Anesthesia management for surgical excision in patients with Rosai-Dorfman disease involving multiple organs throughout the body: a case report and literature review

Abstract

Background

Rosai-Dorfman disease (RDD) is a rare, benign multisystem histiocytic disorder. Cases involving multiple organs and requiring multiple surgeries are uncommon, resulting in limited clinical experience.

Case presentation

We report the case of a 62-year-old male with RDD involving multiple sites, including the nasopharynx, palate, neck, craniocervical junction, anterior brainstem, thoracic spine (T4–T7) within the spinal canal, and mediastinum. The patient was admitted due to ureteral compression by a pelvic RDD mass, causing urinary obstruction and necessitating surgical intervention. Airway management posed a major anesthetic challenge, as the nasal cavity, palate, pharynx, and larynx were all affected by the disease, making tracheal intubation under general anesthesia particularly difficult.

Conclusions

A personalized, multidisciplinary approach—incorporating thorough preoperative evaluation, advanced imaging, and flexible intubation strategies—is essential for managing RDD patients with complex multi-organ involvement. Preparation for potential airway compromise or edema is critical to achieving safe perioperative outcomes.

Trial registration

Not applicable.

Supplementary Information

The online version contains supplementary material available at 10.1186/s12871-026-03656-8.

Introduction

Rosai-Dorfman disease (RDD) is a rare, multisystem histiocytic disorder with an estimated prevalence of approximately 1 in 200,000 individuals [1]. First described by Desombes in 1965 and later characterized by Rosai and Dorfman in 1969 as “sinus histiocytosis with massive lymphadenopathy,” RDD was classified as a non-Langerhans cell histiocytosis by the Histiocyte Society Working Group in 1987 [2]. The clinical manifestations of RDD are highly heterogeneous, ranging from painless lymphadenopathy to life-threatening multiple organ failure. Extranodal involvement occurs in approximately 40% of patients, commonly affecting the skin, central nervous system, orbit and eyelids, upper respiratory tract, and gastrointestinal tract [3]. Notably, about 20% of cases may be self-limited, while nearly 70% follow a relapsing-remitting course [4]. Treatment options include surgery, chemotherapy, and radiotherapy, often requiring multimodal approaches for symptomatic patients or those with vital organ involvement. Although the etiology and pathogenesis of RDD remain incompletely understood, current hypotheses implicate immune dysregulation, viral infection [5] or IgG4-related disease [6]. Recent studies have identified mutations in the mitogen-activated protein kinase (MAPK) pathway in some patients with RDD [7], suggesting a potential neoplastic origin, indicating that this condition may indeed represent a neoplastic process.

Here, we report the case of a 63-year-old male diagnosed with RDD who presented with extensive multisystem involvement and underwent multiple surgical interventions, including procedures involving the nasopharynx, craniocervical junction/anterior brainstem region, the T4–T7 thoracic intradural space, and mediastinal areas. We also discuss pertinent airway management and anesthetic considerations in such complex clinical presentations.

Case presentation

The patient, a 62-year-old man was hospitalized for one week with dysuria due to a space-occupying lesion in the pelvic cavity of approximately 6.1*4.8 cm. His medical history includes tuberculosis and an appendectomy performed 30 years ago, as well as hypertension lasting over 20 years. He has been compliant with his medication regimen. In terms of surgical history, the patient underwent three operations in 2012, 2014, and 2015 for rhinosclerosis. In 2017, he had a thymus tumor resection followed by the resection of an intraspinal space-occupying lesion located at T4-T7 in 2018. Most recently, in 2022, he underwent resection of space-occupying lesions situated in the anterior brainstem at the craniocervical junction. Surgical scars are present over his body. Pathological examination of specimens from these surgeries confirmed Rosai–Dorfman disease (RDD).

Upon examination during hospitalization, vital signs were as follows: temperature 36.4 °C, heart rate 76 bpm, respiratory rate 18 breaths/min, and blood pressure 122/74 mmHg. The patient was alert and spoke fluently. Facial pigmentation was dark red without ulceration. Palpation revealed possibly enlarged bilateral superficial lymph nodes, palpable bilateral parotid glands, and involvement extending from the right mandibular angle into the right supraclavicular fossa. Oral examination with mouth open and tongue slightly protruded to the right showed pink strawberry-like masses on the anterior pharyngeal wall, obscuring the tonsils and uvula. Several pale red masses were also noted on the hard palate, contributing to hoarse pronunciation. Muscle strength and tone in all extremities were normal.

In the operating room, peripheral intravenous access was established and routine monitoring was initiated, including ECG, pulse oximetry, non-invasive blood pressure, and temperature monitoring. After local anesthesia with lidocaine, real-time radial arterial blood pressure monitoring was instituted. Due to RDD involvement of the oropharynx and upper palate, the Mallampati grade was IV and the modified Cormack–Lehane grade was III (Fig. 1C). Head and neck CT imaging showed nasopharyngeal cavity occupation by RDD (Fig. 1A, B), indicating a potentially difficult airway. Given the pharyngeal, palatal, and cervical involvement, preoperative airway assessment based on neck CT revealed the narrowest segment measured less than 8 mm. Although a 5.5 mm internal diameter (ID) tracheal tube would fit the narrowed airway, concern for impaired CO₂ clearance during the anticipated prolonged surgery led to an initial attempt with a 6.0 mm ID tube. If unsuccessful, a switch to a 5.5 mm ID tube was planned. A non-reinforced tracheal tube was chosen to minimize outer diameter.

Fig. 1.

A, B Nasopharyngeal CT scan images presented; C Preoperative laryngoscopy images of the patient, where only the epiglottis is barely visible, while bilateral vocal cords are not discernible; D Postoperative pathology and immunohistochemistry results of the patient’s pelvic space-occupying lesions are displayed as HE staining on the left at 10x magnification and CD68 immunohistochemical staining on the right at 10x magnification

General anesthesia was induced slowly using an amnesic-analgesic technique: preoxygenation via face mask at 2 L/min, followed by dexmedetomidine infusion at 1 µg/kg over 10 min via micropump. Subsequently, sufentanil (5 µg) and remimazolam (5 mg) were administered. The patient entered a sleep state but remained responsive to verbal commands. Meanwhile, 0.5% tetracaine was applied to the oropharynx using an atomizer, and 5 mL of 1% tetracaine was injected via cricothyroid membrane puncture. The patient was asked to cough to distribute the anesthetic for subglottic airway anesthesia. With adequate preparation confirmed, the patient maintained spontaneous ventilation and followed commands cooperatively.

Video laryngoscopy revealed only the epiglottis without glottic exposure. End-tidal CO₂ waveforms remained stable and regular during tracheal catheter manipulation. During right internal jugular vein catheterization, sufentanil (20 µg), rocuronium (50 mg), and ciprofol (30 mg) were administered continuously. Anesthesia was maintained with intravenous ciprofol and remifentanil, along with sevoflurane inhalation at 1–2%. Rocuronium was given intermittently as needed.

The procedure lasted five hours, with stable intraoperative access. The patient was transferred to the ICU postoperatively. At ICU admission, he was hemodynamically stable with approximately balanced intraoperative fluid intake and output. The patient had not yet regained consciousness. Given the difficult airway and risk of airway edema from prolonged surgery, dexmedetomidine was continued at 0.4 µg/kg/h and remifentanil at 0.5 µg/kg/min. Ventilation was supported using SIMV mode with 50% oxygen. Furosemide 10 mg and dexamethasone 5 mg were administered to promote diuresis and reduce edema.

The following morning, after discontinuation of sedatives and analgesics, the patient regained consciousness, followed commands, and exhibited grade 5 muscle strength. The tracheal tube was successfully removed. Pathological examination of the resected pelvic lesion confirmed Rosai–Dorfman disease (Fig. 1D).

Discussion

Rosai-Dorfman disease (RDD), a rare histiocytic disorder characterized by S100+/CD68+ histiocyte proliferation [8], presents significant airway management challenges due to its propensity for cervical lymphadenopathy and extranodal involvement in regions such as the mediastinum and larynx [9]. We report a case of induction of general anesthesia by awake tracheal intubation in RDD with massive cervical lymphadenopathy using topical anesthesia, a strategy undertaken to avoid dangerous cervical manipulation. This report highlights the critical need for meticulous preoperative planning, which is multidisciplinary and integrates advanced imaging like CT or MRI to identify tracheal narrowing or mediastinal extension, as utilized in a cardiac RDD case by Sarraj et al. [10]. Pulmonary function tests and arterial blood gas analysis are crucial for patients with pulmonary involvement, while risk stratification must prioritize features such as > 50% tracheal compression or stridor, which correlate with intraoperative airway collapse [11]. Collaboration with otolaryngologists is vital for complex cases, emphasizing preparedness for awake fiberoptic intubation or tracheostomy [12]. An approach underscored by cases such as that reported by John et al., which highlighted the need for biopsy under spontaneous ventilation in a pediatric patient with neck lymphadenopathy [11]. Intraoperative management necessitates adaptive techniques, for awake intubation, tetracaine nebulization or nerve blocks can enhance tolerance [13]. While video laryngoscopy can aid in limited neck extension, its utility may be reduced in severe stenosis. For patients with mediastinal masses, maintaining spontaneous ventilation during induction—using inhalational agents like sevoflurane in pediatric populations—is critical to prevent airway collapse [14]. Pharmacologic choices should avoid ketamine in cases with CNS involvement due to its potential to elevate intracranial pressure [15], favoring instead short-acting agents like propofol and remifentanil. Postoperative care focuses on complication mitigation, considering delayed extubation for airway edema or tracheomalacia and utilizing high-flow nasal oxygen for respiratory support [16], with neurological monitoring being imperative for patients with CNS involvement [17].

Management must further be tailored for special populations, for pediatric patients require careful consideration due to smaller airway anatomy [18], and immunosuppressed patients need stringent aseptic techniques. Emerging therapies, including BRAF inhibitors like vemurafenib, may reduce lymph node burden and simplify future airway management [19].

Significant research gaps persist, underscoring the need for multicenter studies to establish evidence-based algorithms, as current guidance relies on case reports. The role of preoperative steroids in reducing lymph node size remains controversial yet merits investigation [20], and advanced imaging like PET-CT could help differentiate active disease to inform surgical timing.

The primary limitations stem from the inherent constraints of a case report format: lack of generalizability, absence of comparative data, and a narrative review style that cannot provide high-level evidence. Its value lies in raising awareness, suggesting a plausible management strategy for a rare condition, and highlighting areas for future study, rather than in establishing standard-of-care protocols.

Conclusion

Effective airway management in RDD requires a tailored, multidisciplinary strategy centered on advanced preoperative imaging, adaptive intubation techniques, and robust contingency planning.

Authors’ contributions

Yu Wu and Jinbao Wang wrote the main manuscript text, and Yang Runmin prepared Fig. 1. Zhihui Zhang and Yongzhong Gao revised the revised manuscript. All authors reviewed the manuscript.

Funding

The Key Research Topic of Medical Science in Hebei Province (20180913).

Data availability

The datasets are available from the corresponding author on request. All data is provided within the manuscript.

Declarations

Ethics approval and consent to participate 

not applicable.

Competing interests

The authors declare no competing interests.