[Appendicovesicostomy (Mitrofanoff procedure) in children: Long-term follow-up and specific complications]. / Dérivations urinaires continentes trans-conduit selon Mitrofanoff chez l'enfant : suivi à long terme et complications spécifiques.

OBJECTIVE: In children, intermittent catheterization by appendicovesicostomy according to Mitrofanoff is an interesting alternative to the urethral approach. Objective of the study was to evaluate the rate of appendicovesicostomy's specific complications. METHOD: From 1997 to 2017, data on children treated and followed for an appendicovesicostomy in an academic institution were collected retrospectively. Rates of surgical complications specifically encountered on appendicovesicostomy, time of onset, frequency, and necessity of surgical reinterventions have been reported. RESULTS: Thirty-four patients were operated on and followed for a median of 6.2 years [0.3-24]. Fifty percent had a complication, occurring after a median of 8 months [2-90], and 38% required at least one surgical revision. If complication occurred, adjustment of medical treatment and intermittent catheterization was effective in 12% of patients, endoscopic or over-fascial surgery was necessary in 17% of cases, and under-fascial revision in 21% of cases. Median time to complication was 4 months [1-90] after creation or revision of appendicovesicostomy. Thirty-height percent of difficult channel catheterization were reported, of which 46% were over or under-fascial stenosis. Inaugural urinary incontinence was 18%, and only 9% if using the appendix. At the end, 97% of appendicovesicostomy were continent. CONCLUSIONS: Appendicovesicostomy is a high risk of complications and postoperative revisions surgery, in order to have a functional continent channel. LEVEL OF EVIDENCE: 4.

Long-term results with the laparoscopic transposition of renal lower pole crossing vessels.

BACKGROUND: For the treatment of ureterovascular pelviureteric junction obstruction (PUJO), transposition of lower pole crossing vessels (LPCV) has been described as an alternative to dismembered pyeloplasty. PURPOSE: To report on the long-term follow-up of children after laparoscopic transposition of LPCV. METHODS: A retrospective analysis of 70 children consecutively treated by laparoscopic transposition of LPCV. Candidate patients were selected on the basis of clinical history, renal ultrasound (US), and pre-operative mercaptoacetyltriglycine (MAG-3) scan. Selection criteria included: presence of LPCV with SFU Grade 1-2 hydronephrosis, impaired drainage on MAG-3 and intraoperative normal pelviureteric junction (PUJ) and ureter peristalsis. Thinned parenchyma, impaired renal function, or history of prenatal hydronephrosis were not considered as exclusion criteria. Children were clinically followed up with US and MAG-3 scan. Success was defined by symptom resolution with improvement in hydronephrosis. RESULTS: Seventy children, aged 8.3 years (range 2.75-16.0), were selected. Procedures were performed through transperitoneal laparoscopy (n = 42) or were robotic-assisted (n = 28). Operative time was 120 min and length of hospital stay was 2 days. The outcome was successful in 67/70 patients (96%), with a median follow-up of 52 months (range 13-114). There were three failures in children who eventually underwent dismembered pyeloplasty for a symptomatic, undiagnosed, intrinsic PUJ obstruction. Two of them had been postnatally followed for a resolving prenatally diagnosed hydronephrosis. Three children became free of symptoms, had improved hydronephrosis, but still showed impaired drainage on MAG-3 and are being closely followed up. DISCUSSION: Although this procedure proves to have long-term efficiency in selected indications, the main challenge is to intraoperatively ascertain the absence of associated intrinsic stenosis. Objective criteria remain difficult to establish, but intraoperative findings, including dependent, funnel-shaped, normal-looking PUJ with decreasing hydronephrosis after pelvis and LPCV mobilisation, and efficient peristalsis across the PUJ under intraoperative diuretic test, represent a low likelihood of associated intrinsic stenosis. Ipsilateral impaired renal function doesn't seem to be associated with an adverse outcome. In contrast, a prenatal history of mild or self-resolving hydronephrosis in a patient later presenting with intermittent dilatation, raises the suspicion of associated intrinsic PUJ obstruction, as it is associated with a higher risk of failure. CONCLUSION: With a long postoperative follow-up, the robotic-assisted or laparoscopic vascular hitch procedure has been successful in treating a selected group of children with obstructive LPCV, and represents a safe and reliable alternative to standard dismembered pyeloplasty in the absence of intrinsic PUJO suspected on prenatal US.

[Research on embryonic cells. Legal aspects]. / Recherches sur les cellules souches embryonnaires humaines. Aspects législatifs.

Whilst maintaining the principle of a ban on embryo research, the bioethics law of August 6th 2004, updating the first bioethics laws passed in 1994, authorises French research teams to carry out studies and research on embryos or embryonic cells generated as part of an assisted reproduction programme, under special dispensation and for a maximum of five years from the date of publication of the decree of application of this text (Decree n(o) 2006-126, February 6th 2006, JO of 07/02/06). It also permits the importation and exportation of embryonic tissues and cells for such research and the storage of stem cells for scientific purposes. This highly supervised disposition is subject to control by the Biomedicine Agency, which is responsible for delivering and controlling authorisations for embryo research.

Retroperitoneoscopic nephroureterectomy of a horseshoe kidney in a child.

Retroperitoneoscopic heminephrectomy used to manage a horseshoe kidney in a 15-month-old boy is reported. The surgery was decided after a progressive increase of arterial blood pressure in the patient followed for poor kidney functioning resulting from vesicoureteral reflux in both ureters of the left duplex moiety of a horseshoe kidney. The retroperitoneoscopy was conducted with three trocars: a 10-mm port inserted under direct vision at the extremity of the left 12th rib and two 5-mm operating trocars. Division of the isthmus was performed using an ultrasonic scalpel. Duration of the pneumoretroperitoneum was 115 min. The patient was discharged on postoperative day 4. At 2 years postoperatively, the patient's arterial blood pressure was normal without treatment. The authors conclude that retroperitoneoscopic nephrectomy for horseshoe kidney is safe and feasible in children, offering all the advantages of minimally invasive surgical procedures.

[Acute intestinal invagination revealing celiac disease in a 9-month-old infant]. / Invagination intestinale aiguë révélant une maladie coeliaque chez un nourrisson de neuf mois.

UNLABELLED: In most cases bowel intussusception is idiopathic in children. Indirect causes, such as celiac disease, are rare. CASE REPORT: A nine-month-old girl was admitted for a bowel intussusception diagnosed by ultrasonography, yet not confirmed by barium enema. The girl underwent a thorough check up, due to a loss of weight and denutrition symptoms which evidenced immunoglobulin A anti-gliadin, immunoglobulin G anti-gliadin, immunoglobulin A anti-endomysium and antireticulin positive antibodies. Celiac disease was confirmed by bowel biopsy, which revealed a subtotal villous atrophy. Evolution was favourable under a gluten free diet. COMMENTS: The description of a bowel intussusception associated with celiac disease is common. This observation appears to be the earliest case ever described: the patient was only nine months old. The initial procedure is routine: diagnosis by ultrasonography, therapeutic enema and surgery only in case of failure of medical treatment. Intussusception associated with celiac disease often presents in an atypical way: elementary forms, spontaneously resolvent and recidivious. The knowledge of this clinical set of symptoms leads to an early diagnosis of celiac disease. The gluten free diet prevents the recurrence of intussusception.

Postpneumonectomy syndrome in children: advantages and long-term follow-up of expandable prosthesis.

BACKGROUND/PURPOSE: Pneumonectomy in children can be complicated by a severe mediastinal shift, which leads to bronchial stretching resulting in severe respiratory failure. This postpneumonectomy syndrome can be corrected by inserting a prosthesis in the empty side of the chest. METHODS: Forty-two children, from 6 months to 15 years old, underwent a pneumonectomy. Seven of these patients were treated surgically for severe manifestations of postpneumonectomy syndrome. First insertion of an expandable prosthesis was followed up in 5 cases by its replacement with a breast prosthesis in adolescence. The expandable prosthesis was injected periodically with saline solution to maintain the mediastinum in a midline position as the children grew. RESULTS: The mean delay between pneumonectomy and first prosthesis implantation was 5 years (range, 11 months to 8 years). Pulmonary function tests showed a substantial improvement in the obstructive syndrome in all patients except one, in whom the functional improvement was moderate. The mean follow-up after the expandable prosthesis implantation was 6 years (range, 6 months to 10 years) and all patients are doing well. CONCLUSIONS: The insertion of an intrathoracic prosthesis can dramatically improve the clinical symptoms and reduce the functional obstructive syndrome. The expandable prosthesis allowed for progressive, well-tolerated recentering of the mediastinum and adjustment for growth.

Blockade of the integrin alphaLbeta2 but not of integrins alpha4 and/or beta7 significantly prolongs intestinal allograft survival in mice.

BACKGROUND: Small bowel transplantation remains a difficult therapeutic option endangered by a high rate of rejection and infectious complications. To improve these clinical results, it is mandatory to set up animal models to test alternative immunosuppressive regimens which may lead to immunotolerance. AIMS: To determine the value of blockade of alphaLbeta2 (LFA-1) and alpha4 and beta7 integrins (alpha4beta1, alpha4beta7, and alphaEbeta7) in the prevention of rejection of fetal small bowel grafts in mice and the effect of the association of calcineurin dependent drugs in anti-LFA-1 treated mice. METHODS: Adult recipient mice engrafted with allogeneic fetal small bowel received a short course of anti-alpha4 and/or anti-LFA-1 monoclonal antibodies (mAb) with or without FK506 or cyclosporin A. In addition, in a set of experiment, beta7-/- mice were used as recipients. Graft biopsies were performed and processed for standard histology. RESULTS: Blockade of the pathways of the integrins alpha4 and beta7 had a modest or no effect on intestinal graft survival. In contrast, transitory, short administration of anti-LFA-1 monoclonal antibody alone, when started before engraftment (day -1), allowed long term survival of intestinal grafts, even when associated with calcineurin dependent drugs. However, early withdrawal of FK506 reversed the immunosuppressive effect of anti-LFA-1 treatment. CONCLUSION: These results suggest that firstly, anti-LFA-1, but not anti-alpha4 mAb treatment, may be useful in improving the results of intestinal transplantation, and secondly, that this treatment is not incompatible with long term administration of tacrolimus currently used in the prevention of small bowel graft rejection in humans.

[Role of calcineurin-dependent drugs on the immunosuppressive effect induced by the anti-LFA-1 antibody in a fetal intestinal transplantation model in mice]. / Rôle des drogues calcineurine-dépendantes sur l'effet immunosuppresseur induit par l'anticorps anti-LFA-1 dans un modèle de greffe intestinale foetale chez la souris.

STUDY AIM: We have previously demonstrated that anti-LFA-1 monoclonal antibody (mAb) can efficiently protect against rejection of small bowel allograft in a mouse model. The aim of the present work was to determine, in the same model, the optimum conditions for utilisation of anti-LFA-1 mAb and the effects of calcineurin-dependent drugs on the immunosuppression induced by anti-LFA-1 mAb treatment. MATERIALS AND METHODS: Foetal small intestines of C57Bl/6 (H-2b) mice were transplanted into adult C3H/He (H-2k) mice. Recipients were treated with anti-LFA-1 mAb alone (with or without day-1 injection), or combined to cyclosporin (20 mg.kg-1.j-1 for 14 days), or to tacrolimus (1 mg.kg-1.j-1 from day 0 to day 7). Biopsies were performed after engraftment from day 5 to day 30. RESULTS: Administration of anti-LFA-1 mAb alone is sufficient to induce significant prolongation of intestinal allograft survival, provided that the treatment starts one day before engraftment. This tolerogenic effect is reversed by the transitory administration of tacrolimus (p = 0.008). CONCLUSION: Treatment with anti-LFA-1 mAb has to be started before the allogeneic response has begun. Calcineurin-dependent drugs can modulate the tolerogenic effect induced by anti-LFA-1. A transgenic mice model should give precise details about underlying mechanisms of these interactions, before a possible utilisation of anti-LFA-1 mAb in intestinal transplantation in humans.