Amniotic band syndrome

Classification according to ICD-10
Q79.8	Other congenital malformations of the musculoskeletal system - Amniotic lacing
ICD-10 online (WHO version 2019)

Amniotic ligament syndrome (ABS) are congenital (more precisely: congenital ) birth defects ( malformations ) that arise mechanically during pregnancy when strongly adhesive fibrous ligaments (tying rings) parts of the body (arms, legs, toes or fingers, i.e. dysmelia) ) of the unborn child.

Amniotic ligament syndrome in a newborn after removal of the amniotic ligaments. Tibial and fibular bones, muscles and nerves are completely severed.

Synonyms are: amniotic lacing or lacing rings ; Amniotic band syndrome ; English amniotic deformity adhesions mutilations; ADAM ; Streeter Bands ; Streeter's Syndrome ; Streeter dysplasia

The uncommon name refers to a theory on cause proposed by G. Streeter in 1930.

distribution

The frequency is given as 1 in 10,000–15,000.

causes

The ligaments are caused by tearing the inner membrane of the egg , the amnion , during pregnancy, the causes are difficult to clarify. The following causes are discussed for a somewhat more frequent probability of occurrence:

Metabolic diseases in the mother (e.g. diabetes mellitus )

Exposure to teratogenic factors during pregnancy, e.g. B. X-ray exposure or taking medication (Note: the thalidomide active ingredient thalidomide does not work mechanically by constricting limbs, but by inhibiting the growth factor, so that other typical malformations arise during the growth of limbs, see phocomelia )

inherited susceptibility to the formation of amniotic ligaments

mechanical impact (accidents during pregnancy, attempted abortion, injuries to the unborn child from the needle during amniocentesis )

Symptoms

Body parts (arm, leg, fingers, toes, etc.) are wrapped around by the cord-like, strongly adhesive, fibrous ligaments and grow together (e.g. syndactyly ), are hindered in growth (e.g. hypoplasia , club feet or other forms of dysmelia ) or are completely severed (congenital amputation, e.g. aplasia ). The ligaments can also cause deformities such as cleft lip or jaw dysplasia on the face, open back or abdomen , as well as lighter forms such as distal lymphedema .

diagnosis

The ligaments can be diagnosed on ultrasound . They are rarely visible as constrictions even after birth. Sometimes a pinched limb has to be amputated after birth.

treatment

Treatment options vary from case to case. They consist in the surgical correction of malformations (e.g. separation of fused fingers and toes) or malpositions (e.g. clubfeet ) after the birth, if necessary in the use of aids such as orthoses , prostheses , in physiotherapy and / or psychological Consultation.

In specialized centers, it is also possible to cut the amniotic ligaments by fetal surgery before birth, thus avoiding further impairment of the affected limbs.

Classification

ICD-9 : 658.8
ICD-10 : Q65-Q79.8

literature

PG Gabos: Modified technique for the surgical treatment of congenital constriction bands of the arms and legs of infants and children. Orthopedics. 2006 May; 29 (5): 401-4 (English)
TR Light, JA Ogden: Congenital constriction band syndrome. Pathophysiology and Treatment. Yale J Biol Med. 1993 May-Jun; 66 (3): 143-155 (English)
JH Walter Jr, LR Goss, AT Lazzara: Amniotic band syndrome. J Foot Ankle Surg. 1998 Jul-Aug; 37 (4): 325-333 (English)

Web links

Amniotic ligament syndrome
ABS homepage
emedicine
Rare Diseases
Constricting Bands, Congenital. In: Online Mendelian Inheritance in Man . (English)
medlineplus

Individual evidence

↑ G. Streeter: Focal deficiencies in fetal tissues and their relation to intrauterine amputations. In: Contributions to Embryology Carnegie Institution , Vol. 22, 1930, pp. 1-46
↑ Podiatry Institute
↑ K. Kawamura, KC Chung: Constriction band syndrome. In: Hand Clinics. Volume 25, number 2, May 2009, pp. 257-264, doi : 10.1016 / j.hcl.2008.10.007 , PMID 19380064 (review).
↑ A. Strauss: Amniotic band syndrome. In: IM Heer, S. Müller-Egloff, A. Burges (Ed.): Ultrasound practice . 2004, p. 73, doi: 10.1007 / 978-3-662-10678-5_19
^ University Hospital Mannheim: Surgical treatment of complex malformations (hand and foot surgery). Retrieved August 8, 2018 .
↑ German Center for Fetal Surgery & Minimally Invasive Therapy at the University Hospital Mannheim: Prenatal treatment of the amniotic band syndrome. Retrieved August 8, 2018 .

[1] G. Streeter: Focal deficiencies in fetal tissues and their relation to intrauterine amputations. In: Contributions to Embryology Carnegie Institution , Vol. 22, 1930, pp. 1-46

[2] Podiatry Institute

[3] K. Kawamura, KC Chung: Constriction band syndrome. In: Hand Clinics. Volume 25, number 2, May 2009, pp. 257-264, doi : 10.1016 / j.hcl.2008.10.007 , PMID 19380064 (review).

[4] A. Strauss: Amniotic band syndrome. In: IM Heer, S. Müller-Egloff, A. Burges (Ed.): Ultrasound practice . 2004, p. 73, doi: 10.1007 / 978-3-662-10678-5_19

[5] University Hospital Mannheim: Surgical treatment of complex malformations (hand and foot surgery). Retrieved August 8, 2018 .

[6] German Center for Fetal Surgery & Minimally Invasive Therapy at the University Hospital Mannheim: Prenatal treatment of the amniotic band syndrome. Retrieved August 8, 2018 .