The skin that covers this cartilage is extremely thin with virtually no subcutaneous fat while also strongly attached to the perichondrium, which is richly vascularized to supply the avascular cartilage.Cauliflower ear can also present in the setting of nontraumatic inflammatory injury of auricular connective tissue such as in relapsing polychondritis (RP), a rare rheumatologic disorder in which recurrent episodes of inflammation result in destruction of cartilage of the ears and nose. Joints, eyes, audiovestibular system, cardiovascular system, and respiratory tract can also be involved. Mechanism
The components of the ear involved in cauliflower ear are the outer skin, the perichondrium, and the cartilage. The outer ear skin is tightly adherent to the perichondrium because there is almost no subcutaneous fat on the anterior of the ear. This leaves the perichondrium relatively exposed to damage from direct trauma and shear forces, created by a force pushing across the ear like a punch, and increasing the risk of hematoma formation. In an auricular hematoma, blood accumulates between the perichondrium and cartilage. The hematoma mechanically obstructs blood flow from the perichondrium to the avascular cartilage. This lack of perfusion puts the cartilage at risk for becoming necrotic and/or infected. If left untreated, disorganized fibrosis and cartilage formation will occur around the aforementioned cartilaginous components.Consequently, the concave pinna fills with disorganized connective tissue. The cartilage then deforms and kinks, resulting in the distinctive appearance somewhat resembling a cauliflower.
Rachischisis can develop in conjunction with anencephaly, increasing the amount of area that is exposed. Although these conditions closely resemble one another, cases of rachischisis are morphologically different from cases of anencephaly, so they are indeed two separate conditions. Rachischisis is also a contributing factor to the onset of iniencephaly – a condition in which the spine is malformed. Further abnormalities such as rib malformations can occur with rachischisis. Stomach deformities can also develop with rachischisis. Risk factors
If a sibling of the fetus has been affected by rachischisis, there is increased risk that another child will develop the condition. There is also a slightly higher chance that females will develop the condition over males.Folate deficiency is also a well-accepted risk factor of all neural tube defects, including rachischisis. It is this reason that has caused many health policies regarding folic acid during pregnancy to be developed. Diagnosis
Initial diagnosis is typically by ultrasonography to reveal any congenital abnormalities. Fetuses affected typically show cranial abnormalities and deformities in brain tissue as well as spinal abnormalities. Following ultrasonography, magnetic resonance imaging (MRI) is commonly used to confirm the diagnosis. The presence of rachischisis is indicated in imaging by the absence of an arch-cranial line.Other neural tube defect diagnostic tools such as assays of α-fetoprotein or acetylcholinesterase may also be helpful in determining any other conditions that can lead to development of rachischisis. Classification
Rachischisis occurs most commonly in utero during the development of the child.