Severe neonatal hypercalcemia revealing congenital mesoblastic nephroma: A case report and management of neonatal hypercalcemia: Severe neonatal hypercalcemia revealing congenital mesoblastic nephroma.

Congenital mesoblastic nephroma is a rare pediatric renal tumor and has been reported in patients presenting with palpable abdominal mass, arterial hypertension, hematuria, polyuria, or hypercalcemia. Here we present the case of a 1-month-old neonate with suspected parathyroid hormone (PTH)-related peptide (PTH-rp)-mediated severe hypercalcemia revealing congenital mesoblastic nephroma. Preoperatively, hypercalcemia was corrected with hydration, furosemide, pamidronate, and low-calcium infant formula. Unilateral nephrectomy led to the resolution of hypercalcemia, transient hyperparathyroidism, and transient vitamin D and mineral supplementation. We conclude that congenital mesoblastic nephroma can secrete PTH-rp that can cause severe hypercalcemia.

Acute Urinary Retention in the Male Child from Urethral Calculi: A Report of Three Cases.

Urinary stones are uncommon in children. Urethral location of calculi can give rise to various clinical manifestations. We report three cases of urethral lithiasis presenting with acute urinary retention in children.

TTC7A mutation must be considered in patients with repeated intestinal atresia associated with early inflammatory bowel disease: Two new case reports and a literature review.

TTC7A mutations cause multiple neonatal intestinal atresias with early inflammatory bowel disease and severe combined immunodeficiency. There are no treatment protocols for this rare disease. Two new cases are described for which radical early treatment measures - total enterectomy, home parenteral nutrition, immunoglobulin therapy and intravenous antibiotic prophylaxis - have allowed both patients to develop optimally.

Proximal hypospadias repair using the koyanagi-hayashi technique. A review of 15 cases.

BACKGROUND: Several surgical approaches or modifications of existing techniques have been described for the repair of hypospadias. In Sub-Saharan Africa, a two-stage approach is the preferred option in proximal cases with severe penile curvature. OBJECTIVE: The authors describe their experience with one-stage repair of proximal hypospadias with severe penile curvature using the Koyanagi-Hayashi technique. METHODOLOGY: Three hundred and ten patients were seen at the outpatient clinic from November 2009 to November 2015. All patients aged between 2 and 17 years with a confirmed diagnosis of proximal hypospadias and operated according to the Koyanagi-Hashashi technique were included in the study. RESULTS: The mean age at diagnosis was 6.7 ± 4.35 years (81 months). The level of the hypospadias was penile in 60%, scrotal 33.5%, and posterior in 6.6% of cases. The most common complications after primary repair were partial breakdown of the urethroplasty (44.8%), urethra-cutaneous fistula (3.3%), dehiscence of the glanuloplasty (22.2%), and recurrent penile curvature (11.1%). DISCUSSION: The high psychological implication of this condition in children and adolescents at the time of diagnosis in our context was a major weakness of our study. However, it turned out to be an advantage as the patients could be involved in the decision-making in as much as the previous gender was taken into consideration. CONCLUSION: A one-stage repair approach as described by Koyanagi-Hayashi also provides good aesthetic and functional outcome. It thus stands out as an alternative even in our African setting.

Challenges in the Diagnosis and Management of Acquired Nontraumatic Urethral Strictures in Boys in Yaoundé, Cameroon.

Introduction. Urethral strictures in boys denote narrowing of the urethra which can be congenital or acquired. In case of acquired strictures, the etiology is iatrogenic or traumatic and rarely infectious or inflammatory. The aim of this study was to highlight the diagnostic and therapeutic difficulties of acquired nontraumatic urethral strictures in boys in Yaoundé, Cameroon. Methodology. The authors report five cases of nontraumatic urethral strictures managed at the Pediatric Surgery Department of the YGOPH over a two-year period (November 2012-November 2014). In order to confirm the diagnosis of urethral stricture, all patients were assessed with both cystourethrography and urethrocystoscopy. Results. In all the cases the urethra was inflammatory with either a single or multiple strictures. The surgical management included internal urethrotomy (n = 1), urethral dilatation (n = 1), vesicostomy (n = 2), and urethral catheterization (n = 3). With a median follow-up of 8.2 months (4-16 months) all patients remained symptoms-free. Conclusion. The authors report the difficulties encountered in the diagnosis and management of nontraumatic urethral strictures in boys at a tertiary hospital in Yaoundé, Cameroon. The existence of an inflammatory etiology of urethral strictures in boys deserves to be considered.

Mixed gonadal dysgenesis in Yaoundé: A preliminary experience about three cases.

UNLABELLED: Mixed gonadal dysgenesis is characterised by unilateral chromosomal abnormality, which is probably the result of anaphase lag during mitosis. The 45, XO/46, XY karyotype is the most common form of mosaicism involving the Y chromosome. It is a rare clinical entity with a worldwide incidence of 1.5/10,000 live births. Its epidemiology in Sub-Saharan Africa is not known. This study reports experience in the management of 3 cases at the Yaounde Gynecologic-Obstetric and Paediatric Hospital. From November 2009 to November 2014, 3 cases were successfully managed at our institution. RESULTS: All patients presented with asymmetrical gonadal differentiation. On one side of the body, a poorly-developed testicular gonad and on the other side a gonadal streak. A persistent Mόllerian remnant was equally found in the 3 cases. Management of mixed gonadal dysgenesis should be done in tertiary health care centres. A multidisciplinary team approach is recommended.

A case report of Cowper's syringocele in an 18-months old infant at the Yaoundé Gynaeco-Obstetric and Pediatric Hospital.

Syringocele or dilatation of the duct of the bulbo-urethral (Cowper's) gland is usually of congenital origin but can be acquired. It is a very rare deformity, <10 cases have been reported in literature. The main objective is to describe an additional case of syringocele of Cowper's glands and review the literature. An 18-month-old infant presented with a history of acute urinary retention 3 days after birth and a cystostomy was done. Voiding cystourethrogram was normal and cystourethroscopy showed a syringocele. Endoscopic incision was performed in our patient with satisfactory results. No complications were noted. Syringocele or cystic dilatation of Cowper's gland duct usually has a congenital aetiology. Diagnosis is confirmed by endoscopy. Treatment is by marsupialisation in the urethra by endoscopy. Syringocele is a rare pathology usually congenital. It should be suspected in all case of lower urinary tract obstruction in children.

Our experience of proximal hypospadias repair using the Cloutier-Bracka technique at the Gynaeco-Obstetric and Paediatric Hospital, Yaounde-Cameroon.

BACKGROUND: In parts of Africa, routine circumcision is practised and sometimes even on children with hypospadias. The lack of preputial foreskin renders urethroplasty more difficult and often requires to use of a mucosal graft as described by Bracka. OBJECTIVE: The authors describe their experience of hypospadias repair using Bracka's technique. MATERIALS AND METHODS: Over a period of 5 years, 100 cases of proximal hypospadias were operated in our institution. All patients aged 0-18 years who had already been circumcised were included in this study. RESULTS: The outcome of the 12 cases operated according to Bracka's technique was analysed. The mean age was 11.5 years. The ectopic meatus was penoscrotal in three cases, scrotal in one case and perineal in eight cases. After reconstruction, the new meatus was sutured at the top of the glans in one case, at the prepuce in seven cases and at the penile midshaft in one case. The main complications noted were surgical site infection, wound dehiscence, residual chordee and urethrocutaneous fistula. No neourethral stenosis nor uretrocele was recorded. DISCUSSION: The buccal mucosal graft urethroplasty as described by Bracka is associated with a lower risk of meatal strictures compared to other free mucosal grafts. The buccal mucosa is easier to harvest and causes less scarring than bladder mucosa. CONCLUSION: Repair of severe hypospadias remains a challenge for paediatric surgeons. The functional and cosmetic outcomes depend on the choice of the donor site for the graft and objective assessment of successful reconstruction criteria during follow-up.

Continent cystostomy (Mitrofanoff's procedure) for neurogenic bladder in children and adolescent (56 cases: long-term results).

Between 1978 and 1991 we made a continent cystostomy according to Mitrofanoff's technique in 56 patients - children or adolescents - with a neurogenic bladder. We study the complications and the long-term results (follow-up ranging from 3 to 17 years - average 10 years). One patient died from a urological complication, another one died 2 years after from a neurological complication. Two patients were lost to follow-up after 3 years. Among the other patients 36 i.e. 64.3% have had urological complications. Analysis of these complications and their treatment demonstrates that most could now be avoided. At the present time the continent cystostomy with an appendicular or ureteral conduit is the safest and most comfortable way to treat the incontinence of wheel-chair bound patients who cannot or do not wish to use transurethral catheterisation.

[Malformations of male internal genitalia originating from the Wolffian duct]. / Malformations des organes génitaux internes masculins issus du canal de Wolff.

This review includes the description and treatment of the various malformations of the male internal genitalia of Wolffian origin. Congenital epididymal cyst is the most frequent anomaly; it is usually asymptomatic so that surgery is seldom necessary. Agenesis of the deferent is usually discovered during surgery for undescended testis and is frequently associated with a homolateral renal agenesis. Ectopia and duplication are other possible anomalies affecting the deferent. Seminal vesicle cysts is mainly discovered after puberty; pelvic pain and/or urinary symptoms are the main revealing signs. It is frequently associated with other uro-genital anomalies, mainly renal agenesis.

[Severe bladder dysfunction in the child abuse victim. Hinman syndrome]. / Dysfonctionnements vésicaux graves chez l'enfant victime de maltraitance. Le syndrome de Hinman.

Severe bladder dysfunction with possible consequence on the upper urinary tract caused by psychological trauma were recently identified. Hinman in 1973 was the first to report a series of 14 patients with severe abnormal bladder and bowels behaviour with organic cause. He described the most common symptoms met in this group of patients and their management using a non surgical approach based on bladder reeducation and hypnosis. Other authors such as Allen in 1977 reported a similar experience and found severe detrusor-sphincter dyssynergia. In 1995, Ellsworth reported a relation between these abnormal bladder behaviour and a medical history of sexual aggression. We report here in a series of 7 patients with Hinman syndrome associated with aggression. Diagnosis and management by a multidisciplinary team involving paediatricians, paediatric urologists, physiotherapists, paediatric psychologists are detailed [corrected].

[Drainage of pyelo-ureteral junction surgery: personal technique and review of the literature]. / Drainage après chirurgie de la jonction pyélo-urétérale: technique personnelle et revue de la littérature.

OBJECTIVES: To compare the various Stenting techniques used in ureteropelvic junction (UPJ) surgery and to present a personal series of 54 cases in which the Multipurpose Blue Stent (BARD, ANGIOMED) was used. PATIENTS AND METHOD: Between 1994 and 1998, 54 patients were treated for UPJ obstruction (52 underwent a Anderson-Hynes pyeloplasty and 2 a ureterocalicostomy). The Multipurpose Blue Stent insertion technique is described. The stent is removed on postoperative day 10 in the ward without cystoscopy. RESULTS: Clinical, isotopic and ultrasound improvement was obtained in 98% cases. Seven complications were reported: 4 urinary tract infections and 2 stent displacements without noticeable detrimental consequences on the outcome; 1 anastomotic leakage following a ureterocalicostomy requiring a redo operation (which was successful). The average hospital stay was 3 days. DISCUSSION: This stenting method is compared to those reported in the literature and proves to be more satisfactory in terms of hospital stay and removal. CONCLUSION: Postoperative leakage in non-stented operated UPJs is an under-reported but significant complication. Trans-anastomotic stenting has proven to be much safer than non-stenting. The Multipurpose Blue Stent reduces hospital stay and does not require a second hospitalisation to be removed.

[Current approach to hypospadias in children]. / Approche actuelle de l'hypospade chez l'enfant.

Hypospadias surgery has dramatically changed over the last 15 years with a new anatomical approach of the ventral penile anomalies and new techniques of repair. The description of the hypoplasia of the tissues forming the ventral aspect of the penis (ventral radius of the penis) beyond the division of the corpus spongiosum, and the concept of urethral plate are the two key-elements to select the most appropriate technique of reconstruction. These modern concepts and the various surgical techniques used are reported here with their results.

[Koff's urethral mobilization: report of 26 hypospadias presenting a distal division of the corpus spongiosum]. / Translation uréthrale de Koff: à propos de 26 hypospades présentant une division distale du corps spongieux.

AIM: To evaluate the Koff urethral mobilization in 26 patients with distal division of the corpus spongiosum. MATERIAL AND METHOD: Twenty six hypospadias underwent Koff's urethral mobilization between January 1st 1999 and January 31st 2001. All cases were performed by the senior author. The mean age at surgery was 36.3 months (14 to 117 months). All cases had a distal division of the corpus spongiosum. RESULTS: The mean follow-up was 4.64 months (1-24 months): Four cases (15%) presented with a penile bleeding when the dressing was removed 4 days after the procedure. One of them went back to theatre to stop the bleeding. Five (19.2%) presented with a late meatal stenosis requiring a secondary meatotomy. One had a urethral fistula which disappeared spontaneously a few months later. All had a satisfactory cosmetic result. DISCUSSION AND CONCLUSION: These results are compared with other series published and it seems that a full penile urethral mobilization is a significant alternative procedure whose main advantage is to avoid the use of any non-urethral tissues to reconstruct the hypospadiac urethra. The selection of cases must be cautious to avoid secondary meatal stenosis which seems to be related to ischaemia of the distal hypoplastic urethra.

[Treatment of neurogenic urinary incontinence in children]. / Traitement de l'incontinence urinaire neurogénique de l'enfant.

The current management of neurogenic urinary incontinence is exclusively urological and therefore palliative. However, urological techniques have considerably advanced over the last 20 years and now allow continence with protection of the upper urinary tract in the very great majority of cases. The authors review the various techniques proposed in achieve acceptable continence and define the indications based on published results and their personal experience.

[Analysis of the failure of endoscopic treatment of vesico-renal reflux in children using injections of teflon and collagen and the preliminary results of injections of Macroplastic]. / Analyse des échecs du traitement endoscopique du reflux vésico-rénal chez l'enfant par injections de téflon ou de collagène et résultats préliminaires des injections de Macroplastique.

This study of 785 cases of vesicorenal reflux in 494 children treated endoscopically over a 7-year period was designed to evaluate the results obtained with three products used successively: Teflon, collagen and Macroplastic. Following Teflon injection, despite a 90% short-term success rate, recurrent reflux was subsequently observed in 16.71% of the ureters reviewed. The failure rate was 52.63% after collagen injection and 11.77% after Macroplastic. After one or two injections, complete resolution of reflux was obtained in 48% of children treated with collagen, versus 85.72% with Teflon and 93.33% with Macroplastic. In one half of cases, failure was related to the quality of the product and its modifications after injection. The marked resorption of collagen accounts for the poor results despite the large doses injected. Apart from one case of partial resorption of Teflon paste, the failures with this product were due to lateralisation or secondary elimination of the product from the injection site due to its fluidity. Macroplastic, due to its higher viscosity and absence of retraction, currently provides the best results with doses of less than 0.20 ml in children.

[Malformations of Wolffian duct derived male genital organs (epididymis, vas deferens, seminal vesicules, ejaculatory ducts)]. / Malformations des organes génitaux masculins issus du canal de Wolff (épididyme, déférent, vésicule séminale, canal éjaculateur).

OBJECTIVES: To analyse embryological phenomena, in order to define a practical approach to management. METHODS: The authors reviewed the files of eight boys with an abnormality of Wolffian duct derived genital organs, consisting of three cases of vas deferens agenesis, two of which were accompanied by homolateral renal agenesis, one case of ectopic vas deferens with contralateral renal agenesis, three cases of duplicated vas deferens and one congenital seminal vesicle cyst with renal agenesis. DISCUSSION: Congenital malformations of the epididymis consist of cysts and agenesis or partial atresia. Cysts essentially raise a problem of differential diagnosis. Treatment is only required in the case of severe discomfort. Vas deferens agenesis is the commonest lesion, an incidental finding in children. An experimental treatment is proposed in adults. Duplications, interdeferential communications and deferentomegaly are much rarer lesions. Seminal vesicle cysts are well visualized by transrectal ultrasonography and should be treated surgically. Lastly, ejaculatory ducts may present urethroseminal reflux or may be ectopic. CONCLUSION: The possibility of absent or ectopic vas deferens should be considered in the case of unilateral renal agenesis. Vas deferens agenesis justifies examination of the contralateral side and investigation to exclude renal agenesis. Congenital cysts of the epididymis only require surgery when they are symptomatic. The presence of ipsilateral renal agenesis should be investigated in the case of cystic dilatation of the seminal vesicle.