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 Table of Contents  
Year : 2023  |  Volume : 3  |  Issue : 2  |  Page : 481-482

A diagnostic impasse: Congenital or acquired Horner's syndrome?

Department of Ophthalmology, Goa Medical College, Bambolim, Goa, India

Date of Submission06-Oct-2022
Date of Acceptance08-Dec-2022
Date of Web Publication28-Apr-2023

Correspondence Address:
Yash K Narvekar
House No. 2/76, Dangui Colony, Mapusa, Goa
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Source of Support: None, Conflict of Interest: None

DOI: 10.4103/IJO.IJO_2605_22

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A four-year-old female presented to the department of ophthalmology with history (given by the mother) of noticing drooping of the left upper lid and small eye since birth. On examination, the patient was found to have mild ptosis, miosis, and mild iris hypochromia of the left eye. A diagnosis of Horner's syndrome was made. Retrospective evaluation revealed that the patient was diagnosed as a case of tracheoesophageal fistula (TEF) and esophageal atresia on first day of life and underwent surgical intervention on the second day of life. We present this case as that of diagnostic challenge with respect to the etiology of Horner's syndrome.

Keywords: Heterochromia, Horner's syndrome, miosis, ptosis

How to cite this article:
Narvekar YK, Usgaonkar UP, Naik A. A diagnostic impasse: Congenital or acquired Horner's syndrome?. Indian J Ophthalmol Case Rep 2023;3:481-2

How to cite this URL:
Narvekar YK, Usgaonkar UP, Naik A. A diagnostic impasse: Congenital or acquired Horner's syndrome?. Indian J Ophthalmol Case Rep [serial online] 2023 [cited 2023 Jun 2];3:481-2. Available from: https://www.ijoreports.in/text.asp?2023/3/2/481/374958

We present a case of a four-year-old female patient diagnosed with Horner's syndrome. From previous medical records, it was found that the patient had been diagnosed with tracheoesophageal fistula and underwent surgical intervention for the same on the second day of life. The early timing of surgical intervention creates a dilemma with respect to the etiology of Horner's syndrome. Horner's syndrome has an incidence of 1 in 6000. Congenital Horner's syndrome accounts for 5% of all cases. Only seven cases of acquired Horner's syndrome have been reported secondary to tube thoracostomy.

  Case Report Top

A four-year-old female patient was brought to the ophthalmology department by her mother, with history of noticing small left eye and drooping of the left upper eyelid from birth, which has remained the same till date. This drooping was not associated with any diurnal variation, abnormal eye movements, or abnormal lid movements on chewing food. No other ocular complaints were elicited. On ocular examination, ptosis of the left upper lid measuring 2 mm was noted.

Margin reflex distance 1 of the left eye was 2 mm and margin reflex distance 2 was 3 mm, with a palpebral fissure height of 5 mm [Figure 1]. Levator action could not be assessed due lack of cooperation. The color of both irises were brown with subtle hypopigmentation of the left iris. The pupil was circular with normal direct and consensual light reaction. Near reflex was normal in both eyes. Anisocoria was noted. In a dark room, the right pupil was 4 mm and left pupil was 1 mm. Visual acuity could not be assessed as the patient was uncooperative; however, the patient fixed and followed the light. There was no limitation to ocular movements. The rest of the anterior segment as well as fundus examination was normal. There was no jaw winking phenomenon.
Figure 1: (a) Clinical photograph showing mild ptosis of left upper lid (b) clinical photograph showing mild hypopigmentation of left iris (c) clinical photograph showing miosis of left pupil

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Based on the patient history and ocular examination, a diagnosis of Horner's syndrome was made.

Thereafter, a retrospective evaluation of the patient's birth history and mother's antenatal period was made, which revealed that the patient's mother had been diagnosed with gestational hypertension. Diagnosis of intrauterine growth restriction was also made based on antenatal ultrasonography. Doppler ultrasonography was done, which showed reversal of umbilical artery end diastolic flow. The patient had been delivered at 33 weeks via lower segment cesarean section. The mother gave history of noticing difficulty in breathing in the patient while breastfeeding at two hours of postnatal life. Chest X-ray was done and revealed coiling of feeding tube in the esophagus. The patient was diagnosed with tracheoesophageal fistula with esophageal atresia and underwent posterolateral thoracostomy with feeding gastrostomy and cervical esophagostomy at 28 hours postnatally. Postoperative period was uneventful based on medical records.

  Discussion Top

Horner's syndrome is a rare entity, having an incidence of 1 per 6000.[1] It can be broadly classified as congenital and acquired.It has been estimated that less than 5% of cases of Horner's syndrome are congenital in the pediatric population.[2] Horner's syndrome is characterized by ipsilateral partial ptosis, miosis, and anhidrosis. Congenital Horner's syndrome is characterized by presence of iris heterochromia, which is seen due to marked iris hypopigmentation in the affected eye.[1] In acquired Horner's syndrome of early onset (under two years of age), iris heterochromia may be present to a varying degree.[1],[3]

In the our case, based on the history of drooping of left upper lid and small left eye since birth, as noticed by the mother, and the ocular examination, a provisional diagnosis of congenital Horner's syndrome was made. However, in view of the finding of subtle iris heterochromia,[4] an extensive review of literature was done with regards to the etiology of Horner's syndrome.

The most common cause of congenital Horner's syndrome is birth trauma,[2],[4] though vascular malformations of the internal carotid artery and neoplasms have been cited as underlying etiologies. Vascular malformations may be associated with other anomalies.[3] Iatrogenic Horner's syndrome is rarely seen secondary to tube thoracostomy. Only seven cases have been described in the literature, of which only two were in the pediatric age group.[5] The incidence of Horner's syndrome secondary to malpositioned chest tube is 1%. Most cases develop within 12–72 hours of the surgical intervention.[6],[7]

There exists a thin endothoracic fascia between the parietal pleura and stellate ganglion. The pressure of the chest tube on the stellate ganglion may lead to ischemia and neuropraxia of the second neuronal pathway. Horner's syndrome due to malpositioned chest tube is reversible. Repositioning of the chest tube after radiological confirmation can lead to partial or complete recovery.[6],[7] The timing of the surgical intervention creates a diagnostic dilemma with respect to the etiology. However, since the patient was not evaluated during her infancy and there are no post- and perioperative records, it is difficult to comment upon the onset, severity, and recovery (if any) of the signs of Horner's syndrome.

[TAG:2]Conclusion [/TAG:2]

In conclusion, presence of early surgical intervention in the cardiothoracic region in the pediatric age group, as in our case, presents a diagnostic dilemma as to the etiology of Horner's syndrome.

Declaration of patient consent

The authors certify that they have obtained all appropriate patient consent forms. In the form, the patient(s) has/have given his/her/their consent for his/her/their images and other clinical information to be reported in the journal. The patients understand that their names and initials will not be published and due efforts will be made to conceal their identity, but anonymity cannot be guaranteed.

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Conflicts of interest

There are no conflicts of interest.

  References Top

Khan Z, Bollu PC. Horner syndrome. In: StatPearls. Treasure Island (FL): StatPearls Publishing; 2022.  Back to cited text no. 1
Shetty S, Vijayalakshmi P. Congenital or acquired Horner's? Indian J Ophthalmol 2007;55:399-400.  Back to cited text no. 2
[PUBMED]  [Full text]  
Deprez FC, Coulier J, Rommel D, Boschi A. Congenital Horner syndrome with heterochromia iridis associated with ipsilateral internal carotid artery hypoplasia. J Clin Neurol Seoul Korea 2015;11:192-6.  Back to cited text no. 3
Mirzai H, Baser E. Congenital Horner′s syndrome and the usefulness of the apraclonidine test in its diagnosis. Indian J Ophthalmol 2006;54:197.  Back to cited text no. 4
[PUBMED]  [Full text]  
Ozel SK, Kazez A. Horner's syndrome secondary to tube thoracostomy. Turk J Pediatr 2004;46:189-90.  Back to cited text no. 5
Levy M, Newman-Toker D. Reversible chest tube Horner syndrome. J Neuroophthalmol 2008;28:212-3.  Back to cited text no. 6
Kaya S. Horner's syndrome as a complication in thoracic surgical practice. Eur J Cardiothorac Surg 2003;24:1025-8.  Back to cited text no. 7


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