Microstomy

Classification according to ICD-10
Q18.5	Microstomy
ICD-10 online (WHO version 2019)

The microstomy (from ancient Greek μικρός micro , small 'and'Mouth', also 'mouth', 'opening') is a congenital or acquired mouth that is too small or an opening that is too small.

If only hypoplasia of the lower jaw is present, it is called micrognathia .

Spread and causes

The frequency is not known. Acquired microstomy can be found in up to 10% of burns and electrical accidents in the mouth area during the growing age and in over 30% of cases of scleroderma . A postoperative growth disorder after tumor removal in the mouth area, a CREST syndrome or forms of epidermolysis bullosa are among the causes of an acquired microstomy.

In the context of syndromes

A microstomy is found as a feature of some syndromes :

• Auriculo-condylar syndrome
• Burton syndrome , synonym: Kniest-like dysplasia with pursed lips and Ectopia lentis
• Desbuquois Syndrome
• Edwards Syndrome
• Femoral-Facial Syndrome
• Fine-Lubinsky syndrome , synonym brachycephaly - deafness - cataract - mental retardation
• Freeman-Sheldon syndrome and its variant Sheldon Hall syndrome
• Hallermann Streiff Syndrome
• Michelin Tire Baby Syndrome , synonyms: Michelin Tire Baby Syndrome; Skin folds, multiple ring-shaped, of the extremities; CCSF; Kunze-Riehm syndrome; Michelin tire baby syndrome; MTBS , AD, mutations in the MAPRE2 gene at 18q12.1-q12.2
• Multiple pterygium syndrome
• Oro-palatal dysplasia Bettex-Graf
• Oto-palato-digital syndrome type 2
• Richieri-Costa-Pereira Syndrome , synonyms: RCPS; Robin sequence with cleft mandible and limb anomalies , AR, mutations in the DDX48 gene at 17q25.3
• Simosa syndrome , synonym: face, flat - microsomia - ear anomalies; Blepharophimosis - Telekanthus - Microstomy;
• Syndromic Microphthalmia 5 , AD, mutations in the OTX gene at 14q22.3
• X-linked OHDO syndrome , XLR, mutations in the MED12 gene at Xq13.1

Clinical manifestations

Clinically, the focus is on the reduced opening of the mouth, in addition there are functional restrictions with articulation difficulty when speaking, problems with brushing teeth and possible dental treatment.

literature

• TA Spanholtz, P. Theodorou, V. Phan, W. Perbix, G. Spilker: reconstruction of the oral commissure in microstomia by deep dermal burn - a modified technique. In: Hand surgery, microsurgery, plastic surgery: Organ of the German-speaking work group for hand surgery: Organ of the German-speaking work group for microsurgery of the peripheral nerves and vessels: Organ of the Association of German Plastic Surgeons. Volume 39, No. 5, October 2007, pp. 350-355, doi: 10.1055 / s-2007-965796 , PMID 17985280 .
• A. Satpathy, AK Gujjari: Complete Denture in a Microstomia Patient. In: Journal of clinical and diagnostic research: JCDR. Volume 9, No. 5, May 2015, pp. ZD16 – ZD18, doi: 10.7860 / JCDR / 2015 / 12280.5910 , PMID 26155580 , PMC 4484172 (free full text).
• LG Branch, LR David: Management of Severe Microstomia in a Ten-Week-Old Infant. In: The Journal of craniofacial surgery. Volume 26, No. 5, July 2015, pp. 1609-1610, doi: 10.1097 / SCS.0000000000001847 , PMID 26114536 .
• KN Nguyen, I. Semenov, B. Blasiole, JG Robison, DH Chi: Congenital microstomia in a neonate with impending respiratory compromise. In: Case reports in anesthesiology. Volume 2014, 2014, p. 739463, doi: 10.1155 / 2014/739463 , PMID 25610661 , PMC 4295428 (free full text).

Individual evidence

1. ↑ Willibald Pschyrembel : Clinical Dictionary . 266th, updated edition. de Gruyter, Berlin 2014, ISBN 978-3-11-033997-0, keyword microstomy
2. ↑ Encyclopedia Dermatology
3. ↑ ^{a b} emedicine
4. ↑ Kniest-Like Dysplasia With Pursed Lips And Ectopia Lentis.  In: Online Mendelian Inheritance in Man . (English)
5. ↑ Femoral-Facial Syndrome. In: Orphanet (Rare Disease Database).
6. ^ Fine-Lubinsky syndrome. In: Orphanet (Rare Disease Database).
7. ↑ Brachycephaly, Deafness, Cataract, Microstomia, And Mental Retardation.  In: Online Mendelian Inheritance in Man . (English)
8. ↑ Sheldon Hall Syndrome. In: Orphanet (Rare Disease Database).
9. ↑ Skin folds, multiple ring-shaped ones, of the extremities. In: Orphanet (Rare Disease Database).
10. ↑ Symmetric Circumferential skin creases, congenital, 2.  In: Online Mendelian Inheritance in Man . (English)
11. ^ Richieri-Costa-Pereira syndrome. In: Orphanet (Rare Disease Database).
12. ^ Robin sequence with cleft mandible and limb anomalies.  In: Online Mendelian Inheritance in Man . (English)
13. ↑ Face, flat - microsomia - ear anomalies. In: Orphanet (Rare Disease Database).
14. ↑ Microphthalmia, syndromic 5.  In: Online Mendelian Inheritance in Man . (English)
15. ^ Ohdo syndrome, X-linked.  In: Online Mendelian Inheritance in Man . (English)

This article is about a health issue. It is not used for self-diagnosis and does not replace a diagnosis by a doctor. Please note the information on health issues !