Login to Access Video or Poster Abstract: MP66-04
Sources of Funding: None.

Introduction

Prune belly syndrome (PBS) is characterized by abdominal wall muscle hypoplasia, urinary tract dilatation and bilateral intra-abdominal testis. No spontaneous paternity has been reported to date and infertility is usually taken for granted. Our purpose was to gain insight on the causes of infertility in PBS by evaluating reproductive system anatomy and gonadal function in a cohort of post puberal PBS patients.

Methods

We contacted all patients 14 years-old or older that had undergone surgical reconstruction at our Institution since 1987. Age at orchidopexy, type of orchidopexy (with or without ligation of gonadal vessels), testicular volumes and positions and last serum creatinin were recorded. A pelvic MRI to evaluate prostate size, seminal vesicles and vas and serum FSH, LH and testosterone were ordered. Sperm analysis and analysis of urine after masturbation were performed when the patient and family consented.

Results

Fifteen patients had data from physical examination and hormonal profile and were included in this study. Mean age was 18.2 years. Mean age at orchidopexy was 17 months. Fourteen (93.3%) patients had both testes in scrotum. Mean testicular volume was 6.9 cc (2.1 to 9.4 cc). Eight patients collected semen. Mean concentration was 5.07 million/mL. Motile sperm was found in 5 patients (62.5%): 3 (37.5%) in the ejaculate a 2 (25%) in urine after masturbation. Mean hormone levels were LH: 5.3 mg/dL, FSH:6.9 mg/dL, testosterone 531 mg/dL. MRI revealed prostates hypoplastic in 55.6% and absent in 22.3%. Absence of at least one seminal vesicle was seen in 55.6%. No vasal abnormality was noted. Mean Cr=1.64 mg/dL.

Conclusions

Patients with PBS may have normal sexual hormonal levels and motile sperm in the ejaculate or post masturbation urine. Our study highlights a high prevalence of prostate and seminal vesical abnormalities that may represent an important cause for their infertility.

Funding

None.