Cutis marmorata telangiectatica congenita is a rare congenital vascular disorder that usually manifests in affecting the blood vessels of the skin. The condition was first recognised and described in 1922 by Cato van Lohuizen, a Dutch pediatrician whose name was later adopted in the other common name used to describe the condition – Van Lohuizen Syndrome. CMTC is also used synonymously with congenital generalized phlebectasia, nevus vascularis reticularis, congenital phlebectasia, livedo telangiectatica, congenital livedo reticularis and Van Lohuizen syndrome.It should not be confused with the more general term "cutis marmorata", which refers to livedo reticularis caused by cold. Signs and symptoms
People with visible marks generally feel fine (physically) and can act normally, but when it is mentioned, they may become withdrawn and self-conscious. Some children may have low self-esteem due to the condition.CMTC is an uncommon, sporadic congenital vascular malformation characterized by a generalized or localized reticulated cutaneous vascular network.Cutaneous lesions described in patients with CMTC include nevus flammeus, hemangioma, nevus anemicus, café-au-lait spots, melanocytic nevus, aplasia cutis and acral cyanosis.It has a marbled bluish to deep-purple appearance. The dark skin lesions often show a palpable loss of dermal substance. The reticulated mottling frequently appears more prominent in a cold environment (physiologic cutis marmorata), but tends not to disappear with warming. Hence, the erythema may be worsened by cooling, physical activity, or crying.CMTC frequently involves the extremities, with the lower extremities involved most commonly, followed by the upper extremities, and then the trunk and face.
Chorioangioma, or chorangioma, is a benign tumor of placenta. It is a hamartoma-like growth in the placenta consisting of blood vessels, and is seen in approximately 0.5 to 1% pregnancies. It is mostly diagnosed ultrasonically in the second trimester of pregnancy. Large chorioangiomas are known to cause complications in pregnancy, while the smaller ones are asymptomatic. Presentation
Most chorangiomas are not clinically significant, i.e. they do not have an adverse effect on placental function. Complications
Large (greater than 4 or 5 cm.) or multiple chorioangiomas may lead to complication. The complications are polyhydramnios, preterm labour, hemolytic anemia, fetal cardiomegaly, fetal thrombocytopenia, intrauterine growth retardation, preeclampsia, abruption of placenta and congenital anomalies. Pathogenesis
The origin of chorioangioma is from primitive chorionic mesenchyme. It develops when the blood vessels and stroma undergo rapid proliferation independent of the surrounding tissue. Based on histological features, chorioangioma is classified by Marchetti into three types:
Cellular type : This type is immature and contains mostly cellular elements packed compactly. Angiomatous (vascular) type : This is the most common type of choriocarcinoma. It is distinguished by the presence of numerous small blood vessels. Degenerative type : This is the mature type with degenerative changes.Each type is believed to represent a phase of tumor development. Chorioangioma has no malignant potential. Diagnosis
Most chorioangiomas are asymptomatic. They are generally picked up in second trimester scan. Chorioangioma is seen as a hypo- or hyperechoic circumscribed mass that is distinct from the placenta at gray-scale US examination. Large lesions may contain fibrous septa.