Gilles Perreault

Heterogeneity in the campomelic syndromes. Long-and short-bone varieties.

Analysis of 9 cases of bilateral bent limbs (campomelia) and dwarfism, as well as a review of the literature, indicate that campomelic syndrome appears to be a well-defined distinct disorder which the authors call long-limbed campomelic syndrome. Other neonates with congenital bent-limbed dwarfism can be classified as having short-limbed campomelic syndrome, and among these at least two distinct forms have been delineated--the craniosynostotic and the normocephalic form. Congenital bent bones also occur in a variety of generalized disorders of ossification that must be distinguished from these well-defined types of campomelic dwarfism.

Investigation of childhood blunt abdominal trauma: a practical approach using ultrasound as the initial diagnostic modality

During a 5.5-year retrospective study (1979–84) 170 children with blunt abdominal trauma were investigated with intravenous urography (IVU), ultrasound (US) and scintigraphy. For the investigation of the last 71 children (after 1982) a 4th generation CT scanner was available in the same department. The results of radiologic investigations were compared with clinical outcome in 157 and results at laparotomy in 13 children. During the study period, real time US became the first line screening tool, and was combined with IVU in suspected renal trauma. In spite of permanent accessibility of CT since November 1982, the latter was used only in complex diagnostic problems or in children with multiple injuries (8% of the series). There were no deaths resulting from abdominal trauma. During the study, the incidence of splenectomy and exploratory laparotomy decreased, and no diagnostic peritoneal lavage were performed after 1980.

Fetal hydronephrosis: is there hope for consensus?

This review article aims at summarizing the data regarding fetal and neonatal hydronephrosis, at correlating controversial data with the differences in the practice of obstetrical sonography from one country to another, and finally, at presenting our own criteria for fetal renal collecting system dilatation along with our own guidelines of postnatal investigation.

Antenatal diagnosis of ovarian cysts: natural history and therapeutic implications

A retrospective study from 1980 to 1990 shows 29 ovarian cysts in 27 patients diagnosed by prenatal ultrasound performed between 28 and 38 weeks of gestation. Ten patients underwent surgery, 17 patients were observed with serial ultrasound. Delayed good quality sonograms after spontaneous resolution of the cyst in a selected group of 7 patients showed restoration of a normal ovarian anatomy. The size of the cyst and/or its sonographic characteristics are the 2 main factors for deciding a conservative or a surgical management.

ACUTE EPIGLOTTITIS IN CHILDREN: MANAGEMENT OF 27 CONSECUTIVE CASES WITH NASOTRACHEAL INTUBATION, WITH SPECIAL EMPHASIS ON ANAESTHETIC CONSIDERATIONS

SummaryTwenty-seven consecutive cases of acute epiglottitis in children were treated by nasotracheal intubation during a 25-month period. The duration of intubation averaged 44.2 hours. No mortality or morbidity occurred. Mean hospitalization was 4.5 days.Anaesthetic techniques are discussed. Ketamine is a somewhat controversial choice for these patients as it can enhance excitement, restlessness, and accidental extubation.This study reconfirms the ease of maintaining an assured airway by nasotracheal intubation in cases of acute epiglottitis.RésuméĽHôpital Sainte-Justine pour les Enfants (Montréal) a admis 27 enfants atteints ďépiglottite aiguë ďorigine infectieuse, du 1er janvier 1974 au 31 janvier 1976 (25 mois). Tous ces enfants ont été traités à ľaide de ľintubation nasotracheal, ďaprès un protocole élaboré conjointement par les départements de pédiatrie, ďanesthésiologie, ďoto-rhino-laryngologie et de radiologie.La durée moyenne de ľintubation a été de 44.2 heures. La mortalité et la morbidité reliées à ce traitement ont été nulles. La durée moyenne de ľhospitalisation a été de 4.5 jours.Différ entes techniques ďanesthésie générale ont été employées et discutées. Le choix de la ketamine, pour ces malades, nous semble pour le moins très discutable dans la mesure où le réveil de la dissociation à la ketamine peut être agité et, sans sédation convenable, peut augmenter ľincidence ďextubations accidentelles ( trois de nos cinq patients ayant reçu de la ketamine ont dû être réintubés ). Cette étude confirme une fois de plus que ľintubation nasotrachéale est une méthode excellente pour le traitement des épiglottites aiguës.

Changes in the presentation of intussusception

Intussusception is most often diagnosed in well-nourished 7- to 10-month-old infants, but may be overlooked in older children. From 1985 to 1991, we treated 118 boys and 62 girls ranging in age from 2 months to 15 years (average, 22.6 months). Thirty-seven percent were older than 2 years, significantly more than in earlier experience at this institution. Overall, children with intussusception had a less than average weight (P < .05). The majority of intussusceptions in all age groups were idiopathic. Seventy-three percent of the patients were treated successfully by barium enema. The presence of air-fluid levels on the plain radiograph decreased the success rate of barium enema reduction from 81% to 49%. Barium enema reduction should nevertheless be attempted regardless of the age of the patient or the duration of symptoms, and routine surgical exploration is never recommended.

Normal portal venous diameter in children

A study was conducted on 156 children, in whom the portal veins were measured using standard real time ultrasonography. Knowledge of the normal dimension may be important in patients with liver disease.

Desbuquois syndrome presenting with severe neonatal dwarfism, spondylo-epiphyseal dysplasia and advanced carpal bone age

An infant with the clinical and radiological findings of short stature, hyperlaxity and osseous disorders typical of Desbuquois syndrome was followed from second trimester intrauterine life to his death at age of 22/12 years old.

Pyeloplasty: To divert or not to divert?

The use of diversion in conjunction with pyeloplasty has been a matter of continuous debate for a number of years. In consequence the matter has been left to the urologists individual preferences with no objective data to support these preferences. Herein the authors present an analysis of 105 pyeloplasties performed by four surgeons over a period of eighteen years with a view to determine the place and type of diversion in relation to the surgical technique used and the long-term effects of such diversion or abstinence thereof.

CT localization of an aldosteronoma in a 10-year-old boy

Primary aldosteronism in children under 16 years of age is usually due to adrenal hyperplasia. We could find only seven cases in the literature where it was caused by a solitary adrenal adenoma; all but one were female. We describe the case of a 10-year-old Caucasian boy in whom a unilateral adrenal adenoma was demonstrated by high resolution computed tomography (CT) alone. The literature is reviewed.