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Case report

Year :2013 Month : April Volume : 2 Issue : 1 Page : 13 - 15

Blue Rubber Bleb Nevus Syndrome - A Rare Cause of Anemia in Children


Ashok V. Badaka li, B.C. Yelamali, Meenakshi. A. Badakali
1. Professor in Pediatrics, 2. Professor in Pediatrics, 3. Associate Professor, in OBGS.N.Medical College, Bagalkot- 587 102, India.
 
Correspondence Address :
Ashok V. Badaka li, B.C. Yelamali, Meenakshi. A. Badakali,
Dr. Ashok V. Badakali, Professor, in Pediatrics B-6, Staff Qtrs., S.N.Medical College Campus, Navanagar, Bagalkot- 587 102 (Karnataka) India. Phone: 09880227403 E-mail: ashok_diya@yahoo.com
 
ABSTRACT

: Morphometry of body and neural arch of lumbar vertebrae is very crucial in manufacturing screws, interspinous implants as well as preoperative planning of surgeries involving dorsolumbar spine.

Aim: To determine various dimensions of typical and atypical lumbar vertebrae.

Materials and Methods: A descriptive osteological study was carried out which included 66 intact adult dry human lumbar vertebrae (53 typical and 13 atypical) which were free of any deformity or pathological features. All the 53 typical vertebrae were randomly obtained. The following parameters were measured with slide callipers- superior transverse diameter and superior antero-posterior diameters of vertebral foramen; transverse diameter, antero-posterior diameter and anterior height of vertebral body; width, height of pedicles; interpedicular distance; maximum thickness of lamina; length of transverse process; maximum length, maximum height and maximum central thickness of spinous process. The data was tabulated and analysed using Microsoft Excel software. Mean and standard deviation was calculated for each parameter. Unpaired t-test was applied and p-value was derived for parameters like width and height of pedicles, thickness of lamina and length of transverse process. The p-value<0.05 were considered as significant.

Results: The vertebral foramen (superior transverse diameter- 20.41±2.54 mm, superior antero-posterior diameter- 13.3±2.04 mm); vertebral body (transverse diameter- 44.43±5.91 mm, antero-posterior diameter- 30.17±3.19 mm, anterior height- 24.01±1.84 mm); pedicle (width- 10.85±3.94 mm on left side and 11.04±4.01 mm on right side, height- 13.84±4.01 mm on left side and 13.8±1.93 mm on right side, interpedicular distance- 29.17±5.06 mm); lamina (thickness- 6.6±1.36 mm on left side and 6.85±1.34 mm on right side); transverse process (length- 20.94±4.01 mm on left side and 21.51±4.5 mm on right side); spinous process (maximum length- 26.01±3.73 mm, maximum height- 19.92±4.03 mm, maximum central thickness- 6.42±1.41 mm). The mean transverse diameter and antero-posterior diameter of vertebral foramen of atypical lumbar vertebrae were higher than those of the typical lumbar vertebrae and these differences were significant (p-value of 0.0001 for transverse diameter and p-value of 0.005 for antero-posterior diameter).

Conclusion: Most of the parameters of atypical lumbar vertebrae were found to be more compared to those of typical lumbar vertebrae. This inference should be kept in mind during fixation of lumbar inter-spinous implants, designing of pedicular screws and spinal grafting.
Keywords : Blue rubber bleb nevus syndrome, GI bleeding, Venous malformations
 
INTRODUCTION

Blue Rubber Bleb Nevus Syndrome (BRBNS) is a rare entity consisting of distinctive venus malformation in the Skin, gastrointestinal tract & less often in other organs, leading to occult or profound GI bleeding & chronic anaemia. So far only 150 cases reported in the world literature (1). Most cases are sporadic but autosomal dominant inheritance has been reported. BRBNS was probably first observed by Gascoyen in 1860 (2). A century later, Bean coined the term blue rubber bleb nevus syndrome for this rare disorder. Diagnosis may not be readily apparent in the absence of sub cuteneous nodules (3). BRBNS is an important syndrome because of its potential for serious or fatal bleeding. Here in we are reporting a case of Blue rubber bleb nevus syndrome in a 10 year old boy. The case is being reported for its rarity and for adding to the existing literature.
 
REFERENCES
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Gupta N,Day AS.Blue rubber bleb nevus syndrome.J Pediatrics Child Health.2010 Jan;46(1-2):67-68.
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Gascoyen GG. Case of nevus involving the parotid gland and causing death; nevi of the viscera. Trans pathol SocLand. 1860;11;267.
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Bean WB. Blue rubber bleb nevi of the skin & gastro intestinal tract: Bean WB Vascular spiders & related lesions of the skin. Spring field: Charles Thomas publishers: 1958: 178-185.
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Mittal RR, Pureet, Blue rubber bleb nevus syndrome. Inaidan J. Dermatol Vernereol Leprol. 2001;67:41-42.
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Suma GN,Raviprakash SM,Rao D,Goel S. Blue rubber bleb nevus syndrome: Prominent oral finding,IJDVL.2010-Apl,76(2):168-71.
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Beck PL, Aspinall AI, Kilvert VM, Dort J. Blue rubber bleb nevus syndrome. Gastrointest Endosc. 2002:56:598-600.
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Daniela Dorbu, Ni Colac Seuchea. Blue Rubber Bleb Nevus syndrome : Case report. Roman J. of Gastroentrology .Sep 2004:13:237-240.
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Amita Krishnappa,Jayachandran Padmini. Blue rubber bleb nevus syndrome: Indian J Pathology &Microbiology.2010:53(1):168-170.
 
TABLES AND FIGURES
[Table / Fig - 1]   [Table / Fig - 2]   [Table / Fig - 3]   [Table / Fig - 4]   [Table / Fig - 5]   [Table / Fig - 6]   [Table / Fig - 7]   [Table / Fig - 8]   [Table / Fig - 9]
 
 
 

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