RESUMEN
INTRODUCTION AND IMPORTANCE: Cloacal exstrophy (CE) is defined as a complex anomaly that affects the urogenital and intestinal tracts. It is the most serious form of anomaly that is described within the so-called exstrophy-epispadias complex. These malformations usually present a challenge in the management of particular conditions, as most of these forms require multiple surgeries, resulting in the use of multidisciplinary approaches, including reconstructive urologists, pediatric surgeons, orthopedic surgeons, endocrinologists, pediatricians, psychologists and nutritionists. Additionally, these patients present with ambiguous genitalia, which is another aspect that needs to be taken into consideration during the management of this condition. CASE PRESENTATION: The first patient, a baby who was 8 days of life and referred from a peripheral hospital, presented with classic features of cloaca exstrophy. He underwent first-stage cloacal exstrophy repair. The intraoperative findings included a bi-halved bladder and phallus, and the ureters were not appreciated, but there was continuous urine leakage from the bi-halved bladder and no uterus or ovaries. Poorly formed cecum, cecal-cutaneous fistula with an everted part of the terminal ileum protruding outside (mucosa-out), no transverse, no descending colon, collapsing small bowel, left undescended testis in the inguinal region, and right abdominal undescended testis. He first underwent surgery, which involved ileostomy, omphalocele closure and proper bladder exstrophy construction. The second patient, a 6-day-old female, had a similar presentation and physical findings as the first patient did, except that she had elephantoid trunk deformity with a cecal fistula, bifid clitoris, two cervical orifices, and two uteri completely separated with ovaries. Mobilization of the hindgut, closure of the cecal fistula, and proper bladder exstrophy after repair of the posterior wall were performed. The third patient was a 10-day-old female, similar to the second patient, but this patient presented with a left leg deformity with wide diastasis. In this case, the urinary bladder was not bivalved, and the cecal fistula had perforated just below the posterior wall of the urinary bladder. A mild omphalocele, bifid clitoris and vagina, one cervical orifice, and two uteri completely separated, with ovaries observed. Mobilization of the hindgut, closure of the cecal fistula, and proper bladder exstrophy after repair of the posterior wall were performed. The postoperative period was uneventful. CLINICAL DISCUSSION: Surgical management of cloacal exstrophy is typically undertaken in the newborn period (48-72 h) as a combined effort between pediatric surgery and urology. In the setting of associated spinal dysraphism, neurosurgical consultation and closure should be undertaken as soon as the infant becomes medically stable. Early operation minimizes bacterial colonization of exposed viscera and may decrease the need for pelvic osteotomy. The goals of treatment include securing the abdominal wall and bladder closure, preserving renal function, preventing short bowel syndrome, creating functional and cosmetically acceptable genitalia, and attaining acceptable urinary and fecal continence. CONCLUSION: Cloacal exstrophy remains a rare and complex congenital anomaly characterized by an array of anatomical defects affecting multiple organ systems. With respect to the approach of this congenital malformation, it is therefore important that these individuals and their families remain under the care of a multidisciplinary team of providers who can offer medical care, counseling and life-long follow-up.
RESUMEN
INTRODUCTION AND IMPORTANCE: Leiomyomas are rare, benign mesenchymal tumors. They represent 1 to 5 % of all urinary bladder tumors. 20 % are asymptomatic, but most presentations are voiding and storage, followed by hematuria. Surgery has been reported to be a standard treatment, depending on the size of the tumor and its location in the bladder. The following case report, we discuss the case of a bladder leiomyoma presenting with storage and voiding symptoms and managed with dual approach of Transurethral resection of bladder tumor (TURBT) and open urethrotomy. CASE PRESENTATION: A 39-year-old female who presented with a one-year history of total hematuria and a blood clot that was ovoid in shape presented with urge incontinence, nocturia, strain during urination, and incomplete bladder emptying. She was mildly anemic, afebrile, with no palpable peripheral lymph node and no palpable mass. PVE revealed an anteriorly located cervix. The patient's Labs revealed to have moderate anemia of 8 g/dl, blood chemistry was uneventful, and ultrasound (USS) revealed that both kidneys were normal. The urinary bladder was well distended, with a mass located at the base, measuring 4.13 cm × 4.14 cm. MRI revealed a well-circumscribed intramural tumor on the left side measuring 4.3 cm × 4.2 cm, close to the bladder neck. A cystoscopy was used to visualize the tumor from the left lateral wall at 3 o'clock, extending to 5 o'clock, and part of the bladder neck. The tumor was solid, easily bled, and had an irregular margin, and the bladder mucosa was normal. The 1st TURBT was used for diagnosis; although she still persistent storage and voiding symptoms, she subsequently underwent 2nd TURBT, which was resected to completion with the aid of ventral urethrotomy. After the TURBT, tumor protruded into the urethra, complete excision was performed through the urethra due to extension of the tumor to the urethra. The patient's postoperative events were uneventful, and the patient was discharged with catheter care for 10 days. On follow-up, hematuria resolved, and there were no lower urinary tract symptoms. CLINICAL DISCUSSION: Leiomyoma of the urinary bladder is a rare, benign mesenchymal tumor. They are the most common type of tumor of the urinary bladder. The most common presentations are storage and voiding symptoms, and hematuria. The initial USS can be used, and CT IVU or MRI is necessary for surveillance of the upper tract, possibly with respect to the tumor location in relation to the ureteric orifice. Tumors can be endovesical, intramural or extravesical, resulting in different presentations. Some tumors are pedunculated and can move close to the bladder neck or even at the urethra and present with urinary retention. Leiomyomas are surgically excised, and sometimes via a dual approach, transurethral resection of bladder tumors is important. These patients have a very low recurrence rate and are symptom free, and they have a good prognosis. CONCLUSION: Leiomyomas are surgically excised, and sometimes via a dual approach, transurethral resection of bladder tumors is important. These patients have a very low recurrence rate and are symptom free, and they have a good prognosis.
