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.

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.

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.

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.

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.

Long-term results of bladder neck reconstruction for incontinence in children with classical bladder exstrophy or incontinent epispadias.

OBJECTIVE: To review the long-term results of bladder neck reconstruction (BNR) in patients with classical bladder exstrophy or epispadias, and to review the concept of continence surgery in these two groups, stressing the difficulty in finding an adequate balance between urine storage (which implies high outlet resistance and low storage pressure) and complete bladder emptying (which implies low outlet resistance and a transient increase in bladder pressure); surgery cannot achieve 'continence' (which implies active mechanisms) but only 'dryness' (which implies passive mechanisms). PATIENTS AND METHODS: Eighty patients with classical bladder exstrophy (52 male, 28 female) and 25 with incontinent epispadias (17 male, 18 female) had their bladder neck reconstructed after a Young-Dees-Leadbetter procedure, subsequently modified by Mollard. The treatment is detailed and results reviewed after a mean follow-up of 11 years. All patients were treated and followed in the same institution. RESULTS: In the exstrophy group, 36 (45%) patients presented with a dry interval of > 3 h, with urethral emptying after one BNR; 52 (65%) presented with recurrent urinary tract infections, 19 (24%) with urinary stones, 21 (26%) with dilated upper urinary tracts, 13 (16%) with bladder perforations and one with an adenocarcinoma of the bladder. Thirty-eight patients (48%) required further surgery; 51% of all patients required an endoscopic procedure within 3 months after the BNR and 26% had endoscopic procedures for late (> 3 months) urine retention. In the epispadias group, 13 (52%) patients presented with a dry interval of > 3 h with urethral emptying after one BNR; 12 (48%) had recurrent urinary tract infections, five (20%) upper tract dilatation, two (8%) bladder stones, one (4%) bladder perforation and one an adenocarcinoma of the bowels after a ureterosigmoidostomy. Ten (40%) children required further surgery. CONCLUSION: We compared the present results for continence with those in other published series; most complications encountered were related to the obstructive pattern of bladder emptying and the abnormal bladder urodynamic behaviour caused by BNR. We consider that BNR is unpredictable and the roles of the other factors in urinary continence are discussed. Alternative procedures are detailed. The concept of continence surgery in exstrophy and incontinent epispadias is reviewed, stressing the importance of favouring bladder development and limiting obstructive patterns of bladder emptying that cause severe and recurrent complications.

The left Monti-Malone procedure: Preliminary results in seven cases.

BACKGROUND/PURPOSE: The management of children who have congenital intestinal dysfunction and continuous feces soiling has been improved significantly by the Malone continent caecostomy. When the appendix is not available, the Monti tube represents a good alternative to create a catheterizable conduit. The authors report here 7 cases of left continent colonic access (left Monti-Malone). METHODS: From July 1999 to January 2001 7 patients have been operated on in our unit: 6 spina bifida, 1 cloacal exstrophy. Technically, a ring of descending colon of 1.5 to 2 cm width is isolated with its meso, the Monti's tube fashioned, and implanted into the left colon according to the Malone's technique. Antegrade bowel washouts start 3 weeks after surgery with standard saline (200 to 500 mL) +/- phosphate enemas. A period of 1 to 3 months is needed to adjust to the right enema regimen to get a satisfactory result. RESULTS: The follow-up range is 18 months to 1.5 months. One patient is excluded. The duration of the enemas varies between 10 and 30 minutes, and the number of enemas varies from 1 to every 2 or 3 days. Five children are clean and perform their antegrade enemas after dinner to avoid the embarrassment of post enema leakages. CONCLUSION: Although it is a short series with a short follow-up, the first results collected show a significant shortening of the duration of the enema with excellent outcomes in terms of continence.

Umbilical reconstruction in patients with exstrophy: the kangaroo pouch technique.

PURPOSE: Before the current attitude of umbilical preservation and transposition at bladder closure in patients with exstrophy the navel was systematically removed. Many patients without an umbilicus complain about this deformity. We report a simple technique of umbilical reconstruction using a rectangular skin flap fashioned as a kangaroo pouch. MATERIALS AND METHODS: Four patients 10 to 20 years old with exstrophy underwent this procedure. A small cutaneous pouch was fashioned by folding a vertical rectangular skin flap and the pouch was anchored deeply to the rectus fascia. A compressive dressing was packed into the new umbilicus and left in place for several days. RESULTS: The 4 patients have an excellent cosmetic result with adequate location, good morphology and sufficient depth at a mean followup of 8 months (range 6 to 11). CONCLUSIONS: Our technique of umbilical reconstruction is easy to perform and provides good mid-term cosmetic results. This technique is particularly adapted for patients with exstrophy who often require external genital reconstruction at the same time.

[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.

Transureteroureterostomy in childhood and adolescence: long-term results in 69 cases.

PURPOSE: We analyzed a series of 69 transureteroureterostomies to evaluate long-term results and specify current indications. MATERIALS AND METHODS: Between 1969 and 1998 transureteroureterostomy was performed in 32 females and 37 males with a mean age of 8.6 years. Surgery was done to avoid repeat or difficult ureteral reimplantation after multiple failed procedures in 22 cases and to create a continent ureteral conduit for intermittent catheterization in 23. Other indications included undiversion in 8 cases, ureterocystoplasty in 6, diversion in 4, the Kropp procedure in 3, massively dilated megaureter in 2 and ureteral necrosis in 1. Of the 69 patients 6 were lost to followup and 63 were followed at least 1 year (median 6). RESULTS: A total of 63 patients were regularly monitored by clinical observation and morphological investigation, including ultrasound, excretory urography and cystography. In 50 cases (79.4%) results were good with no upper urinary tract dilatation. All initially normal donor ureters remained normal. Of 51 initially dilated donor ureters 40 (78.4%) improved or returned to normal, while 20 of 27 initially dilated recipient ureters (74.1%) improved or returned to normal. Serious complications in 3 cases (4.3%) involved anastomotic leakage, ischemic stenosis of the common ureteral trunk and progressive deterioration of function in 1 kidney requiring nephrectomy 3 years postoperatively. Reoperation was successful in the former 2 cases. CONCLUSIONS: With careful attention to technique transureteroureterostomy represents a safe and reliable procedure with well-defined indications in pediatric urology.

[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.

[Proximal hypospadias]. / Hipospadias proximal.

OBJECTIVE: To evaluate the outcome of surgery in posterior hypospadias. METHOD: The technique of preservation of the urethral plate is described following three main steps: 1.-The first step is the correction of chordee which is mainly related to the two fibrous and vascular pillars sitting on each side of the urethral plate and representing the division of the atretic corpus spongiosum. Tethering of the urethral plate onto the anterior surface of the corpora cavernosa is another common cause of chordee which can be relieved by untethering the urethral plate from the ectopic meatus up to the glans cap. The urethral plate can be preserved in most cases of posterior hypospadias (35/43) and dorsal plication of the corpora cavernosa is rarely needed. 2.- The second step of this surgery is urethroplasty, which is usually achieved by using a pediculized preputial patch stitched to the urethral plate (onlay urethroplasty). 3.- Penile cover using the skin sleeve technique completes the procedure. RESULTS: Twenty one per cent fistulae, 1 meatal stenosis, no proximal stenosis are reported and discussed. CONCLUSION: Preservation of the urethral plate is possible in most posterior hypospadias and permits an onlay urethroplasty which remains to date the most reliable procedure to reconstruct the urethra in posterior hypospadias.

Male epispadias: experience with 45 cases.

PURPOSE: We evaluate the long-term results of surgery for isolated (without exstrophy) male epispadias. MATERIALS AND METHODS: A total of 45 male patients with isolated epispadias were treated and followed at Debrousse Hospital between 1971 and 1993, 8 in whom repairs performed elsewhere had failed and 14 had total urinary incontinence. Incontinence was treated with a modified Young-Dees reconstruction of the bladder neck and the ureters were reimplanted in all cases. Long-term followup was available in 18 cases of a Cantwell-Young-Gross, 4 Duplay, 7 full thickness skin graft, 1 bladder graft and 10 Cantwell-Ransley urethroplasties. Penile reconstruction was performed using a 2-stage procedure in 8 patients (freeing of the corpora followed by urethroplasty), and an isolated urethroplasty in 13. All other patients underwent a single stage repair combining dissection of the penis and urethroplasty. RESULTS: All patients were reassessed clinically. Of 13 incontinent patients followed for 1 to 20 years 11 acquired continence, 1 remains incontinent and 1 underwent a repeat procedure recently. Urethroplasty was immediately satisfactory in 66% of the cases, minor complications developed in 25% and serious complications (especially free graft urethroplasty) developed in 10%. Assessment of genital cosmesis is subjective but it was considered acceptable in most cases. All 29 patients with long-term followup had erections, 24 performed regular sexual intercourse, 17 had normal ejaculations and 4 fathered children. CONCLUSIONS: The principles described by Cantwell-Young and Gross, and modified by Ransley, have considerably improved penile reconstruction. Free graft and Duplay urethroplasties provided unacceptable results in our series. Our results of surgery for incontinence associated with isolated male epispadias (84%) were better than for male exstrophy (63%).

Female epispadias.

PURPOSE: We assessed the incidence, anatomical features and treatment of female epispadias. MATERIALS AND METHODS: We studied 10 patients followed from 1 to 18 years. Four were operated on in 2 stages: first, cervicoplasty with original technique, and second, reconstruction of genitalia and distal urethra. Two had only a cervicoplasty. The last 4 patients were operated on in 1 stage according to Hendren's technique but with a simplified cervicoplasty. RESULTS: Eight of 10 patients are totally continent, 1 has some small nocturnal leakage, and 1 is incontinent but the interval of dryness is increasing and is longer than 1 hour. Concerning external genitalia, the result is good but not perfect. CONCLUSIONS: Female epispadias is most often complete with total incontinence. The aim of treatment is to reconstruct the anatomy in 1 stage. Treatment is easier and the results are better than in cases of female exstrophy or male incontinent epispadias.

[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.