International Journal of Health Sciences and Research www.ijhsr.org ISSN: 2249-9571

Case Report

Congenital Bilateral Dacryocystocele

Palak Thakrar1, Rahul Chivate2, Reema Choudhary3, Deepak Patkar4, Sachin Deshmukh3

1Department of , Nirman Hitech Diagnostic Centre, Mumbai, India. 2Department of Radiology, Tata Memorial Hospital, Mumbai, India. 3Department of Radiology, Midtown Diagnostics, Mumbai, India. 4Department of Radiology, Nanavati Hospital and Midtown Diagnostics, Mumbai, India.

Corresponding Author: Palak Thakrar

Received: 28/01/2016 Revised: 16/02/2016 Accepted: 26/02/2016

ABSTRACT

Congenital dacryocystocele is a rare entity and bilateral is rarer. It causes obstruction of lacrimal drainage system leading to swelling of the lacrimal sac. It may present at birth or soon after birth as a bluish swelling in the region of medial canthus. In bilateral cases there can be intranasal extension leading to respiratory distress. We present a rare case of bilateral congenital dacryocystocele with dyspnea due to intranasal extension. Imaging plays an important role in diagnosis. In our case computed tomography scan and magnetic resonance imaging revealed bilateral cystic swellings in the region of medial canthus adjacent to nasolacrimal duct causing its expansion with intranasal bulge. Dacryocystocele is managed conservatively and surgically. When a appears in the region of lacrimal sac differential diagnosis of dacryocystocele should be considered.

Keywords: dacryocystocele; lacrimal; congenital; canthus; bilateral.

Key message: Bilateral congenital dacryocystocele is rare and can be identified along with its extent on imaging by CT (Computed tomography) and MRI (Magnetic resonance Imaging). On CT it appears as cystic medial canthal bulge, dilated nasolacrimal sac and nasal cavity bulge, On MRI it appears hypointense on T1-weighted images, hyperintense on T2-weighted and STIR sequence. Post- contrast studies may show minimal enhancement.

INTRODUCTION bilaterally since birth. The swelling did not Congenital dacryocystocele is a rare show any significant change in size since it occurrence due to nasolacrimal duct was noticed. Infant had difficulty in obstruction. Subsequently, there is breathing since few weeks after birth. There accumulation of fluid leading to swelling was no watering or discharge from either of over medial canthus. It is important to the eyes. On examination, there were exclude intranasal extension leading to bilateral palpable fluctuant blue masses in respiratory distress. CT (Computed the medial canthi. Anterior segments of both tomography) and MRI (Magnetic resonance eyes were normal. Infant was dyspnoeic. imaging) features are diagnostic. There was no regurgitation of fluid on compression. Baby was born full term with CASE HISTORY a normal antenatal history. After clinical 10 month old female infant examination, radiological investigations presented with history of painless, non were advised following which it was tender swelling below the medial canthus diagnosed as dacryocystocele. Imaging

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findings on CT revealed two structures in was opted. Under anaesthesia, following anteromedial aspect (medial canthus) of punctal dilatation, endoscopic nasal both orbits in the region of nasolacrimal examination was performed. After locating duct and lacrimal sac expanding it and the bilateral cystic masses, they were appearing slightly hyperdense to the globe removed endoscopically and sent for bulging into the nasal cavity (Figure1). On histological examination. Post operative MRI, the bilateral anteromedial structures follow up showed resolution of both the appeared hyperintense on T2-weighted and medial canthal masses along with symptoms T1-weighted fat saturated images suggestive of dyspnoea. Histological examination of cystic lesions. (Figure2). Infant was given confirmed the presence of benign lacrimal oral antibiotics and topical antibiotic drops sac tissue. The patient was discharged with as a preventive measure with instructions of an advice of regular follow up for next 6 massage. On follow up after 1 week there months. Currently, there is no evidence of was no resolution. Surgical management recurrence.

Figure1: 10 months old female infant with bilateral dacryocystocele.

Findings: mildly bulging into the nasal cavity. The  Figure 1a: Axial CT images in bone right side lesion is bigger than left. The window settings reveals two structures masses measure 16x24x20mm on the within medial canthal region of bilateral right side and 14x18x15mm on the left orbits adjacent to the nasolacrimal duct side in superior-inferior, antero-posterior (yellow arrows) appearing almost and transverse diameter respectively isodense to muscles and slightly with 20-30 HU. hyperdense to globe. The lesions are

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 Figure 1b: Axial CT images in bone nasolacrimal duct (orange arrows) window settings from a section inferior appearing almost isodense to slightly to the image 1a reveals two structures in hyperdense to muscles, likely due to the region of nasolacrimal duct (blue high proteinaceous content. arrows) causing enlargement of  Figure 1d: Coronal CT images in bone nasolacrimal canal. The masses measure window settings reveals two structures 16x24x20mm on the right side and in the medial canthal region of bilateral 14x18x15mm on the left side in orbits (yellow stars) causing superior-inferior, antero-posterior and enlargement of lacrimal sac fossa and transverse diameter respectively with nasolacrimal canal. 20-30 HU. (Ct machine-16 slice Toshiba Aquilion  Figure 1c: Axial CT images in soft ST-0.mm, mA-200, kVp- 120) . tissue window settings reveals two structures within anteromedial aspect of bilateral orbits adjacent to the

Figure 2: 10 Months old female infant with bilateral dacryocystocele. Findings: superior-inferior, antero-posterior and  Figure 2a, b: Axial T2 weighted images transverse diameter respectively. In the reveal homogeneous, hyperintense inferior section there is extension of the cystic masses in the anteromedial aspect lesion into bilateral nasolacrimal canals of bilateral orbits in the region of which are enlarged. 1.5T GE Signa lacrimal sac and nasolacrimal duct Echospeed LX.TR- 4140, TE- 91.72, (yellow arrows). The masses measure ST-3.mm. 16x24x20mm on the right side and  Figure2c: Coronal T2 weighted fat 14x18x15mm on the left side in saturated images reveal two

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homogeneous, hyperintense cystic masses in the anteromedial aspect of bilateral orbits in the region of lacrimal sac and nasolacrimal duct mildly bulging into nasal cavity (blue arrows). The masses measure 16x24x20mm on the right side and 14x18x15mm on the left side in superior-inferior, antero posterior and transverse diameter respectively. 1. 5T GE Signa Echospeed LX.TR- 5440, TE-86.223, ST-3.0mm.

Figure 3. Schematic drawing of a congenital dacryocystocele.[9] DISCUSSION Congenital dacryocystocele is a rare Dacryocystocele is caused due to condition of nasolacrimal duct obstruction blockage of the lacrimal duct in the nasal affecting new born and bilateral cavity at the level of inferior meatus. There dacryocystocele is rarer. It occurs in 0.08% is a valve of Hasner causing obstruction at to 0.1% of newborns, 25% are bilateral. [1] It this level which eventually undergoes is more common in females and non- perforation between 3rd to 6th months. hispanic whites, while familial cases have Incomplete canalization of this valve leads been described only sporadically. [2] to distal obstruction forming a It is a benign condition. Bilateral dacryocystocele. [6] The obstruction of involvement may have intranasal extension Rosenmuller valve, which normally which might be severe enough to cause prevents reflux from lacrimal sac to obstruction resulting in neonatal respiratory canaliculi, is another mechanism causing a distress requiring surgical intervention. [3] It dacryocystocele. [5] Thus, congenital appears as a swelling with bluish dacryocystocele are due to obstruction discolouration at birth or soon after, below proximally at the valve of Rosenmuller and and nasal to medial canthus. It is often distally at the valve of Hasner forming a one misdiagnosed as a vascular lesion. [4] way valve allowing only fluid to enter in. Congenital type is known to disappear in Congenital type is typically known to occur utero or spontaneously at birth. It is in the third trimester as per the sometimes known to persist and would embryological changes. [10-12] There can be subsequently resolve during the neonatal secondary infection due to stasis of the period with conservative or surgical lacrimal fluid, location near the sinuses and management. [5] It is commoner in females, the presence of lymphatic and vascular could be due to narrower nasolacrimal duct system. It could present as respiratory than males. [6] Various causes of distress, and facial cellulitis. dacryocystocele include congenital [6] It can get infected most commonly by deformities, trauma, primary and recurrent Staphylococcus aureus. [13] tumors affecting the nasolacrimal duct, It is important to look for any idiopathic blockage of the nasolacrimal duct associated syndromes and structural and iatrogenic causes including treatment of abnormality. Sharony et al reported an head and neck cancer in the sinonasal association between dacryocystocele and region. [7,8] genetic conditions such as Canavan disease and polycystic kidneys. [12] Polyhydramnios was associated in few cases. [20,14] Westbrook et al., reported recurrent bilateral dacryocystoceles in Wegener’s granulomatosis. [15] It is important to

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diagnose this condition due to the risk of bony involvement of the nasolacrimal canal intranasal extension causing obstruction to and choanal atresia is easier to detect. [23,24] the nasal passages leading to neonatal CT shows hypodense cystic medial canthal respiratory distress requiring surgical mass, dilated nasolacrimal sac, and intervention. [16] There are various opinions submucosal nasal cavity mass. [25] MRI is regarding treatment of dacryocystocele with the method of choice for soft tissue either conservative or surgical treatment. delineation and to study the relation with Conservative treatment is with antibiotics, central nervous system before carrying out massage, nasal probing and also drainage if any interventions as it also differentiates necessary. [6,17-19] However, if there is a solid tumor from this condition. MR delay in response, early surgical imaging shows a mass with low intensity intervention must be applied or a quick first signal on T1-weighted images and high line surgical especially with intensity signal on T2-weighted. [26] Post intranasal extension. [20,21] As per a study by contrast studies may reveal minimal to no Wong and Vander Veen, 28 of 42 patients enhancement. It is essential to differentiate presented with cellulitis or dacryocystitis from other causes of duct obstruction or and 36 of 46 affected eyes underwent cystic dilatation such as ecephalocele, surgical intervention. According to Schnall hemangioma and dermoid [1] [Use and Christian, 18% presented with differential table]. On CT dacryocystocele dacryocystitis of 21 dacryocystoceles and appears as a cystic hypodense, fluid-filled 16 resolved with conservative therapy. 25% structure with minimal enhancement. On occurrence of dacryocystitis has been MRI it appears hypointense on T1-weighted reported during the management of and hyperintense on T2-weighted dacryocystocele. [6] As bilateral sequences. On CT hemangioma appears as a dacryocystoceles can cause obstruction of well defined hyperdense mass with nasal passages leading to respiratory homogeneous contrast enhancement. [27] On distress, surgical intervention is the MR, it appears hypointense on T1-weighted, treatment of choice. [17] The prognosis is hyperintense on T2-weighted and show good. diffuse homogeneous contrast enhancement. When the diagnosis of [27] On CT appears as dacryocystocele is uncertain, diagnostic hypodense mass. On MR it appears studies such as USG or CT or MRI imaging hypointense on T1-weighted, hyperintense can be useful. [22] Ultrasound provides good on T2-weighted and post contrast rim to information about the lacrimal sac, homogeneous enhancement. [28] On CT nasolacrimal duct and the surrounding dermoid appears hypodense, less dense than structures. Antenatal 2D ultrasound is fluid. On MR it appears hyperintense on T1- performed at 30weeks. It shows uni- or weighted and hypointense on T2-weighted bilateral rounded cystic lesions inferiorly in sequence. It shows post contrast rim the medial canthus region measuring few enhancement. [29] millimeters without vascularity or calcification with normal brain and facial CONCLUSION structures. On 3D ultrasound precise Congenital dacryocystocele is an location with a degree of intranasal extent uncommon entity. Bilateral dacryocystocele and communication with orbits is clearly is rarer. In such cases it is important to depicted. It also helps in better exclude intranasal extension leading to understanding for the parents. [6] However, respiratory distress. Cases may be other examinations such as complicated or uncomplicated both dacryocystography, CT and MR imaging requiring medical and surgical management. may be carried out to diagnose the condition Examining paediatricians and and determine the extent. CT determines the neonatologists should be aware of its

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How to cite this article: Thakrar P, Chivate R, Choudhary R et al. Congenital bilateral dacryocystocele. Int J Health Sci Res. 2016; 6(3):329-335.

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