BIYANI AND KOTWAL : BILATERAL DOUBLE PATELLAE

knees revealed double layered patellae, larger and thicker anterior layers in close relationship with bipartite smaller and thick posterior layers (Fig 1). Lateral films in flexion and extension of the knees did not show movement of one layer on the other. Tibiofemoral epiphysis were not involved. Axial skeleton was normal. Hip deformity was corrected by traction and stretching programme. Gait could be improved in this way. Lateral subluxation of patella did not warrant surgery at this time. Double layered patella is an uncommon accompaniment of multiple epiphyseal dysplasia and dwarfism. Lodes 5 had erroneously suggested that these cases represent Morquio disease. Subsequent studies 4 have shown that this disorder is a distinct entity. Patellar abnormality may not be apparent before patella has significantly ossified. Habitual lateral subluxation of patella in patients with multiple epiphyseal dysplasia should arouse strong suspicion of double layered patellae. Lateral radiographs of the knee should be routinely ineluded in the skeletal survey of epiphyeal dysplasia. One or both layers may be multipartite. Dahners et aF noted synovial joint lined by hyaline cartilage in their case and the two layers of patella glided over each other during movement of the knee joint. Lateral subluxation of the patella often requires surgery for patellar tendon realignment.

A. Biyani and P.P. Kotwal, Department of Orthopaedics All India Institute of Medical Sciences New Delhi 110 029.

REV~NCES 1. Beaupore A, Nausib PAD. One dysplasia

2.

3. 4.

5.

275

and four patellae (Proceedings). J Bone Joint Surg. 1982; 64B : 261-262. Dahners LE, Francisco WD, Halleran WJ. Findings at arthrotomy in a case of double layered patellae associated with multiple epiphyseal dysplasia. J Pediatr Orthop 1982; 2 : 67-70. Ficat P, Hungerford D. Disorders of the Patellofemoml joint. Baltimore : Williams and Wilkins, 1977 : 58-62. Hodkinson HM. Double patellae in multiple epiphyseal dysplasia. J Bone Joint Surg 1%2; 44B : 569-572. Lodes R. Familiares Vorkommen habitu, ellar bilateraler Luxation zweigeteilter Kniescheiben hie Chondrodystrophic. Z Orthop 1949; 78 : 506-523.

Down Syndrome in Dubai Sir - Dubai known as the pearl of the Gulf is one of the seven Emirates in the United Arab Emirates (UAE). In Dubai there are four major hospitals which come under the Department of Health and Medical Sciences ( D O H M S ) in the Government of Dubai. Over a three year period from 1987 to 1989, 1630 cultures were received for chromosomal analysis. Of this 56% were in the pediatric age group and referred as patients with multiple congenital abnormalities, the remaining cases were patients affected with habitual abortions or infertility (Table 1). In the pediatric age group 11.2% of the cases were found to be affected with a gross chromosomal aberration ranging from deletions, duplications, inversions,

276

THE INDIAN JOURNAL OF PEDIATRICS

TABLE2. Incidence of Down syndrome at DOH & MS

TABLE1. Cytogenetic Analysis at DOH & MS Year

No. of Chromosomal chromo- aberrations sorlaal analyses No. %

Down syndrome

No.

%

1987

460

62

13.5

35

7.6

1988

553

52

9.4

30

5.4

1989

617

69

11.18

40

6.5

Total

1630

183

11.23 105

6.4

D

ring chromosomes and various trisomies. The largest group (6.4%) of chromosomal aberrations was that of trisomy 21. Of the 105 registered cases of Down syndrome, 85 patients have been born in DOHMS over these three years (Table 2). Seven patients were found to be cases of 13/21 translocation. None of the parents were found to be translocation carriers. There is an ongoing program, both by maternal serum AFP screening and amniotic fluid cultures, to prenatally detect cases of Down syndrome. Due to the

Vol. 57, No. 2

Year

Total no.of livebirths

Total no. of Down syndrome cases

1987

10,183

21 (9F, 12M) 1 in 485

1988

11,615

23 (llF, 12M) 1 in 505

1989

12,640

27 (17F, 10M) 1 in 485

Total

34,438

71

Incidence DOHMS

1 in 485

cosmopolitan nature of the population it is difficult to categorize various ethnic groups for any meaningful analysis. The problem of Down syndrome in Dubai is an enormous one. T o our knowledge this is the first report from the UAE.

S. EI-Kalla and A.R. Mathews Genetic Unit, Pediatric Department, Department of Hea/th and Medica/ Services, P.O. Box 9115, Dubai, U.A.E.

Down syndrome in Dubai.

BIYANI AND KOTWAL : BILATERAL DOUBLE PATELLAE knees revealed double layered patellae, larger and thicker anterior layers in close relationship with b...
103KB Sizes 0 Downloads 0 Views