PubMed · 5769229
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F Op de Beeck, W Gryspeerdt, L Laridon. 1969. [Acardius monster].. https://pubmed.ncbi.nlm.nih.gov/5769229/
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Here we report on an apparently unprecedented case of virtually complete absence of the left half of the body, with resulting severe deformity of the right half. The fetus was stillborn with polyhydramnios at 31 1/2 weeks of gestational age. A second patient may be a mild example of this extraordinary phenomenon with deficiency of organs, predominantly on the left side. The nosology and developmental implications are discussed briefly.
BACKGROUND: The twin reversed-arterial-perfusion sequence is a complication of monochorionic twin pregnancies characterized by the hemodynamic dependence of a "recipient" twin from a "pump" twin. The recipient twin exhibits lethal abnormalities including acardia and acephaly. The pump twin has a mortality rate of 50% as a result of high-output heart failure. CASE: The blood supply to an acardiac-acephalus twin was interrupted at 24 weeks' gestation using endoscopic laser coagulation. The co-twin was delivered at 35 weeks and had an uneventful neonatal course following correction of a persistent patent ductus arteriosus. Review of the literature reveals 22 cases of acardiac twinning treated with invasive procedures, seven of them using endoscopic laser coagulation. Pump twin mortality with fetal surgery was 13.6% in comparison with 50% mortality with expectant management (P < .001). CONCLUSION: Fetal surgery is the best available treatment for acardiac twinning. Endoscopic laser coagulation at or before 24 weeks and endoscopic or sonographic guided umbilical cord ligation after this gestational age seem to be the best treatments for this condition.
Six fetuses with normal chromosomes were found to have severe craniofacial, limb, and visceral malformations during the second trimester of pregnancy. Two of these fetuses were monozygotic twins while a third one had a healthy dizygotic twin brother. A case with familial recurrence was also observed. Autopsy and skeletal radiographs suggested several diagnoses such as neural tube defect with limb defects or XK aprosencephaly. The development of these severe conditions in monozygotic twins and familial recurrence emphasize the difficulties of genetic counseling in such situations. These cases may suggest autosomal recessive inheritance.