|Year : 2015 | Volume
| Issue : 2 | Page : 118-120
A rare case of congenital insensitivity to pain with anhydrosiss
Govardhani Yanamadala, Varaprasad Ussa, Pavani Medisetty, Bhavya Gatte
Department of Anesthesiology and Critical Care, Dr. Pinnamaneni Siddhartha Institute of Medical Science and Research Foundation, Vijayawada, Andhra Pradesh, India
|Date of Web Publication||15-Apr-2015|
Dr. Govardhani Yanamadala
C/O, Dr. A. Sivaram, D-5 Block, Staff Quarters, Dr. Pinnamaneni Siddhartha Institute of Medical Science and Research Foundation, Chinoutpalli, Gannavaram Mandal, Krishna District, Andhra Pradesh
Source of Support: None, Conflict of Interest: None
Congenital insensitivity to pain syndrome with anhydrosis (CIPA) is a rare inherited disorder. It is characterized by loss of pain and temperature sensation, lack of sweating and mild mental retardation. This disorder belongs to hereditary sensory and autonomic neuropathy family (type IV). Because of these abnormalities, patients require special anesthetic care. They include titration of intraoperative opioids, an anesthetic to ensure co-operation and immobility and intra-operative temperature monitoring. Here we report a 7-year-old female child with CIPA posted for restoration and cementation for dental caries along with sural nerve and skin biopsy.
Keywords: Anesthetics, anhydrosis, congenital insensitivity to pain
|How to cite this article:|
Yanamadala G, Ussa V, Medisetty P, Gatte B. A rare case of congenital insensitivity to pain with anhydrosiss. Indian J Pain 2015;29:118-20
| Introduction|| |
Congenital insensitivity to pain with anhydrosis (CIPA) (or) hereditary sensory and autonomic neuropathy (HSAN) type IV  is a rare pathological condition in which patients do not respond to the painful stimulus. It is a rare autosomal recessive neuropathy characterized by insensitivity to painful stimuli, changes in temperature control and varying degree of mental retardation. It is due to mutation in the neurotrophic tyrosine receptor kinase 1 gene (NTRK1), located in chromosome 1. 
The sensation of pain and temperature are absent over the entire body. All other sensory modalities are intact. Children don't display any function of the sweat glands, suffer from recurrent episodes of unexplained fever. Tendon reflexes vary from normal to depressed. Blood pressure is normal. Fungiform papillae of the tongue are present. Some children display hypotrichosis of scalp.
Due to loss of sensations children are self-mutilated, they bite themselves and suffer from burns and fractures. Fractures are often reduced with difficulty, leading to orthopedic problems and the secondary osteomyelitis.
The reduction in the central and peripheral activities of noradrenaline and anhydrosis lead to the development of perioperative hypotension and hyperthermia. ,
Currently, 5 types of HSAN have been recognized  [Table 1].
Specific treatment is not available, and patients usually do not live past the second decade of life. There are very few reports of anesthesia in patients with this disease, which is very rare, but it is related to some ethnic groups and consanguinity. ,
| Case Report|| |
A 7-year-old female child, weighing 14 kg, 107 cm height, with a history of CIPA was admitted for treatment of bilateral calcaneal osteomyelitis. She was treated with oral antibiotics and dressings. After 1 week, she was posted for restoration and cementation for dental caries and for skin and nerve biopsy under anesthesia. History revealed the girl was first child of consanguineous parents (first degree) and the second male child was normal.
When the child was few days old, the parents noticed that the child failed to cry to painful stimuli, did not sweat and suffered from episodes of unexplained fever. At that time, the child was diagnosed clinically as having CIPA. General examination revealed diffuse lack of sensitivity to pain and self-induced injuries in hands and distal extremities of the fingers.
On arrival to the operating room, monitoring with electrocardiography on lead II derivation, SPO 2 , noninvasive blood pressure was instituted. Baseline parameters were within normal limits. A 22-G catheter was used for venipuncture in the left hand without complaints of discomfort. After venipuncture, the patient received injection ondansetron 1 mg i.v and injection midazolam 0.5 mg i.v, injection fentanyl 15 mcg i.v., while administering 100% O 2 with mask anesthesia was induced with injection propofol 30 mg i.v., followed by injection succinylcholine 25 mg i.v and intubation done with 5 mm cuffed endotracheal tube. Anesthesia was maintained with injection vecuronium at frequent intervals. Monitoring of end tidal CO 2 and esophageal temperature were added. Controlled ventilation with an N 2 O:O 2 2:1 l/min and sevoflurane 0.25% was instituted. Esophageal temperature was measured throughout the surgery and kept around 37°C with proper adjustment of room temperature. Surgical table was cushioned properly, to prevent injury during recovery.
The surgery went on for 2 h. The muscle relaxant was reversed with neostigmine 0.75 mg and glycopyrrolate 0.1 mg and extubation was uneventful. She was transferred to postanesthetic Care Unit (PACU) with monitoring and was receiving oxygen with the nasal cannula (2 L/min). There was no complaint of pain and no analgesics were administered postoperatively. The temperature of the patient remained stable in PACU (around 37°C) and was shifted toward next day.
The biopsy reports of skin and nerve, obtained after 1 week, are suggestive of CIPA (type IV).
| Discussion|| |
Congenital insensitivity to pain syndrome with anhydrosis or HSAN type IV or familial dysautonomia type II. It is due to mutation in the NTRK1 gene. Since neurotrophic receptor is a receptor for nerve growth factor  there is a failure of differentiation and migration of neural crest. Cells resulting in the complete absence of small myelinated and unmyelinated nerve fibers in patients with CIPA. 
Main goals of anesthetic management in patients with CIPA includes the prevention of psychological stress by minimizing apprehension and anxiety, maintenance of normal body temperature in the peri-operative period and adjustment of the anesthetic depth to prevent intra-operative awareness while avoiding high concentration that may cause hypotension, bradycardia or cardiac arrest. ,
Some of the patients have a tactile hyperesthesia which cause an uncomfortable perception during surgical manipulation , and also cause complaints of postoperative pain.
The premedication helps to relieve patients from anxiety and also reduce the risk of fever caused by excitement. In our case, the patient was premedicated with midazolam 0.5 mg i.v. Because of the presence of anhydrosis it has been recommended to avoid anticholinergics in patients with CIPA.  However, our patient received anticholinergics during reversal without any adverse effects, being consistent with the previous study (Hardman et al.). 
Controlling body temperature is another crucial perioperative task. They rely on a strict management of environmental temperature to maintain normal body temperature. They are at risk of both hyperthermia and hypothermia. Therefore, anesthesiologists should pay attention to temperature in the operating and recovery room. Throughout the intra-operative period, we monitored esophageal temperature and was remained within normal limits.
The patient should be carefully placed on the surgical table, whose surface should be padded to prevent pressure injury and reduce the risk of new trauma secondary to involuntary movements during awakening. ,
It has been proposed that patients with CIPA may require lesser concentrations of anesthesia because they do not feel pain.  However Okuda et al.  reported that the anesthetic requirement differed according to the type of surgeries, in their report the required concentration of anesthetics was over 1 minimum alveolar concentration (MAC) was required for orthopedic surgeries and <1 MAC for dental surgeries.
Most anesthetic drugs have been used safely in CIPA patients without unusual effects, , including atropine, meperidine, fentanyl, succinylcholine, atracurium, pancuronium, vecuronium, ketamine, propofol, barbiturate and benzodiazapines. , Modern inhalation anesthetics agents, including enflurane, isoflurane, and sevoflurane have been reported to have the usual effects either with or without nitrous oxide in oxygen. 
In our case, depth of anesthesia was maintained with injection fentanyl 15 mcg i.v, injection vecuronium and sevoflurane 0.25% with N 2 O:O 2 (66:33%).
Bispectral index (BIS) is a reliable monitor for the titration of anesthetics to ensure unconsciousness in pediatric CIPA patients while avoiding excessive concentrations. BIS monitoring was not applied due to unavailability.
| Conclusion|| |
Anesthesia is necessary to provide unconsciousness and to prevent the stress response in patients with CIPA, taking care of temperature control.
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