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Paediatric Amputee Case Study

Paediatric Amputee Case Study. Natasha Hankin 12 th November 2010. Peter. 14 years old Epidermal Naevus Syndrome and Polyostotic Fibrous Dysplasia affecting the right side of his body. Epidermal Naevus Syndrome.

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Paediatric Amputee Case Study

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  1. Paediatric Amputee Case Study Natasha Hankin 12th November 2010

  2. Peter • 14 years old • Epidermal Naevus Syndrome and Polyostotic Fibrous Dysplasia affecting the right side of his body

  3. Epidermal Naevus Syndrome • Defined as a sporadic neurocutaneous linkage of congenital ectodermal defects in the skin, brain, eyes, and/or skeleton • Can have associated skeletal disease focal bone defects that may manifest as fibrous dysplasia

  4. Polyostotic Fibrous Dysplasia • Affects bones and skin pigmentation • Characterized by abnormal fibrous development of the bones → fractures, deformities and abnormal x-rays • Abnormal skull bone growth → blindness or deafness and cosmetic abnormalities • Birthmarks

  5. History • At birth he was noted to have ?? rash and referred to a Dermatologist – organised further testing • Full diagnoses of Epidermal Naevus Syndrome was not made until 18 months with his first fracture of the humerus • Affecting (R) side of the body

  6. History cont. • Coarctation of the aorta repaired at 4yrs • Hypoplastic abdominal aorta, hypertension and suspected nephroblastomatosis • “Lymphedema” presentation • Multiple pathological fractures

  7. Fractures • 18 months: (R) humerus fracture – cystic lesions • 4 years old: (R) Tibial fracture • 5 ½ yrs old: (R) femur fracture

  8. April 2009 • Multiple failed Operations to correct pathological # of (R) tibia • Chronic pain in (R) leg • Leg length discrepancy of 11cm (right side shorter) • Missing large amounts of school

  9. Options: Continue in POP Ilizarov frame BKA Through knee amputation

  10. Ilizarov frame: 21st May 2009 • Surgery for excision of fibrous segment proximal tibia, proximal tibia osteotomy, removal of tibia rod and application of Ilizarov Frame • Femoral extension – bone quality

  11. July 2009 • Severe pain persisted in (R) tibia • Requiring large quantities of pain killers • Tibial # not showing signs healing • Pins loosening • Decision made for through knee amputation

  12. 9th July 2009 • (R) through knee amputation • Revision of (R) femoral intramedullary fixation - removal of rod and insertion of nail

  13. Post-op Problems • Post –op pain (phantom and stump) • Swelling & lymphedema – achieving a static size • Delayed wound healing • Naevus breakdown • (R) arm pain – crutches use

  14. Initial Physiotherapy • Various methods of stump compression & trying to minimise naevus breakdown • Standing balance on (L) / mobility • Hip flexor stretch • Hip extensor and core strengthening exs • Mirror Therapy • Massage/ desensitisation

  15. Gait training • Started 22nd September 2009 (2 ½ months post-op) • Standing balance/ weight transfer • Gait training in // bars → 2 x Canadian crutches → 1 x crutch • Continuing strengthening and stretching Aim: To mobilise unaided across all surfaces

  16. Progress • Peter has been able to return to school and be integrated into social activities • Peter has greatly reduced pain • He has started playing non-contact games/ sports

  17. Delays… • Still requires one crutch to mobilise • Ongoing problems: a growth spurt, Lymphedema swelling, and falls • ? Confidence issues because of consequences of a fall • Mobility progression/ weight bearing limited by tolerance of prosthesis

  18. Chronic Pain in children • Multidisciplinary approach • Social cost to children • Friendships • Peer victimisation • Isolation • School functioning

  19. Currently • Peter has followed his hobby of shooting and is training with the Paralympics shooting squad with an aim to represent Australia

  20. References • Chalkiadis, G.A. (2001). Management of chronic pain in children. MJA. 175, 476-479. • Forgeron, P.A., King, S., Stinson, J.N., McGrath, P.J., Macdonald, A.J, & Chambers, C.T. (2010). Social functioning and peer relationships in children and adolescents with chronic pain: A systematic review. Pain Res Manage, 15(1), 27-41.

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