The human pelvis is a vital structure that supports and protects various organs in the lower abdominal region. In some cases, children may develop a pelvis deformity, particularly in association with a condition known as exstrophy bladder.
Exstrophy bladder is a rare congenital anomaly characterized by the malformation of the urinary bladder and the anterior abdominal wall. In this condition, the bladder is exposed to the external environment outside the body. The deformity primarily affects the pelvic region, leading to structural abnormalities and functional impairments.
The exact cause of exstrophy bladder is unknown; however, it is believed to result from genetic and environmental factors. Some researchers suggest that abnormalities in the development of the cloacal membrane during embryonic growth contribute to the condition. Specific genetic mutations have also been associated with exstrophy bladder, indicating a possible genetic predisposition.
The symptoms of deformity associated with exstrophy bladder are noticeable soon after birth. The sensitive bladder and anterior abdominal wall create a distinct appearance, often called the "bladder exstrophy complex." The key symptoms include.
Bladder exposure: The bladder is visible outside the body, appearing as a red, moist mass in the lower abdominal region.
Pelvic malformation: The pelvis may be widened and flattened, resulting in an abnormal shape.
Urinary dysfunction: Children with exstrophy bladder may experience urinary incontinence, difficulty emptying the bladder, and an increased risk of urinary tract infections.
Hernias: There is an increased risk of developing hernias due to the weakened abdominal wall.
Treating deformity associated with exstrophy bladder is complex and requires a multidisciplinary approach involving pediatric urologists, reconstructive surgeons, and other specialists. The primary treatment goals are to achieve urinary continence, improve bladder function, and restore the typical appearance of the pelvic region.
Surgical intervention is the mainstay of treatment for exstrophy bladder deformity. The initial stage involves a series of surgeries performed within the first few days of life to close the bladder and abdominal wall. This procedure, known as bladder closure, helps protect the sensitive bladder and allows for further reconstructive surgeries in the future.
sensitive bladder and allows for further reconstructive surgeries in the future.
Subsequent surgeries are typically performed during early childhood and adolescence to address specific aspects of the deformity. These procedures may include bladder augmentation, urethral reconstruction, and pelvic osteotomies to correct malformation. The aim is to achieve urinary continence and improve bladder capacity while promoting normal skeletal development.
In addition to surgical interventions, children with exstrophy bladder may require ongoing medical management. It includes regular urological evaluations, bladder training, and medications to control urinary function and prevent complications such as infections.
Psychological support and counselling are crucial to comprehensive care for children and families affected by exstrophy bladder deformity. The condition can significantly impact the emotional well-being and self-esteem of the child, requiring a supportive environment to navigate the challenges associated with the deformity.