Table 1 Patient characteristics, outcomes, and complications in this series. ADHD = attention-deficit/hyperactivity disorder, ID = intellectual disability, HE = hemiepiphysiodesis, TBP = tension-band plating, SSR = sympathetic skin reflex, f/u = follow-up
From: Guided growth in the correction of knee deformity in patients with congenital insensitivity to pain
Case no. | Age at surgery/ gender | Presenting symptoms | Other findings | Phenotypic classification (Dyck [3]) | Knee deformity | Procedure | Outcome | Complications | Comments |
|---|---|---|---|---|---|---|---|---|---|
1 | 11/M | Bilateral knee swelling | No anhidrosis, normal intelligence | V | Bilateral valga Left mLDFA = 73° Right mLDFA = 75° | Bilateral femoral HE with staples | Left mLDFA = 76° Right mLDFA = 80° | Staple extrusion necessitating removal | The patient is ambulatory with braces, right hip dislocation |
2 | 9/M | Bilateral knee deformity, suspected infection | Anhidrosis, lip bites, tooth loss, dystrophic nails, ADHD, previous femoral fracture (Fig. 3) | IV | Bilateral valga Left mLDFA = 69° Right mLDFA = 72° | Bilateral femoral HE with TBP | Left mLDFA = 77° Right mLDFA = 80° | Post-op agitation | Absent SSR was found on neurophysiologic studies |
3 | 12/M | Bilateral knee deformity | Anhidrosis, lip and tongue bites, moderate ID, ADHD, dystrophic nails | IV | Bilateral valga Left mLDFA = 77° Right mLDFA = 76° | Bilateral femoral HE with TBP | Left mLDFA = 82° Right mLDFA = 81° | None | The patient died of injuries following a fight at age 16 |
4 | 11/F | Windswept knee deformity | No anhidrosis, previous femoral fracture, normal intelligence, anosmia | V | Left genu varum (MPTA = 79°), right valgum (mLDFA = 79°) | Left tibial and right femoral HE with TBP | left MPTA = 83° right mLDFA = 85° | None | Lost to f/u after 2.5 years |
5 | 5/F | Left knee swelling and deformity. | Anhidrosis, normal intelligence, bilateral ankle swelling (Fig. 1) | V | Left genu valgum mLDFA = 76° | Left femoral TBP with imbrication of medial structures | mLDFA = 84° | None | The patient had a proximal femoral fracture 1.5 years after the index surgery |
6 | 12/M | Left tibial fracture at age 1, deformity of the same limb | Anhidrosis, bilateral ankle and foot swelling, metatarsal fracture, dystrophic nails (Fig. 2) | IV | Left genu valgum mLDFA = 75° | Left femoral HE | mLDFA = 84° | None | Tibial fracture had abundant callus and was biopsied |