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2015-03-20-22 Answer to the case of the week #392 © Bronshtein www.TheFetus.net 

Answer to the case of the week #392

April 09 - April 23, 2015

Moshe Bronshtein, MD1; Kidron Dvora, MD2.

1. Haifa, Rambam, Israel. 2. Head of the department of pathology, Meir Hospital Kfar Saba.

 

Case report

A 33-year old secundigravida was examined in our office at 14 and 23 weeks of pregnancy. Her first child was born with buried penis and pilonidal cyst (both findings were diagnosed at 15 weeks of the pregnancy). Her family history was unremarkable.

Ultrasonographic examination at 14 weeks did not reveal any anomalies and the spine of the fetus looked normal. At 23 weeks the ultrasonographic examination showed a cysts structure arising from the skin above the lumbar region of the spine.

Our ultrasonographic diagnosis was "dermal sinus" with good prognosis. The patient was also scanned by Dr. Goral from France and her diagnosis was "limited dorsal myeloschisis".

MRI examination of the structure was done and the images were evaluated and concluded by three neurosurgeons from three different institutes. Their conclusion was "spinal cyst" as a variant of open neural tube defect with more than 30% probability of severe handicap. 

The parents opted for the termination of the pregnancy at 31 weeks. Pathological examination confirmed the diagnosis of the dermal sinus or limited dorsal myeloschisis

In the literature there is overlapping between the two names of this anomaly [1, 2] and the only possibility to distinguish these two entities is histological examination.

Images 1, 2 and videos 1, 2: 14 weeks of gestation - normal appearance of the spine.

 

 

Images 3, 4, and videos 3, 4, 5, 6, and 7: 23 weeks - images and videos show ultrasonographic sagittal and transverse scans of the
cystic structure arising from the lumbar region of the spine and representing dermal sinus (or limited dorsal myeloschisis). The clue to the diagnosis is the presence of the stalk between the cystic structure and spinal cord (green arrows on the videos 5, 6) and tethered spinal cord that can be seen on the video 7.

 

 

 



Images 5-9: MRI images - showing normal intracranial morphology and spine with a small cystic structure arising from the lumbar region of the spine and representing dermal sinus or limited dorsal myeloschisis.

 







Images 10, 11, 12, 13, 14, and 15: Post termination images of the fetus - pathological specimens showing the dermal sinus (or limited dorsal myeloschisis) of the fetus. A stalk (white arrow) between the cystic structure and spinal cord can be seen on the images 12-15.

 

 


 

Histology of the findings:








References

1. Pang D, Zovickian J, Oviedo A, Moes GS. Limited dorsal myeloschisis: a distinctive clinicopathological entity. Neurosurgery. 2010 Dec;67(6):1555-79; discussion 1579-80. doi: 10.1227/NEU.0b013e3181f93e5a. PubMed PMID: 21107187.
2. Neil Hamill, John A. Grant, and Stephen A. Myers. Congenital Dermal Sinus. JUM May 2008 27:799-802
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