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【读片】产科疑难超声病例（329）：蝴蝶椎

zhangming123456

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猜一个难道是连体双胎

2012-10-27 08:51

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ydh841215

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猜个，貌似没看到棘突，等待拍砖。。。

2012-10-27 09:03

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xihuansushi

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34-year-old patient (G1P0) without remarkable history. Nuchal transluency, triple test , karyotype was normal.
Final diagnosis: Butterfly veterbra
本例最终诊断为蝴蝶椎。

Image and video 1-3: Arrow showing the butterfly vertebra
箭头所指为蝴蝶椎

Image 4, 5: Butterfly vertebra
蝴蝶椎

Image 6-8: Xray scan; arrows showing the abnormal vertebra.
产后X线所示

2012-10-27 09:15

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xihuansushi 编辑于 2012-10-27 09:21

• 教育部：今年国家定向培养免费医学生，你会报考吗？——回帖

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xihuansushi

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Synonyms: Sagittal cleft, midline vertical cleft

Definition: “Butterfly vertebra” is an uncommon congenital anomaly of the spine. It results from the failure of fusion of the lateral halves of the vertebral body because of persistent notochondal tissue between them forming a cleft in the center.^[^1][2]

On frontal X-ray, the two half-vertebrae resemble the wings of a butterfly wings while the processus spinosus represents the butterfly body.

History: In an early study of the vertebral column in the South African Bantu, Shore (1930) noted an unusual fifth thoracic vertebra in a 40-year-old Basutu male. He described the vertebral body as wedged-shaped, its anterior height reduced by approximately 6.5 mm (40%), and he felt that is clearly represented a “developmental phenomenon”. He also noted that this apparent expression of sagittal clefting was the only one observed in 1,936 Bantu vertebrae studied. An example of sagittal clefting was reported for the skeleton of a middle-aged adult recovered from Krasnojarski Kray near the village of Podgornoe in eastern Siberia. The site is dated from the sixth to fifth centuries BC. Because its appearance resembles the wings of the butterfly, so it is called butterfly vertebrae.^[3]

Prevalence: The prevalence of butterfly vertebra in the general population is uncommon but poorly known with less than 100 case reports, yet some dedicated studies have found numbers as high as 1:5000 .^[3],[4],[5]

Etiology: unknown

Pathogenesis: It results from separate bilateral ossification center that fail to unite ^[1]which should happen around the seventh week of gestational age and end at the 25th week^[6]. Abnormal ossification of the vertebral body happens centrally, probably after incomplete fusion of two cartilaginous centers with hypoplasia at the junctional site. Butterfly vertebrae can result from a developmental abnormality leading to regression of the cauda dorsalis and formation of sagittal clefts in the vertebra into right and left halves. There are usually no symptoms. Butterfly vertebrae of normal height do not necessarily result in deviation of the spinal column axis. These most common occur in the thoracic region, but occasionally will be found in the lumber spine.^[1]

Sonographic findings: In frontal plane, we see a complete or incomplete cleft in the center of the vertebral body. Postnatally however, it is easier to confirm diagnosis by MRI.

Differential diagnosis:

? Other vertebral anomalies :

Spina bifida (posteriorly)

Block vertebra (fused vertebrae)

Hemivertebra (failure of development of one side (front/back or lateral of the vertebra)

? Other rare syndromes:^[7]

Vertical vertebral body fracture (in adult: seatbelt injury, axial loading)

Spondylothoracic dysplasia (Jacho-Levin syndrome): complex vertebral anomalies with hemi-vertebrae, rib anomalies, and absent vertebral body.

Alagille syndrome (arteriohepatic dysplasia): single or multiple butterfly vertebrae. Rickets due to hepatic disease (postnatal diagnosis).

Aicardi syndrome: butterfly vertebra and other vertebral anomalies, restricted to girls, failure to thrive, “salaam” spasms, hemihypertrophy , partly or completely missing the structure that connects the two sides of the brain (corpus callosum)

Associated anomalies: Butterfly vertebral may be associated with other congenital abnormalities such as anterior spina bifida (with or without anterior meningocele), cardiac abnormalities, forgut abnormalities and spinal diastematomyelia^[8][7], neurenteric cysts, anomalies of the lungs, gastrointestinal tract, or genitourinary tract ^[7]or a part of some rare syndromes: Pfeiffer, Jarcho-Levin, Crousen, Alagille^[9], Aicaidi syndrome ^[7], Goldenhar syndrome.^[^10]

This anomaly may occur in other syndromes and also in healthy individuals. It is generally not of structural significance.^[^11][5]12]

Prognosis: The prognosis is related to the presence or absence of associated anomalies. Good prgnosis with isolated butterfly veterbra^[13],[1],[5]

Management: An isolated butterfly vertebra does not require treatment.^[2][5] The intervention only needs for associated anomalies.

References:

Chris J. Hutcheson, Joseph W. Howe. The low back and pelvis: clinical applications, third volume 1997 ; chapter 3, page 7.
Martin Weyreuther, Christoph E. Heyde, Michael Westphal, Jan Zierski, Ulrich Webe: MRI atlas:orthopedics and neurosurgery : the spine, 2007, chapter 2, 12-16
Charles F. Merbs*, Sagittal Clefting of the Body and Other Vertebra. Developmental Errors in Canadian Inuit Skeletons. American Journal of physical anthropology 2004; 123:236-249
PaulCave: Butterfly Vertebra. British Journal of Radiology (1958) 31, 503-506
Suzanne B.Cassidy, Judith E.Allanson. Management of genetic syndromes. Third edition, 2010, chapter 4. 39-49
Keith L.Moore : The developing human:Clinally oriented embryology.Fourth edition. 1988. Chapter 15:334-348.
Klaus-Dietrich Ebel, Gabriele Benz-Bohm: Differential diagnosis in pediatric radiology, 1999, chapter 6, 616.
R Kumar, FC Guinto, Jr, JE Madewell, LE Swischuk, and R David. The Vertebral Body: Radiographic Configurations in Various Congenital and Acquired Disorders, RadioGraphics , 1988. 8: 455
Birkan Sonel Peyman Yal??n, Erhan Arif ?ztürk, I??k B?kesoy: Butterfly vertebra: A case report, Clinical Imaging Volume 25, Issue 3 2001, 206-208
YK Dastur, H Trivedi, N Tapaswi, N Shah. Godenhar's syndrome with unilateral duane retraction syndrome and "butterfly" vertebra ,1985,Volume 33 Issue : 3 ,P.187-189
Przkora R, Schmitz A, Perlick L, Koenig R, Jaeger U, Schmitt O. Butterfly vertebrae and scoliosis: Alagille syndrome.Z Orthop Ihre Grenzgeb 2002 Mar-Apr;140(2):182-4
Ian D Krantz, David A Piccoli, Nancy B Spinner. Alagille syndrome. J Med Genet. 1997; 34(2): 152–157
Wirginia J. Maixner,Spina Bifida. Management and Outcome 2008. Chapter 14: 186-189.

2012-10-27 09:24

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【读片】产科疑难超声病例（329）：蝴蝶椎

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