Anesthesia and Patients with Congenital Hyposensitivity to Pain

Background:Congenital hyposensitivity to pain or hereditary sensory and autonomic neuropathy represents a variety of disorders characterized by decreased perception of nociception, loss of other modalities of sensation, and variable expression of autonomic dysfunction. Sensory loss, especially that of pain, is associated with self-mutilations that may require frequent operations. Little is known about the safety of anesthesia for these patients. Methods:The authors performed a computerized search of the Mayo Clinic medical records database between January 1996 and November 2005 for patients with congenital hyposensitivity to pain and related disorders who underwent general anesthesia. Medical records were reviewed for demographics, anesthetic techniques and agents, use of opioids, and perioperative complications. In addition, the authors conducted a comprehensive review of the literature to summarize the current knowledge regarding anesthesia for patients with congenital hyposensitivity to pain, and compared it with the patients with hyposensitivity to pain identified at the Mayo Clinic. Results:The authors identified seven patients with hereditary sensory and autonomic neuropathy II, IV, or V and undefined variants of congenital pain hyposensitivity who generated 17 anesthesia records: 12 for orthopedic operations, 3 for sural nerve biopsies, and 2 for ophthalmologic procedures. In all patients, standard doses of volatile agents were used during anesthesia. Small amounts of opioids were used during the course of eight operations. Most patients experienced mild hypothermia (lowest temperature 34.7°C), and none experienced hyperthermia. All patients were hemodynamically stable during otherwise uneventful anesthesia. During recovery from anesthesia, opioids were given to only one patient, a single dose of 1 mg morphine. Even after major orthopedic operations, the patient did not require additional analgesia. Conclusions:The patients with profound congenital hyposensitivity to pain underwent anesthesia without any adverse events. The authors found that despite reduced pain perception, the requirements for volatile anesthetics were within the expected range for population with normal pain perception, but they did not require opioids postoperatively. Intraoperative mild hypothermia was easily managed by adjustment of environmental temperature.

[1]  I. Brandes,et al.  Use of BIS monitor in a child with congenital insensitivity to pain with anhidrosis , 2006, Paediatric anaesthesia.

[2]  C. Rodrigo,et al.  Anesthetic management of a child with congenital insensitivity to pain with anhydrosis. , 2005, Journal of oral and maxillofacial surgery : official journal of the American Association of Oral and Maxillofacial Surgeons.

[3]  M. Tsou,et al.  Desflurane used in a patient with congenital insensitivity to pain with anhidrosis during septic shock. , 2004, Journal of the Chinese Medical Association : JCMA.

[4]  M. Klein,et al.  The anaesthetic management of patients with congenital insensitivity to pain with anhidrosis , 2004, Paediatric anaesthesia.

[5]  Naffaa Al-Harbi,et al.  Congenital Insensitivity to Pain in Four Related Saudi Families , 2002, Pediatric dermatology.

[6]  M. Hilz.  Assessment and evaluation of hereditary sensory and autonomic neuropathies with autonomic and neurophysiological examinations , 2002, Clinical Autonomic Research.

[7]  K. Hanaoka,et al.  Anesthesia for Patients with Congenital Insensitivity to Pain and Anhidrosis: A Questionnaire Study in Japan , 2002, Anesthesia and analgesia.

[8]  T. Matsukawa,et al.  [Anesthetic management of a child with congenital sensory neuropathy with anhydrosis]. , 2001, Masui. The Japanese journal of anesthesiology.

[9]  Toshimi Arai,et al.  Anaesthetic management of children with congenital insensitivity to pain with anhidrosis , 2000, Paediatric anaesthesia.

[10]  G. Caruso,et al.  Absent innervation of skin and sweat glands in congenital insensitivity to pain with anhidrosis , 2000, Clinical Neurophysiology.

[11]  I. Morisaki,et al.  Oral manifestations of hereditary sensory and autonomic neuropathy type IV. Congenital insensitivity to pain with anhidrosis. , 1998, Oral surgery, oral medicine, oral pathology, oral radiology, and endodontics.

[12]  M. Takasaki,et al.  [Anesthesia for a child with congenital sensory neuropathy with anhydrosis]. , 1998, Masui. The Japanese journal of anesthesiology.

[13]  S. Rosemberg,et al.  Congenital insensitivity to pain with anhidrosis (hereditary sensory and autonomic neuropathy type IV) , 1994, Pediatric neurology.

[14]  S. Yoshitake,et al.  [Anesthetic consideration of a patient with congenital insensitivity to pain with anhidrosis]. , 1993, Masui. The Japanese journal of anesthesiology.

[15]  R. Caplan,et al.  A Comparison of Pediatric and Adult Anesthesia Closed Malpractice Claims , 1993, Anesthesiology.

[16]  S. Izumo,et al.  Congenital insensitivity to pain with anhidrosis. A case report. , 1990, The Journal of bone and joint surgery. American volume.

[17]  C. Mitaka,et al.  Anesthetic management of congenital insensitivity to pain with anhydrosis. , 1985, Anesthesiology.

[18]  R. Langford,et al.  Anaesthesia in dysautonomia: further complications , 1985, Anaesthesia.

[19]  J. Mcdonald,et al.  Not 'indifference to pain' but varieties of hereditary sensory and autonomic neuropathy. , 1983, Brain : a journal of neurology.

[20]  R. Crago,et al.  Anaesthetic considerations in idiopathic orthostatic hypotension and the shy-drager syndrome , 1979, Canadian Anaesthetists' Society journal.

[21]  G. Kuhlenbäumer,et al.  Hereditary sensory and autonomic neuropathies (HSAN) , 2005 .

[22]  P. Dyck,et al.  HSANs: Clinical Features, Pathologic Classification, and Molecular Genetics , 2005 .

[23]  M. V. Rodríguez Pérez,et al.  [Epidural anesthesia in a child with femoral fracture and congenital pain insensitivity]. , 2002, Revista espanola de anestesiologia y reanimacion.

[24]  E. Jarade,et al.  Indolent Corneal Ulcers in a Patient with Congenital Insensitivity to Pain with Anhidrosis: A Case Report and Literature Review , 2002, European journal of ophthalmology.