Limb Salvage of a Flail Neuropathic Ankle in Congenital Insensitivity to Pain with Anhidrosis Using a Hindfoot Superconstruct.
- 1Department of Orthopaedic Surgery, Institute of Orthopaedics and Traumatology, Madras Medical College, Chennai, Tamil Nadu, India.
Abstract
Congenital insensitivity to pain with anhidrosis (CIPA) is a rare autosomal recessive neurological disorder, with an estimated incidence of 1 in 125 million. The condition is characterized by a clinical feature of unexplained pyrexia, global insensitivity to pain, anhidrosis, intellectual disability, and self-mutilating behavior. While musculoskeletal complications, such as fractures and joint dislocations are common, severe neuropathic arthropathy represents one of the most debilitating skeletal manifestations; however, its specific incidence remains unrecorded in present medical literature. Due to progressive joint destruction and gross instability, limb salvage and functional restoration remain a significant surgical challenge. A tertiary care orthopedic center reported the case of a 24-year-old female, who was wheelchair-bound for 7 years and presented with a painless, severely deformed, and unstable ankle complicated by a history of recurrent infections. A diagnosis of a flail neuropathic ankle secondary to CIPA was established. To achieve limb salvage, the patient underwent hindfoot arthrodesis utilizing the "superconstruct" principle. This approach aimed to provide a stable, plantigrade foot through rigid internal fixation and intensive post-operative rehabilitation. Clinical and radiological union was well-established by 10 weeks, at which point assisted mobilization was achieved. At the 1-year follow-up, radiological assessment confirmed solid bony fusion, and the patient was successfully ambulating without assistance. Managing a neuropathic flail ankle in the context of CIPA requires meticulous pre-operative planning, a detailed diagnostic workup, and aggressive post-operative management with respect to wound care and rehabilitation. This case demonstrates that hindfoot arthrodesis utilizing a plate-and-screw superconstruct provides the mechanical stability necessary to overcome the biomechanical challenges of CIPA, proving that limb salvage and functional restoration are achievable goals.
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BibTeX
@article{tholgapiyan2026limb,
title = {{Limb Salvage of a Flail Neuropathic Ankle in Congenital Insensitivity to Pain with Anhidrosis Using a Hindfoot Superconstruct.}},
author = {T Tholgapiyan and Senthilkumar Sivakumar and Madhan Athmanathan and T Dilip Kumar},
journal = {Journal of orthopaedic case reports},
year = {2026},
month = nov,
doi = {10.13107/jocr.2026.v16.i11.8380},
url = {https://europepmc.org/article/MED/42997090},
note = {CC BY-NC},
}
RIS
TY - JOUR
TI - Limb Salvage of a Flail Neuropathic Ankle in Congenital Insensitivity to Pain with Anhidrosis Using a Hindfoot Superconstruct.
AU - T Tholgapiyan
AU - Senthilkumar Sivakumar
AU - Madhan Athmanathan
AU - T Dilip Kumar
PY - 2026
DA - 2026/11/01
JO - Journal of orthopaedic case reports
DO - 10.13107/jocr.2026.v16.i11.8380
UR - https://europepmc.org/article/MED/42997090
AB - Congenital insensitivity to pain with anhidrosis (CIPA) is a rare autosomal recessive neurological disorder, with an estimated incidence of 1 in 125 million. The condition is characterized by a clinical feature of unexplained pyrexia, global insensitivity to pain, anhidrosis, intellectual disability, and self-mutilating behavior. While musculoskeletal complications, such as fractures and joint dislocations are common, severe neuropathic arthropathy represents one of the most debilitating skeletal manifestations; however, its specific incidence remains unrecorded in present medical literature. Due to progressive joint destruction and gross instability, limb salvage and functional restoration remain a significant surgical challenge. A tertiary care orthopedic center reported the case of a 24-year-old female, who was wheelchair-bound for 7 years and presented with a painless, severely deformed, and unstable ankle complicated by a history of recurrent infections. A diagnosis of a flail neuropathic ankle secondary to CIPA was established. To achieve limb salvage, the patient underwent hindfoot arthrodesis utilizing the "superconstruct" principle. This approach aimed to provide a stable, plantigrade foot through rigid internal fixation and intensive post-operative rehabilitation. Clinical and radiological union was well-established by 10 weeks, at which point assisted mobilization was achieved. At the 1-year follow-up, radiological assessment confirmed solid bony fusion, and the patient was successfully ambulating without assistance. Managing a neuropathic flail ankle in the context of CIPA requires meticulous pre-operative planning, a detailed diagnostic workup, and aggressive post-operative management with respect to wound care and rehabilitation. This case demonstrates that hindfoot arthrodesis utilizing a plate-and-screw superconstruct provides the mechanical stability necessary to overcome the biomechanical challenges of CIPA, proving that limb salvage and functional restoration are achievable goals.
SN - 2250-0685
SN - 2321-3817
ER -