TY - JOUR
T1 - Acardius anceps with neck cyst and cleft palate: Three dimensional skeletal computed tomography reconstruction with discussion of the literature
AU - van Gemert, Martin J. C.
AU - Streekstra, Geert J.
AU - Vandenbussche, Frank P. H. A.
AU - Nikkels, Peter G. J.
AU - van den Wijngaard, Jeroen P. H. M.
PY - 2020/1/15
Y1 - 2020/1/15
N2 - Acardiac twinning is a rare anomaly of monochorionic twin pregnancies. Acardiac fetuses lack a functional heart but are passively perfused by arterial blood from their pump co-twin causing the acardiac body to be hypoxemic. In this report, we present an acardius anceps, therapeutically laser separated from its pump twin at 16 weeks. The healthy pump twin and macerated acardiac body were born at 40 3/7 weeks. A three dimensional (3D) reconstruction was made by CT images, showing cranial bones, spinal column, pelvis and lower extremities but absent arms. A cyst in the neck of the acardiac twin was identified by postnatal sonography; this was also described in four literature cases, and was additionally observed by us in two other acardiac twins. Median cleft palate was identified by oral cavity inspection but undetectable in the reconstruction. In the literature, we found 21 other acardiac anceps twins with a cleft palate. From the two larger published series, with 12 clefts in 21 acardiac anceps twins, a cleft palate occurs in over 50% during acardiac twinning. Our first hypothesis is that acardiac fetuses develop an oral cleft palate when acardiac onset starts prior to 11 weeks, because 11 weeks includes the period of embryonic oral cavity formation, and no cleft occurs when onset starts later than 11 weeks. Our second hypothesis is that cysts and cleft palates are more common in acardiac twins than currently known, likely reflecting that acardiac bodies are hypoxemic and that hypoxia contributes to the development of both cysts and clefts.
AB - Acardiac twinning is a rare anomaly of monochorionic twin pregnancies. Acardiac fetuses lack a functional heart but are passively perfused by arterial blood from their pump co-twin causing the acardiac body to be hypoxemic. In this report, we present an acardius anceps, therapeutically laser separated from its pump twin at 16 weeks. The healthy pump twin and macerated acardiac body were born at 40 3/7 weeks. A three dimensional (3D) reconstruction was made by CT images, showing cranial bones, spinal column, pelvis and lower extremities but absent arms. A cyst in the neck of the acardiac twin was identified by postnatal sonography; this was also described in four literature cases, and was additionally observed by us in two other acardiac twins. Median cleft palate was identified by oral cavity inspection but undetectable in the reconstruction. In the literature, we found 21 other acardiac anceps twins with a cleft palate. From the two larger published series, with 12 clefts in 21 acardiac anceps twins, a cleft palate occurs in over 50% during acardiac twinning. Our first hypothesis is that acardiac fetuses develop an oral cleft palate when acardiac onset starts prior to 11 weeks, because 11 weeks includes the period of embryonic oral cavity formation, and no cleft occurs when onset starts later than 11 weeks. Our second hypothesis is that cysts and cleft palates are more common in acardiac twins than currently known, likely reflecting that acardiac bodies are hypoxemic and that hypoxia contributes to the development of both cysts and clefts.
KW - TRAP sequence
KW - acardiac anceps
KW - neck cyst
KW - oral cleft palate
UR - http://www.scopus.com/inward/record.url?scp=85076422877&partnerID=8YFLogxK
U2 - https://doi.org/10.1002/bdr2.1625
DO - https://doi.org/10.1002/bdr2.1625
M3 - Article
C2 - 31774253
SN - 2472-1727
VL - 112
SP - 137
EP - 140
JO - Birth defects research
JF - Birth defects research
IS - 2
ER -