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下肢尾部样先天性重复畸形(额外肢体或残留寄生性孪生体)

Tail-like Congenital Duplication of Lower Extremity (Extra Leg or Vestigial Parasitic Twin).

作者信息

Mekonnen Temesgen

机构信息

Department of Radiology and Imaging, Myungsung Christian General Teaching Hospital and Medical Collage, Addis Ababa, Ethiopia.

出版信息

Ethiop J Health Sci. 2018 Jan;28(1):103-107. doi: 10.4314/ejhs.v28i1.14.

Abstract

BACKGROUND

Congenital duplication of lower extremity, either complete or incomplete is extremely rare. Only 26 cases had been reported till 2010, of which only 5 cases had feature of complete duplication. Theories have been proposed that the cause of this abnormality includes maternal factors like diabetes and usage of teratogenic drugs. Duplication of lower extremity may mimic that of vestigial parasitic twin; phenotypically, it appears as an individual with extra appendage or limbs. Vestigial parasitic twis are very rare comprising only 10% of all conjoined twins). Conjoined twin by itself is rare because the survival rate after birth is approximately 25%.

CASE DETAILS

A 5-year old female patient came from a rural area of Ethiopia was referred to our hospital for radiological diagnosis and to assess the vascular supply. No family history of similar congenital abnormality was identified. No known history of chronic illness or maternal usage of teratogenic drugs was identified either.

CONCLUSION

A child with parasitic twin and complete duplication of lower limb is reported. Tail-like soft tissue was found arising from sacral area. CT scan findings confirmed the duplication of the lower limb bones. Fatty tissue was the major component of the soft tissue almost replacing the muscle. Reconstructed CT angiography images showed major arterial blood supply of the parasite limb. Surgical intervention was done successfully. This type of case is unusual. It is the rarest type of lower limb duplication.

摘要

背景

先天性下肢重复畸形,无论是完全性还是不完全性都极为罕见。截至2010年,仅报道过26例,其中仅有5例具有完全重复的特征。有理论认为,这种异常的病因包括母体因素,如糖尿病和使用致畸药物。下肢重复畸形可能类似退化寄生双胞胎;从表型上看,表现为个体有额外的附属物或肢体。退化寄生双胞胎非常罕见,仅占所有联体双胞胎的10%。联体双胞胎本身就很罕见,因为出生后的存活率约为25%。

病例详情

一名来自埃塞俄比亚农村地区的5岁女性患者被转诊至我院进行放射学诊断并评估血管供应情况。未发现类似先天性异常的家族史。也未发现已知的慢性疾病史或母体使用致畸药物的情况。

结论

报告了一名患有寄生双胞胎和下肢完全重复畸形的儿童。发现骶尾部有尾状软组织。CT扫描结果证实了下肢骨骼的重复。软组织的主要成分是脂肪组织,几乎取代了肌肉。重建的CT血管造影图像显示了寄生肢体的主要动脉血供。手术干预成功完成。这种类型的病例不常见。它是下肢重复畸形中最罕见的类型。

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