[Anaesthetic management of a child with hemoglobinopathy]. / Comment je prends en charge un enfant qui souffre d'une hémoglobinopathie.

Perioperative management of a child with sickle-cell disease requires close collaboration between hematologist, surgeon and anesthetist. The level of preoperative preparation must consider both the surgical risk and the impact of the disease. Preoperative hydration and blood transfusion are the most important part of preoperative management. Anesthetic technique is not as important as preoperative preparation, management of stress and anxiety, and optimization of intraoperative physiological parameters (oxygenation, acid-base balance, tissue perfusion, normothermia). Pain management must be optimal for these children with increased needs of analgesic. Preoperative assessment of a child with thalassemia must include evaluation of the impact of chronic hemolytic anemia and iron overload induced by repeated transfusions. The most important factor in the management of a child with glucose-6-phosphate dehydrogenase deficiency is to avoid exposure to oxidative stressors. Outpatients should be informed of the risk of hemolytic crisis, and free hemoglobin in the urine should lead to discontinuation of drugs associated with hemolysis and to maintenance of urine output to prevent acute renal failure.

Efficacy and plasma levels of ropivacaine for children: controlled regional analgesia following lower limb surgery.

BACKGROUND: Continuous regional analgesia (CRA) is considered a safe and efficacious technique for postoperative pain relief in children after lower limb surgery. We recently evaluated the feasibility of patient-controlled regional analgesia (PCRA) in a similar acute pain situation and we concluded that PCRA might be advantageous over CRA in terms of lower costs, risk of systemic toxicity while producing similarly adequate analgesia. We therefore prospectively compared both techniques in the paediatric population. METHODS: In total, 30 children undergoing lower limb orthopaedic surgery were randomized to receive PCRA or CRA with ropivacaine 0.2%. Visual analogue scale scores, rescue analgesia, overall satisfaction, motor blockade and plasma ropivacaine concentrations were recorded for 48 h. RESULTS: Adequate analgesia was achieved with both techniques. No significant difference was noted for rescue analgesia, overall satisfaction and motor blockade. In contrast, children in the PCRA group received significantly less local anaesthetics than those in the CRA group. In addition, total plasma concentrations of ropivacaine were significantly reduced in the PCRA group as compared with the CRA group during the 48 h postoperative period. CONCLUSIONS: Both techniques are efficacious and satisfactory. However, PCRA with ropivacaine 0.2% can provide adequate postoperative analgesia for paediatric orthopaedic procedures with smaller doses of ropivacaine than CRA.

[Acute gastric distension necessitating gastrostomy after anesthetic induction for surgical correction of type III esophageal atresia]. / Distension gastrique aiguë nécessitant une gastrostomie après induction anesthésique pour correction chirurgicale d'une atrésie de l'oesophage de type III.

IPPV during anaesthesia for management of oesophageal atresia with tracheo-oesophageal fistula (TOF) can cause gastric insufflation. We report such a complication in a one-day-old newborn, who developed, 15 min after induction, a distension of the abdomen, hypoxia and bracdycardia. An emergency gastrostomy was performed. His status improved rapidly and surgery could be completed. TOF was located at the carina and had a large calibre. To avoid gastric distension in such cases, the tip of the tube is located just proximal to the carina, but distal to the fistula to prevent intubation of the latter. Difficulties are due to position of the fistula (carina, main bronchi) or its large bore. Gastric distension carries a risk of regurgitation and inhalation of gastric contents, elevation of hemidiaphragm and lung compression, decreased tidal volume, decreased venous return, cardiovascular collapse and cardiac arrest. When insufflation peak pressures are low, gastrostomy is benefitful, as in our case, as the tidal volume loss through the stomach is acceptable. In case of high insufflation pressures because of co-existing lung disease, gastrostomy is better avoided, as most if not all the tidal volume may be lost through the stomach.

[Long-term results and prognostic factors after mitral valve replacement using the Starr-Edwards 6120 prosthesis]. / Résultats éloignés et facteurs pronostiques après remplacement valvulaire mitral par la prothèse de Starr-Edwards 6120.

Between 1970 and 1984, 386 mitral valve replacements with the SE 6120 prosthesis were performed with a mean post-operative follow-up of 75 +/- 44 months or a total follow-up of 2520 patient years (PY). Rapid post-operative mortality was 6.7 percent and the delayed mortality 26.5 percent, the 5-year actuarial survival rate was 80 +/- 2% and that at 10 years 67 +/- 2% (including early mortality). There was no significant difference for survival between single-valve replacements (n = 183) and multiple-valve replacements (n = 203). Multifactorial prognosis analysis (Cox) gave two post-operative prognosis: X (n = 386) = 0.0391 (age) + 0.2421 (NYHA stage); Y (n = 147) = 0.8561 (X) + 0.6299 (VG type). The incidence of complications related to the prosthesis is 4.16 per 100 PY, including sudden deaths. The current actuarial analysis shows that at 5 years 91 +/- 1% at 10 years 78 +/- 2% of the operated subjects are devoid of any such complication and of thrombosis of the valve. Statistically, it was found that permanent atrial fibrillation (p = 0.03), the size of the right atrium (p = 0.02) played a promoting role. With a post-operative follow-up in excess of 8 years. 79% of the survivors are symptom-free or present few symptoms and the myocardial response is poor in 52 patients (NYHA stage III or IV).

[Uterine inversion: an anesthetic emergency]. / L'inversion utérine: urgence anesthésiologique.

A case of complete uterine inversion, in a 27-year-old woman after delivery of her third child, is reported. Because of lack of placental separation after 40 min. manual removal was carried out under general anaesthesia (midazolam, ketamine, alfentanil). The placenta was quickly and easily removed, but followed immediately by protrusion of the uterine fundus at the vulva. Simultaneous, blood pressure became unmeasurable, and the patient became cyanosed. The uterus remained impossible to replace until anaesthesia had been deepened. The patient remained shocked despite intravenous fluids and catecholamines. As soon as the uterus was replaced, blood pressure rose to 80 mmHg. The patient was extubated 50 minutes later, blood pressure being 105/80 mmHg. Acute and subacute puerperal uterine inversion is a rare obstetrical emergency (1 in 20,000 deliveries) with a 15% mortality rate. Immediate recognition and early treatment to relax the cervico-uterine junction, should ensure rapid replacement of the uterus.

[Uretero-calicostomy for hydronephrosis caused by junction disease in children and adolescents. Apropos of 35 cases]. / Urétéro-calicostomie pour hydronéphrose par maladie de la jonction de l'enfant et adolescent. A propos de 35 cas.

35 children and adolescents were submitted to ureterocalicostomy for PUJ obstruction. The surgical procedure is explained and specially the importance of performing a regular, muco-mucosal anastomosis to avoid its secondary stenosis. 9 patients have an excellent result with absence of clinical symptoms, sterile urinalysis and normal IVP. 20 patients have a good result with absence of clinical symptoms, sterile urinalysis and improvement of the dilatation of the cavities. 2 secondary nephrectomies have had to be performed because of a dysplastic kidney (1 patient) or of a parenchymal infection (1 patient). 2 patients have no clinical symptoms but have no improvement of their IVP. 3 kinds of indications have been distinguished: failures of previous surgery of the PUJ (5 patients); PUJ obstruction associated with congenital kidney abnormalities as horseshoe kidney (15 patients) and malrotation (3 patients); severe PUJ obstructions with a very thin parenchyma (12 patients). Ureterocalicostomy appears to be a helpful procedure in some defined situations where a declive drainage of the urine essential. No late deteriorations have been recorded in this series.

[Indications and results of the MAGPI intervention in the treatment of minor hypospadias]. / Indications et résultats de l'intervention de MAGPI dans le traitement des hypospades mineurs.

In order to clarify the indications of MAGPI procedure (Meatal Advancement and Glanuloplasty Incorporated), 18 patients operated upon have been reassessed after a long follow-up (mean: 30 months). The cosmetic and functional results are good in 16 cases, 2 non-satisfactory results are noticed with a wrong meatal position. The initial position of the meatus (glanular or coronal), the absence of chordee and the initial aspect of the glans (broad and flat) are the three main conditions to obtain a good result.