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Congenital Abnormalities

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Constrictive Bands - Pectoral Deficiency » General Q79.8

Definition:

Constrictive Bands:
Defects by constricting bands.
Misnamed amniotic adherences since the amniotic pathogenesis is uncertain.
The defects are embryo-fetal scars from constriction and their mechanical and vascular consequences.
Pectoral Deficiency
Hypoplasia of the pectoralis major.
The anterior border of the axilae is not clearly defined.
Notes:
Constrictive Bands
The defects due to constriction bands are in general embryological absurds, because they are product of deformation and/or disruption and not of malformations. For example fenestrated syndactyly cannot be explained by simple detention of the digital rays separation from the primitive hand, which is continuous from tip to base of the fingers. Another example are facial clefts in different location than median cleft lip or paramedian cleft lip, in the areas of fusion of the first branchial arch, or its upper branches with or without absence of filtrum (frontonasal prominence), respectively.
Pectoral Deficiency
Half of cases with pectoral deficiency are associated with upper limb defects and / or nipple agenesis, always on the same side, constituting the Poland syndrome [Q79.8].
Images:
Image 1
Multiple disruptive anomalies, with or without visible amniotic bands.
1: Huge cephalocele through left orbit, atypical cleft (Tessier cleft number 4-10??)
2. Multiple limb amputations and constriction rings, cephalocele with upper face disruption, parietal cephalocele.
3. 4. Limb constriction rings.
5: Constriction ring on distal leg and amputations at toe level
6: Fenestrated syndactyly
7: Constriction rings and distal amputations
8: Amputation at finger level (band circling the stumps)
9: Band extending from right nostril to parietal region, widely spaced micropthalmic eyes, bifid nose (left side hypoplastic).
Image 11
Photos 1, 2, 3.
Three cases with right pectoralis muscle hypoplasia.
2. Asymmetric thorax, absent right nipple and rib anomaly.
Image 15
Poland syndrome on the left side, at birth (Photo 1), and at one year (Photo 2).

Notes:

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Images