Pediatric Nasopharyngeal Cysts Differential Diagnosis and Management Tal Marom*, Eyal Russo*, Douraied Ben Salem ψ, Yehudah Roth*,φ * Department of Otolaryngology-Head and Neck Surgery, Edith Wolfson Medical Center, Tel-Aviv University, Sackler School of Medicine, Holon, Israel ψ Department of Neuroradiology, University Hospital of Dijon, France φ Dalla Lana School of Public health, University of Toronto, Ontario, Canada Good morning. My name is Dr. Tal Marom, and I am from the department of Otolaryngology- Head and Neck Surgery, in the Edith Wolfson Medical Center. I would like to thank my colleagues, who helped me with this work, and especially to Dr. Eyal Russo, who is a senior pediatric otolaryngologist in our department. No potential conflicts of interests

Incidental Findings Nasopharygoscopy Flexible bronchoscopy Adenoidectomy H&N imaging studies Nasopharyngeal cysts are considered to be incidental rare lesions, which are infrequently recognized on routine nasopharygoscopies, flexible bronchoscopies and head and neck imaging studies. On these scenarios, otolaryngologists are requested to integrate the radiological information with the physical examination, to determine the nature of the lesion and to try to clarify the possible associations with the clinical presentation. Nasopharyngeal cysts are more frequently encountered in children. Here are a few examples that we have encountered.

Retention cyst of a seromucinous gland 12 y/o male with epiphora This is a yellowy submucosal cyst discovered during nasopharygoscopy, corresponding to a retention cyst of a seromucinous gland in a 12-years-old boy with epiphora Retention cyst of a seromucinous gland

8 y/o boy with hypogonadism Here is a yellowy submucosal cyst discovered during nasopharygoscopy, corresponding to a retention cyst of a seromucinous gland in a 12-years-old boy with epiphora Persistent hypophyseal (Sternberg) canal

Retention cyst obstructive the eustachian tube 11 y/o girl with left CHL This is an MR study performed in a 11-year-old girl with a left-sided conductive hearing loss. In the left picture, you can see a retention cyst of the left lateral nasopharyngeal recess in the axial T1-weighted spin-echo MR. There is a well-defined hypertense cyst spontaneously hyperintense. The cyst bulges into the nasopharyngeal lumen and compresses the orifice of left eustachian tube. In the right picture, same patient, the axial T2-weighted MR image reveals a hyperintense cystic mass of the nasopharyngeal mucosal space. Note the trapped fluid in left mastoid air cells (arrow). Retention cyst obstructive the eustachian tube

7 y/o girl with incidental finding Tornwaldt’s cyst is a benign developmental lesion within the nasopharynx midline, above the upper border of the superior constrictor muscle. The cyst represents the embryonic communication between mesenchymal notochord remnant and endodermal-derived nasopharyngeal mucosa. A 0.06% incidence of Tornwaldt’s cysts was recently reported in large head and neck MRI series, in contrary to earlier reports which suggested that it is a more common finding, both in autopsies (up to 3.3%) and on imaging incidence studies (up to 5%). The cyst has a high signal intensity on T1- weighted, T2- weighted and fluid-attenuated inversion-recovery images and does not enhance with gadolinium contrast. Most cysts are small and asymptomatic, but if they grow or become infected, they may cause nasal obstruction, postnasal drip, halitosis, occipital headaches, neck stiffness and eustachian tube dysfunction. Tornwaldt’s cyst

4 y/o girl with visual acuity deterioration Nasopharyngeal Rathke’s cleft cysts are rare, since most of them are located intracranially. These benign cysts arise from failure of obliteration of the embryonic Rathke’s pouch. Rathke’s cleft cysts in the nasopharynx may grow and block the orifice of the eustachian tube, induce progressive headaches, ptosis and diplopia. Rathke’s cleft cyst Courtesy: Dr. R. Buhl, Department of Neurosurgery, Kiel, Germany

4 y/o girl with progressive nasal obstruction Dermoids, epidermoids and teratomas are three distinguishable congenital lesions which can be precisely diagnosed only following excision and histopathological examination. Epidermoids are defined as simple inclusion cysts lined only by squamous epithelium derived from the ectoderm. Dermoids are also ectodermally derived, but may contain hair and other dermal appendages. Teratomas are lesions that demonstrate ‘‘histologically divergent differentiation’’, but may not be necessarily contain all three primordial germ layers. Head and neck teratomas are most commonly cervical, with the nasopharynx being the second commonest location. In the nasopharynx, they arise from the midline or lateral nasopharyngeal wall and display a female predominance of six to one, like in this case. This 4 year-old girl presented with progressive nasal obstruction. Sagittal CT scan demonstrates a cystic lesion filling the posterior nasal cavity. Pathology demonstrated a polypoid mass lined by keratinized squamous epithelium, overlying a thickened fibrovascular tissue containing abortive pilosebaceous units and profiles of eccrine sweat glands and ducts, consistent with teratoma. CT Intra-Op Pathology Teratoma

4 y/o boy with rhinorrhea Very rarely, second branchial cysts manifest in the nasopharynx, as shown in these two cases. Recent experiments with human embryos have shown that gene expression in the head region follows that seen in other species embryo models. The Dlx homeobox genes gamily code for regional specification of the branchial arches. Mutations in these genes are associated with aberrant branchial arch structures. It is possible that nasopharyngeal branchial cysts are a result of such mutations. 2nd Branchial cyst

1.5 y/o girl with reddish EAC lesion 1st Branchial cyst Courtesy: Dr. M. Ada, Cerrahpas Medical Faculty, Istanbul, Turkey

1.5 y/o boy with recurrent epistaxis Unlike frontoethmoidal encephaloceles, which are visible externally, nasopharyngeal encephaloceles can be detected only by endoscopy and imaging studies. Their course can be complicated, and pass through the ethmoids, the sella or the sphenoid. To date, only a few dozen cases were reported, and the clinical presentation was mostly associated with nasal obstruction and visual disturbances, as seen in this 1.5 year old male, who presented with epistaxis. Nasopharyngeal extension of encephalocele Courtesy: Dr. S. Durry, Department of Radiology, Toronto Hospital for Sick Children, Canada

5 y/o girl: incidental finding Other cystic lesions in the nasopharynx include retention cysts within the adenoids, which are encountered in up to 6% of individuals. The cysts are usually asymptomatic and located in the midline. If the cystic content is rich in protein, it displays high signal on T1 weighted image, opposing to lymph-containing cysts which are not bright on T1.These cysts do not enhance with gadolinium. The presence of these cysts within the adenoids, their rhomboid shape and the typical MR appearance make them recognizable from other cysts, as shown here. Intra-adenoid cyst

12 y/o girl: incidental finding Other nasopharyngeal cysts include more lateral benign seromucinous retention cysts, as shown here, infectious cysts, pharyngeal bursae of Luschka and other rare lesions. Lateral (retention) cyst

13 y/o boy with chronic cough (bronchoscopy) This is a lateral cystic lesion which was seen during flexible bronchoscopy in a 13-years old boy which was carried out to evaluate a chronic cough. Pedunculated nasopharyngeal lateral cyst

Female embryo, 34th week, polyhydarmnios, fMRI It is important to stress the increasing role of otolaryngologists in the prenatal diagnosis of head and neck congenital lesions, due to the advanced technologies available today, and mainly the fetal MRI. This is a case of a nasopharyngeal teratoma in a fetus. Sequential fetal axial MR coronal views of a heterogeneous mass originating from the nasopharynx which protrudes from the oral cavity, consistent with a teratoma. Intracranial extension of the tumor was not detected. The fetus was successfully delivered by an EXIT procedure. Congenital teratoma, scheduled for EXIT Courtesy: Dr. S.S. Peng, Department of Obstetrics and Gynecology, National Taiwan University Hospital and National Taiwan University College of Medicine, Taipei, Taiwan

Summary Nasopharyngeal cysts are rare Presentation depends on size, location and nearby tissues Biopsy may not be necessary in most cases To summarize, nasopharyngeal cysts are considered to be congenital malformations in most cases. However, their clinical presentation may be postponed to adolescent or adult age, depending on their severity, size and location. Once incidentally discovered, clinical correlation with nasopharygoscopy is required, in concordance to the symptoms and the primary indications for the imaging study. Only in rare cases biopsy is required. THANK YOU