|
Table 1: Clinical characteristics of patients and surgical procedures performed. |
|||||
|
Median age in years (range) |
11 (2-18) |
|
|
|
|
|
No. of females |
10 (55.6%) |
|
|
|
|
|
No. of males |
8 (44.4%) |
|
|
|
|
|
|
Type I |
Type II |
Type IV |
Type VI |
Total |
|
No. of patients |
12 (66.7%) |
2 (11.1%) |
3 (16.7%) |
1 (5.5%) |
18 |
|
Concomitant risk factors |
|||||
|
Short neck |
12 |
2 |
3 |
1 |
18 |
|
Restrictive chest |
11 |
1 |
3 |
1 |
16 |
|
Severe joint manifestations |
10 |
1 |
3* |
1 |
15 |
|
Severe spinal deformities |
8 |
1 |
3 |
1 |
13 |
|
Intellectual disability |
9 |
1 |
– |
– |
10 |
|
Severe sleep apnea |
6 |
1 |
– |
– |
7 |
|
Heart valve condition |
5 |
1 |
– |
– |
6 |
|
No. of procedures |
17 (73.9%) |
2 (8.7%) |
3 (13.1%) |
1 (4.3%) |
23 |
|
Procedure |
|||||
|
Port catheter |
9 |
2 |
– |
– |
11 (47.8%) |
|
Tonsillectomy** |
4 |
– |
1 |
– |
5 (21.7%) |
|
Corneal transplantation |
3 |
– |
– |
– |
3 (13.1%) |
|
Ventriculoperitoneal shunt |
1 |
– |
– |
– |
1 (4.3%) |
|
Cervical laminectomy |
– |
– |
1 |
– |
1 (4.3%) |
|
Posterior arch resection |
– |
– |
1 |
– |
1 (4.3%) |
|
Umbilical hernioplasty |
– |
– |
– |
1 |
1 (4.3%) |
|
* All 3 type IV patients had hypermobile joints and the other types had joint stiffness. ** 3 patients had simultaneous myringotomy, 2 type I and 1 type 4. |
|||||