Annual incidence is estimated at 1/150.000. Intratissular proliferation of mastocytes linked, in 86% of cases, to a mutation of the receptor with C-KIT tyrosine kinase activity. The symptoms (mainly cutaneous and cardiovascular, rarely pulmonary) are related to the release of mediators (histamine, tryptase, prostaglandins, heparin, leukotrienes, cytokines) either spontaneously or in response to immunological or non-immunological stimuli. Two peaks of incidence: before the age of 2 years and in early adulthood. 

Several clinical forms are described:

1. cutaneous mastocytosis, most frequent in children

-         urticaria pigmentosa or  maculopapular mastocytosis: maculopapular red to brown, often pruritic lesions; mainly on the trunk;

-         cutaneous mastocytoma: hemispherical nodule, solitary or multiple, brownish, up to several cm in diameter;

-         diffuse cutaneous mastocytosis: the infiltrated with mast cell skin takes a  yellow-buff orange color peel aspect; occasional bullous lesions. This form of mastocytosis may resolve spontaneously; in case of mutations limited to skin tissue, a treatment with imatinib (a type II kinases inhibitor used in the treatment of chronic myeloid leukemia) has been used successfully [possible side effects: cardiomyopathy, slowing of growth, leukopenia, thrombocytopenia, elevation of liver enzymes].

2. systemic mastocytosis, very rare in childhood

3. systemic mastocytosis associated with a hematological disease, with or without skin damage: acute myeloid leukemia, non-Hodgkin's lymphoma

4. aggressive systemic mastocytosis:  mast cells leukemia, mast cells sarcoma, extracutaneous mastocytoma

However, some children with cutaneous mastocytosis present with episodes of abdominal pain and/or acute watery diarrhea associated with digestive impairment. In children, the prognosis for cutaneous mastocytosis is good because it regresses spontaneously at adolescence; only dermographism and hyperpigmentation persist.

Anesthetic implications: 

-        it is useful to know the patient's basal level of serum tryptase: it is generally increased.

-        premedication with antihistaminics H1 and H2 and/or corticoids ifthey are part of a chronic treatment; otherwise such a  premedication does not seem to be very useful.

-        it is recommended to avoid agents that can cause histaminoliberation (atracurium, mivacurium, nefopam, ACTH) Although many cases have been published where these agents were used uneventfully. Morphine can be used

-        caution with NSAID's and nefopam

-        avoid stress and emotional events.

-        maintain normothermia

-        mechanical factors such as aggressive surgical disinfection, can cause mast cells degranulation; as tissue ischemia and reperfusion may trigger aspecific mast cells degranulation, it is prudent to avoid any arterial tourniquet.

-        increased risk of anaphylactoid reactions: in case of hypotension or collapse, same treatment as for an anaphylactic reaction.

References : 

• Greenblatt EP, Chen L. 
  Urticaria pigmentosa: an anesthetic challenge. 
  J Clin Anesth 1990; 2:108-15.
• James PD, Krafchik BR, Johnston AE. 
  Cutaneous mastocytosis in children: anaesthetic considerations. 
  Can J Anaesth 1987; 34:522-4.
• Tirel O, Chaumont A, Ecoffey C. 
  Arrêt circulatoire au cours d'une anesthésie chez un enfant porteur d'une mastocytose. 
  Ann Fr Anesth Réanim 2001 ; 20 : 874-5.
• Carter MC, Uzzaman A, Scott DD, Metcalfe DD, Quezado Z. 
  Pediatric mastocytosis: routine anesthetic management for a comples disease. 
  Anesth Analg 2008; 107: 422-7.

• Dewachter P, Mouton-Faivre C, Cazalaà JB, Carli P, Lortholary O, Hermine O. 
  Mastocytoses et anesthésie. 
  Ann Fr Anesth Réanim 2008: 28: 61-73.
• Ahmad N, Evans P, Lloyd-Thomas AR. 
  Anesthesia in children with mastocytosis – a case based review.
  Pediatr Anesth 2009; 19: 97-107.

• Morren A-M, Hoppé A, Renard M, Debiec Rychter M, Uyttebroeck A, Dubreuil P, Martin L.
  Imatinib mesylate in the treatment of diffuse cutaneous mastocytosis. 
  J Pediatr 2013; 162: 205-7.
• Bridgman DE, Clarke RC, Sadleir PH, Stedmon JJ, Platt PR. 
  Systemic mastocytosis presenting as intraoperative anaphylaxis with atypical features : a report of two cases. 
  Anaesth Intensive Care 2013; 41: 116-21.
• Soria A, Chanderis M-O, Mouton-Faivre C, Hermine O, Dewachter P.
  Mastocytoses et anesthésie: conduit à tenir au cours de la période périopératoire.
  Le Praticien en anesthésie réanimation 2017 ; 21 : 21-8.
• Unterbuchner C, Hierl M, Seyfried T, Metterlein T.
  Anaesthesia and orphan disease : Rapid sequence induction in systemic mastocytosis.
  Eur J Anaesthesiol 2017; 34: 176-8
• Dewachter P, Kopac P, Laguna JJ, Mertes PM et al.
  Anaesthetic management of patients with pre-existing allergic conditions: a narrative review.
  Br J Anaesth 2019; 123 (1): e65-81.

Updated: August 2019