缩窄环综合征

J. Dhandapani, Venkateshvaran Ponnusamy
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摘要

先天性缩环综合征(CRS)包括在各种合并中发生的集体异常,通常损害下肢(肢体),几乎不损害头部和躯干。这种综合征是一种遗传性肢体畸形。这些异常具有多种诊断表现,可以通过显示身体任何部位的部分到完全、遗传性、纤维性、环状、收缩带或环来描述,即使在上肢和下肢都可以看到特别的偏爱。CRS发病率从每1500 - 15000例活产1例不等。80%的病例发生在手指和上肢。90%以上发生在腕区远端。家族性比例相对较低,并且认为这种疾病没有先天的非自愿成分。超声检查可对CRS的诊断措施进行验证。临床表现可能完全不同,也可能由一种或多种表现引起。这可以在妊娠前三个月结束或妊娠中期开始时进行验证。这种治疗通常在出生后进行,整形和重建手术被认为可以治疗由此产生的变形。根据畸形程度将整形手术分为简单到复杂。物理和职业治疗可能需要很长一段时间。羊膜带综合征被认为是一种意外事件,它似乎不是遗传或遗传的,所以它在另一次怀孕中发生的可能性很小。羊膜撕裂的原因尚不清楚,因此也没有已知的预防措施。先天性CRS病因不明,可导致新生儿发病。该综合征及其并发症可通过矫正手术控制,预后良好。早期干预有利于取得成功的结果。
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Constriction Ring Syndrome
The inborn constriction ring syndrome (CRS) encloses collective anomalies that develop in various amalgamation, typically damaging the lower extremities (limbs) and hardly the head as well as trunk. This syndrome has an inherited limb deformities. These anomalies with a variety of diagnostic presentations were described by showing the partial to complete, inherit, fibrous, circumferential, constriction bands or rings on any parts of the body, even though a particular predilection for both upper and lower extremities is visible. The CRS incidence differs from one case in 1500–15,000 live births. Eighty percent of cases happen in digits and upper extremities. More than 90% presents in the region of distal to wrist area. The familial ratio is relatively low, and it is thought that there is no inborn involuntary component to this disease. The diagnostic measures regarding the CRS can be authenticated with the help of ultrasonography. The clinical features may be totally different, and also, it could be due to one or more manifestations. This can be validated at the end of the first trimester or at the beginning of the second trimester. The treatment commonly follows after birth and where plastic and reconstructive surgery is believed to treat the resulting deformation. Plastic surgery was classified from simple to complex based on the extent of the deformity. Physical and occupational therapy may be necessary for a prolonged period. Amniotic band syndrome is considered an accidental event, and it does not appear to be genetic or hereditary, so the likelihood of it occurring in another pregnancy is distant. The reason for amnion tearing is not known, and as such, there are no known prophylactic measures. Congenital CRS is of unknown etiology and can lead to morbidity in the newborn. The syndrome and its complications are controllable with corrective surgery with good outcomes. Early intervention is agreeable for successful results.
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