Barbara Ludwikowski

The Rectogenital Septum: Morphology, Function, and Clinical Relevance

PURPOSE: The rectogenital septum (known in clinical literature as Denonvilliers’ fascia) forms an incomplete partition between the rectum and the urogenital organs in both men and women. It is composed of collagenous and elastic fibers and smooth muscle cells intermingled with nerve fibers emerging from the autonomic inferior hypogastric plexus. The aim of this study was to investigate the fetal development of the rectogenital septum, and the origin and innervation of the longitudinal smooth muscle cells within the septum, as well as to consider possible effects on function of operations that compromise the integrity of these structures. METHODS: Macroscopic dissections on embalmed human pelves and plastination histology of 40 fetal and newborn pelvic specimens were performed. By means of conventional and immunohistochemical staining methods using monoclonal and polyclonal antibodies for tissue analysis and neuronal labeling, the motor and sensory innervation of the longitudinal muscle bundles within the septum was defined. RESULTS: The rectogenital septum is formed by a local condensation of mesenchymal connective tissue in the early fetal period. The longitudinal muscle bundles could be traced back to the longitudinal layer of the rectal wall, and, using the septum as a guiding structure, it was possible to identify autonomic nerve fibers and ganglion cells innervating the muscle cells and crossing the midline without detectable gender differences. CONCLUSIONS: Because of a coinnervation of the rectal muscle layers and the adjacent longitudinal muscle fibers of the septum, a functional correlation between the two structures during defecation is postulated. On the basis of these findings, a safer dissection of the anterior rectal wall during rectal resection is postulated, thus limiting functional disturbance and preventing neural damage.

Clinical anatomy of the pelvic floor.

The study presented here comparing cross-sectional anatomy of the fetal and the adult pelvic connective tissue with the results of modern imaging techniques and actual surgical techniques shows that the classical concepts concerning the subdivision of the pelvic connective tissue and muscles need to be revised. According to clinical requirements, the subdivision of the pelvic cavity into anterior, posterior, and middle compartments is feasible. Predominating connecting tissue structures within the different compartments are: Paravisceral fat pad within the anterior compartment (Fig. 17, I), rectal adventitia or perirectal tissue within the posterior compartment (Fig. 17, II), and uterosacral ligaments within the middle compartment. The nerve-vessel guiding plate can be found in all of these compartments; it starts within the posterior compartment and it ends within the anterior one. It constitutes the morphological border between the anterior and posterior compartments in the male. This border is supplied by the uterosacral ligaments in the female. Whereas in gross anatomy no further border is discernable between anterior and posterior or middle compartment, the rectal fascia (hardly visible in embalmed cadavers) demarcates the rectal adventitia and is one of the most important pelvic structures for the surgeon. In principle, the outlined subdivision of the pelvic connective tissue is identical in the male and in the female; facts that become clear from early human life and that are already established during this period (Fig. 18). The uterus is interposed between the bladder and rectum and subdivides the pelvic peritoneum into two pouches thus establishing the only real difference between male and female pelvic cavity. The preferential direction of the pelvic connective tissue fibers is not changed by the interposition of the uterovaginal complex. The pelvic floor muscles are composed of the portions of the levator ani muscle, the muscles of the cavernous organs and the deep transverse perineal muscle in the male. The latter does not exist in the female. We have clearly shown that the different muscles can already be found in early human life and that they are never intermingled with the muscular walls of the pelvic organs. The levator ani muscle of the female, however, is intermingled with connective tissue long before the female sexual hormones exert influence. We have also shown that the distinct sexual differences within the pelvic floor muscles as well as within the sphincter muscles can already be found in early human life. Both the external urethral and the external anal sphincter muscles are not completely circular. The external anal sphincter is intimately connected with the internal sphincter as well as with the longitudinal muscle. Whereas the innervation and function of the urethral sphincter muscles are mostly clear, cloacal development, innervation, and function of all parts of anal sphincter complex are not completely clarified. As to the support of the pelvic viscera, we believe that intact pelvic floor muscles, an undisturbed topography of the pelvic organs, and an undisturbed perineum are of more importance than the so-called pelvic ligaments. Our hypothesis points to the fact that the support of pelvic viscera is multistructural. Thus in pelvic surgery, a lot of techniques have to be revised with the aim to preserve or to reconstruct all the structures mentioned. This is a multidisciplinary task that can only be solved by cooperation of morphologists, urologists, gynecologists, and coloproctologic surgeons or by creating a multidisciplinary pelvic floor specialist.

Anal sphincter complex: reinterpreted morphology and its clinical relevance.

AbstractPURPOSE: Recent clinical studies on the anal sphincter complex have criticized the lack of reliable morphologic concepts. The purpose of this study was to determine the anatomy and histology of the anal sphincter complex with the help of undisturbed anatomic preparations. METHODS: The anal sphincter complex was studied in axial, sagittal, and coronal sections of human fetal, newborn, and adult pelves. RESULTS: The anal canal was surrounded by the internal sphincter, the longitudinal muscle layer, and an external sphincter that turned in to become continuous with the internal sphincter and with it to enclose the longitudinal muscle bundles. The classical tripartite subdivision of the external sphincter was not confirmed. The external sphincter seems not to be a complete circle in certain planes, neither in the male nor in the female. Sexual differences of the ventral part of the external sphincter were already present in fetuses. Large lamellated corpuscles were embedded within the interlacing smooth and striated muscles. Branches of the pudendal nerve innervated them. CONCLUSION: Our anatomic and histologic findings highly correlate with the results of magnetic resonance imaging and endosonography as well as with the physiologic findings. Furthermore, they are of great clinical importance for the understanding of sphincter defects during vaginal delivery and for anorectal operations in the adult as well as in the child.

TOTAL UROGENITAL SINUS MOBILIZATION: A MODIFIED PERINEAL APPROACH FOR FEMINIZING GENITOPLASTY AND UROGENITAL SINUS REPAIR

PURPOSE We report a modification of the total urogenital sinus mobilization technique adapted to the repair of isolated persistent urogenital sinus and masculinized external female genitalia. MATERIALS AND METHODS The records of 6 girls undergoing total urogenital sinus mobilization were reviewed. Diagnosis was the adrenogenital syndrome in 4 girls, and persistent urogenital sinus and true hermaphroditism in 1 girl each. Mean patient age at the time of surgery was 5.9 years (range 4.5 months to 19.5 years). The surgical technique was modified by using the perineal approach since all patients had normal anorectal anatomy. A posterior perineal skin flap was used to widen the vaginal introitus. The wall of the mobilized urogenital sinus was opened and used to create a mucous lined vestibule. In those cases of the adrenogenital syndrome reduction clitoroplasty was performed at the same time. Postoperative results were assessed subjectively by observation of the vulvar appearance and objectively by determining the location of the urethral meatus and caliber of the vagina. RESULTS Mean followup was 3.7 months (range 1 to 9). There were no postoperative complications. Most patients were discharged home within 3 days of surgery. All patients have a satisfactory cosmetic appearance. The urethral meatus was situated in the vestibule and easily accessible. There were no changes in voiding habits postoperatively in those patients who were toilet trained before surgery. Vaginal calibration was performed in 4 patients and mean vaginal caliber was 10.5 Hegar (range 6 to 14). CONCLUSIONS The repair of persistent urogenital sinus less than 3 cm. long can be accomplished with total urogenital sinus mobilization through the perineal approach. The technique can be combined with reduction clitoroplasty for the surgical management of girls with masculinized external genitalia.

The development of the external urethral sphincter in humans.

Objective To assess the hypothesis that during fetal development, the external urethral sphincter changes from a concentric sphincter of undifferentiated muscle fibres to a transient ring of striated muscle which regresses caudo‐cranially in the posterior urethra during the first year of life, when the sphincter assumes its omega‐shaped configuration.

Determinants of Success and Failure of Seromuscular Colocystoplasty Lined With Urothelium

PURPOSE Seromuscular colocystoplasty lined with urothelium is a method of bladder augmentation that avoids incorporating intestinal mucosa into the urinary tract. Others have reported a repeat augmentation rate of 23%. We analyzed the results in 20 patients who underwent the procedure, as performed by one of us (RG), at 3 institutions. MATERIALS AND METHODS After receiving institutional review board approval we retrospectively reviewed the charts of all patients operated on since 1998. Preoperative and postoperative bladder capacity at 30 cm H(2)O, expressed as the percent of expected capacity for age using the equation, bladder capacity in ml = (age +1) x 30, as well as prior, concomitant and subsequent bladder or bladder neck procedures, continence and the need for repeat augmentation were recorded. RESULTS There were 20 patients, including 7 females, with a mean age at surgery of 9 years and a mean followup of 53 months. All patients had neurogenic bladder dysfunction. An artificial urinary sphincter was implanted at the time of seromuscular colocystoplasty in 10 patients, preoperatively in 6 and postoperatively in 1. A sling was used in 3 females. Patients were divided into 2 groups. The 15 group 1 patients underwent no concomitant procedure in the bladder and the 5 in group 2 underwent creation of a continent channel at seromuscular colocystoplasty. There were no failures of augmentation in group 1, in which bladder capacity increased from 60% of that expected for age to 100%. All patients were continent. Three of the 5 patients in group 2 required repeat augmentation. CONCLUSIONS Seromuscular colocystoplasty lined with urothelium has proved to be an effective method to augment the bladder in patients who have an artificial urinary sphincter or who undergo simultaneous artificial urinary sphincter implantation. We do not recommend constructing a continent catheterizable channel at the time of seromuscular colocystoplasty lined with urothelium.

The controversy regarding the need for hormonal treatment in boys with unilateral cryptorchidism goes on: a review of the literature

Hormonal treatment for unilateral undescended testes continues to be recommended in some countries. We reviewed the literature in favor and against this recommendation. Since the paternity rate of men with a history of unilateral undescended testes only treated with surgery is normal, the effectiveness of hormonal treatment to produce testicular descent is low, the cost is considerable, and there are potential adverse effects, hormonal treatment for boys with unilateral undescended testes should no longer be recommended.

The Surgical Correction of Urogenital Sinus in Patients with DSD: 15 Years after Description of Total Urogenital Mobilization in Children.

Total urogenital sinus mobilization has been applied to the surgical correction of virilized females and has mostly replaced older techniques. Concerns have been raised about the effect of this operation on urinary continence. Here we review the literature on this topic since the description of the technique 15 years ago. Technical aspects and correct nomenclature are discussed. We emphasize that the term “total” refers to an en-bloc dissection and not to the extent of the proximal dissection. No cases of urinary incontinence have been reported following this operation. It is yet too early to evaluate results regarding sexual function but it is likely that the use of a posterior skin flap to augment the introitus will minimize the development of introital stenosis.

Improvement of continence with reoperation in selected patients after surgery for anorectal malformation.

PURPOSE: Fecal incontinence is a serious complication after repair of anorectal malformations. We investigated whether reoperation can improve fecal continence. METHODS: Medical records of 41 patients (40 children and one adult; 26 male and 15 female) who underwent reoperation after previous reconstruction of an anorectal malformation were reviewed for outcomes of bowel function. Type of primary corrective surgery performed, therapeutic measures, results of physical examination and barium enema, and reoperation procedures were evaluated. A questionnaire was administered to assess stool behaviour and level of continence at follow-up three or more years after secondary operation. RESULTS: Secondary operations in males comprised posterior sagittal anorectoplasty (PSARP) in 16 patients, PSARP with antegrade continent enema in one patient, antegrade continent enema alone in 6, anoplasty in one, rectosigmoid resection in 1, and definitive colostomy in 1 patient. Secondary operations in females included PSARP alone in 4 patients, PSARP with total urogenital mobilization in 4, PSARP with vaginoplasty in 2, PSARP with vaginoplasty and antegrade continent enema in 2, and PSARP with vaginourethroplasty in 3. Of 41 patients 18 (44 percent) were continent at follow-up, 21 (51 percent) were clean with use of enemas, diet, or drug therapy. One patient had a definitive colostomy. One died after kidney transplantation. CONCLUSIONS: Surgery is a good option for improving incontinence in selected patients previously operated for anorectal malformations. Posterior sagittal anorectoplasty is advocated to improve bowel control. Antegrade continent enema is a reliable therapeutic option to maintain clean patients with fecal incontinence.

Should the Genitoplasty of Girls with CAH be Done in One or Two Stages

The debate over the timing of surgical repair in girls with congenital adrenal hyperplasia (CAH) is intense and was in part started by patient advocacy groups largely composed of dissatisfied adults operated in childhood. Their influence has been beneficial to make surgeons reconsider gender reassignment in certain cases of DSD and other genital malformations such as penile agenesis and cloacal exstrophy. Some authors have proposed a ban on all no-medically necessary surgery in infants (1). However, we think this debate as useful as it may be should not include the treatment of girls with CAH. One must keep in mind that girls with 21-hydroxylase deficiency, the most common form of CAH, are genotypically female, and have a normal potential for fertility and sexual function, and to date there have been no instances of gender dysphoria reported in this population. The situation is quite similar to that of proximal hypospadias in boys, a condition that, as is universally accepted, is best repaired in infancy. If we have to ban early genital reconstruction in girls with CAH, surgeons must be rational and consistent in their behavior and also ban hypospadias repair (not to speak of most circumcisions) in infants and children who cannot give consent. Two recent articles in Frontiers in Pediatrics (2, 3) advocate or suggest seriously considering the surgical correction of the genital ambiguity in girls with CAH, in two stages: one early stage, to feminize the appearance of the external genitals, and vaginoplasty as a second stage after puberty. The rationale for this approach is the high reported incidence of post-pubertal vaginal (3) introital stenosis when the vaginoplasty was done in childhood (4–8). The validity of this proposal has not been tested. In fact there is only one report suggesting that vaginoplasty done after puberty carries a lower rate of stenosis (9). On the other hand, several reports indicate that the stenosis detected after puberty is relatively easy to repair (5, 8). But the two-stage proposal to repair CAH in no way addresses the debate on infant genital surgery since it advocates early vulvo and clitoroplasty and only assumes that post-pubertal vaginoplasties will have a better outcome than when done in infancy. The report by Hoepffner et al. (9) on which the recommendation to stage the repair is based is worth close analysis. The authors reviewed 46 patients with CAH aged between 16 and 46 years. Of interest are the 35 women who had a Prader stage of virilization 3 or greater. Most patients had a Fortunoff and Lattimer flap vaginoplasty, two had a vaginal pull through (10), and three had no vaginoplasty. No patient had undergone en-block urogenital mobilization with a posterior flap (11). Thirteen patients had the vaginoplasty at or before the age of 12 years (mean age 8.6, range 2–12) and 19 had it at a mean age of 14.3 years (range 13–27). We used a cut off age of 12 for this analysis since no Tanner stage at the time of vaginoplasty is given in the article. Patients were evaluated by a questionnaire and when not sexually active, a gynecological examination was performed to determine the possibility of having sexual intercourse. Of the 13 patients with a prepubertal vaginoplasty, 6 required a revision after puberty whereas only 1/19 in whom the vaginoplasty was done later needed a revision. Nevertheless, in the end there were no differences between the groups in the ability to have intercourse or having the potential for doing so suggesting that the revisions were successful. In fact, the only four patients who were thought to have a vagina inadequate for intercourse had no vaginoplasty (two) or had it at the age of 15 years (two). Both of these women were classified as Prader 5. Our policy and recommendations are to perform the repair of the genital in CAH when the infant is endocrinologically stable. This is consistent with our policy to repair hypospadias in infancy. In our experience, two-stage surgery has some disadvantages. After the initial vulvo and clitoroplasty, it remains scarring in the area of the future creation of the vaginal introitus. The distal urogenital sinus, which is so useful to create a mucosa-lined vestibule, is often disturbed to the point of making it unavailable at the time of vaginoplasty. For this reason, we favor early one-stage reconstruction. The options available as well of the existing controversies should be thoroughly explained to the parents. If the parents opt for early surgery, they should be informed that an examination under anesthesia is recommended at 3 months and necessary at puberty. The operation consists of a vulvoplasty and vaginoplasty by a Fortunoff flap or en-block mobilization according to the Prader stage. If the degree of clitoral hypertrophy so demands, we do a reduction of the corpora cavernosa with preservation of the dorsal neurovascular bundle (12). The glans clitoris is not reduced but simply hidden by creation of a clitoral hood. We perform a brief examination under anesthesia 3 months later to assess the early result and then perform an examination under at puberty to assess the vaginal introitus in a non-traumatic way before the onset of sexual activity and recommend revision or dilatation when needed. This approach seems to us to be as valid as the proposed two-stage procedures.