Miss Sally Tennant Consultant Paediatric Orthopaedic Surgeon RNOH Stanmore St Marys Hospital Paddington ABNORMAL COLLAGEN Collagen Type 6 Almost all tissues Extracellular matrix Muscle hypotonia ID: 915063
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ORTHOPAEDIC PROBLEMS IN DOWN SYNDROME
Miss Sally Tennant
Consultant Paediatric Orthopaedic Surgeon
RNOH, Stanmore
St Mary’s Hospital Paddington
Slide2ABNORMAL COLLAGEN
Collagen Type 6
Almost all tissues
Extracellular matrixMuscle hypotonia“Lax” Tissues
Slide3MUSCULO-SKELETAL PROBLEMS
HIP
KNEE
FEETSPINE
Slide4HIP INSTABILITY
Capsular insufficiency, ligamentous laxity & muscle hypotonia →
Progressive instability →
Subluxation/Dislocation → Fixed dislocation→Osteo-arthritis
IncidenceInstitutionalised patients-4.5% hip instability
Bennet et al 1982
Hypermobile but stable hips < 2 years
Then painless spontaneous dislocations
Posterior-produced by flexion, adduction, internal rotation
Recurrent Posterior dislocations → dysplasia of the acetabulum (abnormal shape)
Slide5Slide6NATURAL HISTORY/PROGNOSIS
“Hip disease in adults with Down syndrome”
Hresko et al 1993
65 adults-hip abnormalities in 28%, correlated with walking abilityInstability Worsened with timeSometimes started after skeletal maturity
Slide7POTENTIAL SURGICAL MANAGEMENT
Capsular plication
Femoral osteotomy
Pelvic osteotomy
Slide8SURGICAL MANAGEMENT
Complications of surgery
Recurrent subluxation/dislocation-up to 50%
Infection- up to 20%2011-Journal of Paediatric Orthopaedics- Femoral varus derotation osteotomy for the treatment of habitual subluxation and dislocation of the pediatric hip in trisomy 21 (Toronto
)16 hips in 9 children over 10 year period-all but 2 stabilised with femoral osteotomy only1 arthritis, none redislocated2011-Sankar et al, Instability of the hip in patients with Down Syndrome
. Improved results with Complete Redirectional Acetabular osteotomy
Better results with pelvic osteotomy-92% of 18 unstable hips stable after surgery compared with femoral osteotomy only
Slide9OTHER HIP PROBLEMS
PERTHES
SUFE
AVASCULAR NECROSIS
Slide10SUFE (SLIPPED UPPER FEMORAL EPIPHYSIS)
Slide11SUFE
Puberty
Hormonal changes weaken growth plate
ObesityOther hormone disorders eg hypothyroidism↑incidence Down syndrome-probably due to ↑ incidence hypothyroidism
Slide12SUFE
Pain-KNEE or HIP
Limping
Inability to walkChronic, acuteUrgent medical treatment needed Hip xray
Slide13↑ risk of Avascular Necrosis
Slide14OUTCOME
2004-11 hips in 8 patients
6/8 had hypothyroidism
All had ↑BMI (Body mass index)After pinning, 6 slips progressed, 2 had Avascular necrosis, 1 had infectionTreatment difficult Screen for hypothyroidism
2004-8 patientsAfter pinning, 5 hips developed AVNHigh rate of complications
Slide15AVASCULAR NECROSIS (AVN)
Slide16PERTHES
Slide17TOTAL HIP REPLACEMENT
Osteo-arthritis of hip in Down syndrome - 8-28%
Dislocation, dysplasia, Perthes, SUFE, Avascular necrosis
Problems with THR:Distorted femoral necks
Small femoral canals-small stemsCapsular laxity (constrained cups)Compliance
Acetabular deformity (dysplasia)-
Bone graft
Comorbidities (Heart, cervical spine)
Susceptibility to infection
Slide18TOTAL HIP REPLACEMENT IN DOWN SYNDROME
Slide19RESULTS
1987 Skoff and Keggi
- 8 THRs in 5 patients
Average age 46 years, all had subluxationExcellent results -no evidence of infection, loosening, or dislocation at average follow up 4 yearsTotal hip arthroplasty in patients with Down’s syndrome 1999
THR in 9 hips in 6 patients with severe arthritisAverage follow up nearly 8 years (2-14 years)All had pain relief and full hip function
Slide20RESULTS
Total hip arthroplasty in patients with Down syndrome. 2010
9 hips in 7 patients
Osteoarthritis secondary to Hip dysplasia in 6 patients, SUFE in 1 patientAverage follow up 9.9±6.4 years (range, 2-22.5 years). Average patient age at THR was 34.8±7.5 years
Harris Hip Scores improved significantly at 4-year follow-up. Harris Hip Scores remained unchanged at 8-year follow-up.
Two patients required revision THA for stem loosening at 6 and 16 years
No dislocations or deep infections
At last follow-up, all patients had a functional range of motion without evidence of discomfort related to their THR.
Slide21RESULTS
Bone Joint J
2013;95-B, Supple A:41–5.
Four studies –total 42 hips- clinical outcomes of THR in patients with Down’s syndrome. All patients successfully treated with standard acetabular and femoral components.
Extra acetabular screws often used to enhance component stability Constrained liners used to treat intra-operative instability in 8 hips. Survival rates 81% - 100% at a mean follow-up of 105 months (6 to 292) are encouraging.
Overall, while THR in patients with Down’s syndrome does present some unique challenges, the overall clinical results are good, providing these patients with reliable pain relief and good function
.
Slide22SPINE
Slide23SCOLIOSIS
Slide24FEET
CLUB FOOT
METATARSUS PRIMUS VARUS
FLAT FOOT
Slide25CLUBFOOT
Slide26INCIDENCE OF CLUBFOOT IN DOWN SYNDROME
Shipp and Benacerraf
Retrospective review of Antenatal Ultrasound reports over 18 years
34 of 68 fetuses had karyotyping-4 were abnormal (47, XXY, 47, XXX, trisomy 21, trisomy 18)-5.9%Karyotyping indicated when an isolated clubfoot abnormality is identified.
Slide27PONSETI TECHNIQUE
Slide28BOOTS AND BAR
Slide29FLAT FEET
Slide30BUNIONS
Slide31MANAGEMENT
Surgery-painful deformity
Osteotomies
Correction of flat foot and heel valgusMTP joint fusion
Slide32KNEES-PATELLO-FEMORAL DISLOCATIONS
Contributing factors
Capsular laxity
Muscle hypotoniaGenu valgum
Slide33Slide34Slide35MANAGEMENT
Indications for surgery
?Functional impairment
? To prevent Osteo-arthritis
1986 “Instability of the Patellofemoral joint in Down syndrome” Dugdale et al361 people
Dislocatable or dislocated in 8% of institutionalised and 4% of non-institutionalised
In only 3/47 knees did it stop walking
8 knees in 5 patients-surgery-50% satisfactory
1988 “Treatment of patellofemoral instability in Down Syndrome” Mendez et al
26 dislocated or dislocatable patellae in 16 patientsDegree of PF instability not related to walking ability or to form of treatment
Surgery → good walking ability in 86% of knees (67% with non op treatment and less likely to help poor ambulators) but did not effectively correct deformities which resulted in arthritis.
Slide36REALIGNEMENT SURGERY
Slide37RESULTS OF SURGERY
“The Four-in-one procedure for habitual dislocation of the patella in children”
20076 knees in 5 patients-2/6 Down syndromeMean age at surgery 6 years (4.9-6.9)Mean Follow up 54 months
No recurrence-Children satisfied“Surgical treatment of patellar dislocation in children with Down syndrome: a 3-11 year follow up study”
2009
10 knees in 6 children
Roux-Goldthwait-Campbell procedure (proximal & distal realignment)
Mean age at surgery 10 years (6-13)
Average Follow up 8 yearsNo recurrenceImprovement in function
Slightly more successful in patients with more severe disability
Slide38RESULTS
The results of the operative treatment of patellar instability in children with Down’s syndrome
201210 knees in 8 childrenAge range 6-11Quadricepsplasty
Quadricepsplasty & Galeazzi procedureMean follow up 3 yearsNo recurrence of dislocation
SUMMARY
All small series
Never identical procedures
Short Follow up
Slide39OTHER PROBLEMS
Polyarticular arthropathy
Decreased bone density