eMedicine Specialties > Urology > Congenital Urologic Conditions
Prune Belly Syndrome: Follow-up
Updated: Oct 29, 2009
Outcome and Prognosis
The prognosis in patients with prune belly syndrome varies. The spectrum runs from stillbirth to undescended testicles with a minimal degree of abdominal wall laxity.
The authors conducted an anonymous Internet- and postal-based survey of the members of the Prune Belly Syndrome Network, which is a nonprofit organization dedicated to the support of patients with prune belly syndrome. The survey was designed to query adult patients with prune belly syndrome regarding physical, social, and sexual aspects of their lives. General health–related quality of life was assessed with the RAND 36-Item Health Survey 1.0 (SF-36), which is a self-administered 36-item questionnaire that was designed to evaluate and to quantify general health–related quality of life based on 8 scales to rate physical function, role limitations due to physical problems, social functioning, pain, emotional well-being, role limitations due to emotional problems, energy/fatigue, and general health perceptions.
The SF-36 has been widely tested and validated. Each of the 8 scales is scored from 0-100, with a higher score representing a better outcome. The scores themselves have been scaled such that they can be compared with scores from a general US adult population cohort without a chronic medical illness. The average score in the general population is 50.
General questions were asked regarding the number and types of surgeries the respondents had undergone. A current medication profile was obtained. The survey included items pertaining to educational and employment achievements, as well as to participation in various types of physical activities.
The Brief Male Sexual Inventory (BMSI) scale assessed sexual quality of life and sexual function. This questionnaire is an 11-item instrument designed to evaluate sexual drive, erection, ejaculation, and perceptions of problems in these areas, as well as overall sexual satisfaction. Additional questions were asked regarding patient fertility and whether they had children (biological or adopted).
The authors received responses to the survey from 23 patients (20 men, 3 women); the mean age was 32 years (range, 14-62 y). All 20 men surveyed acknowledged a history of undescended testicles. Twelve of these men had undergone some form of orchidopexy. Four men who reported having undergone orchidopexy also reported having undergone unilateral or bilateral orchiectomy. Two men who had not undergone orchidopexy underwent at least one orchiectomy. Fourteen of the 23 respondents reported a history of vesicoureteral reflux. Eleven of these persons went on to undergo some form of ureteral reimplantation. Eight patients went on to undergo at least one nephrectomy. Seven patients (6 men, 1 woman) have had renal failure and have undergone renal transplantation (mean age at the time of survey, 36.3 y).
Four patients acknowledged having undergone some form of abdominal wall reconstruction for their muscle laxity. Three patients reported wearing a scoliosis-type brace, presumably as a noninvasive means of affording abdominal wall support.
Health-related quality of life was determined based on the SF-36. Twenty-two patients responded to the SF-36 portion of the questionnaire. The scores were scaled so that they may be compared with a standard reference population of US adults. The average domain score in the standard population is 50. Above-average scale scores were reported for physical function (13/22), lack of limitations due to physical problems (14/22), lack of limitations due to emotional problems (16/22), energy (15/22), mental health/sense of emotional well-being (15/22), social function (14/22), bodily pain (13/22), and general health perceptions (8/22) versus established norms for the standard population. The 7 patients who required renal transplantation had significantly lower scores (P <.05, Student's T-test) on scales of physical function, general health perceptions, social function, and mental health compared with patients who did not require renal transplantation.
Eighteen patients (16 men, 2 women) responded to the BMSI. Eight of the 16 men noted in their responses that they either have retrograde ejaculation or are anejaculatory. Only 2 of these men reported this as bothersome. Twelve respondents (11 men, 1 woman) reported that they are mostly or very satisfied with their sex lives.
Six respondents (5 men, 1 woman) reported having used artificial reproductive techniques to attempt conception. Three of the male patients reported having children secondary to artificial reproductive techniques. Two of the men fathered children with their own sperm and some form of artificial insemination. Both of these men had undergone some form of undescended testis surgery. One man's wife conceived with the use of donor sperm and artificial insemination.
Twenty-two of those surveyed responded to questions regarding employment status. Of these, 3 are unemployed, 3 are retired, 3 are full-time students, and 13 are currently employed.
Two of the respondents are currently in high school, 2 are in college, 2 did not finish high school, 6 received a high school or equivalency diploma, 7 have a bachelor's or associate's degree, and 3 have a Master's degree.
Prune belly syndrome, along with its associated long-term medical issues, does not necessarily result in long-term perceived physical, psychosocial, or sexual distress or problems. The vast majority of the patients who responded to the authors' survey seemed to function physically and emotionally at levels as high or higher than the standard normative study population on the SF-36. Interestingly, perceived levels of general health were, for the most part, lower than the levels reported by the standard normative study population on the SF-36.
The differences between the authors' study group and the standard population are all the more striking considering the patients who underwent renal transplantation. Only 1 of 7 transplant recipients reported a general health scale score higher than that of the normative population, whereas 7 of 15 respondents who did not require a transplantation reported a higher general health scale score than the normative population (P <.05, Student's T-test). This finding corresponds with the general trend that patients who require renal transplantation are not as healthy overall as those who do not need renal replacement. These differences were also significant on the physical function, social function, and mental health scales.
The authors were somewhat surprised that only 14 men had undergone some type of testis surgery (orchidopexy alone, 8; orchidopexy and orchiectomy, 4; orchiectomy alone, 2) considering that all men reported having a history of undescended testicles. Two men (ages 49 and 53 y) had bilateral intraabdominal testicles and underwent bilateral orchiectomies. One of these men is currently taking testosterone supplementation. Conceivably, orchidopexy may not have been attempted due to unavailability of a surgeon with orchidopexy expertise for intraabdominal testes or due to the belief at that time that fertility was impossible in these patients.
Two men had attempted orchidopexy followed by orchiectomy. One man reported having undergone bilateral orchiectomies after attempted orchidopexy for bilateral intraabdominal testicles. He is taking testosterone supplementation. Another patient reported having had one testicle removed after failed orchidopexy. His other testis is intrascrotal. Nine of the 12 men in the orchidopexy group report having one or both testicles in the scrotum. Of the remaining men, one man has palpable pubic testicles, one still has bilateral intraabdominal testicles, and one man underwent bilateral orchiectomies after failed orchidopexy.
Four of the 6 men who have not undergone testicular surgery reported having bilateral intraabdominal testis. All of these men are postpubertal (age range 29-47 y). The literature quotes at least 4 cases of germ cell malignancy in patients with prune belly syndrome. The authors do not know how these men are being monitored for potential malignancy.
At one time, all patients with prune belly syndrome were believed to be infertile. All men with prune belly syndrome seen by Woodhouse et al were found to have Sertoli cell–only patterns on their testicular biopsy samples.10 Massad et al found germ cells present, but abnormal, in testis biopsy samples of children younger than 1 year with prune belly syndrome.11 Woodard et al reported viable sperm in the postejaculate urine in a patient who had undergone orchidopexy as a young child.12
With advances in assisted reproductive technologies come reports of sperm retrieval and intracytoplasmic sperm injection in patients with prune belly syndrome. Five of the authors' male respondents tried some means of assisted reproductive with sperm retrieval and artificial insemination. Two of these men have successfully fathered children.
Given that some men with prune belly syndrome seem to be capable of fathering children, albeit with the help of assisted reproductive technologies, surgery should be attempted early in patients with prune belly syndrome to preserve fertility. Using current techniques, laparoscopic orchidopexy is apparently an ideal treatment for intraabdominal testis in persons with prune belly syndrome.
Intuitively, with intact Leydig cell function, men with prune belly syndrome should have normal testosterone levels and, therefore, a normal libido. Woodard reported that, following puberty, the average serum testosterone level was higher in patients with prune belly syndrome following prepubertal rather than postpubertal orchidopexy.12 Erection and orgasm are apparently normal. However, retrograde ejaculation secondary to prostatic hypoplasia and an open bladder neck is common. This reported finding was also seen in the authors' patients. Despite findings of retrograde ejaculation and difficulties with fertility, 11 of 16 men who responded were mostly or very satisfied with their sex lives.
Repair of the abdominal wall defect (from which prune belly syndrome derives its name) should be considered to be not only cosmetic but also potentially functional. Smith et al reported improvement in voiding function in patients with prune belly syndrome after abdominal wall reconstruction.13 Woodard and colleagues reported improved pulmonary function after abdominal wall plication.14 Therefore, surprisingly, only 4 patients had undergone some form of abdominal wall reconstruction. If an external support device can be considered as tantamount to a noninvasive approach to abdominal wall management, more than two thirds of those surveyed did not undergo definitive treatment of their abdominal wall defect.
Prune belly syndrome presents with a wide spectrum of severity. Seven of the authors' patients (30%) ultimately required renal transplantation. This number parallels previous estimates regarding the development of renal failure in this population. Noh et al found several factors as prognostic for renal failure, including bilateral abnormal kidneys revealed by imaging studies, nadir serum creatinine levels of greater than 0.7 mg/dL, and clinical pyelonephritis.15 The authors did not ask about these factors in their survey. Of their patients who went on to undergo transplantation, 4 had undergone some form of ureteral reimplantation, 4 had undergone vesicostomy (3) or suprapubic tube (1), and one had an ileal conduit. The role of extensive urinary tract reconstruction in patients with prune belly syndrome is controversial. The authors do not know the chronology of when lower urinary tract reconstructions were attempted relative to the onset of renal failure.
The findings in the authors' study seem to indicate that adults with prune belly syndrome are happy and well-adjusted and are participating in conventional physical, sexual, emotional, educational, and employment roles.
The authors' study has limitations. The data represent the responses of only 23 adults with prune belly syndrome. Whether a larger sample size would have altered the outcome is unknown. Additionally, the BMSI is not specifically designed or validated to answer questions about female sexuality. A questionnaire designed to address female sexuality may have been better to include.
In conclusion, many individuals with prune belly syndrome appear to function at a high level, with good health-related quality of life and good social and sexual function. Persons who require renal transplantation score significantly lower on many indices of health-related quality of life. Artificial reproductive technology appears to be required to conceive children but is possible, contrary to prior beliefs.
Future and Controversies
Persistent advances in laparoscopic surgery may continue to improve the outlook for patients with prune belly syndrome. As more is learned about the natural history of obstructive uropathy, treatment of this condition will be adjusted to offer the most benefit with the least amount of intervention. Whether to institute aggressive early intervention or no intervention will continue to be debated the true pathophysiology of prune belly syndrome is completely understood.
The fact that a decreasing number of patients are being born with prune belly syndrome indicates an ever-increasing ability to diagnose the disease in utero. In utero intervention with vesicoamniotic shunting has had little effect in reducing the incidence of renal failure and the need for transplantation in this group of patients.16 Expertise in treatment will eventually be limited to only a small number of experts. In the future, patients with prune belly syndrome will need to consider obtaining care from such specialized centers.
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Further Reading
Keywords
prune belly syndrome, PBS, Eagle-Barrett syndrome, triad syndrome, undescended testis, undescended testes, abdominal wall reconstruction, percutaneous nephrostomy, standard pyeloplasty, infravesical obstruction, obstruction at the prostatic urethra, trisomy 18, trisomy 21, tetralogy of Fallot, TF, ventriculoseptal defect, ventricular septal defect
