Three-week or one-week bladder catheterization for hypospadias repair? A retrospective-prospective observational study of 189 patients.

PURPOSE: While there is little scientific evidence over the optimal duration for transurethral bladder catheterization after hypospadias repair, most surgeons leave the catheter for 7-10 days. We herein describe our experience with bladder catheterization for three weeks after hypospadias repair, an approach not previously described in the literature. PATIENTS AND METHODS: We reviewed the charts of 189 patients who underwent hypospadias repair by a single pediatric urologist. The study population was divided as follows: group 1 consisted of children operated between March 2007 and September 2010 and whose catheters were left for one week (n=95); group 2 consisted of those operated between September 2010 and July 2013 and whose catheters were left for three weeks (n=94). The primary objective of the study was to compare complication rates between the two groups. Secondary outcomes were evaluation of the effect of age, surgical technique, curvature, and hypospadias degree as potential factors for postoperative complications. RESULTS: Median age at hypospadias repair was 18 months (range, 3-100 months) in group 1, and 16 months (range, 2-96 months) in group 2, P=.209. The complication rate was 22.1% (n=21) for group 1 and 7.4% (n=7) for group 2, P=.005. Complications observed in group 1 and 2 were meatal stenosis (n=4 and 2, respectively) and urethro-cutaneous fistulas (n=17 and 5, respectively). Coronal fistulas manifested more frequently in patients in group 1 compared to those in group 2 (13.7% vs. 3.2%, P=.01). Complications were observed in 20 patients out of 139 (11.5%) after Duplay, and in 8 patients out of 15 (53.3%) after Duckett (P<.001). In Duplay cases, complications were significantly associated with one-week bladder catheterization (OR: 5.00; 95% CI: 1.53-16.32; P=.008) and higher age group at operation (OR: 1.88; 95% CI 1.07-3.28; P=.026). In Duckett cases, number of surgeries, age, severity, curvature and catheter duration were not found to be associated with complications. CONCLUSION: In cases of Duplay, a three-week instead of one-week catheterization and age below 6 months at hypospadias repair are associated with a better outcome and fewer complications.

Growing teratoma syndrome: first case report in a 4-year-old girl.

Growing teratoma syndrome (GTS) consists of germ cell tumors that grow following chemotherapy despite complete eradication of the malignant cells. They can metastasize to any site, particularly the retroperitoneum, mediastinum and cervical region. It typically affects young adults and adolescents. Here we describe the youngest case reported in a 4-year-old girl with an ovarian mixed germ cell tumor who underwent an oophorectomy. Her tumor markers normalized by the end of her chemotherapeutic treatment; however, she developed a retroperitoneal mass that was subsequently resected. Histopathology revealed a mature teratoma, consisting of a GTS. We stress the need for early recognition and treatment of GTS to avoid the subsequent morbidity and mortality associated with it. Although GTS has an excellent prognosis when completely resected, it is essential that the patient be regularly followed-up with serum tumor markers and imaging.

Renal medullary carcinoma in a white adolescent with sickle cell trait.

Renal medullary carcinoma (RMC) is a rare neoplasm of the kidney that has been recently described. It is almost exclusive to young patients of African descent and associated with sickle cell hemoglobinopathy, mainly sickle cell trait and hemoglobin sickle cell disease. The prognosis of RMC is very poor because of the highly aggressive behavior of this neoplasm and its resistance to conventional chemotherapy. Metastatic disease is almost universal at the time of presentation, and the malignancy is minimally responsive to a variety of regimens and/or modalities, including surgery, radiotherapy, chemotherapy, and biological immune-modulation therapy. We report the seventh case of a left RMC occurring in a white child with sickle cell trait, but with a localization of the tumor in the left kidney, considered a nonpredominant side for this type of tumor.

Congenital costo-vertebral fibrous band and congenital kyphoscoliosis: a previously unreported combination.

Congenital kyphoscoliosis (CKS) results from abnormal vertebral chondrification. Congenital fibrous bands occur in several locations with variable impact on vertebral development. We report a previously unreported case of a female infant with CKS presenting with an L2 hypoplastic vertebra and a costo-vertebral fibrous band extending to the skin in the form of a dimple. We also describe the therapeutic approach, consisting of surgical excision of the fibrous band and postoperative fulltime bracing, with a 7-year follow-up. We recommend a high index of suspicion in any unusual presentation of CKS and insist on case by case management in such cases.

Uncommon co-occurrence of ileal atresia and total colonic aganglionosis in two unrelated newborns.

We report two recent cases of distal ileal atresia associated with total colonic aganglionosis (TCA). It is well known that ileal atresia and Hirschsprung's disease (HD) are individually frequent causes of intestinal obstruction. However, the association of both these diseases is an extremely rare event. To our knowledge, only 19 cases of ileal atresia associated with HD have been described so far. When a child is diagnosed having ileal atresia, the possibility of associated TCA should be considered. Therefore, intra-operative staged biopsies should be sent for histological examination in order to rule out or confirm this very rare co-occurrence.

Ectopic gastric mucosa in the cervical esophagus presenting as a recurrent neck abscess: a case report.

We report a unique case of ectopic gastric mucosa (EGM) in the cervical esophagus. The patient presented with a recurrent cervical abscess communicating through a fistula with the EGM. Surgical treatment consisted of complete excision. The postoperative course was complicated by a breach in the hypopharynx, which was treated conservatively, and a stenosis of the esophagus requiring balloon dilation.

Is the Fowler-Stephens procedure still indicated for the treatment of nonpalpable intraabdominal testis?

PURPOSE: There are no formal guidelines for the management of boys with nonpalpable testis. In this article, we report our experience in treating all nonpalpable intraabdominal testis (NPIT) with standard inguinal orchidopexy without dividing the spermatic vessels stating that the Fowler-Stephens technique is no longer indicated for the treatment of the intraabdominal testis. METHODS: Between June 2003 and April 2008, we treated 23 boys with NPIT confirmed by ultrasound or laparoscopy. All cases were treated through an inguinal orchidopexy without division of the spermatic vessels by fixing the testis to the scrotum by 2 absorbable stitches even if there was an upward retraction of the scrotal skin. Location and size of testis were reported at 1 week, 1 month, 3 months, and 6 months through physical examination. RESULTS: Average age at presentation was 24 months. Ten patients (43%) had the NPIT on the right side, 8 (35%) on the left side, and 5 (22%) were bilateral. We had no intraoperative complications. All patients were discharged on the same day. In all cases, the testis was normal in size and found in the scrotum after 6 months of follow-up. CONCLUSION: On the basis of our experience, we believe that the Fowler-Stephens procedure is not indicated anymore in the management of NPIT. Orchidopexy without division of the spermatic vessels should be the treatment of choice even for the cases of very high intraabdominal testis because it does not affect normal testicular vascularization and is minimally invasive.

Prenatal diagnosis of an intrathoracic gastric duplication: a case report.

We report an intrathoracic gastric duplication cyst detected antenatally on obstetrical ultrasonography at 33 weeks of gestation. After birth, the newborn was directly intubated. At day 19, the intrathoracic cyst was removed, and the patient discharged on his 27th day of life. Histologic findings confirmed the diagnosis of a gastric duplication cyst. This appears to be the first reported case of an intrathoracic gastric duplication detected antenatally. Intrathoracic gastric duplication should be among the differential diagnosis of any mediastinal cyst discovered antenatally; its detection allows appropriate management at birth.

An unusual case of bilateral primitive obstructed megaureters due to ureteral valves presenting with anuria at the age of 1 month.

Ureteric valves represent a very rare etiology of ureteral obstruction. We experienced an unusual case of bilateral distal ureteric valves that presented as bilateral primitive obstructed megaureters with anuria at the age of 40 days. To our knowledge, this is the second case of bilateral involvement of distal ureteric valves reported in the literature. Bilateral ureteral valves should be included in the differential diagnosis of bladder outlet obstruction, as well as bilateral primitive obstructed megaureters in children. Excision and ureteral reimplantation is curative.

Topical application of mitomycin C in the treatment of esophageal and tracheobronchial stricture: a report of 2 cases.

We present 2 cases of successful treatment of recurrent anastomotic strictures using a topical application of mitomycin C. In the first case, a 4-year-old boy had a cervical cyst excised, which appeared to be an ectopic gastric mucosa. He consequently presented severe stenosis at the origin of the cervical esophagus that needed repeated balloon dilatations. The second case is about a 12-year-old girl who presented a traumatic complete rupture of the right mainstem bronchus managed by primary repair, with subsequent anastomotic stricture. Both patients were successfully managed with topical application of mitomycin C (1 mg/mL), and needed no more dilatations.

Upper airway obstructive symptoms because of ectopic gastric mucosa in a newborn: a case report.

An 8-month-old female infant was referred after unsuccessful attempts of extubation owing to upper airway obstruction by a pharyngeal soft tissue mass. Laryngoscopic examination revealed an obstructing mucosal fold originating from the posterior pharyngeal wall. The lesion was totally excised with remarkable improvement of the infant's deglutition and respiration. Histologic examination revealed an ectopic gastric mucosa. To our knowledge, this is the first report of ectopic gastric mucosa causing upper airway obstruction in a newborn.