Treatment outcome of neurogenic bladder dysfunction in children; a five-year experience

A neurogenic bladder is one which functions abnormally due to disorders of sacral nerves that control the bladder's ability to fill, store and empty urine. Abnormal bladder function can cause the bladder to be underactive or overactive. This study was planned to evaluate the treatment outcome of our patients with neurogenic bladder dysfunction [NGBD]. Thirty three patients who have been treated for NGBD were evaluated. Diagnosis was confirmed by voiding-cysto-urethrography [VCUG] and urodynamic study. The patients were treated medically and all had clean intermittent catheterization [CIC]. Data regarding age, sex, clinical and paraclinical findings, sonography, imagings, renal scan, associated anomalies, treatment and outcomes were collected and entered in SPSS software version 18 and analyzed by descriptive statistical. Totally 33 patients aged three days to four years [mean 6.8 months] were included in this study. There were 20 [61%] males and 13 [39%] females. Mean follow-up period was 3.4 +/- 1.2 years [1.5 months to 5 years]. Eighty two precent cases had bilatral and 18% unilatral hydronephrosis and bilatral vesicouretral reflux [VUR] existed in 67% and unilatral in 33% of the patients. Treatment consisted of antibiotherapy and CIC in all patients, which was only in 33% of the cases succesful. The most common associated anomaly was meningomyelocle in 8 patients. Vesicostomy was performed in 22 [67%] cases. Kidney scan showed scar in 10 patients at follow-up study. Complete continence on follow-up was achieved in 24 [71%] patients, and it was improved in 6 [18%] cases. Mortality rate was 9% [3 cases]. Cure rate was 85% in urinary tract infection, 82.7% in hydronephrosis, 80% in VUR and 86.5% in kidney function. Anticholinergic medications was not effective in all our patients. We believe that permanent vesicostomy is an effective and acceptable surgical intervention for protection of upper urinary tract decompression, especially in those who do not respond to medical treatment and have high risk position

Posterior urethral valves; a single center experience

Posterior urethral valves [PUV] are the most common cause of bladder outlet obstruction in infancy that impair renal and bladder function. This study was planned to evaluate and record the various clinical presentations and management, complications, and surgical management and long-term outcome of PUV. In a retrospective study, 98 patients who have been treated for PUV are evaluated in Mofid Children's Hospital from January 2007 to December 2012. Detailed history taken and paraclinical examinations were performed in each patient and diagnosis was confirmed by voiding-cysto-urethrography [VCUG]. PUV had been ablated in 62 patients by electric hook, and diversion was performed in 42 [42.85%] cases. Data were analyzed by SPSS software versionlS. Totally 98 patients with mean age at diagnosis 62 [ +/- 13] days were included in this study. Fifty seven cases had been catheterized within one to 6 days of life [mean age one day], PUV was ablated in 62 patients by electric hook, and dive rsion was performed in 42 cases. The most common symptom in our group was dribbling poor stream 51% and urinary tract infection [UT1] 40.8%. There was vesicoureteral-reflux [VUR] in 61.2%, and hydronephrosis in 82.6%. Most common associated anomaly was kidney anomalies [multicystic kidney disease and renal agenesis/dysplasia] in 8 [8.2%] patients. Twenty patients had prenatal diagnosis of PUV. Complication occurred in three [3.1%] patients. Mortality occurred in 5 [5.1%] patients. Mean follow-up period was 3.4 +/- 1.2 years [1.5 months to 5 years]. Urinary drainage by feeding tube in early days of infancy, followed by valve ablation is the best treatment in PUV, and urinary diversion improves the outcome. VCUG is still the gold-standard imaging modality for documenting PUVs. The factors like renal dysplasia and UTI have their role in final outcome

Abdominal inflammatory myofibroblastic tumor: report on four cases and review of literature

The Abdominal Inflammatory Myofibroblastic Tumor [AIMT] is a rare tumor with unknown etiology which usually occurs in children and adolescents. It is composed of myofibroblastic spindle cells intermixed with inflammatory cells. We present four cases of AIMT. We herein present four cases of AIMT in different ages [range: 3.5 to 13 years] and in different organs [stomach, periduodenal, mesenteric, and colon]. There were two females and two males. The main symptoms were abdominal pain/mass/obstruction, vomiting, and weight loss. In all four patients, diagnosis was made by laparatomy and pathologic examination of excised mass lesion. Three patients underwent complete excision and no residual disease was present, one patient received chemotherapy due to tumor recurrences. The patients were followed up in average for four years. As the imaging and laboratory tests are non-specific, the diagnosis of AIMT is rarely made before surgery. AIMT should, therefore, be considered when a mass arises in an unusual location in the pediatric age group. Complete surgical resection should be performed whenever possible and the child should be kept on long-term follow-up

Congenital nasopharyngeal teratoma in a neonate

Congenital germ cell tumors are uncommon. The most common site of teratoma is in the sacrococcygeal region. Teratoma arising from the head and neck comprises less than 10% of reported cases and of these, nasopharyngeal lesions are rare. Teratomas are generally benign, and have a well recognized clinical and histopathological entity. We present a case of nasopharyngeal teratoma [NPT] associated with a wide cleft palate. A 20 day old female neonate with a teratoma of the nasopharyngeal area, and wide cleft palate was referred to our center. The protruded mass which measured 6x4x3cm, was of soft consistency, blocked the airway, and prevented oral feeding. Preoperative evaluation and imaging was performed and mass was excised 2 days after admission. Pathology revealed a well-differentiated mature solid teratoma [hairy polyp]. The patient had no complication in the post-operative period. Cleft palate was surgically repaired when 2 years old. She is now a six year old girl with normal development. Congenital nasopharyngeal teratomas are usually benign. Surgery is the treatment of choice, and should be undertaken on an urgent basis, especially in a patient who presents with signs and symptoms of airway obstruction

Diphallus: report on six cases and review of the literature

Diphallus is an extremely rare anomaly. Numerous associated genitourinary, gastrointestinal and other anomalies have been described with diphallus. These patients need several investigations, and finally surgical intervention. In this report we discuss six patients with diphallus which evaluated retrospectively. Five patients had complete diphallia, and one had bifid diphallus. Meatus was normal in 3, hypospadiac in 2, and epispadiac in one patient. The most common associated anomaly was bifid scrotum [5 cases], and other common anomalies consisted of bladder duplication [3 cases], imperforate anus [2 cases], and hypospadias [2 cases]. Phalloplasty was performed for all but one. All the patients with urethral duplication have to be evaluated carefully because of the high incidence of other systemic anomalies