Evaluating the impact of pop-off mechanisms in boys with posterior urethral valves.

Introduction: Posterior urethral valves are urethral leaflets that cause Lower Urinary Tract Obstruction (LUTO) in boys and are associated with congenital renal dysplasia and abnormal bladder function. They affect 1:4,000 to 1:25,000 births and can be responsible for End-Stage Renal Failure in childhood. There have been several studies on the effect of pop-off mechanisms in boys with posterior urethral valves, but results are contradictory. We aimed to assess and discuss the effect of pop-off mechanisms on renal function in a large cohort of patients. Patients and method: Boys with PUV with and without pop-off mechanisms (urinoma, VURD or giant bladder diverticula) were divided into three severity groups for renal function according to their nadir creatinine (low-risk NC < 35 µmol/L, intermediate-risk NC between 35 and 75 µmol/L, and high-risk NC > 75 µmol/L). We compared children with and children without pop-off mechanisms for mean renal function as well as patient distribution within each severity group. Results: We included 137 boys of which 39 had a pop-off mechanism. Patients had complete data for at least 5 years follow-up. Though there was no significant statistical difference in mean renal function between the pop-off and non-pop-off group, patient distribution within each severity group varied according to whether patients had a pop-off mechanism or not. Conclusion: Though there was no significant difference in mean renal function between boys with and without pop-off mechanisms, it is possible that these are two different patient populations and direct comparison is not possible.

Nadir creatinine predicts long-term bladder function in boys with posterior urethral valves.

INTRODUCTION: Posterior urethral valves (PUV) cause lower urinary tract obstruction leading to increased intravesical pressure during fetal urinary tract development. Though the bladder and kidneys are separate organs, with different embryological origins, they are complementary and influence each other both before and after birth. We aimed to assess the relationship between renal and bladder function in boys with PUV and whether early renal markers could predict future bladder function. PATIENTS AND METHODS: We included all boys with prenatally suspected lower urinary tract obstruction, born between 2000 and 2013, in two University Hospitals, with at least 5 years follow-up. We excluded patients who presented a Lower Urinary Tract Obstruction other than PUV, children who presented multiple birth defects and neonatal deaths and those with incomplete long-term renal or bladder function data. We included data on nadir creatinine (NC), long-term renal function and long-term bladder function (defined by Uroflow parameters). Boys with PUV were divided into three severity groups for renal function according to their NC and three severity groups for bladder function as determined by Uroflow. RESULTS: We included 73 boys. Average nadir creatinine was 43.4 ± 26.1 µmol/L. Twenty-nine boys (49.3%) presented a NC < 35 µmol/L, thirty-eight (52.1%) a NC between 35 and 75 µmol/L, and 6 (8.2%) a NC > 75 µmol/L. Thirty-eight (52.1%) presented normal bladder function, 23 (31.5%) presented moderately impaired bladder function and 12 (16.4%) presented severely impaired bladder function. 41.4% of boys with NC < 35 had abnormal bladder function vs 46.2% of those with an NC between 35 and 75 µmol/L and 83.3% of boys with NC > 75 µmol/L. Nadir creatinine both predicted both bladder function and renal status (table 1). Correlation between presence of grade 3-5 CKD and poor uroflow was also significant (p < 0.005). DISCUSSION: Nadir creatinine was significantly correlated to bladder function at 5 years of age. What this study suggests is that as nadir creatinine increases so does the risk of severe bladder dysfunction. Our results, though limited to flowmeter and renal function, could help pediatric urologist tailor bladder function monitoring, and indicate which patients could benefit from more aggressive bladder therapy. CONCLUSION: Bladder and renal function are linked in boys with posterior urethral valves. Boys with high nadir creatinine could benefit from early bladder function evaluation and management.

Circumcision and Risk of Febrile Urinary Tract Infection in Boys with Posterior Urethral Valves: Result of the CIRCUP Randomized Trial.

BACKGROUND: Boys with posterior urethral valves (PUVs) have an increased risk of febrile urinary tract infections (fUTIs). Circumcision is believed to reduce the risk of fUTIs in boys, although there are no randomized trials demonstrating this. OBJECTIVE: To determine the effect of circumcision on the risk of fUTIs in boys with PUVs. DESIGN, SETTING, AND PARTICIPANTS: A clinical randomized trial that ran between August 2012 and July 2017 was conducted. The trial was multicentric, including 13 referral centers for pediatric urology. Male boys, aged 1-28 d, diagnosed with posterior urethral valves, confirmed by voiding cystogram, were included. The exclusion criteria included presence of a genital malformation contraindicating performing a circumcision. INTERVENTION: Participants were randomized to neonatal circumcision + antibiotic prophylaxis (CATB) or antibiotic prophylaxis alone (ATB), and followed for 2 yr. OUTCOME MEASUREMENTS AND STATISTICAL ANALYSIS: The primary outcome was a risk of presenting fUTIs in each group. An fUTI was defined as fever (>38.5 °C) with evidence of pyuria and culture-proven infection on urinalysis, obtained by urethral catheterization or suprapubic aspiration. A bivariate analysis of the primary outcome was performed using the Kaplan-Meier method. RESULTS AND LIMITATIONS: In total, 91 patients were included: 49 in group CATB and 42 in group ATB. The probability of presenting an fUTI was 20% in group ATB versus 3% in group CATB. The hazard ratio of presenting an fUTI within 2 yr in the ATB group compared with that in the CATB group was 10.3 (95% confidence interval: 1.3-82.5). Sixty-four children (70.3%) had a complete follow-up at 2 yr of age. CONCLUSIONS: Circumcision significantly decreases the risk of presenting an fUTI in boys with PUVs. PATIENT SUMMARY: In this report, we compared, in a multicentric trial, the number of febrile urinary tract infections (UTIs) in boys with posterior urethral valves who had either antibiotic prophylaxis alone or antibiotic prophylaxis and circumcision. We found that those who had a circumcision had a significantly lower risk of febrile UTIs.

[French linguistic validation of the Index of Sexual Satisfaction (ISS)]. / Validation linguistique française de l'indice de satisfaction sexuelle (ISS).

INTRODUCTION: The objective of this study was to propose a French version of the Index of Sexual Satisfaction (ISS). MATERIAL AND METHOD: The translation from English to French of the ISS was performed independently by three pediatric surgeons whose mother tongue is French. Then, an English speaker, fluent in French, who was unaware of the original questionnaire, translated the reconciled French version of the ISS into English. RESULTS: The final French version was successfully tested on 25 French-speaking volunteers. CONCLUSION: We present a validated translation of the Sexual Satisfaction Index.

The fallow mitrofanoff.

BACKGROUND: Treatment of severe congenital dysfunctional bladders often requires bladder drainage to maintain low bladder pressure, thus preserving renal function. Although clean intermittent catheterization is the ideal choice, this can be especially challenging in the younger pediatric population or in children with neurological impairment. Alternatives such as incontinent vesicostomy, long-term suprapubic catheterization, or button cystostomy exist, but these are rarely very long-term options. OBJECTIVE: The objective of this study is to report the authors' experience with children who underwent a 'fallow' Mitrofanoff, meaning an appendicovesicostomy in which an indwelling catheter was placed for several months or years, allowing for bladder emptying several times a day, until the child was ready for clean intermittent catheterization (CIC). STUDY DESIGN: All patients who underwent a Mitrofanoff appendicovesicostomy with or without concomitant bladder augmentation, for whom there was a significant delay (≥6 months) between surgery and implementation of CIC, were reviewed. In all these cases, the child showed obvious opposition to CIC. An indwelling catheter was left in place, with a stopper allowing for bladder emptying 5-6 times a day as would happen with CIC. The catheter was changed once a week until CIC was implemented. Complications including febrile urinary tract infections (fUTIs) during the fallow period and complications including leakage or stenosis during the CIC period were noted. RESULTS: The series includes 10 patients (7 boys and 3 girls), aged a median 41 ± 34 months (range: 23-144) at the time of the appendicovesicostomy (6 posterior urethral valves and 4 non-neurogenic neurogenic bladders). All underwent classic appendicovesicostomies. The delay before full implementation of CIC was a median 29.5 ± 24 months (range: 6-72). During the fallow period, 3 patients presented fUTIs. The catheter was closed, allowing for bladder drainage 4-6 times a day. There were no episodes of leakage from the Mitrofanoff or stomal stenosis during the fallow period or CIC period. Mean follow-up since the appendicovesicostomy is 66 ± 33 months and since initiation of CIC is 26 ± 26 months (range: 4-94). DISCUSSION: For children who require bladder drainage, an appendicovesicostomy can be performed even if CIC is not initiated immediately and be used as a suprapubic catheter or button cystostomy. When the child is ready, CIC can be initiated without need for further surgery and without risk for the conduit. The limitations of this study include its retrospective nature and the low number of patients. CONCLUSION: A Mitrofanoff appendicovesicostomy can be performed in a child requiring long-term bladder drainage and in whom classic CIC is not possible, even if CIC is not initiated immediately.

Is peritoneal dialysis feasible after laparotomy in children? A case-control series to compare outcomes.

OBJECTIVES: Peritoneal dialysis (PD) is the modality of choice for children with end-stage renal disease (ESRD) awaiting renal transplant; however, this option is sometimes avoided for those with previous laparotomy. The goal of this study was to compare the outcomes of PD in patients with and without previous laparotomy. PATIENTS AND METHODS: Twenty-four patients who had been started on peritoneal dialysis were retrospectively analysed. Group LAP consisted of six patients with previous laparotomy, and Group NO-LAP of 18 controls with either retroperitoneal or no abdominal surgery. The percentage of theoretical maximum volume of infusion, time to reach it, complications (infection and drainage difficulties), and number of catheters needed to finish therapy were analysed. RESULTS: The characteristics of patients and technique of insertion are presented in Table. The percentage of maximum theoretical volume of infusion was similar in both groups. Median of catheter survival was similar in both groups. Complications were divided into malfunction (slow drainage, obstruction or leak) and infection. Incidence of complications per catheter and per month of dialysis was ten times lower in Group NO-LAP. Peritoneal dialysis failed in one patient with recurrent intraperitoneal adhesions after adhesiolysis in Group LAP. CONCLUSION: Despite a higher incidence of complications (malfunction and infections), PD remains an acceptable option after laparotomy. In this series, it was sufficient in achieving adequate filtration in five patients.

Urethral duplication in girls: Three cases associating an accessory epispadiac urethra and a main hypospadiac urethra.

INTRODUCTION: Urethral duplication is extremely rare in girls, with less than 40 cases reported so far. Most of them present as a prepubic sinus. Literature is scare regarding aetiology, classification and management in other forms. This study presents three cases of sagittal urethral duplication in girls presenting a main hypospadiac urethra and an accessory epispadiac urethra. PATIENTS AND METHODS: Medical records were retrospectively reviewed of three girls with urethral duplication managed over a 30-year period at a single institution. Circumstances of diagnosis, management and outcomes were analysed. RESULTS: The oldest case presented as a neonatal retrovesical mass with an accessory clitoral stream, whereas the two more recent cases presented with antenatal hydrocolpos and bilateral ureterohydronephrosis. Cases 1 and 3 had an incomplete duplication, while Case 2 had a complete form. In Case 3, the duplication was associated with a urogenital sinus and an anteriorly placed anus. Management involved resection of the epispadiac accessory urethra to achieve continence, with dilatation and/or mobilisation of the hypospadiac one. All girls are now aged >5 years old and are continent, and one is old enough to have normal menstruation. Renal function is normal in all. The summary table presents the schematic anatomical description as shown on micturating cystourethrogram and endoscopy, as well as the management for each patient. DISCUSSION: Step-by-step management is necessary in urethral duplication. The neonatal emergency is to release the urinary tract compression by evacuating urinary retention or hydrocolpos. Later in infancy, decision has to be taken regarding the urethras. If the resection of the epispadiac accessory urethra seems acceptable to achieve continence, the attitude towards the hypospadiac channel is more controversial and should be individualised. Embryologic and aetiopathogenic pathways are still missing to uniformly characterise the malformation. CONCLUSION: Paediatric urologists should remember that there is a wide spectrum of urethral duplication in girls, and that various presentations exist beside the more classic prepubic sinus.

[Clinical and biochemical characterization of childhood urolithiasis]. / Caractéristiques cliniques et biochimiques des lithiases urinaires de l'enfant.

OBJECTIVES: Urolithiasis is rare in children, but the incidence has increased over the past few decades. This study aims at describing the clinical and biochemical characteristics, etiology, and treatment of urolithiasis in children. METHODS: This was a retrospective study of all children under 16 years of age seen at the Bordeaux University Children's Hospital with a diagnosis of urolithiasis. The diagnosis was confirmed either radiologically or clinically by the expulsion of the stone. RESULTS: A total of 186 children with a diagnosis of urolithiasis between 1994 and 2012 were included. The median age at diagnosis was 7.4 years. The male-to-female ratio was 1.9. The estimated annual incidence was around 5.5/100,000 children under 15 years of age in the past 5 years. The main presenting feature was nonspecific abdominal pain (71%). Metabolic calculi accounted for 48% of the patients with idiopathic hypercalciuria as the main cause. Genetic diseases accounted for 15% of cases. The proportion of infectious calculi was estimated at 33% and decreased in the past two decades. Stone fragments were sent for analysis in 86 children, and calcium oxalate was the major component (37%), followed by calcium phosphate (33%), purine (9%), and struvite (8%). At least 26% of patients experienced recurrence of stone passage. CONCLUSION: This retrospective study highlighted changes in characteristics of pediatric urolithiasis over time. Childhood-onset urolithiasis requires complete etiological work-up so that a metabolic cause with a high risk of recurrence does not go unrecognized.

Proximal humerus fractures in children and adolescents.

Proximal humerus fractures are rare in paediatric traumatology. Metaphyseal fractures account for about 70% of cases and epiphyseal separation for the remaining 30%. The development and anatomy of the proximal humerus explain the various fracture types, displacements, and potential complications; and also help in interpreting the radiographic findings, most notably in young children. Physicians should be alert to the possibility of an underlying lesion or pathological fracture requiring appropriate diagnostic investigations, and they should consider child abuse in very young paediatric patients. Although the management of proximal humerus fractures remains controversial, the extraordinary remodelling potential of the proximal humerus in skeletally immature patients often allows non-operative treatment without prior reduction. When the displacement exceeds the remodelling potential suggested by the extent of impaction, angulation, and patient age, retrograde elastic stable intramedullary nailing (ESIN) provides effective stabilisation. As a result, the thoraco-brachial abduction cast is less often used, although this method remains a valid option. Retrograde ESIN must be performed by a surgeon who is thoroughly conversant with the fundamental underlying principles. Direct percutaneous pinning is a fall-back option when the surgeon's experience with ESIN is insufficient. Finally, open reduction is very rarely required and should be reserved for severely displaced fractures after failure of closed reduction. When these indications are followed, long-term outcomes are usually excellent, with prompt resumption of previous activities and a low rate of residual abnormalities.

Evolution of the management of acquired neurogenic bladder in children using intradetrusor botulinum toxin type A injections: 5-year experience and perspectives.

OBJECTIVE: To report the clinical and urodynamic results of repeated intradetrusor botulinum toxin type A injections in children with an acquired neurogenic bladder. PATIENTS AND METHOD: We reviewed the data of 8 patients presenting an acquired neurogenic bladder treated between 2005 and 2010. Their mean age was 12.4 years old (range: 5-18). They were all on clean intermittent catheterization. All patients presented detrusor overactivity resistant to oral anticholinergic treatment. They received between 2 and 6 injections at a dose of 12 botulinum toxin units (BU)/kg (maximum 300 BU). Cystometry was performed 4-8 weeks after treatment. RESULTS: Five patients became completely dry, 2 were only rarely wet, and data are lacking for 1 patient. Febrile urinary tract infections ceased after 1 or 2 injections. The mean maximal detrusor pressure decreased below 40 cmH(2)O after 1, 2 and 3 injections. The normalized safe capacity rose significantly after 1, 2 and 3 injections. The normalized maximal bladder capacity rose similarly after 1, 2 and 3 injections although not always significantly. CONCLUSION: Intradetrusor botulinum toxin-A injections significantly reduce detrusor pressure and can be repeated with efficacy. They have their place in between anticholinergic treatment and surgery. The procedure could be simplified and the dosage reduced.

Long-term outcome of severe hypospadias.

OBJECTIVE: To evaluate, from the patient's point of view, long-term urinary and sexual outcome of surgery for severe hypospadias. PATIENTS AND METHOD: Patients treated for severe posterior hypospadias during childhood and now aged 20-35 years were included. Outcome was evaluated using the Hypospadias Objective Scoring Evaluation and our own quality of life questionnaire. RESULTS: Of 48 patients, 13 were lost to follow up. The questionnaire was thus sent to 35 patients of whom 27 agreed to answer (77% response rate). Fifteen presented proximal and 12 scrotal hypospadias. Average age at surgery was 3.3 years and the children underwent an average of 3.7 procedures. There were 40% early complications, and 59% late complications, including 33% fistulae and 26% stenosis. At present, 70% of patients have an apical meatus, 82% have a straight penis, 37% present difficulty initiating voiding, and one patient has a persistent fistula; 74% say their penis looks abnormal, either because of the absence of foreskin, penis size or scarring. Sexual and global satisfaction rates, determined using a visual analogue scale, are respectively 68% and 63%. Satisfaction is statistically correlated to the presence of early complications or stenosis, but not fistula. It decreases if the patient thinks his penis is not normal, even if the anatomical result is good. CONCLUSION: Urinary and sexual outcome is good for most patients though the results are variable. Satisfaction seems more affected by subjective feelings than the anatomical result.

[Indications and results of intradetrusor of botulinum toxin type A injections in children]. / Indications et résultats des injections intradétrusoriennes de toxine botulique type A chez l'enfant.

OBJECTIVE: The authors report their experience of intradetrusor injections of botulinum toxin type A (TBA) in children. This treatment was deliberately limited to patients with acquired neurogenic bladder, high detrusor pressures and urinary incontinence despite anticholinergic therapy. MATERIAL: Six children with a mean age of 11.6 years (range: five to 18 years) treated by intermittent catheterization presented an indication for intradetrusor injection of TBA. One half of these patients presented recurrent urinary tract infections. Their leak point pressure was greater than or equal to 40 cmH(2)O (mean: 67+/-33.6) and the bladder capacity of these children was less than the capacity predicted for age (mean: 68%+/-32.8). TBA was injected into 20 to 30 sites by cystoscopy at a dose of 12 IU/kg of body weight (maximum dose: 300 IU). RESULTS: No adverse effects were observed. Only one child still presented incontinence, but only during urinary tract infections. Four children were able to stop their anticholinergic treatment. Two months after the injection, all children had normal detrusor pressures (mean: 24.2+/-7.4 cmH(2)O). The maximum cystomanometric capacity then exceeded the predicted capacity (113%+/-22). CONCLUSION: In acquired neurogenic bladder with detrusor hyperactivity, TBA protects the upper urinary tract (by decreasing detrusor pressures) and controls urinary incontinence (by increasing the functional bladder capacity), without preventing subsequent bladder augmentation.

Intravesical instillation of ropivacaine reduces bladder spasms following paediatric ureteroneocystostomy.

OBJECTIVE: Bladder spasms are a common cause of pain after surgical procedures that call for postoperative catheter drainage. Several therapeutic methods have been used to lessen these spasms but none have received widespread success. PATIENTS AND METHODS: Twenty-six children were included in a prospective randomized trial to evaluate the safety and efficacy of daily intravesical instillation of ropivacaine as prophylactic treatment for bladder spasms following ureteroneocystostomy. RESULTS: Although six patients experienced mild transient pain during instillation, there was no systemic toxicity attributable to the ropivacaine. The average number of spasms per day fell by half in the instillation group (p<0.01). CONCLUSION: Intravesical instillation of ropivacaine is a feasible alternative prophylactic treatment for postoperative bladder spasms.

Complications occurring during conservative management of splenic trauma in children.

AIM: Conservative management of splenic rupture in haemodynamically stable children is now generally accepted. However, during follow-up, many complications can occur. The aim of this study was to describe the complications we observed and to propose a standardised follow-up adapted to them. METHODS: Between March 1992 and December 2002 we managed 65 children (aged between 3 and 15 years old) with accidental splenic rupture. Follow-up and treatment consisted of a 10-day bed rest with sonogram and Doppler controls on the 5th and 10th day and subsequently every month until complete healing. Complications included secondary haemorrhage in 3 cases, cystic evolution in 5 cases, and pseudo-aneurysm in 2 cases. All were treated by renewed bed rest. Three of the cysts resolved spontaneously, the other two underwent cystic resection and epiploplasty by laparotomy (n = 1) or laparoscopy (n = 1) and both pseudo-aneurysms were selectively embolised. There were no splenectomies. CONCLUSIONS: Complications essentially occurred in older children and were not related to gender, type of fracture, or extent of bleeding. Cystic evolution of the sub-capsular haematomas can appear up to 1 month after trauma. Peripheral pseudo-aneurysms which could be responsible for secondary haemorrhages were selectively embolised. We favour the use of the Doppler sonogram for follow-up until total recovery of these patients, even in low-grade traumas. Considering the number of complications encountered we do not believe the American evidence-based guidelines are suitable for our population.

The parapelvic renal cyst. A rare aetiology of blood hypertension in children.

Parapelvic renal cysts are uncommon in children. We report a case occurring in a 2-year-old girl, diagnosed during follow-up for blood hypertension. The cyst measured 8 cm in length. There was associated hydronephrosis, due to obstruction of the renal pelvis. Surgical resection was performed. Normotension returned within 3 months, and the obstruction disappeared. A renovascular mechanism can be evoked to explain hypertension.

Sacral neuromodulation in lower urinary tract dysfunction.

Vesico-urethral dysfunction is a major problem in daily medical practice due to its psychological disturbances, its social costs and its high impact on quality of life. Recently, sacral neuromodulation, namely the electrical stimulation of the sacral nerves, appears to have become an alternative for radical bladder surgery particularly in cases of idiopathic bladder overactivity. The mechanism of action is only partially understood but it seems to involve a modulation in the spinal cord due to stimulation of inhibitory interneurons. Temporary sacral nerve stimulation is the first step. It comprises the temporary application of neuromodulation as a diagnostic test to determine the best location for the implant and to control the integrity of the sacral root. If test stimulation is successful, a permanent device is implanted. This procedure is safe in experienced hands. So-called idiopathic bladder overactivity still the major indication for this technique. Patients not likely to benefit from the procedure were those with complete or almost complete spinal lesions, but incomplete spinal lesions seemed to be a potential indication. This technique is now also indicated in the case of idiopathic chronic retention and chronic pelvic pain syndrome. When selection is performed, more than three-quarters of the patients showed a clinically significant response with 50% or more reduction in the frequency of incontinent episodes, but the results vary according to the author's mode of evaluation. From the economic point of view, the initial investment in the device is amortized in the mid-term by savings related to lower urinary tract dysfunction. Finally, this technique requires an attentive follow-up and adjustments to the electric parameters so as to optimize the equilibrium between the neurological systems.

Distribution and regulation of L-type calcium channels in deep dorsal horn neurons after sciatic nerve injury in rats.

Deep dorsal horn neurons are involved in the processing of nociceptive information in the spinal cord. Previous studies revealed a role of the intrinsic bioelectrical properties (plateau potentials) of deep dorsal horn neuron in neuronal hyperexcitability, indicating their function in pain sensitization. These properties were considered to rely on L-type calcium currents. Two different isotypes of L-type calcium channel alpha 1 subunit have been cloned (Ca(V)1.2 and Ca(V)1.3). Both are known to be expressed in the spinal cord. However, no data were available on their subcellular localization. Moreover, possible changes in Ca(V)1.2 and Ca(V)1.3 expression had never been investigated in nerve injury models. Our study provides evidence for a differential expression of Ca(V)1.2 and Ca(V)1.3 subunits in the somato-dendritic compartment of deep dorsal horn neurons. Ca(V)1.2 immunoreactivity is restricted to the soma and proximal dendrites whereas Ca(V)1.3 immunoreactivity is found in the whole somato-dendritic compartment, up to distal dendritic segments. Moreover, these specific immunoreactive patterns are also found in electrophysiologically identified deep dorsal horn neurons expressing plateau potentials. After nerve injury, namely total axotomy or partial nerve ligation, Ca(V)1.2 and Ca(V)1.3 expression undergo differential changes, showing up- and down-regulation, respectively, both at the protein and at the mRNA levels. Taken together, our data support the role of L-type calcium channels in the control of intrinsic biolectrical regenerative properties. Furthermore, Ca(V)1.2 and Ca(V)1.3 subunits may have distinct and specific roles in sensory processing in the dorsal horn of the spinal cord, the former being most likely involved in long-term changes after nerve injury.

Right pulmonary agenesis associated with remaining bronchus stenosis, an equivalent of postpneumonectomy syndrome. Treatment by insertion of tissue expander in the thoracic cavity.

We report a case of right pulmonary agenesis with worsening of mediastinal shift during a viral bronchiolitis at the age of 5 months. The respiratory failure was due to stretching and narrowing of the remaining bronchus, as in postpneumonectomy syndrome. Weaning of respiratory assistance was possible after inserting a tissue expander in the empty side of the thoracic cavity. The correction of the shift brought a liberation of the left main stem bronchus. The main risk is heart compression. The child is now 3 years old and is at home. Filling of the prosthesis is done according to the clinical evolution.