Woakes' Syndrome: A Systematic Review of Reported Cases

Woakes' syndrome is a rare form of chronic rhinosinusitis with nasal polyps (CRSwNP) characterized by the progressive expansion of the nasal framework. We aimed to systematically review the literature on Woakes' syndrome to characterize its clinical features, comorbidities, management strategies, and outcomes. A literature search was conducted in accordance with the Preferred Reporting Items for Systematic Reviews and Meta-Analyses (PRISMA) guidelines. A systematic search of several databases (PubMed, Embase, Cochrane Library, etc.) was conducted for all reports of Woakes' syndrome. All study types (case reports, case series) were included if they provided clinical data on Woakes' syndrome. Non-English-language papers were excluded. Data on patient demographics, comorbidities (e.g., asthma, aspirin-exacerbated respiratory disease), treatments, and outcomes were extracted and synthesized descriptively. Twenty-three studies met the inclusion criteria, comprising 39 unique patients (mean age 39.5 years; range 5-81; 65% male). Both paediatric and adult-onset cases were identified. Common comorbidities included Samter's triad (seven cases) and bronchiectasis or sinobronchial disease (two cases). All patients underwent surgical treatment, most commonly functional endoscopic sinus surgery, with adjunctive procedures including digital nasal bone compression (six cases) and formal rhinoplasty or septorhinoplasty (seven cases). Pre- and postoperative steroid therapy was variably reported with most prescribed topical or systemic corticosteroids. Follow-up data were inconsistently reported; where available, the mean follow-up was approximately 12.5 months, with seven documented recurrences (six despite nasal steroid therapy). Woakes' syndrome is an extremely rare but distinct clinical entity in rhinology, representing an aggressive phenotype of CRSwNP that causes facial deformity. Both paediatric and adult-onset cases occur, frequently associated with underlying conditions such as aspirin-exacerbated respiratory disease in adults. Management requires a combination of aggressive surgical polyp removal and prolonged treatment with steroids to maintain remission. Long-term disease control remains challenging, and emerging treatments such as biological therapies to target chronic inflammation may provide benefit in the most refractory cases.