Percutaneous nephrolithotomy in horseshoe kidney: comparing ultrasound-guided access in flank position with conventional fluoroscopic-guided in prone position.

We conducted this study to compare radiation-free US-guided percutaneous nephrolithotomy (PNL) in the flank position with conventional PNL in the prone position for the treatment of renal stones in patients with horseshoe kidneys. In a retrospective study, 14 HSK patients that were treated with conventional fluoroscopy-guided PNL in the prone position (group A) were compared with twenty-four HSK patients that were treated US-guided PNL in the flank position (group B). Data on baseline characteristics, percentage of successful entries, stone-free rate, duration of admission and complication rate were obtained from data registry. The average duration of the operation was 57.6 min in group B, which was statistically less than group A with 65.9 min (P = 0.001). Access time varied from 10 to 32.4 min (mean = 17.1 min) in group A and 5-29.5 min (mean = 10.9 min) in group B (P < 0.001). Access length had a significant relation to the surgery method so PNL with US-guided had less access length (P = 0.002). There was no significant relationship between the surgery guide and the residual stone rate (P = 0.6). Hemoglobin decrease (P = 0.5), hospitalization duration (P = 0.5) and need for blood transfusion (P value = 0.6) were not statistically different between the two groups. PNL with US guidance in flank position is a safe and effective technique in HSK patients and is associated with fewer complications than the traditional approach as well as reduced operating time, radiation exposure, and its complications.

Riñón en herradura: casualidad o embriopatía por fenilcetonuria materna / Horseshoe kidney: Chance or embryopathy due to maternal phenylketonuria

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Otofaciocervical syndrome and metachondromatosis in a girl: Presentation of a novel association and remarks on clinical variability of branchial-arch disorders.

Otofaciocervical syndrome (OFCS) is a rare disorder characterized by facial, ear, branchial, and musculoskeletal anomalies, along with hearing loss and mild intellectual disability. Clinically, its distinction from branchiootorenal syndrome can be difficult. To date, the coexistence of OFCS and metachondromatosis has not been reported. Here, we describe a sporadic patient with both OFCS and metachondromatosis. This novel association prompts us to do some remarks on the clinical variability of branchial-arch disorders; in fact, our observations are consistent with the highly variable expressivity of OFCS and illustrate the need of a more accurate characterization of these branchial-arch disorders. In the meantime, involvement of clavicles, scapulae and shoulders remains a distinctive feature of OFCS.