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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