Indian Journal of Radiology and Imaging Indian Journal of Radiology and Imaging

RADIOLOGICAL QUIZ
Year
: 1999  |  Volume : 9  |  Issue : 2  |  Page : 79--80

Radiological quiz: Musculoskeletal


NA Patel, NU Bahri, Deepak M Murjani, Tejal S Shah 
 Department of Radiology, Shri M.P. Shah Medical College and Irwin Group of Hospital, Jamnagar, India

Correspondence Address:
N A Patel
Khambhalia Gate, C/o Dr.K.N.Baxi, Jamnagar 361 005, Gujrat
India




How to cite this article:
Patel N A, Bahri N U, Murjani DM, Shah TS. Radiological quiz: Musculoskeletal.Indian J Radiol Imaging 1999;9:79-80


How to cite this URL:
Patel N A, Bahri N U, Murjani DM, Shah TS. Radiological quiz: Musculoskeletal. Indian J Radiol Imaging [serial online] 1999 [cited 2019 Nov 22 ];9:79-80
Available from: http://www.ijri.org/text.asp?1999/9/2/79/28339


Full Text

A five-years-old boy was brought to the hospital with a history of fall followed by convulsions. When the child was seen by the attending doctor, he was fully conscious but the doctor noted a swelling of the right index finger. The patient's radiograph of the hand with wrist was obtained [Figure 1].

 RADIOLOGICAL DIAGNOSIS



Tuberous Sclerosis

The radiograph of right hand showed irregular areas of cortical thickening with periosteal reaction and areas of sclerosis in the phalanges of the index and middle finger and second metacarpal. Multiple cystic areas were also seen in the tubular bones with soft tissue swelling in the index and middle fingers

The following diseases may present with this appearance; Caffey's disease, congenital syphilis and tuberculosis. In Caffey's disease, the mandible, ribs and clavicle are common sites of involvement showing increased bone density. The hands are usually spared. In congenital syphilis, periosteal reaction is bilaterally symmetrical and associated with metaphysitis and epiphysitis. In tuberculous dactylitis, osteoporosis, medullary expansion and ballooning with cortical thinning of involved tubular bones is commonly noted.

Detailed examination of the child revealed a large patch of thick skin with coarse appearance in the lower lumbar region typical of a "shagreen patch". Adenoma sebaceum was seen on the face.

Tuberous sclerosis, first described by Von Recklinghausen in 1863, then again by Bourneville in 1880, is characterized by the clinical triad of mental retardation, skin lesions (adenoma sebaceum) and seizures. It is inherited as an autosomal dominant trait with a variety of clinical manifestations. The basic lesion of the disease is characterized by the development of hamartomas in various tissues and organs in the body.

Radiological manifestations in the hands and feet are characterized by irregular deposition of subperiosteal bone commonly in the 2nd, 3rd and 4th metatarsals and metacarpals. In the phalanges, changes are characterized by uneven thickness of the cortical bone and change in bone density and trabecular pattern with "cyst" formation. There are also areas of sclerosis in bones adjacent to the areas of lucency. The terminal phalanges may show cyst formation due to subungual fibromas.

Other radiological manifestations which may be seen include calcifications on skull radiographs, sclerotic lesions throughout the skeleton, renal cystic disease and renal masses especially renal angiomyolipomas. In the chest, the lungs may show a honey-comb pattern and the heart may show developmentment of rhabdomyomas.[3]

References

1Nathanson Noel and Avnet Norman L. An unusual X-ray finding in Tuberous Sclerosis BJR 1966; 39: 786-787.
2Hawkins T Desmond. Radiological bone changes in tuberous sclerosis. BJR 1959; 32: 157-161.
3Poranski. The hand in radiological diagnosis. 2nd Edition, Vol. 2, p. 594-595, 1984.