by ultrasound.

The authors report a case of tuberous sclerosis (TS), diagnosed by prenatal ultrasound, which was suspected by the detection of intracardiac tumours and confirmed by the family investigation. Cardiac rhabdomyomata can be visualized early on echography and must suggest this diagnosis. The place of genetic counselling and prenatal diagnosis in TS is examined.

[Prenatal diagnosis of ovarian cysts]. / Les kystes de l'ovaire à diagnostic anténatal.

The authors report 10 cases of ante-natally diagnosed ovarian cysts. Ultrasonography greatly contributed to the diagnosis of this condition rarely described before. These cysts are usually follicular cysts. Physiopathological explanation still remain unclear: excessive fetal gonadotrope activity, enzymatic abnormality of the theca interna, abnormal stimulation by the mother's HCG are the main hypothesis. Ultrasonographic prenatal diagnosis is based on the discovery of an intra-abdominal round liquid area in a female fetus with normal kidneys and bladder. Obstetrical management is very simple: observation and vaginal delivery. After birth, diagnosis is assessed by clinical examination and ultrasonography which may be able to recognize a possible torsion (intracystic fluid septation). Treatment is conditioned by two facts: first these cysts are usually follicular benign lesions, second there is a high risk of complications, mainly torsion or rupture. Non-operative treatment and observation can be justified for very small cysts which have low risk of torsion. Laparotomy confirms the diagnosis: regarding non complicated cysts, surgery must be as conservative as possible: cystectomy is often feasible, leaving a laminated but functional ovary. Percutaneous puncture under ultrasonography could be considered.

[Abnormalities of the central nervous system. Results of 3 years of genetic counseling at the University Hospital of Rennes: 192 cases]. / Les anomalies du système nerveux central. Bilan de trois années de la Consultation de Génétique du C.H.U. de Rennes: 192 dossiers.

The authors present their view of screening for central nervous system malformations in Brittany, having studied 192 case histories of subjects seen in the three years of genetic counselling in Rennes. Ultrasound usually manages to demonstrate anencephaly but all too often it fails to demonstrate spina bifida. Furthermore serum or amniotic fluid alphafetoprotein levels are often poorly interpreted. Microcephaly and encephaloceles occur rarely. The ultrasound diagnosis of the latter is easy whereas it is more difficult to diagnose microcephaly. The authors point out that there are familial forms of hydrocephaly and of holoprosencephaly which are not all that rare and fairly easy to diagnose so long as one remembers this very serious abnormality.