EPISPADYYa (epispadia; Greek epi-on, from above, atop + spadon an opening, a crack) — the difficult rare malformation of urinogenital system, the main sign to-rogo is splitting (not fusion) of a front wall of an urethra (urethra). Approximately in 50% of cases E. it is combined with other malformations of urinogenital system (see).
Emergence E. connect with disturbance of embryonic development of a germ, a cut occurs on 3 —
4th week and consists in the shift of primary pair rudiment of a sexual hillock in the caudal direction whereas the urinogenital sine remains on the usual place. Such shift and the slowed-down formation of a sexual hillock are followed by disturbance of laying of a mesodermal plate that leads to a rassasyvaniye ekto-and ento-dermalny plates of an urinogenital sine. Further it causes lack of a front wall of an urethra and a muscular layer of a front wall of a bladder in his neck with education submukoz-ache cracks. If pathology of primary sexual hillock is not followed by disturbance of the course of a mesodermal bookmark, process of a nesmykaniye is limited to limits of an urethra.
Classification E. considers extent of splitting of an urethra, size of muscular defect of a bladder and dysfunction of its sphincter. At men distinguish E. balanus, AA. penis, chlenolobkovy E. (subtotal), total E.; at women — E. heads of a clitoris, AA. clitoris, subpubic E. (subtotal) and total E. To 90% of all forms of an epispadiya makes total and subtotal E. with an incontience of urine.
The wedge, a picture depends on a form of an epispadiya. In case of E. a balanus the outside opening of an urethra opens on its dorsal surface at a coronal furrow (a neck of a head), the head at the same time is split (fig. 1, a). In some cases there is no splitting of a prepuce in this connection E. can remain unnoticed. The urination is not broken, small spraying of a stream of urine can be observed. In treatment at E. there is no balanus usually need.
At E. a penis its expressed deformation is noted: on
Lowai the member is shortened, brought up to a front abdominal wall. On its dorsal surface there passes the depression in the ground 0,7 — 1,5 cm wide, edges is covered by a mucous membrane of an urethra and comes to an end at a root (basis) of a penis where also the outside opening of an urethra is located (fig. 1, b). Sphincter to urine -
Fig. 1. The diagrammatic representation of external genitals at various forms of an epispadiya at men: and —
an epispadiya of a balanus; — an epispadiya of a penis; in — a total epispadiya (on the dorsal surface of a penis the fillet of the split urethra is visible, splitting of a neck and a front wall of a bladder in the drawing is not visible).
the ispuskatelny channel it is kept, an urination any, however patients with this form E. complain of spraying of a stream of urine during an urination. Sexual intercourse is complicated, but is possible.
At chlenolobkovy E. partial splitting of a sphincter of an urethra and the related more or less expressed incontience of urine is characteristic. The penis is shortened, deformed; the outside opening of an urethra in the form of funneled deepening is located under a pubic symphysis. At adult patients in connection with shortening and deformation of a penis sexual intercourse is complicated, and is sometimes impossible. Quite often at this form E. discrepancy of pubic bones and direct muscles of a stomach is observed.
At total E. (fig. 1, c) splitting of an urethra, neck and a front wall of a bladder is available that is followed by a full incontience of urine. The penis is shortened, bent, tightened to a pubis and covers an entrance to a bladder. Cavernous bodies on the dorsal surface of a penis are divided by the deep longitudinal crack covered by a mucous membrane of an urethra, passes edges directly into a mucous membrane of a bladder. The scrotum at a part of patients is underdeveloped, testicles often of a gipoplazirovana or are not lowered and at a palpation easily escape to the pakhovy canal.
At E. a clitoris the outside opening of an urethra is displaced up, to urine
emission is not broken; wedge, values E. has no clitoris. At sub-total E. at women splitting of a sphincter of an urethra and a partial incontience of urine is noted; the clitoris is completely split, the nesmykaniye of big and small vulvar lips is observed; there is no discrepancy of pubic bones.
At all patients with total E. there is a discrepancy of a pubic symphysis with diastases between pubic bones to 2 — 8 cm in this connection gait becomes «duck». The neck of a bladder is absent. Not fusion of an urethra extends to a front wall of a bladder in the form of the latent defect of muscular layers replaced with the strip of fibrous fabric which is narrowed up, edges is covered by a mucous membrane from within, and outside adjoins to an abdominal wall. The urethra has an appearance of a trench, on Krom urine constantly flows down. In position of patients costing a bladder always it is completely emptied; in a prone position in it about 70 — 80 ml of urine (false deduction) can collect.
The continuous expiration of urine, wet clothes, irritation of skin and generative organs at total E. limit mobility of the child of the house and its stay on air, deprive of society of peers. The mental condition of children suffers, especially from 8 — 9 years when they begin to understand the situation, notice that they are avoided by peers, see reaction of adults. Children become closed, unsociable, visit of school turns for them into a painful duty. Especially sharply teenagers endure the state.
At men at total E. sex life is often impossible or complicated that is connected with an underdevelopment, a chordee and morbidity of an erection. Women can lead normal sex life, pregnancy and childbirth at them proceed, as a rule, without complications.
Diagnosis E. at men it is not difficult. As E. at women it is characterized by less expressed anatomic changes, its diagnosis is complicated, especially at early children's age. Difficulties can arise also at differential diagnosis with the hypospadias which is followed by an incontience of urine and combined with a malformation of a vagina. At the same time the back
the ny wall of an urethra and a part of a front wall of a vagina are absent; the outside opening of a neck of a bladder opens in a vagina on all its extent. The entrance of the vagina has the involved, funneled appearance, urine follows from it. Small vulvar lips are underdeveloped, but unlike E. are connected in front; the clitoris is underdeveloped (can be absent), the outside opening of an urethra is absent. At a combination of a hypospadias to doubling of a vagina an upper (front) underdeveloped and narrow part of a vagina, from a cut urine expires, mistakenly take for an urethra.
Treatment operational. At total and subtotal forms E. its main objective is elimination of an incontience of urine. At E. a penis operation of creation of missing department of an urethra promotes improvement of an urination and at the same time allows to eliminate cosmetic defect. Operations at E. it is necessary to carry out on reaching 6 — 7-year age.
At presurgical inspection of patients E. make a X-ray analysis of sacrococcygeal department of a backbone, a pubic symphysis, excretory urography (see) and a tsistografiya (see) for the purpose of detection of the accompanying malformations of kidneys, ureters, an agenesia of a sacrum, etc. In case of detection of the accompanying malformations of kidneys and ureters make their correction in the beginning. However an agenesia of a sacrum (see. Sacral area) if it is followed nevrol. frustration, is a contraindication to operation; but sometimes make attempts of elimination of an epispadiya at these patients aged not earlier than 14 — 15 years. In the preoperative period it is necessary to liquidate maceration of skin and an intertrigo, to make sanitation of an oral cavity and treatment of the inflammatory centers in an organism.
For elimination of an incontience of urine at E. a number of the operative measures directed to creation of a mechanical obstacle to outflow of urine by formation of an urethral tube and its excess through bottom edge of a pubic symphysis is offered; lengthenings and narrowings of an urethra; turn of its (torsion) clockwise on 360 — 450 °C the subsequent fixing on the former place, etc. Other group of operations is based on creation of an artificial sphincter of a bladder by means of the mobilized muscle of a stomach, a hip or a crotch. However these operations do not eliminate an incontience of urine or at best give only temporary effect.
The operations directed on sozda-
to Fig. 2 were more effective. The diagrammatic representation of one of stages of plastics of a sphincter and a neck of a bladder according to Derzhavin at a total epispadiya: on area of a neck of a bladder put the narrowing noose sutures.
ny a sphincter from tissues of a neck of a bladder. The founder of these operative measures consider X. Young, to-ry in 1908 the fibrous site of a front wall and a neck of a bladder with the subsequent sewing up for recovery of a continuity of the circular course of muscle fibers of an internal sphincter suggested to exsect. It should be noted that the operation known in literature as Young's operation, was for the first time developed, carried successfully out and described in 1876 by A. Savostitsky. As a result of this operation the incontience of urine manages to be eliminated at 30 — 40% of patients. Insufficient efficiency of operation of Young is explained by the aspiration to recover a tsirkulyarnost of a sphincter at an underdevelopment or lack of its muscular layer that often does not provide deduction of urine. The positive take at operation, apparently, is observed only when as a part of a neck of a bladder at least muscular elements of a sphincter, rapprochement and sewing together partially remain to-rykh leads to recovery to circus-lyarnosti of their course and function of a sphincter. Young's operation is finished with imposing of suprapubic fistula for drainage of a bladder within 12 — 14 days. In the postoperative period formation of uric fistulas is possible.
Operation at E is more physiologic., the urine which is followed by an incontience, offered by V. M. Derzhavin in 1965. It differs in simplicity — is carried out without opening of a bladder and imposing of suprapubic fistula. The principle of this operation consists in creation of a sphincter from the muscular tissue of a vesical triangle functionally different from muscular tissue of a detruzor. After allocation of a neck and the lower third of a bladder on the centerline impose a number of noose silk sutures and immerse inside the longitudinal site of a front wall of a bladder and its
neck 3 — 4 cm wide (fig. 2). The second row of the narrowing seams, immersing the first row, try to obtain hard embracing by fabrics of the rubber catheter which is previously entered into a bladder. As a result of the region of the distal site of a vesical triangle form the funneled circular muscular press fixed to the longitudinal postoperative hem which is formed in front. Through a puncture of skin enter rubber drainages into paravesical (paravesical) cellulose. In the postoperative period the bladder is emptied through an urethral catheter therefore special attention is paid it to passability — for prevention of obturation of a catheter salts it is washed out by each 4 — 6 hours of 0,3% solution of acetylsalicylic acid. In
Fig. 3. The diagrammatic representation of the main stages of an urethroplasty across Dyupley at an epispadiya of a penis: and — a section of skin and future rags of skin at the edges of the split urethra (are shown by a dotted line); — from side rags of skin on a catheter the urethra is created, the wound is taken in by noose sutures.,
10 — the catheter, and then drainages delete the 12th day from paravesical cellulose.
In 3 — 4 months at the patients who underwent this operation normally adjustable act of an urination is established. Capacity of a bladder
increases and reaches 250-— 300 ml. The periods between urinations make from 3 to 5 hour. Full elimination of an incontience of urine is reached almost at 90% of patients.
In cases of inefficiency of plastics of a sphincter of a bladder urine is taken away in a large intestine (see the Ureteroplasty).
At the age of 13 — 14 years is also more senior carry out the second stage of operational treatment, on Krom carry out surgical correction of external genitals and an urethroplasty. For creation of missing department of an urethra many ways are offered, however the greatest application was found by plastic surgeries with use of skin of a penis, a scrotum, front abdominal wall. By Dyupley (1880) method the urethra is formed by broad mobilization of edges of a mucous membrane of an urethral trench and skin along all trunk of a penis. On a catheter create an urethral tube, over a cut sew skin (fig. 3, and, b). At an urethroplasty by Nelaton's method the front wall of an urethra is created from the skin rag found on skin of a stomach with the basis at an epispadichesky opening (fig. 4, a). The rag is turned off from top to bottom, the epidermal party close defect of an urethra, hem a rag to edges of the mobilized urethra; the skin wound on a front abdominal wall is taken in (fig. 4, b); the penis together with a wound surface of a skin rag is temporarily placed in the tunnel created under skin of a scrotum (fig. 4, in, d). After healing of wounds the penis is separated in the acute way from a scrotum. Rosenberg's operation is modification of a method of Nelaton; defect of an urethra is also closed a piece of leather of a stomach, but at the same time the penis is raised up also by
Fig. 4. The diagrammatic representation of the main stages of an urethroplasty on Nelatona at an epispadiya of a penis: and — a type of a penis before operation (the dotted line showed the line of a section of skin on a penis with cutting out of a rag on a stomach); — defect of an urethra is closed by a skin rag (the epidermal party inside), hemmed to edges of the split urethra; the wound of an abdominal wall is pulled together with noose sutures; in — between cross sections on a front surface of a scrotum the tunnel is created; — * the penis is shipped in the tunnel under skin of a scrotum, edges of wounds are taken in.,
hem edges of an urethral trench to skin of a stomach. After healing of wounds find and otseparovy-vat a piece of leather of a stomach together with a penis; the wound of a penis is closed by sewing together of edges of a rag, defect of skin on a wall of a stomach is pulled together with noose sutures.
The forecast after recovery of an urethra, external genitals and elimination of an incontience of urine favorable.
Bibliography: Derzhavin V. M. Operational treatment of a full epispadiya at children, Urol. and nefrol., No. 5, page 32, 1965, bibliogr.; Savostitsky G. A. A case of an epispadiya at women, the Chronicle the surgeon, about-va in Moscow, t. 1, JNb 1, page 292, 1875; Savchenko H. E. and Derzhavin of V. M. Epispadiya, Minsk, 1976, bibliogr.; H at x r and e N to about D. P. and Lyulko A. V. The atlas of operations on bodies of urinogenital system. M, 1972; Campbell M. Clinical pediatric urology, Epispadias, p. 285 a. o., Philadelphia — L., 1951.
V. M. Derzhavin, I. V. Kazanskaya.