Hereditary woakes syndrome; our experience

Chronic rhinosinusitis (CRS) with or without nasal polyp is one of the most prevalent chronic disease worldwide, causing a significant impact on quality of life. CRS is broadly divided into CRS with nasal polyps (CRSwNP) and without nasal polyps (CRSsNP) (1).CRSwNP represents a more severe medical condition. Woakes described a rare form of nasal polyposis which, arises in childhood and produces severe nasal deformity and necrosis of ethmoidal cells. in 1885.B. Kellerhals and De Uthemann defined Woakes syndrome as severe recurrent infantile nasal polyposis with broadening of the nasal pyramid causing pseudohypertelorism, frontal sinus aplasia, bronchiectasis and dyscrinia.Herewith we are presenting three siblings who came with nasal polyposis and facial deformity with nasal obstruction. We did FESS and Rhinoplastysurgery and successfully treated them without any complications. Case Report: 17 year old male came with complaints of nasal obstruction and progressive deformity with broadening of nasal bridge, recurrent rhinitis, since 3 years & hyposmia and hyponasal voice since 1year. Second patient was15 year old female, sister of case -1, came with similar complaints with severe broadening of nasal bridge, recurrent rhinitis, hyposmia and hyponasal voice since 1year. Third patient was 13 year old female sibling came with complaints bilateral nasal obstruction, recurrent rhinitis, slight nasal deformity, hyposmia and hyponasal voice for 6 months. Compared to her siblings she only had minimal nasal deformity (Figure.1). on anterior rhinoscopy polypoidal mass seen in both the nostrils (Figure.2).CT scan of paranasal sinuses and nose (PNS) revealed nasal polyposis occupying bilateral nasal cavity with resultant maxillary, frontal, sphenoid and ethmoid sinusitis (Figure. 3) High resolution CT thorax showed bilateral symmetrical central bronchiectasis with upper lobe predominance (Figure.4). All three of them were operated. FESS and Rhinoplasty surgery were done. Intraoperative findings were showing thick tenacious secretions and fungal concretions (Figure.5). In all three cases bilateral ethmoidal, spehoid , frontal and Maxillary sinuses were full of polypoidal mass . Complete clearance of all sinuses was done (Figure .6) & good frontal glow was achieved Figure.7). Postoperatively they were given oral steroids for15 days along with nasal douching and nasal steroidal spray was given for three months. Initially azelastine & fluticazone steroidal spray was given for one month. And then only fluticazone steroidal nasal spray was given for next 2 months. They were followed at interval of onw week, one month, 3 months and 6 months. Every follow up check nasal endoscopy was done. They all had good outcome without any complications. Conclusion: Woakes syndrome is a clinical condition characterised by a tetrad of recurrent nasal polyposis, nasal pyramid deformity, frontal aplasia or hypoplasia and bronchiectasis. Etiopathogenesis of this syndrome still remains unclear due to its rarity and sparse documentation. Non-inherited unknown genetic factor may be a possible etiology, and needs further detailed genetic studies. As these cases are usually refractory to medical management, meticulous surgery in the form of Functional endoscopic sinus surgery forms the mainstay of the treatment with regular post-operative care for prolonged duration carrying equal importance. Rhinoplasty surgery helps the patient to give cosmetic results and better face. Furthermore, detailed study over a prolonged period is required in such patients to comment upon specific duration of follow-up required.