Aplasia Cutis Congenita of the Trunk with Fetus Papyraceus

Kelly, Benjamin J.; Samolitis, J B S Nancy; Xie, Don-Ling; Skidmore, Robert A.

doi:10.1046/j.1525-1470.2002.00093.x

Cited by 36 publications

(29 citation statements)

References 18 publications

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“…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 4,12 . In our patient, the concomitant osseous defect was a secondary challenge.…”

Section: Discussionmentioning

confidence: 77%

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

et al. 2011

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Section: Discussionmentioning

confidence: 77%

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

et al. 2011

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“…3 A total of 10% of all non-scalp variants of ACC are associated with fetus papyraceus. 4 This cutaneous type is usually linked with the death of a second fetus in utero, which results in the delivery of a fetus papyraceus. 5 The vascular theory is upheld as a likely etiology for type V ACC.…”

Section: Discussionmentioning

confidence: 99%

Prenatal diagnosis of aplasia cutis congenita of the trunk

Liu

Chen

et al. 2012

Int J Dermatology

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Aplasia cutis congenita (ACC) is a rare malformation characterized by the localized absence of skin, which may extend through the underlying tissues to the musculature and bone.1 Aplasia cutis congenita associated with fetus papyraceus is classified as type V ACC. 2 We present a case of type V ACC with extensive truncal lesions. Ultrasonic examination has become a conventional method in prenatal diagnosis. Many congenital malformations can be detected by ultrasound (US). Abnormalities in the structure of the abdominal wall can be detected in prenatal US examination. In the normal fetus, the abdominal skin surrounding the trunk generates strong echoes on US. In the present case, strong echoes were unobtainable for part of the abdomen. Case reportA 28-year-old woman (gravida 3, para 1, abortus 1) presented for US examination at 13 and 32 weeks of gestation. At 13 weeks of gestation, US showed a twin pregnancy with one death. At 32 weeks, US examination displayed only one fetus. The surviving fetus was smaller than expected for the post-menstrual period. Measurements calculated in US indicated a fetus at 28 weeks of gestation. When we examined the trunk, sagittal and cross-sectional US scans demonstrated that the abdominal skin was absent. In a normal fetus, the skin surrounding the trunk generates strong echoes. In the present case, strong echoes were absent for some of the abdominal circumference. The liver appeared to be exposed. No strong echo was generated between the liver and amniotic fluid. We designated this US characteristic as indicating absence of skin (Fig. 1). The positions and shapes of intra-abdominal organs were normal. No other structural abnormality was found. We excluded gastroschisis, omphalocele and Prune belly syndrome. Ultrasonic diagnosis indicated that: (i) fetal intrauterine growth was retarded, and (ii) fetal abdominal skin was absent.The patient was delivered vaginally at term with a macroscopically normal placenta. Apgar scores were 10 and 10 at one minute and five minutes, respectively. The infant was male and weighed 1850 g. A 95 9 45 9 10-mm fetus papyraceus was also delivered.

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“…Several theories have been suggested in an attempt to explain the cause 4 . The vascular theory suggests that there is vascular insufficiency to the skin, perhaps from placental compromise or a thromboplastic material 5,6 .…”

Section: Discussionmentioning

confidence: 99%

A Case of Aplasia Cutis Congenita, Type VII

et al. 2008

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Aplasia cutis congenita (ACC) is a rare congenital defect in which localized or widespread areas of the skin are absent at birth. In the majority of cases, it is limited to the scalp especially on the vertex although other areas of the body may also be involved. Other congenital malformations can be associated with ACC. We present herein the case of a new born male with unilateral absence of skin on the extensor surface of the right lower leg. There was no associated malformation or skin disease such as blistering or nail abnormailty. According to the classification outlined by Frieden, the condition was diagnosed as type VII aplasia cutis congenita. The treatment of this large ulcer was conservative, wet dressing and prophylactic topical antibiotics. On follow up after 2 years showed that the patient was nearly cured of the ulcer and had only minimal scar formation.

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Aplasia Cutis Congenita of the Trunk with Fetus Papyraceus

Cited by 36 publications

References 18 publications

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

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

Prenatal diagnosis of aplasia cutis congenita of the trunk

A Case of Aplasia Cutis Congenita, Type VII

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