Craniofacial Anomalies of the Amniotic Band Syndrome in Serial Clinical Cases

Abstract

Amniotic band syndrome has been proposed as a sequela of intrauterine rupture of the amnion, resulting in oligohydramnios and passage of the fetus into the chorionic cavity. Amnion disruption with loss of amniotic fluid, causing fetal compression and localized fetal ischemia, possibly results in a pathogenic mechanism of extremely variable malformations. The prominence of the nasal processes and the adjacent stomodeal orifice facilitates free band attachment and adherence, resulting in a spectrum of similarly oriented facial defects. The authors present six consecutive cases of amniotic band syndrome with cleft lip and palate (facial cleft 3, 5, 7, and 10, isolated or combined) that were associated with other craniofacial anomalies, such as craniosynostosis and hypertelorbitism. They also present limb malformations and discuss the proposed pathogenesis and the surgical challenges in functional and aesthetic restoration.

Revisiting Amniotic Band Sequence: A Wide Spectrum of Manifestations

“…Although the majority of anomalies in our series suggested late compromise, in 3 cases (33%) there was craniofacial involvement suggesting an earlier insult and worst prognosis. This is in good agreement with other series that found that in up to one third of the cases engagement of craniofacial region can preferentially occur [23]. …”

“…The degree of deformity depends on the severity and location of the band formation and the gestational age at which the amniotic rupture occurs. Early amniotic rupture, within the first 45 days of gestation, leads to the most severe defects, including craniofacial and visceral defects [4], whereas when it occurs after 12 weeks of gestation it tends to cause constriction rings and limb amputations [22,23]. In our series, the structural abnormalities have been found most frequently at the level of the upper extremities, what is consistent with the literature [24].…”

The Anaesthesiologist and Palliative Care in a Newborn with the Adam “Sequence”

“…The ADAM sequence occurs in 1 in 1,200–15,000 in live births [1, 5, 6] and 1 : 70 in stillbirths [7]. It affects both genders in the same proportion.…”

A Case of Amniotic Band Syndrome with Cleft Lip and Palate

“…Forty-nine cases (27 males and 22 females) of oro-facial anomalies associated with amniotic band syndrome were reported in the literature over the past 20 years [1][2][3][4][5][6][7][8][9][10][11][12][13][14][15]. These cases included 37 cases of facial cleft and 9 cases of cleft lip and palate.…”

“…This syndrome is a sporadic condition and is reported to occur in approximately 1 in 1,200-15,000 births [1][2][3][4][5]. The patient may present with craniofacial, thoracic, abdominal, and/or limb involvement.…”