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Total Hip Arthroplasty in an Inveterate Femoral Neck Fracture in a Patient with Congenital Insensitivity to Pain with Anhidrosis

Overview
Journal Joints
Publisher Thieme
Date 2017 Dec 23
PMID 29270564
Citations 1
Authors
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Abstract

Congenital insensitivity to pain with anhidrosis (CIPA) is an extremely rare disorder characterized by autonomic and sensory nerves malfunction with insensitivity to both deep and superficial painful stimuli, inability to sweat and produce tears, and mild to moderate mental retardation with self-mutilating behavior. Related consequences of inveterate musculoskeletal injuries represent a major issue for these patients, since pain cannot act as a protection mechanism. For the same reason, the patients are at risk during postoperative rehabilitation, which should be taken into account when selecting an orthopaedic implant. To our knowledge, only one case of total hip arthroplasty has been reported in the literature to date. A 21-year-old Caucasian male patient affected with CIPA arrived at our attention complaining about a functional limitation of the left hip. No history of trauma was reported. The X-rays showed an inveterate femoral neck fracture with a severe necrosis and resorption of the femoral head. We decided to perform a total hip arthroplasty with a cemented stem and a cemented dual mobility cup. The postoperative course and rehabilitation were satisfactory, with excellent clinical results, measured with the Harris Hip Score at 1 year.

Citing Articles

Effectiveness of Total Hip Arthroplasty Combined With Hip Braces for Hip Charcot Arthropathy.

Chao L, Zhuang T, Huan S, Luo S, Wu C, Wu W Orthop Surg. 2024; 17(3):790-800.

PMID: 39711443 PMC: 11872383. DOI: 10.1111/os.14329.


A 10-year follow-up of asymptomatic Charcot hip joints caused by CIPA syndrome (congenital insensitivity to pain with anhidrosis) with failure of any surgical reconstructive treatment.

Delniotis I, Leidinger B J Surg Case Rep. 2019; 2019(5):rjz154.

PMID: 31186831 PMC: 6537910. DOI: 10.1093/jscr/rjz154.

References
1.
Bar-On E, Weigl D, Parvari R, Katz K, Weitz R, Steinberg T . Congenital insensitivity to pain. Orthopaedic manifestations. J Bone Joint Surg Br. 2002; 84(2):252-7. DOI: 10.1302/0301-620x.84b2.11939. View

2.
Shaaban H, Bayat A, Davenport P, Shah M . Necrotising fasciitis in an infant with congenital insensitivity to pain syndrome. Br J Plast Surg. 2002; 55(2):160-3. DOI: 10.1054/bjps.2001.3771. View

3.
Thompson C, Park R, Prescott G . Oral manifestations of the congenital insensitivity-to-pain syndrome. Oral Surg Oral Med Oral Pathol. 1980; 50(3):220-5. DOI: 10.1016/0030-4220(80)90373-4. View

4.
Machtei A, Levy J, Friger M, Bodner L . Osteomyelitis of the mandible in a group of 33 pediatric patients with congenital insensitivity to pain with anhidrosis. Int J Pediatr Otorhinolaryngol. 2011; 75(4):523-6. DOI: 10.1016/j.ijporl.2011.01.011. View

5.
Zhang Y, Haga N . Skeletal complications in congenital insensitivity to pain with anhidrosis: a case series of 14 patients and review of articles published in Japanese. J Orthop Sci. 2014; 19(5):827-31. DOI: 10.1007/s00776-014-0595-2. View