[Downloaded free from http://www.aeronline.org on Monday, October 12, 2015, IP: 117.192.194.

137]

A E R Editor‑in‑Chief :
Mohamad Said Maani Takrouri
(KSA)
Open Access
HTML Format Anesthesia: Essays and
For entire Editorial Board visit : http://www.aeronline.org/editorialboard.asp
Researches

Case Report
Calcinosis cutis: A rare cause for difficult airway
Nethra H. Nanjundaswamy

Department of Anaesthesiology, Bangalore Medical College and Research Institute, Bengaluru, Karnataka, India
Corresponding author: Dr. Nethra H. Nanjundaswamy, #SF4, VV Apartments, Contour Road, Padmanabhanagar, Bengaluru ‑ 560 070, Karnataka, India.
E‑mail: drnethrahn@gmail.com

Abstract
Calcinosis cutis is a rare clinical condition wherein calcium is deposited in the layers of the skin.
Various types of calcinosis cutis have been described based on histology and location. Airway may
be involved only if calcinosis cutis is associated with overlapping syndrome of connective tissue
disorders and muscular dystrophy. I report a rare case of dystrophic calcinosis cutis without any
connective tissue disorders with the unanticipated difficult airway. A young lady with dystrophic
calcinosis cutis of the gluteal region was posted for surgical excision and flap cover procedure
under general anesthesia. Unanticipated difficult airway situation arised and was effectively
managed. Patient had an uneventful recovery.

Key words: Calcinosis cutis, Raynauds phenomenon Esophageal dysmotility, Sclerodatyly and
Telengiectasia syndrome, connective tissue disorders, dystrophic calcinosis cutis, idiopathic
calcinosis cutis

INTRODUCTION CASE REPORT

Calcinosis cutis is a clinical entity wherein the insoluble
calcium salts are deposited in the layers of the skin.
Calcinosis cutis rarely exists as a single disease process.
It is frequently associated with autoimmune connective
tissue diseases or secondarily to imbalances in electrolyte
physiology. There are very few case reports describing the
clinical presentation and treatments. The difficult airway
is very rarely encountered in calcinosis cutis. However,
the difficult airway has to be anticipated in cases with
associated connective tissue disease.
Access this article online
Website DOI
www.aeronline.org 10.4103/0259-1162.159768
An 18‑year‑old female presented to the Department of
Plastic Surgery in surgical super specialty block. History
revealed repeated administration of intramuscular
injections at bilateral gluteal regions. Clinical examination
showed hard mass in bilateral gluteal area. Ultrasound
scan of the gluteal areas showed hard lesions with
pockets of necrosis within. Biopsy revealed calcified
tissue. Patient was further evaluated by dermatologists
for connective tissue disorders and muscular dystrophies.
Laboratory investigations for rheumatoid arthritis,
systemic lupus erythematousus (SLE) were done. Patient’s
clinical examination, blood investigations for connective
Quick Response Code
tissue disorders and muscular dystrophies were negative.
The lady was posted for bilateral gluteal mass excision
and flap cover procedure under anesthesia. Patient’s
preanesthesia check‑up was done a day prior to surgery.
Lady gave a history of getting intramuscular injections
for fever and body ache from adolescence. Lady

405
[Downloaded free from http://www.aeronline.org on Monday, October 12, 2015, IP: 117.192.194.137]
Anesthesia: Essays and Researches; 9(3); Sep-Dec 2015 Nanjundaswamy: Difficult airway in calcinosis cutis
weighed 40 kilos and height of 150 cm. General physical
examination showed thickened skin and small discrete
bony masses beneath the skin in areas like face, elbows,
knees and surgical site. Airway examination‑mouth opening
vertically allowed three fingers. Angles of the mouth
were inelastic and allowed three fingers with difficulty.
Jaw and neck were normal with a normal range of neck
movements. Systemic examination was normal. There was
restriction in the movement of the hip joint due to hard
mass over gluteals. Fungal infection was present on the
back till lumbar region. Anesthesia technique planned was
general anesthesia with the endotracheal tube as position
needed for surgery was the prone position.
Nil per oral guidelines were advised, and a written
informed consent was taken from the patient Tablet
Ranitidine 150 mg and Tablet Alpralozam 0.5 mg was
advised for the night. On the day of surgery intravenous
line was secured in preoperative holdup area and
intravenous premedication of injection midazolam 1 mg
was administered. Patient was shifted to operation
theater and preinduction monitors for pulse oximetry,
electrocardiography and noninvasive blood pressure
were placed. Premedicated with the injection fentanyl
2  mcg/kg; preoxygenated for 3  min and induced with
injection propofol 2.5 mg/kg. After confirming the ease of
mask ventilation, injection rocuronium bromide 0.9 mg/kg
was administered. Ventilated with air‑oxygen mixture and
sevoflurane. Laryngoscopy was tried with Macintosh
blade 3 at a neuromuscular score of 0 and MAC of 1.
Cormack–Lehane view was grade 4. BURP maneuver
did not improve the view. Second dose of injection
propofol 1 mg/kg was administered, and laryngoscopy
was reattempted with MacCoy blade 3. Bougie was used
and no. 7.5 Endotracheal tube was placed and secured
with tape. Positioning of the tube was confirmed with
capnography tracing and auscultation. Patient was
positioned in a prone position with adequate padding at
pressure points. Intraoperatively patient was maintained
on IPPV with isoflurane, air‑oxygen and intermitted doses
of rocuronium and injection fentanyl.
Surgical duration lasted 3  h. Patient was turned supine
and extubated when fully awake. Patient was discharged
on 7th post‑operative day. Postprocedure follow‑up was
done and histopathology report showed calcification
in the dermis layer. This confirmed the diagnosis of
calcinosis cutis.
DISCUSSION
Calcinosis cutis refers to a rare clinical condition in which
calcium salts are deposited in the skin.[1] Calcium deposits
are often found in the dermis. The etiopathogenic
classification for deposition of calcium in the dermis may
be divided into dystrophic, metastatic, iatrogenic and
idiopathic.[1,2]
406
Serum levels of calcium and phosphates may be altered
in cases of calcinosis cutis. In dystrophic calcinosis
cutis, calcium deposits take place secondary to tissue
injury following infection, inflammation, injury, trauma
and venous stasis. In metastatic calcinosis cutis, the
serum calcium levels are elevated, and changes in serum
phosphorus levels are also seen. The metastatic calcinosis
occurs in renal failure, hyperparathyroidism, sarcaidosis
and excessive intake of milk and alkali. Iatrogenic
calcinosis occurs secondary to occupational exposure, local
tissue damage and elevated levels of serum calcium levels.
Iatrogenic calcinosis cutis arises secondary to a treatment
or procedure like parenteral administration of calcium
or phosphate, parental inorganic phosphate tumor lysis
syndrome and repeated heel pricks in newborn. Idiopathic
calcinosis occurs without any predisposing factor for
calcium deposition, the absence of known tissue injury or
systemic metabolic defect. Serum calcium and phosphate
levels will be normal in idiopathic calcinosis.[2,3] The
reported sites for idiopathic calcinosis are eyelid, vulva,
scrotum, subepidermoid nodules and around tumors.[1‑4]
Depending on the type of deposition calcinosis cutis
is divided into calcinosis circumscripta and calcinosis
universlis. In calcinosis circumscipta the deposition is in a
well‑demarcated area and in calcinosis universalis there is
widespread deposition of calcium in the skin.[4‑6]
Calcinosis cutis may be found in various overlapping
connective tissue disorders like scleroderma, rheumatoid
arthritis, and dermatomyositis and rarely in SLE.[6]
Pathophysiology for ectopic calcium deposit is not clear.
There are a few proposed hypotheses for calcinosis cutis.
These hypotheses propose that an alteration in tissue
alkaline phospatase secondary to genetic predisposition,
tissue necrosis, decreased vascularity, tissue trauma
may lead to inhibition of the activity of tissue alkaline
phosphatase and lead to deposition of calcium. Other
proposed mechanism is that in areas of tissue damage,
cell phosphates may bind to denatured protein and lead
to calcium deposits. Genetic predisposition may lead to
an abnormality in the proportion of collagen and elastin
deposits at the site of trauma and inflammation, leading
to thick and calcified skin.[1,2,5]
Pertaining to anesthesia considerations, calcinosis cutis
may be associated with CREST syndrome which involves
Calcinosis cutis, Raynauds phenomenon Esophageal
dysmotility, Sclerodatyly and Telengiectasia. CREST
syndrome may be associated with difficult airway as
microstomia, stiff temporomandibular joint and ineffective
BURP maneuver.[6,7] Other problems in CREST syndrome
are related to thick skin, cutaneous bleeding and stiff
joints, which pose problems for securing arterial, venous
lines and for positioning.[7]
Our patient did not have symptoms and signs of
CREST syndrome. Patient was also evaluated for SLE,
[Downloaded free from http://www.aeronline.org on Monday, October 12, 2015, IP: 117.192.194.137]
Anesthesia: Essays and Researches; 9(3); Sep-Dec 2015 Nanjundaswamy: Difficult airway in calcinosis cutis
muscular dystrophy, and rheumatoid arthritis. Clinical
examination and blood tests were negative for above
mentioned diseases.
Treatment for calcinosis cutis includes oral therapy with
probenecid, warfarin, diltiazem, biphosphonates and
colchicides. However, these treatments are not good enough
to prevent the progress or to cure. Other modalities for
treatment are intravenous immunoglobulins, intraleisonal
instillation of corticosteroids, carbon‑di‑oxide laser.[3‑5]
For large lesions‑extracorporeal lithotripsy, curettage and
surgical excision have been tried with equivocal results.
Surgical excision of the calcified part is the last resort.
However, calcinosis cutis may occur at the operated site
and needs follow‑up of the patient.
Lagoo and George[7] has discussed the anesthetic
considerations in the management of calcinosis cutis
with SLE. There are not many reports of calcinosis cutis
case requiring anesthesia. This case report insists on
the anesthetist to check for overlapping syndromes and
possibility of airway involvement in a simple case of
calcinosis cutis.
SUMMARY
Calcinosis cutis is a rare clinical entity encountered in
surgical practice. It is rarer to get calcinosis cutis case
requiring anesthesia for surgical procedure. This clinical
condition needs to be evaluated for connective tissue
disorders, muscular dystrophies, CREST syndrome and
chronic systemic illness altering calcium metabolism.
A careful evaluation of airway should be done and difficult
airway cart needs to be kept available while anesthetising
calcinosis cutis case.
REFERENCES
1. Muddegowda PH, Lingegowda JB, Ramachandrarao RK, Konapur PG.
Calcinosis cutis: Report of 4 cases. J Lab Physicians 2011;3:125‑6.
2. Firdous F,  Afroz IA, Quadri SS, Begum A. Mixed variant calcinosis cutis.
J Med Allied Sci 2012;2:84‑6.
3. Kayhan TC, Temiz P, Ermertcan AT. Calcinosis cutis on the face. Indian J
Dermatol Venereol Leprol 2009;75:180‑1.
4. Coban YK, Aytekin AH, Aydin EN. Idiopathic calcinosis cutis of the vulva.
Indian J Dermatol 2013;58:464‑6.
5. Jun I, Kim SE, Lee SY, Kim GJ, Yoon JS. Calcinosis cutis at the tarsus of the
upper eyelid. Korean J Ophthalmol 2011;25:440‑2.
6. Carocha AP,   Torturella DM, Barreto GR, Estrella RR, Rochael MC.
Calcinosis cutis universalis associated with systemic lupus erythematosus:
An exuberant case.  An Bras Dermatol 2010;85:883‑7.
7. Lagoo JY, George B. Ultrasonography Guided Supraclavicular Brachial
Plexus Block for Excision of Multiple Calcinosis Cutis in CREST Syndrome:
A Safe Approach. Available from: http://www.slja.sljol.info/articles/
abstract/10.4038/slja.v22i1.6112/. [Last accessed on 2015 Jun 15].
How to cite this article: Nanjundaswamy NH. Calcinosis cutis: A
rare cause for difficult airway. Anesth Essays Res 2015;9:405-7.
Source of Support: Nil, Conflict of Interest: None declared.
407