Aplasia cutis in association with a triplet pregnancy and fetus papyraceus

Aplasia cutis congenita of the trunk and scalp associated with fetus papyraceus

“…Most of the described cases of type V ACC present solely with symmetric areas of cutaneous skin defects involving the trunk. This case of the additional implication of the scalp represents a rare variant of type V ACC among published cases of the literature 1–11 …”

“…This case of the additional implication of the scalp represents a rare variant of type V ACC among published cases of the literature. [1][2][3][4][5][6][7][8][9][10][11] Treatment of ACC affecting the scalp is controversial with regard to aggressive and conservative management. Some authors recommend skin grafting in face of large defects considering the risk for sagittal sinus thrombosis and cerebral hemorrhage.…”

Fetus Papyraceus: Congenital Pulmonary Anomalies Associated with Congenital Aplasia Cutis on the Surviving Twin

“…Placental vascular anastomosis is almost always present in monochorionic and rarely in dichorionic twin gestations. Three main hypotheses have been suggested : the death of one twin in utero allows thrombogenic material to pass from the dead to the living fetus through placental vascular anastomosis, which may cause ischemia of the developing skin and rarely of internal organs; rapid exsanguination of the surviving twin into the relaxed circulation of the dying twin could induce acute hypovolemia of the living twin and thus ischemia of skin and organs; and perturbations in fetofetal transfusion could be the cause of ischemia of both twins, with only one able to survive, with aplasia areas . In our case, all three theories could explain the ischemia of the developing skin and lungs responsible for bilateral congenital skin and lung aplasia.…”

The Vanishing Twin Syndrome

“…16,17 The association between fetus papyraceus and aplasia cutis congenita, as described by Maccario et al, has been documented through many case reports and reviews. [18][19][20][21][22][23][24][25] Aplasia cutis congenita in the context of co-twin demise with fetus papyraceus most often has a well-described distribution of symmetrical truncal, buttock, and leg lesions, with occasional descriptions of scalp defects as well. 20,25 While the etiology of this association is to be discovered, vascular compromise likely underlies these conditions.…”