Rosai-Dorfman's disease presenting as vallecular mass: An anesthetic overview.

Rosai-Dorfman's disease (RDD) is a rare multisystemic histiocytic disorder, commonly presenting as cervical lymphadenopathy in a young male. Its extranodal form is very unusual. We report a case of extranodal RDD presenting as a large vallecular mass. Management of an airway mass is always a challenge to the anesthesiologist. This problem is accentuated when the mass is due to RDD, as RDD can involve multiple system of the body such as central nervous system, upper respiratory tract, orbit and eyelid, gastrointestinal tract, and skin. Autoimmune phenomenon though rare is also an important concern. Individualized plan based upon thorough evaluation and anticipation is the key for better outcome.

INTRODUCTION

Rosai-Dorfman's disease (RDD) is a rare multisystem histiocytic disorder. RDD most commonly presents as bilateral cervical lymphadenopathy in young males. Extranodal involvement is seen in 40%; the skin, central nervous system (CNS), orbit and eyelid, upper respiratory tract, and gastro intestinal tract are the common sites.[1] RDD could be self-limiting (20%) or may have a relapsing and remitting course (70%). Treatments such as surgery, chemotherapy, and radiotherapy. Are required in symptomatic patients or when the vital organ is involved. To say the least, the etiology of RDD is suspected to be immune dysfunction or often correlated with Epstein-Barr virus, Parvovirus B19 and human herpes virus infection. However, still the exact etiology is not known.[2] We report a rare case of RDD as vallecular mass and have discussed airway management and other anesthetic considerations in detail.

CASE REPORT

A 42-year-old male of 60 kg weight was posted with a plan for excision of a vallecular mass, on the 2nd day of his admission. He had a history of dysphagia to solid, plummy quality of voice since a month. He had no history of any other major illness. The patient had mild breathing difficulty. On local examination, there was no cervical lymphadenopathy. Airway examination revealed mouth opening more than three fingers and Mallampati grading-1. Electrocardiogram and chest X-ray were normal. Radiography of lateral neck was showing a large soft tissue mass in hypopharynx extending to the larynx. Contrast enhanced computed tomography (CECT) [Figure 1] showed a large mass of size 3.4 cm × 3.7 cm × 2.7 cm in hypopharynx, extending toward right side filling the valleculae, and posteriorly up to inlet of larynx. Routine hematological investigation showed raised erythrocyte sedimentation rate, leukocytosis, and hypergammaglobulinemia (with a reversal of immunoglobulin G/immunoglobulin A ratio). The renal and liver function test of the patient was within normal limit.

Figure 1

Contrast enhanced computed tomography of the neck showing a large vallecular mass

The patient was planned for awake fiberoptic-guided tracheal intubation (AFOI) in propped up position by the senior anesthesiologist. As the mass was significantly large, surgical tracheostomy was also planned as the second step as per the difficult airway algorithm. The patient was explained about AFOI, the planned alternative of surgical tracheostomy and consent was obtained. He was premedicated with IV ranitidine 50 mg, ondansetron 4 mg and glycopyrrolate 0.2 mg. The airway was nebulized with 5 ml of 4% lidocaine, and xylometazolin nasal drops were applied before admitting to operation theater (OT). In OT, the routine including an electrocardiogram, pulse oximeter, and automated noninvasive blood pressure monitoring was done. Then dexmedetomidine infusion was started as loading dose µg/kg over 10 min followed by 0.5 µg/kg/1 h as a maintenance dose. As the mass was large, pediatric fiberoptic probe (2.7 mm) was chosen, and 5 mm cuffed endotracheal tube was kept ready. In fiberoptic, a large vallecular mass was seen with a small air column through which the pediatric fiberoptic could not be negotiated, considering the large size and risk of bleeding further attempt was not made. Oxygenation by poly mask at 4 L/min continued and surgical tracheostomy done with 7 mm of cuff tracheal tube. Bilateral air entry was checked, and the tracheal tube was secured. Propofol (100 mg) and fentanyl (100 µg) was administered intravenously. Lungs were ventilated with 1.2% isoflurane in oxygen and nitrous oxide (50–50). Neuromuscular blockade was achieved with 6 mg vecuronium. A direct laryngoscopy was done which showed the mass completely blocking the airway [Figure 2]. The mass was completely removed surgically. No transfusion of blood was required. The total duration of surgery was 1 h and was otherwise uneventful. After completion of surgery, the residual neuromuscular blockade was reversed with neostigmine and glycopyrrolate. The patient was conscious, oriented and shifted to the Post-Anesthetic Care Unit with a tracheostomy tube in room air.

Figure 2

Direct laryngoscopy image of the mass

On the 5th postoperative day, the patient was decannulated and discharged on the 8th postoperative day. The diagnosis of RDD was established by histopathology and immunehistochemistry of S-100 and CD-68 positive histiocyte cells [Figure 3].

Figure 3

Immunohistochemistry slide showing S-100 positive histiocytes

DISCUSSION

RDD is a rare proliferative sinus histiocytic disorder presenting as massive lymphadenopathy, described by Foucar et al.[3] Presentation of RDD as vallecular mass is unusual. In airway management perspective, patients of RDD can have difficult bag mask ventilation due to lymphadenopathy and soft tissue mass but in our patient we had no such problems. We had anticipated the possible difficult intubation based on the evidence of location and size of the mass by neck X-ray and CECT. Accordingly, we had prepared for surgical tracheostomy as per the difficult airway algorithm.[4] We preferred dexmetomidine during the attempt of AFOI. Dexmetomidine was chosen as it provides a moderate level of conscious sedation without causing respiratory distress or hemodynamic instability during AFOI.[5] Dexmetomidine also provides better intubation condition without desaturation.[6]

As RDD affects multiple organs, it has many anesthetic implications. The possible complication thereof should be well apprehended, and the plan should be modified accordingly. RDD if involves CNS, it present with a headache, seizures, cranial nerve deficits, or paraplegia. In this scenario, a proper neurological assessment and specific perioperative care is needed. When RDD involves eyes and orbital structures, it can present with exophthalmos, blepharoptosis, conjunctivitis, etc. This may create difficulty in mask placement and the possible risk of injuries to eyes. RDD can also involve immune systems leading to autoimmune hemolytic anemia[7] and polyclonal gammopathy. In such case, the anesthesiologist should have proper hematological monitoring and have strict asepsis to avoid complication.[8]

Patients of RDD may have a history of treatment of chemotherapeutic and radiotherapy which may beget side effects such as stiffness in the neck tissue and reduced submandibular space along with long-term effects of steroids and other chemotherapeutic agent. The anesthesiologist should keep these things in mind to avoid unforeseen complications.[8]

CONCLUSION

The airway management in case of an airway mass should be thoroughly planned to avoid landing up at an unforeseen situation. Though rare, RDD should be kept as a differential diagnosis in the etiologies of airway mass. The multisystemic character of RDD mandates a thorough investigation. Then accordingly pre- and post-anesthetic care should be planned individually for the better patient outcome.

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Conflicts of interest

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