The following information is provided in order to assist physicians and anesthesiologists when performing surgery on children with mastocytosis. All journal citations are listed below.

Our first section is dedicated to the information found in the article:

Anesthesia and Analgesia (1986) 65:S1-S170, "Anesthetic management of systemic mastocytosis: Experience with 42 cases," written by W.C.V. Parris, MD, et al.

The article says:

Both regional and general anesthesia can produce life-threatening complications in mastocytosis patients undergoing surgical procedures. In an attempt to decrease the morbidity and mortality associated with this clinical syndrome, the following perioperative measures were adopted for the management of our patients:

Preoperative control with H1 and H2 histamine antagonists.

The use of intradermal skin tests to predict the patients who may develop reactions to drugs that may be used electively or on an emergency basis if a complication develops.

Preoperative sedation with oral diazepam.

Administration of pre-induction dose of 25-50 mg diphenhydramine IV.

Continuous perioperative availability of epinephrine infusion (1 mg in 25 mg of normal saline) and epinephrine for bolus administration (1:100,000). The dose used to manage acute exacerbation of systemic mastocytosis was 1 - 3 micrograms/kg IV.

The use of Isoflurane for the induction (including the intubation of the trachea) and maintenance of general anesthesia.

The use of regional anesthesia when appropriate in order to minimize the number of pharmacological agents used for a given procedure.

Anesthesia in UP/SM

This patient suffers from urticaria pigmentosa which is a form of mastocytosis. In these patients anesthesia poses a special risk, precautions that should be taken are listed below:

The temperature in the OP room should not be too cold (this also includes all the metal parts the patient has contact to, as well as the infusions).
Avoid skin irritation (rubbing etc.).
Avoid upsetting the patient; be calm and quiet.
Careful with nasal tubes (easy bruising and nose bleeds).
Before narcosis H1 and H2 blockers should be administered intravenously.
Steroids should be administered pre-operatively.
Pre-operative sedation with diazepam.
Try to avoid atropine.
If muscle relaxing agents are used, don’t give them as a bolus, but slowly over a period of about 1 minute.
Use a relaxing agent with low potential of histamine liberation (e.g., atracurium). Do not use pancuronium.
If local anesthesia is needed, use amide-type and not ester-linked local anesthetic.
Stay away from medication with preserving additives methylparaben and para-amino-benzoic acid.
An intra-dermal skin test of drugs likely to be used during anaesthesia can be indicated.
Have i.v. adrenaline/epinephrine at hand in case histamine release occurs.

The following drugs have been described to be directly or indirectly associated with mast-cell degranulation: Lidocaine, morphine, oxymorphone, codeine, d-tubocurarine, metocurine, acetylsalicylic acid, etomidate, thiopental, succinylcholine, enflurane, and isoflurane.

The following articles deal with anesthesia in mastocytosis/urticaria pigmentosa patients:

2001

Auvray, L., Letourneau, B., and Freysz, M. Mastocytose. Anesthésie générale par rémifentanil et sévoflurane. Ann Fr Anesth Réanim 2001; 20:635-638. (French.)

2000

Krauss, B. and Green, SM. Sedation and analgesia for procedures in children. N Eng J Med 2000; 342:938-945. (Not specific for UP/SM.)

1998

Borgeat, A. and Ruetsch, Y.A.. Anesthesia in a patient with malignant systemic mastocytosis using a total intravenous anesthetic technique. Anesth Analg 1998; 86:442-444.

Vaughan, S.T.A. and Jones, G.N. Systemic mastocytosis presenting as profound cardiovascular collapse during anaesthesia. Anaesthesia 1998; 53:804-809.

1995

Koitabashi, T. and Takino, Y. (Anesthetic management of a patient with urticaria pigmentosa). Masui. Japanese Journal of Anesthesiology 1995; 44:270-281. (Japanese.)

1994

Sala, X., et al. Anestesia de una paciente afectada de mastocitosis sistémica. Revista Espanola de Anestesiologia y Reanimacion 1994; 41:337-8. (Spanish.)

1993

Brodier, C., et al. Anesthésie d’un enfant porteur d’une mastocytose cutanée. Cahiers d’Anesthésiologie 1993; 41:77-79. (French.)

1992

Delalande, J.P., et al. Absence de dégranulation des mastocytes lors d’une anesthésie générale chez une enfant atteinte de mastocytose. Ann Fr Anesth Réanim 1992; 11:393-394.

Yaniv R., et al. Anesthetic considerations in mast-cell proliferative disease (urticaria pigmentosa and mastocytosis). Harefuah 1992; 122(12):780-784. (Hebrew.)

1991

Goins, V.A. Mastocytosis- perioperative considerations. AORN-J 1991; 54:1227-1238.

1990

Desborough, J.P., et al. Massive histamine release in a patient with systemic mastocytosis. Brit J Anaesth 1990; 65:833-836.

Greenblatt, E.P. and Chen, L. Urticaria pigmentosa: an anesthetic challenge. J Clin Anesth 1990; 2:108-115.

Lerno G., et al. Anaesthetic management of systemic mastocytosis. Brit J Aneasth 1990; 65:254-257.

1987

Hosking, M.P. and Warner, M.A. Sudden intraoperative hypotension in a patient with asymptomatic urticaria pigmentosa. Anesth Analg 1987; 66:344-346.

James, P.D., et al. Cutaneous mastocytosis in children : anaesthetic considerations. Can J Anaesth 1987; 34:522-524.

1986

Parris, W.C.V., et al. Anesthetic management of systemic mastocytosis: experience with 42 cases. Anesth Analg 1986; 65:S117. (Abstract.)

1983

Basta, S.J., et al. Histamine-releasing potencies of atracurium, dimethyl tubocurarine and tubocurarine. Br J Anaesth 1983; 55:105S-106S.

Moss, J. and Rosow, C.E. Histamine release by narcotics and muscle relaxants in humans. Anesthesiology 1983; 59:330-339.

Scott H.W., et al. Hazards in operative management of patients with systemic mastocytosis. Ann Surg 1983; 197:507-514.

1982

Roberts L.J., et al. Shock syndrome associated with mastocytosis: pharmacologic reversal of the acute episode and therapeutic prevention of recurrent attacks. Advances in Shock Research 1982; 8:145-152.

1981

Parris, W.C.V., et al. Anesthetic management of mastocytosis. Anesth Rev 1981; 8:32-35.

1980

Coleman, M.A., et al. General anesthesia in a child with urticaria pigmentosa. Anesth Analg 1980; 59:704-706.

1973

Rosenbaum, K.J. and Strobel, G.E. Anesthetic considerations in mastocytosis. Anesthesiol 1973; 38:398-401.