Congenital Insensitivity to Pain and Anhydrosis: A Report of Two Cases

Maharajan, Karthikeyan; Sreenivas, T.; Menon, Jagdish; Patro, Dilip K.

doi:10.1177/230949901302100132

Cited by 8 publications

(5 citation statements)

References 18 publications

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“…Therefore, CIP can manifest with orthopaedic problems such as fractures, joint dislocations, avascular necrosis, heterotopic ossification, osteomyelitis and Charcot's joints [5,6]. Therefore, CIP can manifest with orthopaedic problems such as fractures, joint dislocations, avascular necrosis, heterotopic ossification, osteomyelitis and Charcot's joints [5,6].…”

Section: Discussionmentioning

confidence: 99%

“…In 2006, Minde et al [11] on the basis of a study of a Swedish family with CIP type V, suggested that children usually present between the ages of 4 and 6 years. These patients can also present with chronic or acute on chronic osteomyelitis as the delayed bone healing encourages pathogen colonization and worsening joint destruction [3,5]. A high index of suspicion is essential to establishing an early diagnosis.…”

Section: Discussionmentioning

confidence: 99%

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Congenital insensitivity to pain in a child attending a paediatric fracture clinic

Abdulla¹,

Khaled²,

Khaled

et al. 2014

Journal of Pediatric Orthopaedics B

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Congenital insensitivity to pain is a rare condition that is often undiagnosed until patients present with a variety of musculoskeletal problems. A major sequel of these orthopaedic manifestations is the development of heterotopic ossification and callus formation following fractures, eventually leading to the development of a Charcot's joint. This case reports on a 7-year-old child who was diagnosed with congenital insensitivity to pain type V, after he presented in our clinic with fractures of the metatarsals in his left foot while continuing to weight bear, without any discomfort. The patient failed to attend the follow-up in paediatric neurology clinic despite multiple invitations. This case highlights the importance of establishing an early diagnosis and keeping a close eye on this rare entity, which can present for the first time in a paediatric fracture clinic.

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Section: Discussionmentioning

confidence: 99%

Section: Discussionmentioning

confidence: 99%

Congenital insensitivity to pain in a child attending a paediatric fracture clinic

Abdulla¹,

Khaled²,

Khaled

et al. 2014

Journal of Pediatric Orthopaedics B

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“…Makari et al described two brothers with CIPA who suffered from child abuse [20]. In recent days, two cases of CIPA patients who continue treatment have been reported [21]. Generally, effective CIPA treatment has been established within the framework of family education and patient education [22].…”

Section: Discussionmentioning

confidence: 99%

Congenital insensitivity to pain syndrome accompanied by neglected orthopedic traumas and complications

et al. 2017

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Pain is a protective mechanism. Congenital insensitivity to pain syndrome is a very rare disease in which there is no ability to feel physical pain. It has been reported that it occurs with an incidence of 1 in 125 million newborn. Patients with congenital insensitivity to pain may have various orthopedic complications such as recurrent fractures, osteomyelitis and neuropathic joints. The most frequently affected body parts are lower extremities. Besides, curvature of spine can be seen. Injuries in epiphyseal points may cause incompatibility of extremity. Charcot joints may develop, which can lead to neuropathic arthropathy as a result of insensitivity to pain. Here, we present a patient with traumatic fracture-dislocation on left hip that neglected the treatment, had a bilateral femur fracture and then had septic arthritis of knee.

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“…Corneal ulcers are also relatively frequent in patients with CIPA. A differential diagnosis of neurotrophic keratitis may be taken into consideration [ 33 , 34 ]. Among all these diagnostic possibilities and according to Raspall-Chaure [ 29 ],…”

Section: Discussionmentioning

confidence: 99%

Update Review and Clinical Presentation in Congenital Insensitivity to Pain and Anhidrosis

Perez-Lopez

Cabrera-González

Iglesia

et al. 2015

Case Reports in Pediatrics

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Introduction. Congenital insensitivity to pain and anhidrosis (CIPA) or hereditary sensory and autonomic neuropathy type IV is an extremely rare syndrome. Three clinical findings define the syndrome: insensitivity to pain, impossibility to sweat, and mental retardation. This pathology is caused by a genetic mutation in the NTRK1 gene, which encodes a tyrosine receptor (TrkA) for nerve growth factor (NGF). Methods. The consultation of a child female in our center with CIPA and a tibia fracture in pseudoarthrosis encouraged us to carefully review literature and examine the therapeutic possibilities. A thorough review of literature published in Pubmed was done about CIPA and other connected medical issues mentioned in the paper. Conclusions. The therapeutic approach of CIPA remains unclear. The preventive approach remains the only possible treatment of CIPA. We propose two new important concepts in the therapeutic approach for these patients: (1) early surgical treatment for long bone fractures to prevent pseudoarthrosis and to allow early weight bearing, decreasing the risk of further osteopenia, and (2) bisphosphonates to avoid the progression of osteopenia and to reduce the number of consecutive fractures.

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Congenital Insensitivity to Pain and Anhydrosis: A Report of Two Cases

Cited by 8 publications

References 18 publications

Congenital insensitivity to pain in a child attending a paediatric fracture clinic

Congenital insensitivity to pain in a child attending a paediatric fracture clinic

Congenital insensitivity to pain syndrome accompanied by neglected orthopedic traumas and complications

Update Review and Clinical Presentation in Congenital Insensitivity to Pain and Anhidrosis

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