Lacrimal Gland Choristoma in the Setting of Acute on Chronic Sinusitis: A Rare Case Report

    

    

    Figure 1: A coronal CT scan of the head depicting potential periorbital
abscess and/or lesion of the left upper lateral portion of the eyelid.
    

Patients presenting with sinusitis and presumed orbital complications should be admitted to hospital and started on broad-spectrum intravenous antibiotics [2]. Surgical drainage is recommended when there is CT evidence of abscess formation, worsening visual acuity or blindness, rapid progression of orbital signs and symptoms or lack of improvement despite aggressive medical therapy [1]. Regardless of the method, underlying sinus pathology and abscesses must be addressed as at least 10% of untreated cases result in blindness [2]. While sinusitis is a common cause of orbital infection, it is important to rule out other possible causes of ophthalmological symptoms. The differential diagnosis can include orbital pseudotumor (pediatrics), orbital rhabdomyosarcoma, ethmoid mucocele, allergic fungal rhinosinusitis, iatrogenic injury and neoplasm [2]. Choristomas are benign masses of histologically normal tissue located at abnormal sites [3]. Choristomas consisting of more than one type of tissue are considered complex. Choristomas containing ectopic lacrimal glands have been reported in the orbit, outer eye, conjunctiva, cornea, sclera or in the intraocular tissue [4]. The following case is the first reported incident of an incidental Lacrimal Gland Choristoma (LGC) in the setting of acute on chronic sinusitis with presumed orbital involvement.

Case Report

Demographics

A 12-year-old male recently immigrated from France to Canada of African descent.

Medical History: Chronic rhinosinusitis patient who previously underwent Functional Endoscopic Sinus Surgery (FESS) in Europe. Otherwise healthy with no allergies and up-to-date vaccinations.

Signs and Symptoms

The patient was admitted 8 days prior to surgery for retro-orbital eye collection. The patient presented with purulent nasal discharge, throbbing facial pain, intermittent episodes of spiking fevers, swelling of left upper and lower eyelids and some difficulty opening his eyelids. CT scan revealed evidence of potential periorbital abscess and/or lesion of the left upper lateral portion of the eyelid.

Treatment and Test results: The patient was treated with a course of IV ceftriaxone, moxifloxacin, and vancomycin. The patient then underwent image-guided FESS, septoplasty and submucosal resection of turbinates, left maxillary antrostomy, left anteriorposterior ethmoidectomy, left frontal sinusotomy and antral lavage 8 days after admission. The left maxillary sinus had a significant amount of pus which was drained out and sent for culture and sensitivity. Intra-operative drainage incidentally revealed an orbital tumor. A left superior orbital mass resembling lymphoma was submitted to the pathology department. Histopathological analysis of excisional biopsy revealed a lacrimal gland choristoma with focal acute inflammation. More specifically, the tumor tissue showed mainly acini glands resembling lacrimal glands with focal reactive changes subsequent to acute inflammation. Some decompression of the eye was noted after the procedure. There were no intraoperative complications. The patient was handed over to ophthalmology for the management of eye pathology.

Discussion and Conclusion

Lacrimal Gland Choristomas (LGC) are rare congenital lesions thought to be caused by sequestration of parts of the lacrimal anlagen [5]. Macroscopically these specimens resemble normal lacrimal gland tissue [5]. Microscopic examination generally reveals normal lacrimal gland tissue, commonly accompanied by moderate nonspecific inflammatory cell infiltration [5]. Alyahya et al. in their study reviewed 61 ectopic lacrimal gland specimens collected over 50 years. 62% of these specimens were considered complex choristomas, while the remainder contained only ectopic lacrimal gland tissue [6]. In our case, the histopathological analysis revealed only ectopic lacrimal gland tissue. Although symptoms of LGC’s can manifest at any age, they most commonly present during the first 3 decades of life, the reason for their often-delayed presentation is unknown [5]. Generally, the complications of an LGC are inflammatory in nature [5]. However, vascular lesions, pleomorphic adenomas and very rarely carcinomas have developed in ectopic LGCs [5]. Our patient presented with suspected orbital involvement presumably attributable to an inflammatory process from acute on chronic sinusitis. Incidentally, a LGC was found that was likely contributing to his orbital symptoms. This case emphasizes that in the context of presumed orbital complications of sinusitis, orbital tumors must be considered. All sinus cases involving the orbit require ophthalmology consult to ensure appropriate diagnostic and surgical management for these patients.

References

1. Giannoni CM, Weinberger DG, Ferguson BJ, Ryan MW. Complications of Rhinosinusitis editors. Head & Neck Surgery- Otolaryngology Lippincot Williams & Wilkins. 2006; 2: 493-504.
2. Goldstein BJ, Goldenberg D, Goldstein BJ, Goldenberg D. Orbital Complications of Sinusitis editors. Handbook of Otolaryngology Thieme. 2018; 228-230.
3. Zwaan J, Karcioglu ZA. Orbital Tumors editor. In Benign Pediatric Tumors Springer. 2015; 337-348.
4. Ranganathan D, Lenhart P, Hubbard G, Grossniklaus H. Lacrimal gland choristoma in a preterm infant, presenting with spontaneous hyphema and increased intraocular pressure. Journal of Perinatology. 2010; 30: 757-759.
5. Verdijk RM, Grossniklaus H , Heegaard S. The Orbit, Including the Lacrimal Gland and Lacrimal Drainage System In Eye Pathology. 2015; 547-731.
6. Alyahya G, Bangsgaard R, Prause J, Heegaard S. Occurrence of lacrimal gland tissue outside the lacrimal fossa. comparison of clinical and histopathological findings Acta Ophthalmologica Scandinavica. 2005; 83: 100-103.