Uterine prolapse

The presence of genital prolapse in neonates and young women poses a challenging management problem to the gynecologist. Neonatal uterine prolapse is associated with congenital spinal defects, and successful correction has been achieved mainly with simple digital reduction or the use of a small pessary. Uterine prolapse can also occur in young or nulliparous woman who wish to preserve their fertility. Operations using sling, sacral cervicopexy, or transvaginal sacrospinal fixation techniques seem to provide excellent repair for these patients, including the possibility of childbearing. A review of the pathophysiology of genital prolapse in neonates and young women with emphasis on the surgical and nonsurgical options for management is presented.

Chief Editor’s Note: This article is the 11th of 36 that will be published in 1996 for which a total of up to 36 Category 1 CME credits can be earned.

Uterine prolapse is a condition usually associated with multiparity in the postmenopausal woman. Its diagnosis in neonates and nulliparous women poses a significant management problem to the gynecologist. Neonatal genital prolapse is a rare condition that usually occurs immediately after birth or during the first few days of life. Most cases have been reported in association with congenital spinal defects.

Genital prolapse can also occur occasionally in young and/or nulliparous women. Insufficient emphasis has been given to the contribution of the inherent strengths of the pelvic floor’s musculofascial support as underlying cause for prolapse in this age group. Standard vaginal operations for the correction of procidentia due to congenital weakness of the pelvic supports are frequently not successful, resulting in high failure rates and reduction in fertility. Recently described sling, sacral cervicopexy, and transvaginal sacrospinal fixation operations provide excellent repairs for these women, with the possibility of childbearing. In this review, our objective is to present the history, current understanding, and pathophysiology of genital prolapse in neonates and young women and discuss the surgical and nonsurgical options for treatment.

The first reports of genital prolapse are those of the Ebers papyrus, dated 1500 BC. No discussion regarding possible causes was made, but the suggested treatment was to smear the fallen womb with a mixture containing honey and subsequent replacement. Hippocrates (400 BC) mentioned genital prolapse in his writings and described three methods of treatment. The first was by tying the patient to a ladder that was then turned upside-down and shaken violently hoping to reduce the uterus. He also described the placement of suction cups to the buttocks and lower abdomen expecting to replace the prolapse. A third option described was sponing the prolapse with cold wine, raising the foot of the bed until the uterus was completely reduced, then it was supported in position by a half pomegranate soaked in wine and introduced into the vagina. This was the first time that the concept of a vaginal pessary was used in the management of genital prolapse. Celsus (25 BC) and Soranus of Ephesus (98 AD) used a very similar method of treatment, where the prolapse was washed with an astringent lotion and manual reduction of tissues into proper place, followed by the introduction of a medicated pessary into the vagina and binding the legs firmly together. Aretaeus (100 AD) and Galen (130 AD) believed that the prolapse was able to reduce itself and used fumigations to promote this. Haby Abbas (932 AD), a well-known Persian physician, wrote extensively about genital prolapse. He described prolapse as a condition that was related to birth trauma and managed the prolapse by cleaning, manual reduction, introduction of a wool pessary and binding the legs together. Later Caspar Stromayr of Lindau recommended the use of tightly rolled sponges dipped in wax and covered with oil or butter as pessaries Figure 1 and Figure 2. This was the accepted mode of treatment for hundreds of years, until the first surgical methods of treatment appeared in the 1800s when Donald of Manchester and his assistant, Fothergill, described what became known as the Manchester operation for the treatment of genital prolapse [1].

The first case of genital prolapse in a newborn was reported by Conovius in 1723 [2]. The first formal review on the condition was written in 1897 by Ballantyne and Thomson [3], where they reported the autopsy findings of two neonates who presented with spina bifida and genital prolapse and reviewed the six previously reported cases. They also described a possible pathophysiology, suggesting that disturbances in the innervation of the genital area secondary to spina bifida were responsible for this condition. Findley [4], in 1917, found that prolapse of the uterus in the newborn and nulliparous women constituted 3.45 percent to 13.15 percent of all cases of prolapse and of these, 82 to 86 percent of cases were associated with spina bifida. He also described several cases of procidentia that occurred about the time of puberty, where the girls were poorly nourished, some had pulmonary tuberculosis associated with persistent coughing, and others were simply involved in hard labor duties. Kepler [5], in 1911, described a total of 151 cases of procidentia uteri in nulliparous women and classified the cases into two groups; those due to congenital defects occurring in the newborn period or at the time of puberty, and the second group were cases due to congenital defects occurring later in life. He also related mental defects to prolapsed uteri and described 38 cases where the patients were mentally retarded. Noyes [2], in 1927 reviewed extensively the anatomic dissections of several other authors and concluded that atrophy of the ligaments, connective tissue and muscles from defective innervation, secondary to spina bifida, contributed to the uterine prolapse Figure 3 and Figure 4. All the cases reported to that point in the literature, had a universally fatal outcome secondary to complications from meningomyelocele.

Inherent to the understanding of prolapse is a basic knowledge of the functional anatomy of the pelvic floor. The pelvis itself is made up of two innominate bones anteriorly and laterally, and by the sacrum and coccyx posteriorly. The base is provided by the platform of the levator ani and coccygeus muscles and by the pelvic fascia. The muscle support consists of the ischiococcygeus, iliococcygeus, and pubococcygeus blended on each side into a shelf which slopes immediately downward, producing a gutter effect in the midline, penetrated by the urethra, vagina, and anal canal. The muscles arise from the ischial spine and the pelvic bones. They are inserted into the coccyx and the anococcygeal raphe, innervated by the perineal branch of the fourth sacral nerve and also by branches of the inferior rectal and perineal nerves. Normally any elevations in intraabdominal pressure such as sneezing, laughing, or coughing are counteracted by contractions of the pelvic floor muscles. If these nerves or their spinal cord components are defective from myelodysplasia, lumbosacral segmentation anomalies or trauma, flaccid paralysis of the pelvic floor permits downward protrusion of the lower abdominal and pelvic viscera. In neonates, this is manifested by a poorly developed intergluteal cleft and a bulging of the perineum that is accentuated by crying or straining for a bowel movement. In severe cases, even rectal prolapse may be present.

A layer of fascia also lies between this muscular floor and the pelvic peritoneum, forming a loose areolar tissue surrounding the pelvic viscera. Condensations of this areolar tissue form the ligaments supporting the viscera, (for example, the round ligaments, the cardinal ligaments, and the uterosacral ligaments). Failure in the development of these supportive ligaments from denervation or obstetrical trauma will also lead to genital prolapse.

Some authors have studied the radiographic anatomy of normal infants and neonates with genital prolapse Figure 5 and Figure 6 [6,7]. In neonates and infants, the position of the pelvic organs is higher than in adults. For example, the urinary bladder is an intraabdominal organ with its space laying above the symphysis pubis; it does not reach its adult position in the pelvic floor until around 20 years of age. Zerin et al. [8] found an association between the descent of the bladder neck, denervation of the urethral sphincter, and the incidence of genital prolapse in children with myelodysplasia. Genital prolapse is present in 86 percent of infants with complete lower motor neuron denervation of the urethral sphincter, compared with only 13 percent when there is partial or lack of denervation. These studies support the reported observations where genital atrophy and prolapse are secondary to pelvic denervation from spinal defects. Noyes [2] also stressed the importance of spina bifida occulta as a cause for genital prolapse. They studied 28 cases of uterine prolapse and found that 28 percent of them showed spina bifida occulta whereas, an equal number of women without prolapse were used as controls, showing the condition only in 10 percent of cases.

Patients with bladder extrophy also have associated genital prolapse [9]. These patients usually lack the lower inferior abdominal wall, the anterior portion of the bladder and the urethra. There is often an anterior displacement of the anus and deficient pelvic musculature resulting in rectal prolapse. The pelvic bones are widely separated anteriorly and the junction between the separated bony ends being filled with fibromuscular tissue. These patients usually develop prolapse from a huge defect in the anterior supporting structures of the pelvic floor.

Digital reposition of the prolapse was the first reported intent for managing this condition. Noyes [2] and Ballantyne and Thomason [3] were the first to describe this mode of management, unfortunately babies died from meningitis in the first days of life Table 1. Fraser [10] was the first to describe the successful digital replacement of a prolapsed uterus in an otherwise normal newborn. More recently, Bayatpour et al. [11] reported the successful treatment of a child with no apparent neurologic anomalies by multiple digital reductions of the prolapse (the mother was trained to reduce the prolapse during diaper changes). When the tissues are edematous, the approach suggested by Ajabor and Okojie [12] with the application of hypertonic saline packs to the vulvar area, followed by digital repositioning of the mass with partial labial fusion with sutures would be appropriate. Dixon et al. [13] was the first to report the use of a pessary for the reduction of genital prolapse in a neonate. The pessary was made with a 1-inch, rolled, sterile Penrose drain with a 1-0 silk guy string attached Figure 7. Carpenter and Rock [16] reported an alternative approach by placing a nipple in the vagina, which was fixed with adhesive paper tape across the infant’s buttocks.

It seems, as evidenced in the literature, that simple digital replacements of the prolapsed genitalia or the use of small pessaries Figure 8 in normal neonates is successful. In the past, cervical amputation or hysterectomy were used as the ultimate treatment for genital prolapse in the neonatal period [2,10]. In patients with neurological impairment or myelomeningocele, nonsurgical treatment methods should also be attempted, given the good long-term outcomes obtained with conservative management and preservation of fertility. Surgical management is reserved until they reach an older age, if necessary.

Conservative approaches, like the use of a pessary, is certainly a feasible option. The proper care and frequent follow-up that the use of a pessary requires, makes it difficult for young patients to accomplish this in the long term, therefore, surgical approaches are preferred. Of course, pessaries provide good temporary relief of the prolapse, and can be used to assist in identifying other anatomic defects or urinary incontinence, assisting in the surgical planning for the individual needs of the patient.

The surgical treatment of genital prolapse in young women, particularly for those who want to preserve their fertility, is a real challenge to the gynecologist. In general, there are techniques reported for both abdominal and vaginal approaches. The most common operation used in the past was the Manchester operation described in 1888 by Donald [1], which consisted in the plication of the uterosacral and cardinal ligaments with a partial amputation of the cervix. This operation had many drawbacks including reduction in fertility (pregnancy rates of 10-20 percent) [17], increased miscarriage rates, premature labor and dystocia leading to a cesarean section rate of 20 to 55 percent [18]. Enterocele formation and recurrence of the prolapse were frequent [19]. Description of more extensive techniques for the Manchester operation, like the one described by O’Leary and O’Leary [20], are also available Table 2, and they address the problem of recurrence by excising the enterocele sac and closing the cul-de-sac at a high level with a purse-string suture that included the uterosacral ligaments and the uterine serosa.

More recent techniques have been described for the management of prolapse in young women. Durfee [22] and Nassar [23] advocated uterine suspension via Dacron tape or fascia passed through the round ligaments with attachment to the posterior border of the cervix. Unfortunately, these operations change the normal vaginal axis and allow the abdominal pressure to be transmitted directly to the cul-de-sac, potentiating enterocele formation. Chaudhuri [18] described both vaginal and abdominal internal oblique fascia sling operations, with good anatomical results and 66 percent pregnancy rates with only two recurrences. Other techniques for abdominal hysterosacropexy operations were described by Aboulghar and El-Kateb [21] and Dewhurst et al. [9] with similar results.

More recently, Richardson et al. [24] and Kovac and Cruikshank [25] advocated transvaginal sacrospinous fixation approaches for the correction of genital prolapse in young women. Both reports stressed that each individual defect of the pelvic floor had to be addressed in order to obtain optimal results. The report by Kovac and Cruikshank [25] was larger, and described 19 patients of whom 5 had a total of 6 deliveries with good outcomes and adequate anatomical support postdelivery. In that series, six patients were nulliparous, although none were pregnant at the time of publication. The sacrospinous fixation is simple with an average operating time of 15 minutes and minimal blood loss Figure 9 and Figure 10. This approach should be given serious consideration for the management of young patients with symptomatic uterine prolapse who wish to preserve their fertility, because it provides adequate anatomical restoration, avoiding an intraabdominal procedure that can potentially generate pelvic adhesions and enterocele formation.

In summary, genital prolapse in neonates and young women occurs when the innervation of the pelvic floor is defective from myelodysplasia, lumbosacral segmentation anomalies or trauma, causing a flaccid paralysis of the muscles permitting downward protrusion of the lower abdominal and pelvic viscera. Pelvic relaxation can also occur from direct damage to the fasciae from obstetrical lacerations and trauma. Successful treatment in normal neonates or infants with myelomeningocele is obtained by nonsurgical means, such as simple digital replacements of the prolapsed genitalia or the use of small pessaries. In young adults who want to preserve their fertility potential, pessaries provide temporary relief and assist in the detection of associated abnormalities before surgical correction. Although abdominal approaches appear to provide satisfactory results, more recent experience with the transvaginal sacrospinous fixation operations for the correction of genital prolapse in young women provide better anatomical repairs, with the possibility of childbearing.

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