Oligohydramnios sequence
| Classification according to ICD-10 | |
|---|---|
| Q60 | Renal agenesis and other kidney reduction defects |
| Q60.6 | Potter Syndrome |
| O41.0 | Oligohydramnios |
| P01.2 | Oligohydramnios harm to the fetus and neonate |
| ICD-10 online (WHO version 2019) | |
The oligohydramnios sequence or Potter sequence describes a number of effects that are initiated by insufficient production of amniotic fluid (with the formation of oligohydramnios ) during pregnancy .
Originally described by Edith Potter , an American pathologist , in a series of 5,000 autopsies in 17 male and three female fetuses or newborns with bilateral kidney agenesis ( bilateral renal agenesis ) , the symptom complex was initially referred to as Potter's syndrome or renofacial dysplasia . It is characterized by:
- Malformations in the urogenital tract , especially agenesis (absence or very severe underdevelopment) of both kidneys
- Club feet
- Hypoplasia (underdevelopment) of the lungs
- possible further malformations and / or incorrect posture of the extremities and the spine
- Malformations of the face (the so-called Potter faces ):
- special shape of the auricles , comparatively deep and flat ears, missing cartilage substance
- Epicanthus medialis
- Hypertelorism (comparatively large eye relief)
- Microgenius (underdevelopment of the lower jaw , which appears shortened as a result)
All of these peculiarities are due to the restricted space conditions due to the lack of amniotic fluid in the uterus and the resulting unusual growth conditions for the unborn child.
Since the etiology is not limited to the absence of the kidneys, even if the final path is pathogenic , it is usually referred to as an oligohydramnios sequence today.
In the context of syndromes , a combination with other malformations can occur, for example in Thomas syndrome .
