From Big Medical Encyclopedia

ECTOPIA OF THE BLADDER - (ectopia vesicae urinariae; Greek ektopios remote, remote from the place; a synonym an ekstrofiya of a bladder) — a difficult inborn malformation — lack of a nizhnesredinny part of a front abdominal wall and front wall of a bladder. AA. the m of the item can be combined with a cryptorchism (see), a hypoplasia or an aplasia of a prostate (see), doubling of a vagina (see), a two-horned uterus (see).

AA. the m of the item meets approximately in 1 case on 30 thousand childbirth, is more often at boys. It arises at zaro breathing in the first 4 — 7 weeks of pregnancy and is caused by the caudal shift of hearth hillocks.

AA. the m of the item is shown as the defect of a front abdominal wall which is located over a pubis, through to-ry the back wall of a bladder and area of a vesical triangle with mouths of ureters prolabirut. From ustiya of ureters (ostiums ureteris, T.) urine is constantly emitted. The mucous membrane of a bladder in the field of edges of defect passes into skin of a stomach; muscular layers of a wall of a bladder are delimited from muscles of a front abdominal wall by an aponeurotic ring. Muscles of a front abdominal wall above defect are underdeveloped and replaced with an aponeurotic membrane, the navel is absent or is located on border of skin and mucous membrane of a vesical wall. The mucous membrane of a bladder, usually smooth, light pink color, soon after the birth of the child becomes friable, edematous, easily bleeding; its surface becomes covered by polypostural growths, in depth to-rykh mouths of ureters are hidden. In the subsequent the mucous membrane of a bladder gradually is flattened, becomes smooth, and the muscular layer is sclerosed and loses sokratitelny function. At boys the penis is underdeveloped, pulled in, its front surface is deprived of skin and covered by the mucous membrane passing from a wall of a bladder. Girls have a clitoris and front commissure of vulvar lips are split. AA. m and. always is followed by discrepancy of bones of a pubic joint, at Krom of diastases can reach several centimeters.

Treatment only operational is also shown at early age (at newborns and babies) before development of the heavy complications caused by hl. obr. the ascending infection of uric ways. There are 3 groups of operative measures at E. m of the item: 1) a cystoplasty from local fabrics; 2) creation of the isolated bladder from a segment of a gut; 3) removal of urine in intestines.

Reconstructive plastic surgeries with use of local fabrics are most physiologic, but with their help seldom it is possible to eliminate an incontience of urine. Among these interventions the greatest distribution was gained by the operation offered by G. A. Bairov (1966). After introduction of polyethylene catheters to ureters will mobilize edges of a bladder, separating them from a vagina of direct muscles of a stomach and a peritoneum. The neck of a bladder is allocated together with sfink a shooting gallery, cutting from edges of pubic bones. For creation of a cavity of a bladder sew the mobilized edges of a bladder a chrome-plated catgut, without taking a mucous membrane, and impose suprapubic fistula (see Cystostomy). Through a puncture of a wall of a bladder and skin the right ureteric catheter is removed outside, near it place a drainage tube of suprapubic fistula. The left ureteric catheter is stacked on the centerline and sewed on it a thin catgut of edge of an urethral plate and a sphincter of a bladder. The created urethral tube is immersed between cavernous bodies of a penis, to-rye pulled together rare catgut seams. Pubic bones pull together, squeezing a basin from sides, and sew. Paravesical spaces drain the tubes brought outside to skin of a crotch. Defect of an abdominal wall is layer-by-layer taken in.

Operation is contraindicated at the expressed prematurity, the heavy combined malformations, considerable polypostural changes of a mucous membrane of a bladder.

To. V. Konstantinova (1955) at the small sizes of the vesical platform suggested to create a front wall of a bladder with use of a free musculoaponeurotic rag, to-ry find from a direct muscle of a stomach and shroud in the site of an epiploon on a leg.

One of the most perspective ways of operational treatment E. m of the item — creation of a bladder from a segment of a gut. However during the use of this method heavy postoperative complications are often observed and it is not always possible to eliminate an incontience of urine and to recover the adjustable act of an urination. Many methods of operations with use of segments of thin, blind, sigmoid and direct guts are offered. In particular, M. S. Subbotin's methods

(1901), A. V. Melnikova (1924) are based on creation of the isolated bladder from a rectum, to-ruyu later crossing on border with a sigmoid gut take in tightly and implant into it ureters. Through a tunnel between a rectum and a sacrum remove outside the distal end of the mobilized sigmoid gut, fixing it in the field of a section on border of a mucous membrane and skin of an anus. As a result of intervention deduction of urine and a calla is reached by an outside sphincter of an anus. Operation is traumatic, often is complicated chastich by ny cicatricial wrinkling of distal department of the partition dividing the created bladder and the reduced sigmoid gut.

The most productive consider the operation offered by Hendren (W. H. Hendren, 1976) though experience of its performance is not big yet. The isolated bladder is created from a segment of a sigmoid gut length apprx. 10 cm. Its proximal end is taken in tightly and fixed to the cape (promontorium), distal — brought to a front abdominal wall in suprapubic area in the form of fistula. Previously implant ureters into a gleam of a segment of a gut. Operation is carried out after preliminary (for 2 — 3 weeks) by switching off of a large intestine by imposing of fistula on the ascending colon. The second stage of operation is made 6 months later. The fistula of an intestinal segment created in suprapubic area is allocated from surrounding fabrics throughout 6 — 7 cm; the allocated end of fistula is anastomosed isoperistaltically with a rectum.

The operations directed to removal of urine in intestines make in case of impossibility of creation of a bladder with use of local fabrics or the isolated segment of a gut; at an incontience of urine after unfortunate attempts of its elimination in the operational way. Such interventions are contraindicated at an incontience a calla in connection with dysfunction of an outside sphincter of an anus and at its weakness (not deduction liquid a calla). However in some cases it is possible to recover function of an outside sphincter by electrostimulation (see). Removal of urine in intestines is made by change of ureters in a gut (see the Ureteroplasty).

The forecast considerably is defined by timeliness of operational treatment. Optimum results manage to be achieved by means of a cystoplasty and creation of the isolated bladder from a segment of a gut. The operations directed to removal of urine in intestines predictively are least favorable since at such patients the phenomena of persistent giperkhloremichesky acidosis quite often develop, the ascending infection leads to frequent aggravations hron. pyelonephritis and a chronic renal failure (see).

Bibliography: Bairov G. A. Reconstructive plastic surgery at an ekstrofiya of a bladder at newborns. Vestn. hir. t. 97, No. 12, page 85, 1966; Doletsky S. Ya., Gavryushov V. V. and Akopyan V. G. Hee rurgiya of newborns, page 218, M., 1976; Konstantinova K. V. To operational treatment of patients with an ekstrofiya of a bladder, Urology, No. 2, page 71, 1955; The Guide to clinical urology, under the editorship of A. Ya. Pyteli, page 5, M., 1970; Suubbotin M. S. Formation of a bladder and urethra with a press from a rectum at ektopiya, epispadiya and in general at an incontience of urine, the First congress rossiysk. hir., page 155, M., 1901; Hendren W. N of Exstrophy of the bladder — in alternative method of management, J. Crol. (Baltimore), v. 115, p. 195, 1976.

V. M. Derzhavin, I. V. Kazanskaya.