Potter's syndrome digital illustration

Potter's syndrome Save


ICD-10 code: Q60.6

Disease category: Q60: Renal agenesis and other reduction defects of kidney

Potter's Syndrome: A Rare and Devastating Condition

Potter's syndrome, also known as bilateral renal agenesis, is a rare and heartbreaking condition that affects the development of a baby's kidneys and lungs. This congenital disorder occurs when both kidneys fail to form properly during pregnancy, leading to a series of complications that are often fatal for the affected fetus.

While the exact cause of Potter's syndrome is not fully understood, it is believed to be a result of genetic abnormalities or environmental factors. The absence of both kidneys prevents the production of amniotic fluid, which is essential for the development of the lungs. Without amniotic fluid, the lungs cannot expand properly, leading to underdeveloped lungs, a condition known as pulmonary hypoplasia.

Here are some key points to know about Potter's syndrome:

  1. Rare occurrence: Potter's syndrome is an extremely rare condition, with an estimated incidence of 1 in 4,000 to 1 in 20,000 pregnancies. It affects both males and females equally.
  2. Physical characteristics: Babies with Potter's syndrome often have distinct physical features, such as flattened noses, low-set ears, and folds of extra skin around the neck.
  3. Other complications: In addition to kidney and lung abnormalities, Potter's syndrome can lead to skeletal malformations, facial deformities, and impaired growth.
  4. Prenatal diagnosis: Potter's syndrome can be detected during routine prenatal ultrasound examinations. The absence of amniotic fluid and the presence of characteristic physical features may raise suspicion.
  5. Impact on pregnancy: Unfortunately, most cases of Potter's syndrome result in stillbirth or neonatal death. In rare cases where the baby survives, the prognosis is extremely poor, and long-term complications are common.

Due to the severity of the condition and the limited treatment options available, the focus of managing Potter's syndrome is primarily on palliative care and support for the parents. Genetic counseling may be offered to families affected by the syndrome to help them understand the underlying causes and assess the risk of recurrence in future pregnancies.

In conclusion, Potter's syndrome is a distressing condition that significantly impacts the lives of affected families. Increased awareness and further research are crucial to better understand the causes and potential treatments for this devastating disorder.

Treatment of Potter's syndrome:

Treatment Options for Potter's Syndrome

Potter's syndrome, also known as Potter sequence or Potter's facies, is a rare and serious condition that affects fetal development. It is characterized by a group of physical abnormalities that can lead to life-threatening complications. While there is no cure for Potter's syndrome, there are several treatment options available to manage the...

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