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prune belly syndrome
ICD-10 Codes
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Description
Prune Belly Syndrome (PBS) Description
Prune belly syndrome, also known as Eagle-Barrett syndrome, is a rare congenital condition that affects the abdominal muscles and urinary tract in infants. The condition gets its name from the wrinkled appearance of the abdominal skin, which resembles a prune.
Key Features:
- Weak or absent abdominal wall muscles: This leads to a characteristic wrinkled appearance of the abdominal skin.
- Undescended testes: In males, the testicles do not descend into the scrotum.
- Urinary tract problems: The condition often affects the kidneys and bladder, leading to issues such as hydronephrosis (swelling of the kidney due to a blockage in the urinary tract) and megaureters (enlarged ureters).
Symptoms:
- Weak or absent abdominal muscles
- Undescended testes
- Urinary tract problems, including:
- Hydronephrosis
- Megaureters
- Enlarged bladder
Prevalence: Prune belly syndrome is a rare condition that mostly affects males. According to studies, the condition occurs in approximately 1 in every 40,000 to 50,000 births.
References:
- [10] Prune belly syndrome is characterized by poor development of the abdominal muscles, undescended testicles and an abnormal, expanded bladder.
- [11] Prune belly syndrome is a rare condition that gets its name because weak or absent abdominal wall muscles make your infant's belly look wrinkled.
- [12] Poor development of the abdominal muscles, causing the skin of the belly area to wrinkle like a prune. Undescended testicles. Urinary tract problems.
- [13] A baby born with prune belly syndrome has weak belly muscles and problems with their bladder and kidneys. The condition mostly affects males.
- [14] Sep 25, 2023 — Prune belly syndrome is characterized by elongated, dilated, and tortuous megaureters, which affect 81% of patients. Studies indicate that the ...
Additional Characteristics
- Weak or absent abdominal wall muscles
- Undescended testes
- Urinary tract problems
- Hydronephrosis (swelling of the kidney due to a blockage in the urinary tract)
- Megaureters (enlarged ureters)
- Enlarged bladder
Signs and Symptoms
Prune Belly Syndrome Signs and Symptoms
Prune belly syndrome, also known as Eagle-Barrett syndrome, is a rare condition characterized by the absence or underdevelopment of abdominal muscles, undescended testicles in boys, and an abnormal expanded bladder. The symptoms of this condition can vary from person to person but often include:
- Abdominal abnormalities: A wrinkled appearance with multiple folds of skin on the abdomen, making it look like a prune.
- Urinary tract problems: An abnormal, expanded bladder that can cause urinary tract issues, such as:
- Urinary tract infections
- Difficulty urinating
- Frequent urination
- Abdominal mass: A bulge above the pubic bones caused by the bladder or other abdominal organs.
- Visible outline of intestines: The intestines may be visible under the skin due to the absence of abdominal muscles.
In addition to these physical symptoms, prune belly syndrome can also cause:
- Constipation
- Delay in sitting and walking
- Difficulties coughing
It's essential to note that every individual with prune belly syndrome may not exhibit all of these symptoms. If you suspect that your child or someone else has this condition, it's crucial to consult a medical professional for proper diagnosis and treatment.
References:
[2] Prune belly syndrome is characterized by poor development of the abdominal muscles, undescended testicles and an abnormal, expanded bladder. [6] What are the signs and symptoms of Prune Belly syndrome? · a wrinkly abdomen with multiple skin folds · urinary tract organs, such as the bladder and kidneys, ... [7] Belly (abdomen) that looks wrinkly with multiple folds of skin · Bladder causing a bulge in the belly above the pubic bones · Urinary tract organs easy to feel ... [8] What are the symptoms of prune belly syndrome? · The abdomen may have a wrinkly appearance with multiple folds of skin. · An abdominal mass may sit above the ...
Additional Symptoms
- Urinary tract problems
- Abdominal abnormalities
- Visible outline of intestines
- Delay in sitting and walking
- Difficulties coughing
- abdominal mass
- constipation
Diagnostic Tests
Prune belly syndrome (PBS) can be diagnosed through various diagnostic tests, which are essential for confirming the condition and planning appropriate treatment.
- Prenatal Ultrasound: PBS is often diagnosed during routine fetal ultrasounds, as the distinctive appearance of the bowels hanging over the pubic bone can be observed [1].
- Kidney, Ureter, and Bladder (KUB) Test: The KUB test findings can usually be used for diagnosis based on the typical appearance of the bowels hanging over the pubic bone [2].
- Blood Tests: Blood tests may be conducted to rule out other conditions that may present with similar symptoms.
- Imaging Studies:
- Intravenous Pyelogram (IVP): An IVP can help evaluate the urinary tract and identify any abnormalities [3].
- Ultrasound: Ultrasound imaging, particularly renal US, is used to evaluate the kidneys and urinary tract [4].
- Voiding Cystourethrogram (VCUG): A VCUG may be performed to assess the bladder and urethra for any abnormalities.
- X-ray and CT scan: These imaging studies can help evaluate the abdomen and chest, and are used in conjunction with other diagnostic tests [5].
- Renal Ultrasound: This diagnostic tool allows the images of blood vessels, tissues, and organs of the renal system to be visualized, aiding in the diagnosis of PBS [7].
These diagnostic tests are essential for confirming prune belly syndrome and planning appropriate treatment. The goal is to preserve kidney function and prevent urinary tract infections.
References: [1] Context 1 [2] Context 2 [3] Context 3 [4] Context 4 [5] Context 5 [7] Context 7
Additional Diagnostic Tests
- Blood Tests
- Ultrasound
- X-ray
- CT scan
- Prenatal Ultrasound
- Voiding Cystourethrogram (VCUG)
- Kidney, Ureter, and Bladder (KUB) Test
- Intravenous Pyelogram (IVP)
- Renal Ultrasound
Treatment
Treatment Options for Prune Belly Syndrome
Prune belly syndrome, also known as Eagle-Barrett syndrome, is a rare congenital disorder characterized by the absence or underdevelopment of abdominal muscles, urinary tract abnormalities, and undescended testicles. While there is no cure for this condition, various treatment options are available to manage its symptoms.
Antibiotic Therapy
In mild cases of prune belly syndrome, antibiotic therapy may be prescribed to control and prevent urinary tract infections (UTIs) [1]. This is particularly important for infants with vesicoureteral reflux, a common complication of the condition [2].
Surgical Management
Early surgery is often recommended to address weak abdominal muscles, urinary tract problems, and undescended testicles [7]. The type and timing of surgical interventions depend on the severity of symptoms and individual patient needs. Some patients may require multiple surgeries throughout their lifetime [8].
Targeted Therapies
While not yet widely available, targeted drug therapies and gene therapies hold promise for modulating or curing prune belly syndrome in the future [6]. Researchers continue to explore innovative treatment approaches to improve outcomes for affected individuals.
Maintenance Suppressive Antibiotics
Children with prune belly syndrome who experience vesicoureteral reflux may benefit from maintenance suppressive antibiotics to prevent recurrent UTIs [2].
In summary, the drug treatment of prune belly syndrome primarily involves antibiotic therapy to manage urinary tract infections and surgical interventions to address associated complications. While targeted therapies are being researched, they are not yet widely available.
References: [1] - Context 1 [2] - Context 2 [6] - Context 6 [7] - Context 7 [8] - Context 8
Recommended Medications
- Antibiotic Therapy
- Targeted Therapies
- Maintenance Suppressive Antibiotics
💊 Drug information is sourced from ChEBI (Chemical Entities of Biological Interest) database. Always consult with a healthcare professional before starting any medication. Click on any medication name for detailed information.
Differential Diagnosis
Prune Belly Syndrome Differential Diagnosis
Prune belly syndrome (PBS) is a rare congenital disorder characterized by the absence or underdevelopment of abdominal muscles, undescended testicles, and urinary tract abnormalities. When diagnosing PBS, it's essential to consider other conditions that may present with similar symptoms.
- Lower Urinary Tract Obstruction (LUTO): LUTO can cause similar sonographic findings to PBS, including hydronephrosis and renal dysplasia [1].
- Eagle-Barrett Syndrome: This is another name for prune belly syndrome, indicating its rarity and the need for differential diagnosis.
- Triad Syndrome: This term refers to the combination of abdominal muscle deficiency, urinary tract abnormalities, and undescended testicles seen in PBS. However, other conditions may also present with this triad [8].
- Obrinsky Syndrome: This is another name for prune belly syndrome, highlighting its unique characteristics.
Key Features to Distinguish from Other Conditions
To accurately diagnose prune belly syndrome, consider the following distinct features:
- Weak or absent abdominal wall muscles, giving the appearance of a wrinkled "prune" skin [6]
- Undescended testicles
- Urinary tract abnormalities, including hydronephrosis and renal dysplasia
References:
[1] Sep 25, 2023 — On prenatal ultrasound, fetuses with prune belly syndrome have many sonogram findings comparable to those with lower urinary tract obstruction [1]. [8] Prune-Belly syndrome is also known as triad syndrome or Eagle-Barrett syndrome. It is characterized by the presence of three abnormalities [8].
Additional Differential Diagnoses
- megacystis-microcolon-intestinal hypoperistalsis syndrome
- congenital megabladder
- vesicoureteral reflux
- Lower Urinary Tract Obstruction (LUTO)
- N syndrome
Additional Information
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It is not intended to be a substitute for professional medical advice, diagnosis, or treatment. Always seek the advice of your physician or other qualified healthcare provider with questions about your medical condition.