Pagad H et al Duane’s Retraction Syndrome with Crocodile Tears Nepal J Ophthalmol 2018; Vol 10 (19): 90-93 Case Report

Bilateral Type-I Duane's Retraction Syndrome with bilateral Crocodile Tears: A Case Report Harsha Pagad1, Pooja Ramnani1, Sumita Karandikar1, Archana Tadwalkar1, Nita Shanbhag1, Akshay G Nair2 1Dr. D Y Patil Medical College and Hospital and Research Centre, 2Advance Eye Hospital Navi Mumbai

Abstract Background: Duane's retraction Syndrome is a congenital form of characterized by horizontal limitation and retraction with palpebral fi ssure narrowing in attempted adduction. It may be associated with co-existing ocular and systemic pathologies. Crocodile tears, or a paradoxical gustatory lacrimal refl ex can be either congenital or acquired. The congenital cases typically are associated with Duane’s syndrome in most of the cases. Case: We present in our case report, uncommon case of bilateral Duane’s syndrome with bilateral crocodile tears - both of which can be assumed to be of probably central origin. Though several cases have been reported, there are very few from the Indian subcontinent. Here, we also review the literature of the syndrome with crocodile tears. Conclusion: All cases of Duane’s retraction syndrome warrant a thorough screening for coexisting ocular and systemic abnormalities.Ours is a classic report of bilateral Duane’s syndrome with bilateral congenital crocodile tears, the coexistence of these two anomalies emphasising the aetiology to be anomalous innervation occurring at central level i.e. is brain stem Key words: Duane’s syndrome, congenital tears, paradoxical innervation, central origin

Case report off spring of her parents and was born out of A 6 year old girl was brought to our non-consanguineous marriage. Antenatal and clinic by her mother with postnatal history were unremarkable. There history of watering from both the eyes while was no family history of squinting and no eating food. This was noted from a very associated systemic complaints. early age. The mother also gave a history of On examination, the child had no anomalous eyes appearing small and some limitation head posture and no facial asymmetry. Anterior of eye movement. The child was the only segment and dilated fundus examination of both eyes were normal. Her uncorrected visual Financial interest: Nil acuity was 6/6 N6 in both eyes. She exhibited Confl ict of interest: Nil fusion which was examined using the Worth Received: 12.09.2017 Accepted: 17.12.2017 four dot test for distance. Her stereoacuity Corresponding author Dr Harsha Pagad measured with Randot stereoacuity chart Dr D Y Patil Medical College showed 50 arc/seconds. She was orthophoric Sector 5 nerul, Navi Mumbai 400706 Ph 08879029689 for near and distance on alternate cover test E-mail: [email protected] examination (Figure 1).

90 Pagad H et al Duane’s Retraction Syndrome with Crocodile Tears Nepal J Ophthalmol 2018; Vol 10 (19): 90-93

Figure 1: Orthophoric in primary gaze Figure 3: Limitation of abduction in left Extra ocular motility examination demonstrated eye with widening of palpebral fi ssure and limitation of abduction in both eyes with narrowing of palpebral fi ssure with globe narrowing of palpebral fi ssure on adduction retraction right eye and widening of palpebral fi ssure on attempted abduction. Globe retraction was noted in both eyes but more signifi cant in the left eye with minimal upshoot in left eye (Figure 2 and 3). In order to assess specifi c complaints of mother, the child was asked to eat biscuits in the presence of the examing doctor and profuse lacrimation from both eyes was noted (Figure 4). The in both eyes was functionally and anatomically normal and a thorough systemic examination did not reveal Figure 4: Hyper lacrimation following any other anomalies. A conclusive diagnosis of ingestion of biscuits bilateral Type-I Duanes Retraction Syndrome (DRS) with congenital aberrant tearing Owing to the fact that patient had no anomalous (crocodile tears) was made. head posture and globe retraction which was not cosmetically disfi guring, she was advised a regular follow up and no surgical intervention. The option of lacrimal gland botulinum toxin injection for the aberrant tearing was off ered to the parents after due counselling, however, they refused any surgical intervention. Discussion DRS may be seen as an isolated fi nding or in association with both ocular and systemic anomalies (Sachsenweger et al,1966; Kirkham TH,1970). Numerous concomitant ocular and Figure 2: Limitation of abduction in right systemic anomalies have been reported with eye with widening of palpebral fi ssure and DRS. Some ophthalmic manifestations include narrowing of palpebral fi ssure in left eye dysplasia, , , ,

91 Pagad H et al Duane’s Retraction Syndrome with Crocodile Tears Nepal J Ophthalmol 2018; Vol 10 (19): 90-93 anomalies, colobomata, congenital diff erent levels and the use of anticholinergic , microphthalmia, and Marcus-Gunn drugs (.Axelsson A et al, 1962; McCoy FJ et al, jaw winking syndrome(Gutowski NJ, 2000 1979). Recent reports (Boroojerdi B et al, 1998; ; Jafari AK et al ; 2010). Systemic anomalies Hofmann RJ, 2000 ; Reimann R et al, 1999) include Goldenhar’s syndrome, Klippel–Feil suggest that injection of botulinum toxin type syndrome, Noonan’s syndrome, Rubinstein– A (BTX-A) into the lacrimal gland of patients Taybi syndrome, spinal and rib malformations, with CTS may eff ectively and safely abolish sensory hearing loss, facial nerve palsy, the refl ex by chemodenervating the cholinergic genitourinary dysfunction, malformations of neurons of the parasympathetic nervous system the limbs, and facial deformities (Gutowski to the aff ected gland. NJ,2000). The aetiology of DRS was thought to If we assume the presence of paradoxical be replacement of by fi brous innervation as the etiology of our retraction tissue but recent studies point to co-contraction syndrome, a fi nding proved by electromyography of medial and lateral rectus due to paradoxical in a similar case reported by Regenbogen and innervation as to the main reason responsible Stein, then we have the simultaneous presence for the features of this syndrome. The cause of of two syndromes of paradoxical innervation this anomalous innervation is varied, but the (Regenbogen T et al, 1968 ). This points to evidence has pointed to a supranuclear lesion a central location as a possible anatomic site presumably in the brainstem (Pfaff enbach DD et to explain both anomalies. Such a location al,1972). Duane’s syndrome warrants surgical in the pons was fi rst suggested by Lutman management only if there’s unacceptable head (Lutman FC,1947). The obvious explanation posture, large primary gaze squint, upshoots is an abnormality in the brain stem involving and downshoots and severe globe retraction the embryonic development of the superior which is cosmetically unacceptable being some salivary nucleus and the supranuclear center of the primary indications. for lateral gaze. Aberrant tearing or gustatory lacrimation The other explanation of the lacrimal and eye (Crocodile tears) can be congenital or acquired. movement disorders in Duane’s syndrome The congenital variant is very rare as compared would be a lesion causing a nuclear degeneration to acquired type which is seen typically after or dysgenesis in the immediate vicinity of the facial nerve paresis secondary to lesions at or abducens nucleus, the paradoxical aspects of proximal to the geniculate ganglion. Congenital Duane’s syndrome and the lacrimal disorder crocodile tears is usually associated with cases being the result of a substitute innervation of of Duane’s retraction syndrome (Awan KJ, the lateral rectus by fi bres from the oculomotor 1975 ). Various studies done till date have nerve and the lacrimal gland by fi bres sub tried to determine the cause of simultaneous serving salivation (Ramsay J et al,1980). occurrence of these two anomalies of Duane’s and crocodile tears. Many attempts have been To summarise, the rare association of two made to abolish the annoying manifestations of syndromes of paradoxical innervation, bilateral this refl ex.These include intraorbital injection Duane's retraction syndrome and congenital of alcohol or cocaine to destroy postganglionic crocodile tears, is presented here. This is fi bres of the sphenopalatine ganglion, subtotal probably due to a central anomaly at nuclear resection of the lacrimal gland, sectioning level in the pons. Very few cases have been of the glossopharyngeal and facial nerves at reported so far from the Indian subcontinent.

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