Parul Sevashram's Case of Bladder Exstrophy

In the tapestry of medical ailments that question our wisdom of the human body, one rare and complex abnormality stands out – Bladder Exstrophy.

It is a hereditary birth defect wherein the bladder and the front wall of the abdomen do not correctly close during foetal maturation. As a result, the bladder is revealed and situated outside the body. This condition is rare, and demands specialised medical supervision, often involving surgical intervention to fix the anatomical abnormality and encourage normal urinary and pelvic operation. Bladder exstrophy can have noteworthy physical, emotional, and social influences on individuals and their families, making exhaustive medical management and support critical for those affected.

Signs and symptoms

Visible Bladder: The most distinctive sign is the exposure of the bladder on the lower abdominal wall, resembling an open “Y” shape.
Abdominal Wall Defect: A gap or separation in the abdominal muscles and pelvic bones, resulting in the exposed bladder.
Incontinence: Difficulty in controlling urine due to the bladder’s position, often leading to continuous or frequent leakage.
Genital Abnormalities: In males, the penis may be split or have an unusual appearance, while in females, the clitoris and labia may be separated.
Pelvic Floor Issues: Weakened pelvic muscles may lead to difficulties in controlling bowel movements as well.
Pubic Bone Abnormality: The pubic bone may be divided, leading to an atypical appearance of the pelvis.
Recurrent Urinary Tract Infections: Due to the exposed bladder, infections can occur frequently.
Psychosocial Impact: The physical differences and ongoing medical care can have emotional and social effects on individuals and families.

Causes

Genetic Factors: There may be a genetic predisposition for bladder exstrophy, as it sometimes runs in families. However, no specific gene or genetic mutation has been consistently identified as the sole cause.
Abnormal Development: During the early stages of pregnancy, the abdominal wall and bladder should close and form properly. In bladder exstrophy, this process is disrupted, leading to the failure of the abdominal wall to fully enclose the bladder.
Environmental Factors: Certain environmental factors or prenatal exposures might play a role in the development of bladder exstrophy. However, no specific environmental triggers have been definitively identified.
Multifactorial Nature: Bladder exstrophy is considered a complex, multifactorial condition, meaning that both genetic predisposition and environmental influences interact to contribute to its development.

Treatment options

Primary Repair Surgery: This involves surgically closing the bladder and abdominal wall, repositioning the pelvic bones if necessary, and reconstructing the genitalia. The first surgery is often performed shortly after birth.
Ongoing Surgical Interventions: Multiple surgeries may be needed as the child grows to address functional issues, improve continence, and optimise bladder and pelvic function.
Medical Management: Individuals with bladder exstrophy might require ongoing medical care to manage urinary continence, prevent infections, and address any associated issues.
Physical and Occupational Therapy: These therapies can aid in pelvic muscle strengthening, mobility, and daily living skills.
Psychosocial Support: Given the potential emotional and social impact, individuals and families may benefit from counselling and support groups.

Risk factors

The complexity of the Condition: The severity of bladder exstrophy can impact the number and complexity of surgeries needed.
Surgical Complications: Surgical interventions carry risks such as infection, bleeding, scarring, and anaesthesia-related complications.
Urinary and Bowel Function: Achieving optimal urinary and bowel continence might require ongoing management and potential additional surgeries.
Psychosocial Impact: The condition can have emotional and social implications, necessitating support for individuals and families.
Long-Term Follow-Up: Lifelong monitoring and medical care are often necessary to address any potential complications and ensure optimal function.

How Parul Sevashram Hospital solved the case of Bladder Exstrophy?

At the heart of medical splendour lies stories that boost wonder and gratitude, where reliable professionals and cutting-edge techniques converge to fix the problems of human health. Such is the extraordinary tale of a 2-year-old girl from Madhya Pradesh who found her way to the Pediatric Department of Parul Sevashram Hospital. Born with a condition as rare and complex as Bladder Exstrophy, her voyage toward a healthier life was a testament to the inexhaustible commitment of medical experts and the astonishments of modern medicine.

Dr Mishal Patel and Dr Karthik Vishvanathan, the guiding lights of her medical journey, navigated her course with mastery and compassion. Bladder Exstrophy had left her with a missing front abdominal wall, exposing her urinary bladder on the front of her abdomen. Such a condition would deter most, but not the skilled team at Parul Sevashram Hospital.

The collaborative effort of the medical team culminated in a Complete Primary Repair of Exstrophy (CPRE) surgery – a transformative procedure where the bladder was turned in, closed, and the bladder neck repaired. With post-operative care, the patient emerged with newfound continence, passing urine through the natural passage. Yet, the challenges extended beyond the bladder, with the pelvic bones widely separated due to the long-standing neglect of her condition. The intricate orthopaedic procedure involved a controlled alteration of the pelvic bones’ direction, and the ingenuity of using a thick Mersilene Tape to bring the bones together, sparing her from the usual trauma caused by stainless steel wires. An external fixator ensured stability during her recovery.

Conclusion

Witnessing her triumphant journey, one can’t help but express profound appreciation for the dedication of Parul Sevashram Hospital which has emerged as the best orthopaedic hospital in Vadodara. The child, once burdened by her condition, now walks, runs, and plays alongside her friends – a testament to the power of collaboration, innovation, and compassionate care. The story of this little girl echoes the broader message that, through expertise and dedication, even the most intricate medical challenges can be overcome, redefining lives and inspiring hope for a brighter, healthier future.