التفاصيل البيبلوغرافية
| العنوان: |
In utero Diagnosis of Spinal Dermal Sinus. |
| المؤلفون: |
Apte, Anisha, Fauser, Tobias, Carson, Quinlan, Liechty, Kenneth W., Simpson, Lauren N., Avellino, Anthony M. |
| المصدر: |
Fetal Diagnosis & Therapy; 2024, Vol. 51 Issue 3, p235-242, 8p |
| مصطلحات موضوعية: |
FETAL MRI, NEURAL tube defects, FETAL ultrasonic imaging, NEURAL tube, SPINA bifida |
| مستخلص: |
Introduction: Congenital dermal sinus (CDS) is an open neural tube defect (NTD) that occurs in 1 in 2,500 births a year and often goes undetected until patients present with complications like infection and neurological deficits. Early diagnosis and repair of CDS may prevent formation of these complications. In utero diagnosis of these lesions may improve long-term outcomes by enabling referral to specialty services and planned postnatal repair; however, only 2 such cases have been reported in the literature. We present a third case of in utero diagnosis of CDS with a description and discussion of findings from surgical exploration and pathology. Case Presentation: Routine prenatal ultrasound scan detected a tethered cystic structure arising from the back of the fetus at 20 weeks of gestation. Dedicated fetal ultrasound confirmed the presence of a cystic lesion protruding through a lamina defect, while fetal magnetic resonance imaging showed an intact spinal cord and meninges, suggesting a diagnosis of CDS. Neurosurgery followed along closely and took the child for surgical exploration on day 2 of life. A fibrous stalk with an intradural component and associated cord tethering was excised. Histology showed fibrous tissue without an epithelial-lined lumen. Conclusion: CDS is a form of NTD that occurs from nondisjunction of the cutaneous ectoderm and neuroectoderm during formation of the neural tube. Slight differences in how this error occurs can explain variations seen in this spectrum of disease, including CDS without an epithelial-lined lumen as seen in this case. Newborns with CDS can go undiagnosed for years and present with long-term complications. Fetal imaging can assist in early recognition and surgical excision of CDS in newborns. Established Facts: Congenital dermal sinuses are rare forms of spinal dysraphism with multiple different manifestations that are difficult to diagnose in utero. Unrecognized, congenital dermal sinuses can lead to devastating spinal cord infections and long-term neurological deficits from intradural lesions and cord tethering. Congenital dermal sinuses are thought to occur from an error in primary neurulation during the separation of the cutaneous ectoderm and neuroectoderm in a process called disjunction. [ABSTRACT FROM AUTHOR] |
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| قاعدة البيانات: |
Complementary Index |
