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Journal of Disability and Oral Health (2007) 8/3 124–128
Mastocytosis – oral and dental
manifestations and medical
considerations for dental treatment: a
case report
Esti Davidovich DMD, MSc1, Lev Ronin MD2 and Diana Ram Dr.
Odont3
1
Clinical Instructor, 3Senior Clinical Lecturer, Department of Paediatric Dentistry, The Hebrew UniversityHadassah School of Dental Medicine, Jerusalem, Israel. 2Department of Anaesthesiology and Critical Care
Medicine, Hadassah Medical Centre Jerusalem, Israel
Abstract
The present case emphasises the importance of a multidisciplinary approach in treating a 6-year-old boy
suffering from systemic mastocytosis. Mastocytosis is characterised by mast cell proliferation and
accumulation within various organs, the most commonly affected of which is skin. The patient was
scheduled for treatment under general anaesthesia. The medical, dental and anaesthetic considerations
and treatment are presented.
Key words: Mastocytosis, oral findings, dental treatment, anaesthesia, perioperative medicine
Introduction
Mastocytosis is a clonal disorder of the mast cell due to
proliferation and accumulation of these cells and their
precursors within different tissues (bone marrow, skin, GI
tract and spleen). The skin is the most frequently affected
organ. The most common cutaneous form of mastocytosis is urticaria pigmentosa (UP), and is characterised by
red-brown macules, papules, or plaques ranging in number
from a few to thousands. Lesions may vesiculate in infancy
(Hogan, 2005; Metcalfe, 2005; Valent et al., 2005).
The increase in mast cell numbers in most patients is
probably due to activating mutations in the c-kit receptor
(Worobec et al., 1998). Another important pathogenetic
cause is the abnormal expression of cell surface adhesion
antigens on neoplastic mast cells (Metcalfe, 2005).
Mast cells contain a range of active mediators in their
granules, which are preformed and stored. They produce
generated membrane-derived lipid mediators and are a
source of multifunctional cytokines. The pathophysiology
of mast cell disease can be divided into systemic and local
effects. The systemic effects are related to the response of
the tissue to the release of the above-mentioned mediators
from the mast cells, or to local mast cell burden, or associated non-mast cell haematological disorders (Braverman,
1998; Koyamangalath, 2005). Mediator-related symptoms
can include pruritus, flushing, syncope, gastric distress,
nausea and vomiting, diarrhoea, bone pain and neuropsychiatric disturbances. Systemic manifestations may cause
severe end-organ dysfunction, for example, hepatosplenomegaly and bone marrow dysfunction (Escribano, 2006). Local
manifestations of this disease arise from the effects of local
collections of mast cells (Braverman,1998; Koyamangalath,
2005).
The onset of mastocytosis occurs before the age of 2
years in 55% of the patients and from 2 to 15 years old in
10% of the patients. Most of the reported cases are in
Caucasians, with males and females being equally affected.
The prognosis depends on the age of onset; and is usually
more transient and self-limited in children than adults (Hogan,
2005; Koyamangalath, 2005). Systemic persistent disease,
higher risk of malignant transformation and poorer prognosis are found when the onset is after the age of 10-yearsold (Hogan, 2005; Metcalfe, 2005; Valent et al., 2005).
Therapy is primarily symptomatic since no therapy is
curative. Symptoms are variably controlled by adequate
medications. Management of patients within all categories
of mastocytosis includes:
• Careful counselling of patients (or the parents in case of
paediatric patients) and care providers
• Avoidance of factors triggering acute mediator release
(Table 1 summarises the substances that induce mast cell
mediator release)
Davidovich et al.: Mastocytosis, oral findings, dental treatment
125
Table 1. Factors that may induce mediator
release from mast cells and should be avoided
Pharmacological agents
salicylates (Aspirin)
NSAIDs
codeine
morphine
thiamine
quinine
opiates
gallamine
decamethonium
procaine
radiographic dyes
dextran
polymyxin B
scopolamine
D-tubocurarine
Food and beverages
crawfish
lobster
alcohol
spicy foods
hot beverages
cheese
Activity
physical stimuli
emotional stress
temperature extremes
physical exertion
Miscellaneous
bacterial toxins,
insect stings
Table 2. Indication for the use of different kind of medications in mastocytosis
Drug
Indication
H1 and H2 antihistamines
Corticosteroids
Epinephrine
To decrease pruritus, wealing, flushing, GI symptoms
Injections to cutaneous lesions, for the control of malabsorption
Acute anaphylaxis
• Treatment of acute and chronic mast cell-mediator
symptoms
• If necessary, treatment of organ infiltration by mast cells
(Escribano, 2006).
The different medications and indications for use are
presented in Table 2. (Hogan, 2005; Metcalfe, 2005; Valent et
al., 2005). Complications include possible transformation
to a haematologic malignancy, death secondary to mast cell
degranulation and Mast Cell Leukaemia (Braverman, 1998;
Koyamangalath, 2005; Metcalfe, 2005).
There is a paucity of reports in the dental literature
regarding mastocytosis. A case of systemic mastocytosis of
a 4.5-year-old paediatric dental patient with mastocytosis
has been reported (Nelson and Savelli-Castillo, 2002). In this
reported case, the planned dental treatment was provided
in an ambulatory setting utilising only nitrous oxide-oxygen
analgesia to provide sedation, with no local anaesthesia, and
H1 and H2 antihistamines were used to prevent mast cell
degranulation. The dental treatment provided included
fissure sealants and shallow, resin-composite fillings. During
general anaesthesia, trauma, stress, extreme temperatures
and drugs may precipitate intra-operative mast cell degranulation.
The aim of our case report is to describe the dental
treatment under general anaesthesia of a child with systemic
mastocytosis, and the medical considerations associated with
the dental treatment.
Case report
A 6-year-old boy was referred to the Department of
Paediatric Dentistry at the Hebrew University, Hadassah
School of Dental Medicine in Jerusalem by his paediatri-
126 Journal of Disability and Oral Health (2007) 8/3
cian for dental treatment due to a dento-alveolar abscess
and tooth pain. The boy was diagnosed as suffering from
systemic mastocytosis, and no clinical signs of the disease
were apparent. Consultation with his paediatrician revealed
that he only suffered from cutaneous manifestations and no
other organs were involved. He was mainly treated with
antihistamines as necessary.
On his first visit, the boy was not cooperative. Clinical
examination revealed extensive caries and poor oral health.
A dento-alveolar abscess related to the second maxillary
primary left molar was clinically diagnosed and Amoxicillin
750mg/day was prescribed. Due to the extensive dental
treatment plan, the lack of cooperation of the child, and
the possibility of flare up of the disease with a concern of
oedema, the boy was scheduled for dental treatment under
general anaesthesia (GA).
The treatment plan included a strict prevention protocol: prophylaxis, restorative treatment, extractions, fluoride
application and meticulous oral hygiene instructions.
Figure 1. Carious lesions in the mandibular incisors as well
as in the maxillary incisors and canines. Right maxillary
incisor is missing.
Anaesthetic management
The child was pre-medicated with Midazolam syrup 0.4mg/
kg. The induction of anaesthesia was performed by inhalation of sevoflurane and nitrous-oxide followed by the
insertion of an I.V. catheter; 2mg/kg of Propofol I.V.
was administrated followed by nasal intubation with careful fixation.
Anaesthesia was maintained by propofol infusion at 10–
15mg/kg/h. Mechanical ventilation of lungs was instituted
using a 2:1 nitrous-oxide/oxygen mixture. Standard monitoring (E.C.G., pulse oximeter, NJBP, Et CO2 analyser) was
used. During the anaesthesia the patient was haemodynamically stable and the recovery was uneventful.
Dental management
Local anaesthesia (lidocaine 2% with epinephrine 1:100,000)
was added to perform the dental extractions. The child had
deep carious lesions in all his primary teeth.
The right maxillary incisor was missing. (Figures 1–3a and
b). A rubber dam covered with Vaseline was used in order
to prevent direct contact with the skin.
The carious and mobile upper primary lateral and
central left incisors were extracted. The maxillary primary
second molars were extracted due to severe pulp inflammation and poor prognosis. Since extensive bleeding due
to heparin release from the mast cells could be expected,
local haemostatic agents (Gel-foam, topical thrombin,
microfibrillar collagen and sutures) were prepared in
advance. No excessive bleeding was observed and no
haemostatic agents were used.
Pulp haemostasis was achieved in the mandibular
primary second molar, and a pulpotomy and placement of
a stainless steel crown were successfully performed on this
tooth. The other decayed teeth were restored with resinbond-composite restorations.
Figure 2. Deep carious lesions involving the maxillary
primary teeth
After two hours supervision in the recovery room the
child was admitted to the Paediatric Department of the
Hadassah Children’s Hospital, and remained under observation for 24 hours. Four days later, when he was seen at
a recall examination, the oral mucosa appeared intact.
Neither pain nor sensitivity was reported.
Discussion
This case report presents the dental management of a 6year-old boy with systemic mastocytosis who urgently
needed dental treatment due to extensive dental caries and
pain. The complexity of the disease and the clinical manifestations in this patient required a multidisciplinary approach
before dental treatment could be performed.
The main goal in treating a child with mastocytosis is to
avoid triggers that can cause mast cell degranulation. Dental
treatment in general and dental treatment under general
anaesthesia (GA) in particular are stressful events that may
cause acute mediator release. However in this case, GA was
preferred in order to minimise the number of stressful
episodes, as well as to allow meticulous monitoring and the
possibility of appropriate reaction to an anaphylaxis shock.
In addition, due to the lack of cooperation of the child, this
Davidovich et al.: Mastocytosis, oral findings, dental treatment
Figure 3a. Deep carious lesions involving the right
mandibular teeth.
127
Figure 4. lack of pulp haemostasis after pulp exposure due
to deep caries.
and pressuring additives such as methylparben) should be
avoided. Amide-type of local anaesthetics with adrenaline
were used as recommended. Relaxation agents, with a low
potential for histamine release, for example, veceronium,
were used. All these precautions were helpful in providing
the child with a safe dental treatment with minimum risk.
Dental considerations
Figure 3b. Deep carious lesions involving the left
mandibular teeth.
form of behaviour management was deemed necessary.
Previous reports had presented a number of complications
in patients with mastocytosis undergoing GA and include
anaphylaxis, cardiovascular collapse, bleeding and even death
(Borgeat and Ruetsch,1998; Auvray et al., 2001).
Pre-medication, to control anxiety, is generally recommended before general anaesthesia in children. However, in
children suffering from mastocytosis it is imperative to
provide it since anxiety increases mast cell activity. Intubations
should be performed with special care so as not to bruise
or to provoke bleeding, or skin irritation.
The patient should be kept stable, avoiding low temperatures. It is recommended to provide preventive H1 and
H2 blockers and to have IV adrenaline ready to use in the
case of emergency. Drugs that are directly or indirectly
associated with mast cell degranulation (including atropine
Primary and permanent teeth were restored with resin
bonded composite restorations, pulpotomies and stainless
steel crowns. Extraction of primary molars was indicated,
since haemostasis of the pulp was not achieved after pulp
exposure due to deep caries (Figure 4). The reason for the
bleeding can be attributed to heparin release from mast cells,
or the status of the dental pulp. However, no bleeding problems were observed after extractions, and spontaneous
haemostasis was observed after a normal time.
Our dental findings were dissimilar to Nelson and SavelliCastillo (2002), who reported dental treatment performed
in the dental chair without local anaesthesia.
In the present case, pulp treatment and extractions were
needed, and local anaesthesia was imperative. In addition
the child was not cooperative, and nitrous-oxide sedation
was insufficient to control the child’s behaviour.
Meticulous oral hygiene instructions were given to the
parents, including the need to use a fluoridated dentifrice
daily to help to prevent caries and to brush in order to
prevent gingivitis. The child was scheduled for periodic
follow up examinations.
Conclusion
The present case describes the general and oral manifestations in a 6-year-old, uncooperative boy who suffered from
mastocytosis, and who needed full mouth restorative treatment. The importance of a multidisciplinary approach is
128 Journal of Disability and Oral Health (2007) 8/3
emphasised: the roles of the paediatrician, the anaesthesiologist and the paediatric dentist were crucial in the planning and performance of the dental treatment.
References
Auvray L, Letourneau B, Freysz M. Mastocytosis: general anesthesia
with remifentanil and sevoflurane. Ann Fr Anesth Reanim 2001;
20: 635–638.
Borgeat A, Ruetsch YA. Anesthesia in a patient with malignant systemic
mastocytosis using a total intravenous anesthetic technique. Anesth
Analg 1998; 86: 442–444.
Braverman IM. Hypersensitivity syndromes. In: Braverman IM (eds)
Skin Signs of Systemic Disease, 3nd edn. Philadelphia: WB Saunders
Co; 1998:465–466.
Hogan D. Mastocytosis. eMedicine 2005.com.Inc; http://www.
emedicine.com/derm/topic258.htm#www. Last accessed June,
2005.
Escribano L, Akin C, Castells M et al. Current options in the treatment
of mast cell mediator-related symptoms in mastocytosis. Inflamm
Allergy Drug Targets 2006; 5: 61–77.
Koyamangalath K. Systemic mastocytosis. eMedicine 2005.com.Inc;
http://www.emedicine.com/med/topic1401.htm. last accessed
June, 2006
Metcalfe DD. Regulation of normal and neoplastic human mast cell
development in mastocytosis. Trans Am Clin Climatol Assoc 2005;
116: 185–203.
Nelson LP, Savelli-Castillo I. Dental management of a pediatric patient
with mastocytosis: a case report. Pediatr Dent 2002; 24: 343–
346.
Valent P, Akin C, Sperr W et al. Mastocytosis: Pathology, genetics,
and current options for therapy. Leuk Lymphoma 2005; 46: 35–
48.
Worobec AS, Semere T, Nagata H et al. Clinical correlates of the
presence of the Asp816Val c-kit mutation in the peripheral blood
mononuclear cells of patients with mastocytosis. Cancer 1998;
83: 2120–2129.
Address for correspondence:
Dr. Diana Ram
Department of Paediatric Dentistry
The Hebrew University-Hadassah School of Dental Medicine
P.O.B 12272
Jerusalem 91120, Israel
Email: [email protected]