Our initial experience with the technique of complete primary repair for bladder exstrophy

ObjectiveWe reviewed our initial results with complete primary repair of exstrophy in regard to continence status and the need for subsequent continence procedures.Patients and methodsWe performed a retrospective review of our surgical records from 1996 to 2008 to identify all patients with bladder exstrophy managed at our center.ResultsSixteen children were closed successfully. Six patients (37.5%) experienced complications: umbilical hernias in two, transient penopubic fistula in three, and subcoronal fistula due to meatal stenosis in one. Of the 12 males, seven (58.3%) were left with a hypospadias at the time of primary closure. Two (22.2%) children required a formal bladder neck reconstruction to achieve continence. Bladder augmentation and continent catheterizable stoma was performed in four cases (44.4%), and bladder neck injection in one case (11.1%). Bladder neck closure was also performed in another child following primary closure. Three of these children are continent and void spontaneously (33.3%). The remaining six require clean intermittent catheterization four to six times a day, resulting in four (44.4%) being continent. The number of continence procedures and mean number per patient were 15 and 1.66, respectively.ConclusionOur early experience with this technique has been encouraging, with few major complications, a highly successful closure rate and a cosmetically normal result.     

Anterior urethral valves: an uncommon cause of obstructive uropathy in children

PurposeAnterior urethral valves (AUV) are rare entities generally described in case reports. They are an uncommon cause of lower urinary tract obstruction in children and can be difficult to diagnose. In the present study, we present our experience in four children with AUV along with a literature review.Materials and methodsWe retrospectively identified four children with AUV presented between 1998 and 2005 at age 4–9 years.ResultsHematuria, urinary tract infection and weak voiding stream were the most common symptoms. Voiding cystourethrography (VCUG) confirmed the diagnosis of AUV. On cystourethroscopy, cusp-like valves in the anterior urethra were seen in all children. Transurethral endoscopic resection of the valves was carried out in three children using a pediatric resectoscope. In one child with a massive anterior urethral diverticulum, open resection of the valve, diverticulectomy and urethroplasty were performed. All patients were cured, none had complications as a result of surgery, and all reported a normal urinary stream at follow-up.ConclusionsChildren with poor stream and recurrent infections should be evaluated carefully and anterior urethral valves should be considered in differential diagnosis of obstructive lesions.     

Global aphasia due to left thalamic hemorrhage

Global aphasia is an acquired language disorder characterized by severe impairments in all modalities of language. The specific sites of injury commonly include Wernike's and Broca's areas and result from large strokes--particularly those involving the internal carotid or middle cerebral arteries. Rarely, deep subcortical lesions may cause global aphasia. We present three cases with global aphasia due to a more rare cause: left thalamic hemorrhage. Their common feature was the large size of the hemorrhage and its extension to the third ventricule. HMPAO-SPECT in one of the cases revealed ipsilateral subcortical, frontotemporal cortical and right frontal cortical hypoperfusion. Left thalamic hemorrhage should be considered in the differential diagnosis of global aphasia.     

Effect of lower infundibulopelvic angle, lower infundibulum diameter and inferior calyceal length on stone formation

The effect of anatomical factors such as lower infundibulopelvic angle (LIPA), lower infundibulum diameter (LID) and inferior calyceal length (ICL) on renal stone formation was investigated. These parameters were measured from noncalculous kidneys of 40 healthy kidney donors. The same parameters from 119 patients with single, unilateral, nonobstructive lower calyceal stone were also measured. LID and ICL were significantly higher in calculous kidneys when compared to the control group. On the other hand, the difference between the LIPA of the two groups was not significant. It is concluded that LID and ICL could be good indices in determining lower calyceal stone formation. Copyright Copyright 1999 S. Karger AG, Basel     

99mTc tetrofosmin scintigraphy in acute leukaemia: the relationship between marrow uptake of tetrofosmin and P-glycoprotein and chemotherapy response

BACKGROUND: The non-invasive detection of P-glycoprotein (Pgp) and multidrug resistance related proteins in vivo, will represent the greatest challenge in overcoming multidrug resistance. Although 99mTc tetrofosmin has been used previously as a myocardial perfusion agent, it is now also being used in the imaging of various tumours. In the current study, Tc tetrofosmin was used in the investigation of acute leukaemia. AIM: To show the uptake pattern of 99mTc tetrofosmin in the bone marrow of patients with acute leukaemia, and to ascertain the relationship between 99mTc tetrofosmin uptake and the level of Pgp expression and their relation to the response to chemotherapy. In addition, CD95, which is an indicator of apoptosis (programmed cell death), has also been assessed. MATERIALS AND METHODS: Pgp and CD95 were detected by using flow cytometry. Of the 27 acute leukaemia patients assessed, nine had previously received chemotherapy, and 18 had had an initial diagnosis. All patients had undergone 99mTc tetrofosmin scintigraphy, and their Pgp and CD95 levels had been determined. The same parameters were studied again for 14 patients. The responses to chemotherapy were assessed by patients' clinicians. A control group of 37 patients without bone marrow pathology was also studied in order to provide comparisons for the scintigraphy results. The control images were assessed only qualitatively. RESULTS: In leukaemia patients the uptake of 99mTc tetrofosmin into bone marrow was found to be considerably higher than in control patients (P=0.000). An analysis of the relationship between Pgp, CD95, and the qualitative and quantitative tetrofosmin uptake ratios (URs) showed that there was an inverse correlation only between Pgp and the quantitative uptake ratio (P=0.016, r=-0.461). When the patients were grouped as 'good' and 'poor', as related to the chemotherapy response, there were no meaningful differences between these two groups regarding Pgp, CD95 and tetrofosmin URs (P>0.05). By evaluating the scintigraphic findings of the 'repeated' 14 patients, we showed that if the 99mTc tetrofosmin UR in the second imaging test was reduced by >0.08, the response to chemotherapy tended to be good. This method, based on follow-up scanning with tetrofosmin, showed a sensitivity of 83% and a specificity of 62% in the prediction of a 'good' response, if a decrease of 0.08 was taken into consideration. CONCLUSION: In this study, patients with acute leukaemia showed significant uptake of tetrofosmin into the bone marrow. The addition of basal and repeated 99mTc tetrofosmin scintigraphy to the management protocol for leukaemia could lead to the preferential determination of responses to chemotherapy, by evaluating whole bone marrow non-invasively. This method seems promising, but it needs further support from various similar investigations comprising more patients in order to confirm our results.