Mikrognathie

Micrognathia • Micrognathia - Ganzer Fall bei Radiopaedia

Micrognathia

• Micrognathia - Ganzer Fall bei Radiopaedia

Patel M, Micrognathia. Case study, Radiopaedia.org (Accessed on 12 Dec 2022) https://doi.org/10.53347/rID-19728 • CC by-nc-sa 3.0 (modified)

Radiopaedia • CC-by-nc-sa 3.0 • de

siehe auch

Pätau-Syndrom
Ehlers-Danlos syndrome
Herzfehler
Marfan-Syndrom
mandibuläre Retrognathie
Turner-Syndrom
Polyhydramnion
Trisomie 18
Franceschetti-Zwahlen-Syndrom
Embryopathia rubeolosa
Pena-Shokeir-Syndrom
Noonan-Syndrom
Mikrodeletionsyndrom 22q11
Goldenhar-Gorlin-Syndrom
Smith-Lemli-Opitz-Syndrom
Fetales Alkoholsyndrom
Fryns-Syndrom
microgenia
Juvenile idiopathische Arthritis
hydrolethalus syndrome
Coffin-Lowry-Syndrom
Stickler-Syndrom
camptomelic dysplasia
Pierre-Robin-Sequenz
Akrogerie
Achondrogenesie
Prader-Willi-Syndrom
seckel syndrome
Graham-Cox-Syndrom
lethal multiple pterygium syndrome
Bloom syndrome
Metaphysäre Chondrodysplasie Typ Jansen
Thrombozytopenie-Radiusaplasie-Syndrom
Neu-Laxova-Syndrom
Russell-Silver syndrome
Hemifaziale Mikrosomie
Yunis-Varon-Syndrom
Hallermann-Streiff-Syndrom
Wolf-Hirschhorn-Syndrom
OFM Nager-Syndrom
Progerie
Melnick-Needles-Syndrom
Mesomele Dysplasie Typ Verloes-David-Pfeiffer
COFS-Syndrom
Katzenschrei-Syndrom
Dinno-Syndrom
Frontometaphysäre Dysplasie
Johanson-Blizzard-Syndrom
SGFLD-Syndrom

The term micrognathia describes a small mandible.

Epidemiology

Associations

Micrognathia is associated with a vast array of other congenital anomalies which include:

aneuploidic syndromic
non-aneuploidic syndromic
non-syndromic non-aneuploidic
- congenital cardiac anomalies
- certain skeletal dysplasias
  - achondrogenesis

Pathology

A small mandible occurs secondary to abnormalities of the first branchial arch which in turn are caused by deficient or insufficient migration of neural crest cells and usually occur around the 4 week of gestation.

Radiographic features

Antenatal ultrasound

Due to a large portion of normal mandibular growth occurring in the 3 trimester, the condition is best diagnosed towards the latter half of pregnancy

a true sagittal facial image would show a receding chin
the facial profile view is most useful in evaluating the mandibular size

Micrognathia is often a subjective finding best appreciated on a midline sagittal view.

Parameters used for objective measurement include:

jaw index: (mandibular anteroposterior diameter/biparietal diameter) x 100
frontal nasomental angle

Ancillary sonographic features

If fetal swallowing is impaired there may be evidence of polyhydramnios.

Significance

Due to a high association rate with other anomalies, the detection of micrognathia warrants a careful search for other fetal abnormalities.

Treatment and prognosis

The overall prognosis is highly variable dependent on the presence of other associated anomalies. Even when there is isolated fetal breathing (respiratory) difficulty at the time of birth, it is a concern . In selected case a genioplasty may be an option in later life.

Complications

Severe micrognathia can potentially compromise neonatal respiration after birth.