Calcinosis cutis: A rare cause for difficult airway.

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.

INTRODUCTION

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.

CASE REPORT

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 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 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.[12]

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.[23] The reported sites for idiopathic calcinosis are eyelid, vulva, scrotum, subepidermoid nodules and around tumors.[1234]

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.[456]

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.[125]

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.[67] 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, 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.[345] 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.