Antley-Bixler syndrome: case report and review of the literature.

A female newborn presenting a malformative syndrome which groups trapezoidocephaly, midfacial hypoplasia, radiohumeral synostosis, multiple joint contractures and femoral bowing is described as being affected of an Antley-Bixler syndrome. We compare this case with the seventeen others cases reported.

[Extensive Hirschsprung's disease associated with intestinal malrotation]. / Maladie de Hirschsprung à forme iléocolique associée à une malrotation intestinale.

Hirschsprung's disease (HD) involves the entire colon in less than 5% of cases, and the association of extensive HD with intestinal malrotation is very rare. This association of symptoms may delay both diagnosis and treatment. An infant presented with an intermittent occlusive syndrome that began neonatally. Intestinal malrotation was diagnosed radiologically, and treated surgically when the child was 2 months old. However, a chronic occlusion persisted. Biopsies of the rectum and the appendix demonstrated an absence of neurons in intestinal plexi. When the child was 17 months old, ileostomy and surgical excision of the segment affected by HD (the colon and terminal ileum) were performed. An ileoanal anastomosis was performed at the age of 29 months, with favorable outcome. The persistence of symptoms of intestinal occlusion after attempted treatment of intestinal malrotation must therefore suggest the possibility of associated HD in a young child.

[Extramucosal pylorotomy via the umbilical route under general anesthesia and para-umbilical cord: evaluation of intra- and postoperative analgesia]. / Pylorotomie extramuqueuse par voie ombilicale sous anesthésie générale et bloc para-ombilical: évaluation de l'analgésie per- et postopératoire.

OBJECTIVE: To assess both intra- and postoperative analgesia in infants undergoing extramucosal pylorotomy via a circumumbilical incision under general anaesthesia with neither opioid nor muscle relaxant, associated with a paraumbilical block. STUDY DESIGN: Open prospective study covering a 1-year period. PATIENTS: The study included 32 infants (age = 1.1 +/- 0.7 months, body weight = 4,427 +/- 876 g). METHOD: General anaesthesia was induced with either thiopentone or halothane and, after tracheal intubation, maintained with halothane in a N2O-O2 50 vol% mixture. Para-umbilical block was obtained using 0.5 mL.kg-1 of 1% lidocaine with epinephrine. Pain was assessed using time course of respiratory rate, heart rate and mean arterial pressure. A change of more than 20% in one of these variables was considered as criterion for poor analgesia. RESULTS: Intraoperative analgesia was adequate in all patients but one, 3 minutes after incision. Surgical conditions were considered as being good or satisfactory in 76% and 24% of cases, respectively. Postoperative analgesia, assessed 1 and 6 hours after completion of surgery, was convenient in 90% of infants, the end of the action of the local anaesthetic resulting in a poor analgesia at the 6th hour in four of them. CONCLUSION: Provided a short bevel needle is used and basic safety rules of local anaesthesia are followed, the para-umbilical block provides adequate intra and postoperative analgesia in infants undergoing extramucosal pylorotomy via circumumbilical route.

[Urinary lithotripsy in children. Multicenter study of the Pediatric Urology Study Group]. / Lithotritie urinaire de l'enfant. Etude multicentrique du Groupe d'Etudes en Urologie Pédiatrique (GEUP).

The authors present the results of a survey conducted among French paediatric urologists belonging to the Groupe d'Etudes en Urologie Pédiatrique (GEUP) (Paediatric Urology Study Group). This study, based on 122 cases observed in 13 centres, is not exhaustive, but is nevertheless statistically significant. The preoperative assessment confirms the usual findings of urinary stones in children: pyelonephritis, haematuria and abdominal pain, the usual presenting complaint, concomitant malformative uropathy (10% of cases) and a predominance of calcium stones. More than 200 stones were treated, larger than 10 millimeters in diameter in one-third of cases. Renal stones, mainly caliceal (more than 50%), included 11 staghorn calculi. This study also included 22 ureteric stones, mainly in the pelvic ureter, and 2 bladder stones. Lithotripsy was ultrasound-guided in 2/3 of cases and required general anaesthesia in about 3/4 of cases. Ureteric catheterization was required in 19 infants preoperatively, but in only 2 infants (stein strasse) postoperatively. One or two lithotripsy sessions were sufficient in most cases, but 4 sessions were necessary in 5 patients, to the same kidney in 1 case. The mean hospital stay was 2 to 3 days, but the procedure was performed on an outpatient basis in 15 cases. The immediate postoperative course was uneventful and asymptomatic. This survey revealed about 10% of complete failures, corresponding to solitary caliceal stones in 2/3 of cases; 29 partial failures were essentially due to lower caliceal stones and staghorn calculi; 84 successes (stone-free), mainly pelvic or simple caliceal stones. Scintigraphy did not reveal any immediate postoperative impairment of renal function. This study reported a success rate of about 70%, regardless of the type of apparatus used. Assessment of the results of ESWL requires sufficient follow-up both concerning the outcome of fragmented stones and evaluation of possible functional repercussions. This survey defines the main indications: although ESWL can be applied to most stones, some stones constitute poor indications (cystine stones, stenotic malformative uropathy) or dubious indications: small lower caliceal stones, densely calcified staghorn calculi in older children. This study confirmed the efficacy and low morbidity of ESWL in children. A prospective study needs to be conducted according to a rigorous protocol in order to refine the technique and indications while reducing the possible long-term risks.

[A giant cyst of the seminal vesicle associated with ipsilateral uretero-renal agenesis]. / Un kyste géant de la vésicule séminale associé à une agénésie urétéro-rénale ipsilatérale.

The authors present a case of seminal vesicle cyst and discuss the polymorphic clinical features and the examinations required to establish the diagnosis. Treatment is surgical and reserved to cysts with severe symptoms.

[Epidural anesthesia in children under 3 months of age. Apropos of 49 cases]. / Anesthésie péridurale chez l'enfant de moins de trois mois. A propos de 49 observations.

This retrospective study includes 45 patients (age: 32 +/- 28 days; weight: 4,077 +/- 988 g) who underwent epidural analgesia with a catheter for various surgical operations. Epidural space was detected by the loss of resistance to air technique. The anaesthetic solution administered was bupivacaine 0.25% with 1/200,000 epinephrine. Four technical failures were recorded; intraoperative analgesia was adequate for all other patients. In 17 patients, postoperative analgesia was maintained by continuous infusion (0.125% bupivacaine or 0.25% lidocaine: 0.2 mL.kg -1.h-1). In 18 patients, it was managed by iterative injections (0.25% bupivacaine with epinephrine: 0.25 mL.kg -1.h-1). In 10 patients, a single shot was sufficient. Postoperative analgesia was found to be inadequate in four patients. No incidents or accidents were recorded. A benefit-risk analysis with the support of the literature attempts to define the main indications of this method.

[Epidural anesthesia and esophageal atresia. Apropos of a case]. / Anesthésie péridurale et atrésie de l'oesophage. A propos d'une observation.

We report the good results of a thoracic epidural analgesia for oesophageal atresia repair in a 3-day old girl. Surgery was performed under light general anaesthesia combined with a single epidural injection of bupivacaine 0.25% (0.5 ml.kg-1) with epinephrine through a 19 G catheter (T7 space, descending to T5). During the three following days, a continuous epidural infusion was used (0.2 ml.kg-1.h-1 bupivacaine 0.125%). Intraoperative analgesia and postoperative analgesia were adequate, the latter being assessed by the Barrier-Amiel-Tison pain scale. Thus heavy postoperative care could be avoided, namely mechanical ventilation. Recovery was uneventful.

[Value of ilio-hypogastric block in appendectomy in children]. / Intérêt du bloc ilio-hypogastrique pour appendicectomie chez l'enfant.

This prospective study aimed to evaluate the efficiency of ilio-hypogastric nerve block for control of post appendicectomy pain in children. Forty-two children aged 3-15 years scheduled for appendicectomy were anaesthetized in the same way. After randomization, a preoperative ilio-hypogastric nerve block was performed in 21 patients. Twenty one were not blocked. The postoperative pain assessment showed a better analgesia in the blocked children group. Analgics were required less in group. Five inefficient blocks were recorded. No complications were noted. Ilio-hypogastric block was found to be safe and efficient for control of post-appendicectomy pain in most children.

[Urothelial tumor of the bladder in children]. / Tumeur urothéliale vésicale chez l'enfant.

A papilloma of the bladder was discovered in 10-year-old child who presented with haematuria. The review of the literature from 1955 produced 131 similar cases. The particular situation of this usually adult tumour in paediatric patients was emphasized by this case and those reported in the literature: good prognosis, rare invasion, lower rate of recurrence than in the adult and habitual effectiveness of endoscopic resection.

Para-umbilical block: a new concept for regional anaesthesia in children.

This preliminary study describes a new technique to provide analgesia in children undergoing umbilical hernia repair. The para-umbilical block consists of infiltrating the anterior cutaneous branches of the two tenth spinal roots over and under the rectus sheath far from the operative field. Intra and postoperative analgesia as well as operative conditions were assessed in 11 children 16.7 +/- 31 months old, weighing 8421 +/- 6941 g, the block being performed before surgery under light general anaesthesia. Intraoperative analgesia, operative conditions and recovery were good in all patients. Analgesia was adequate one h after surgery in ten patients, six h after surgery in eight. The block proved to be safe and on the whole effective in this short series. The study should proceed on a multi-centre basis if possible. Indications can be extended.

[Drainage of the erectile organs in the rat]. / Le drainage des corps érectiles chez le rat.

The retro-pubic veinous circle of the male rat was investigated on the both sides: anatomic and functional aspect. The dorsal vein of penis in the male rat run into a retro-pubic veinous circle. This circle receive the anterior vesical veins too. The internal pudendal vein and the anastomotic vein begin in this circle, and go together to the iliac vein. From the retro-pubic veinous circle, there is a triangular circulation between the internal pudendal vein, the anastomotic vein and the iliac vein. Angiographies performed on rats in vivo, indicates that the anastomotic vein is effical for the drainage of the retro-pubic circle only if the flow of the penis dorsal vein is sufficient. These conclusions are proved by experimentation on dead rats.

[Cystic ectasia of the rete testis: apropos of a case]. / Ectasie kystique du rete testis: à propos d'un cas.

We report a case of a simple testicular cyst developing on the rete testis. Nine cases have been reported in children. In our patient, the cyst was associated with aplasia of the homolateral kidney and termination of the left ureter in the left deferens. According to the literature, the diagnosis is usually fortuitous at histology examination. The cause is probably embryonic malformation. Castration is the therapeutic solution. In our case, simple enucleation allowed preservation of the deferens since the controlateral tree was damaged. There have been no complications with a 6-year follow-up.