Exstrophy epispadias complex with adenocarcinoma in adults: a case series and repair technique

نویسندگان

چکیده

The exstrophy-epispadias-complex (EEC) is a rare congenital malformation with spectrum of abdominal-pelvic fusion abnormalities, an incidence 1/46,000 LB. Recurrence 1 in100 & the male-to-female ratio 2.3:1. Patients rarely may present in adulthood due to lack knowledge or financial constraints; it increases risk bladder carcinoma mostly adenocarcinoma squamous carcinoma, chronic inflammation, infection, and metaplasia. It has significant physical, functional, social, sexual, psychological burden. Three patients have been operated on our institute. A 37-year-old male, 19-year-old 23- year-old female patient. operation planned was radical cystectomy ileal conduit expanded local lymphadenectomy all three patients. pelvis abdominal wall defects were repaired differently post-radical 3 using different techniques. first patient went through primary tension closure, second bilateral rectus muscle used as flap reinforced mesh, third patient, we rotation skin flap. All followed for year. developed wound dehiscence by fecal fistula during follow-up succumb sepsis, recovered well postoperative period, unfortunately, recurrence locally multiple metastases, develops necrosis at tip day 5, however, recover after dressing, no seen year regular follow up. As result, mesh recovers better than flap, which closure.

برای دانلود باید عضویت طلایی داشته باشید

برای دانلود متن کامل این مقاله و بیش از 32 میلیون مقاله دیگر ابتدا ثبت نام کنید

اگر عضو سایت هستید لطفا وارد حساب کاربری خود شوید

منابع مشابه

The Exstrophy-epispadias complex

Exstrophy-epispadias complex (EEC) represents a spectrum of genitourinary malformations ranging in severity from epispadias (E) to classical bladder exstrophy (CEB) and exstrophy of the cloaca (EC). Depending on severity, EEC may involve the urinary system, musculoskeletal system, pelvis, pelvic floor, abdominal wall, genitalia, and sometimes the spine and anus. Prevalence at birth for the whol...

متن کامل

Bladder exstrophy-epispadias complex.

The bladder exstrophy-epispadias complex (BEEC) represents an anterior midline defect with variable expression comprising a spectrum of anomalies involving the abdominal wall, pelvis, urinary tract, genitalia, and occasionally the spine and anus. The vast majority of BEEC cases are classified as non-syndromic and the etiology of this malformation is still unknown. This review presents the curre...

متن کامل

Modern Management of the Exstrophy-Epispadias Complex

The exstrophy-epispadias complex is a rare spectrum of malformations affecting the genitourinary system, anterior abdominal wall, and pelvis. Historically, surgical outcomes were poor in patients with classic bladder exstrophy and cloacal exstrophy, the two more severe presentations. However, modern techniques to repair epispadias, classic bladder exstrophy, and cloacal exstrophy have increased...

متن کامل

Surgical embryology of the exstrophy-epispadias complex.

Among the developmental anomalies seen in children, few has as broad a spectrum as the exstrophy-epispadias complex which encompasses numerous abnormalities arising in the hind-end of the embryo. Table 1, the scheme proposed by Marshal and Muecke, depicts the developmental relationships of the variations within the complex. One can readily appreciate the multifaceted nature of a syndrome which ...

متن کامل

Bladder Exstrophy and Epispadias

Introduction Exstrophy and epispadias are part of a spectrum of anomalies characterised by exposure of part or all of the mucosa of the lower urinary tract (bladder and urethra) to the external environment through a defect in the anterior abdominal wall. At one end of this spectrum is cloacal exstrophy, which is the most complex of these anomalies. It is characterised by exposure of the bladder...

متن کامل

ذخیره در منابع من


  با ذخیره ی این منبع در منابع من، دسترسی به آن را برای استفاده های بعدی آسان تر کنید

ژورنال

عنوان ژورنال: International Surgery Journal

سال: 2023

ISSN: ['2349-2902', '2349-3305']

DOI: https://doi.org/10.18203/2349-2902.isj20232335