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Introduction

Currarino's syndrome (CS) is an autosomal dominant disorder of embryonic development causing a rare malformating syndrome characterized by a triad of an anorectal malformations, presacral mass (most commonly an anterior sacral meningocele) and sacral bony defects. Mutations of the HLXB9 gene have been identified in most CS cases, but a precise genotype-phenotype correlation has not been described so far. Family screening is obligatory. The diagnosis is usually made during childhood and rarely in adulthood. In this context, imaging, and especially MRI plays a major role in the diagnosis of this syndrome. Surgical management is provided by pediatric surgeons or neurosurgeons.

Familial case report

Here, we present a family case report with CS requiring different neurosurgical management. The son, a 3-year-old boy, developed a tethered spinal cord syndrome associated to a lipoma of the filum terminale, a sacro-coccygeal teratoma and an anal adhesion. A combined surgical approach permitted a good evolution on the urinary and digestive functions despite a persistent fecal incontinence. The 2-year-old daughter presented with a cyst of the thyreoglossal tract infected and fistulized to the skin. She was also followed for a very small lipoma of the filum terminale that required a neurosurgical approach. The father, 44-year-old, manifested functional digestive and urinary disorders caused by a giant anterior sacral meningocele. The ligation of the neck of the cyst and aspiration of the liquid inside in full through a posterior partial approach permit a complete collapse of the cyst with an instantly satisfactory clinical outcome.

Conclusion

In these cases, cooperation between pediatric surgeons and neurosurgeons was crucial. The follow-up of these patients should be done in a spina bifida clinic. A geneticist evaluation must be offered to the patient in the case of a CS as well as a clinical evaluation of the relatives (parents, siblings).  相似文献   
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Objective

To assess the functional effects of prostatic cryosurgery on micturition.

Material and methods

Prospective study of men who underwent cryosurgery (CS) for prostate cancer between 2013 - 2015. Low urinary tract symptoms (LUTS) and quality of life (QoL) were assessed 1 month before surgery using IPSS questionnaire, a three-day voiding diary (3 DVD) and uroflowmetry with ultrasound-measured postvoid residual volume. Need of medical treatment for LUTS was also recorded. The same assessment was performed at 3, 6 and 12 months after CS. Outcomes after surgery were compared to those prior to surgery.

Results

Forty-five patients underwent a CS during the study period, of whom 25 patients could be recruited in the study. Mean age was 73.5 years (range 66-84). Nineteen CS (76%) were performed as a primary procedure, while 6 CS (24%) as a salvage procedure. No statistical differences were found comparing results of IPSS, QoL, D3vd or uroflowmetry and PVR at 3, 6 or 12 months after CS compared to before surgery. Before CS, 8 (32%) patients were on medical treatment for LUTS, while at 6 and 12 months after surgery, 3 (13.6%) and 2 (9.5%) patients required some medication, respectively.

Conclusion

According to the punctuation of IPSS, QoL questionnaire, and a 3-day voiding diary, LUTS does not worsen after CS. Prostatic cryosurgery does not seem to impact uroflowmetry results.  相似文献   
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