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Year : 2006  |  Volume : 16  |  Issue : 4  |  Page : 587-588
Case presentation:Langer's type mesomelic dysplasia


Department of Radio diagnosis & Imaging, B. J. Medical College, Civil Hospital, Ahmedabad, India

Click here for correspondence address and email

Date of Submission07-Jun-2006
Date of Acceptance10-Oct-2006
 

Keywords: Dysplasia, Langer′s, Mesomelic

How to cite this article:
Ravalji M, Makwana G J, Shah H R. Case presentation:Langer's type mesomelic dysplasia. Indian J Radiol Imaging 2006;16:587-8

How to cite this URL:
Ravalji M, Makwana G J, Shah H R. Case presentation:Langer's type mesomelic dysplasia. Indian J Radiol Imaging [serial online] 2006 [cited 2019 Aug 24];16:587-8. Available from: http://www.ijri.org/text.asp?2006/16/4/587/32276

   Introduction Top


Mesomelic dysplasia is a distinct heritable bone dysplasia, characterized by shortening of all the intermediate segments of long bones characterized by shortening of all the long bones associated with hypoplasia of the ulna, fibula and mandible.

There are six types of Mesomelic dysphasia are described like Langers type, Nievergelt type, Reinhardt type, Robinow type, Werner type, Lamy-Bienefeld type which differ from each other by some of the characteristics and associated anomalies.


   Case history Top


A 50 yrs old female patient presented with diarrhea, generalized weakness, admitted to our hospital for the treatment of hypotension. The female had history of short stature and failing to gain height since childhood. No any other significant history was associated with her.

So along with continuing treatment for hypovolemia, bone survey was done which shows hypoplasia of radius, fibula and hypo plastic mandible. Patient was also mentally retarded but able to do routine household work without help of others. These findings are in favor of the diagnosis of Mesomelic type of dwarfism - Langer type.


   Discussion Top


The word Mesomelic dysplasia means group of skeletal dysplasia characterized by shortening of the middle segments of the limbs together with other dysmorphic features[1]. These varieties of dwarfism are associated with normal life span and an inheritance pattern mostly Autosomal dominant type.

The six types of Mesomelic dysplasia are as below[2] :

1).Langer type :

-Autosomal recessive

-mental impairment

-hypoplasia of ulna and fibula

-hypoplasia of mandible with short condyles.

2).Nievergelt type:

-Autosomal dominant

-Most severe of all the type characterized by severe shortening of lower limbs, marked thickening of tibia and fibula in central portion and club foot seen frequently.

3).Reinhardt type:

-Autosomal dominant

-Characterized by short stature, forearm shortening with radial bowing, ulnar deviation with moderate restriction of pronation and supination of the hands and lateral bowing of the fibula.

4).Robinow type:

-Autosomal dominant

5).Werner type:

-Autosomal dominant

-Characterized by absent or extremely hypo plastic tibiae. Polydactyl and absence of the thumb[3]. Congenital heart disease and Hirschsprung's disease may be associated with it.

6).Lamy-Bienenfeld type:

-Autosomal dominant

-Characterized by ligamentous laxity, shortening of radius, ulna and tibia.

Absent fibula and normal femur and humerus[4].

 
   References Top

1.James Edekein: Roentgen diagnosis of diseases of bone, 3rd edition, the Williams & Wilkins Co. Volume 2, 1223-25.  Back to cited text no. 1    
2.Silverman F.N. Pediatrics radiology, Espiritu chem. volume, 5th edition Volume 2, 3460.  Back to cited text no. 2    
3.Caffey's pediatric radiology,Manroteux, 3rd edition,Volume 2nd, page 561-564.  Back to cited text no. 3    
4.Wolfgang Danhert., Radiology review manual, Lippincott Williams & wilkins 5th edition, 116.  Back to cited text no. 4    

Top
Correspondence Address:
M Ravalji
Room No-6, D-10, PG Doctor's Hostel, Civil Hospital Building, Asarwa, Ahmedabad-380016
India
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Source of Support: None, Conflict of Interest: None


DOI: 10.4103/0971-3026.32276

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    Figures

  [Figure - 1], [Figure - 2], [Figure - 3]

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[Pubmed] | [DOI]



 

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    Introduction
    Case history
    Discussion
    References
    Article Figures

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