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.

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.

Cardiac transplant vasculopathy treated by percutaneous coronary intervention.

AIM: Cardiac transplant vasculopathy is a limit to long-term survival in heart transplantation (H-Tx) recipients. PTCA results in our H-Tx population were retrospectively analyzed. METHODS: From November 1985 to May 2004, 767 patients underwent heart transplantation. All patients received immunosuppressive therapy with cyclosporine or tacrolimus, azathioprine, steroids and mycophenolate mofetil. Lymphocyte was administrated by 3-7 days course of either rabbit antithymocyte globulins or anti-lymphocyte globulins or by a 14 days course of OKT3. Coronary angiograms were performed every year and more frequently if graft vasculopathy was already diagnosed or suspected. RESULTS: Fifty-two coronary artery lesions were treated during 42 percutaneous transluminal cardioangioplasty (PTCA)/stent procedures in 36 patients. Mean time since heart transplantation to PTCA was 80 +/- 27 months. Indication to PTCA was asymptomatic angiographic graft vasculopathy in 34 patients (94%) and acute myocardial infarction in 2 patients (6%). PTCA was performed on left anterior descending artery in 34 cases (65.4%), on circumflex artery in 10 cases (19.2%), on right coronary artery in 8 cases (15.4%). There were no procedure related deaths. None of the patients required emergency bypass surgery. Two patients had transient acute renal failure. Patient follow-up showed 10 deaths after 1 +/- 54 months from PTCA. Six died for progression of graft vasculopathy, three for cancer and one for gastrointestinal bleeding. Two patients underwent heart retransplantation after 20 and 107 months from the first procedure. Mean follow-up of the remaining patients is 78.3 +/- 50.3 months. CONCLUSION: PTCA may represent a reasonable treatment for graft vasculopathy in selected heart transplant recipients.

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.

[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].

[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.