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 Table of Contents  
Year : 2021  |  Volume : 1  |  Issue : 2  |  Page : 277-279

Lens particle glaucoma as a presenting feature of Hallermann–Streiff syndrome

Department of Ophthalmology, Lady Hardinge Medical College and Associated Hospitals, University of Delhi, New Delhi, India

Date of Submission19-Jun-2020
Date of Acceptance13-Sep-2020
Date of Web Publication01-Apr-2021

Correspondence Address:
Dr. Siddharth Madan
Department of Ophthalmology, Lady Hardinge Medical College and Associated Hospitals, University of Delhi, New Delhi - 110 021
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Source of Support: None, Conflict of Interest: None

DOI: 10.4103/ijo.IJO_1983_20

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Hallermann–Streiff syndrome (HSS), an asymmetric second branchial arch defect affects growth, cranial and dental development. A 40-year-old lady with HSS developed severe pain and redness in her right eye due to raised intraocular pressure (IOP; 51 mmHg). A ruptured anterior capsule with cortical lens matter and granulomatous reaction was seen in anterior chamber (AC) along with absorbed cataract. Treatment with antiglaucoma medications, steroids followed by AC wash, anterior capsulotomy with anterior vitrectomy normalized IOP and inflammation reduced. Lens particle glaucoma is a rare presentation of HSS. Therefore, early cataract surgery is recommended that also avoids the risk of suppression amblyopia.

Keywords: Amblyopia, bilateral cataracts, Hallermann–Streiff syndrome, lens-induced glaucoma, lens particle glaucoma, microphthalmia, vitrectomy

How to cite this article:
Kapoor M, Madan S, Singh P, Beri S, Nayana T K. Lens particle glaucoma as a presenting feature of Hallermann–Streiff syndrome. Indian J Ophthalmol Case Rep 2021;1:277-9

How to cite this URL:
Kapoor M, Madan S, Singh P, Beri S, Nayana T K. Lens particle glaucoma as a presenting feature of Hallermann–Streiff syndrome. Indian J Ophthalmol Case Rep [serial online] 2021 [cited 2023 Feb 7];1:277-9. Available from: https://www.ijoreports.in/text.asp?2021/1/2/277/312348

Hallermann–Streiff syndrome (HSS) is a result of an asymmetric second branchial arch defect seen during the fifth to sixth gestational week.[1] The most likely hypothesis is that a single mutant (dominant) gene is responsible, with most cases representing fresh sporadic mutations.[2],[3] Recently, a defect of elastin and abnormal glycoprotein metabolism has been reported. HSS is characterized by seven essential signs as described by Francois. These include dyscephaly (scaphocephaly or brachycephaly with frontal bossing) {98-99%}, typical bird-like facies (micrognathia, condylar aplasia, and thin pointed nose) (98-99%); dental anomalies (80-85%); proportionate nanism (45-68%); hypotrichosis (80-82%); atrophy of the skin (68-70%) localized to the head and nose; bilateral microphthalmia (78-83%); and congenital cataracts (81-90%).[1],[2],[3] Ocular anomalies are a major problem in patients with HSS.[4],[5],[6] Glaucoma is an uncommon presenting feature of HSS.[7]

  Case Report Top

A 40-year-old lady presented with complaints of severe pain and redness in the right eye (OD) for 3 days. She had typical features of HSS on examination [Figure 1]a, [Figure 1]b, [Figure 1]c, which include bird-like face [Figure 1]b and [Figure 1]c, dental anomalies [Figure 1]c, hypotrichosis, and short proportionate stature [Figure 1]a. The mouth was small with thin lips. Hypoplasia of the mandible (micrognathia) and a beak-like nose was observed. Movements of temporo-mandibular joint were minimally limited. Several persisting deciduous teeth with irregular dentition and poor oral hygiene were noted after an opinion with a dentist. A psychiatry consultation was sought and she was assessed for her intelligence that was found to be normal.
Figure 1: (a-c) The patient had a short proportionate stature (a), bird-like face (b), and dental anomalies (c). The mouth was small with thin lips (a). Hypoplasia of the mandible (micrognathia) and a beak-like nose was observed (b). Irregular dentition and poor oral hygiene were noted (c)

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She disclosed a history of bilateral congenital cataract; however, the left eye (OS) was pseudophakic, as she underwent an uneventful cataract surgery 20 years back although the relevant operative records were unavailable. Occlusion therapy was advised along with the use of corrective spectacles following cataract surgery in view of low-vision OS, but the patient did not use glasses. She denied to get operated for cataract in her right eye at the same time inspite of being advised by the operating surgeon. On presentation, best-corrected visual acuity (BCVA) OD and OS was finger counting at 2 m and 20/120 (-1.00 diopter spheres {DS}; Near vision with addition of +2.50 DS -20/80), respectively, with accurate projection of rays (PR) in both eyes (OU). Her intraocular pressure (IOP) on presentation was 51 mmHg OD; 18 mmHg OS. A ruptured anterior capsule along with cortical lens matter and granulomatous reaction was seen in the anterior chamber (AC) OD [Figure 2]. Gonioscopy revealed widely open angles without any evidence of neovascularization in both eyes (OU). Fundus details were unclear OD due to the presence of corneal edema and a dense AC reaction. Ultrasound B-scan was anechoic. Fundus examination OS was normal. In the current presentation, the patient was diagnosed with lens particles glaucoma OD, was admitted, and treatment with oral acetazolamide along with intravenous mannitol initiated. Simultaneously, oral and topical steroid, topical antiglaucoma drugs, and cycloplegic agent were started. On day 2 of admission, her IOP reduced to 15 mmHg and so did the AC reaction with a faintly visible fundal glow on indirect ophthalmoscopy. IOP OS was 18 mmHg. By fifth day, granulomatous reaction in the AC disappeared. During the hospital stay, medicine, dental, and psychiatry opinion confirmed the diagnosis of HSS. The patient was nondiabetic, nonhypertensive. AC wash along with anterior capsulotomy, anterior vitrectomy with posterior chamber intraocular lens implantation in the ciliary sulcus was performed through the anterior route by making a clear corneal entry using MVR blade measuring 19G. On the first postoperative day, VA OD was 20/120 and IOP was 13 mmHg with a normal appearing fundus. Patient was discharged on topical steroid and antibiotic eye drops, topical nepafenac, and carboxymethylcellulose. Her IOP on the third day following surgery was found to be 20 and 19 mmHg OD and OS, respectively. Follow-up [[Figure 3] OD] IOP at six weeks was 18 mmHg OU. All other topical medications were stopped. BCVA OU was 20/120 (OS: -0.75 DS/-0.50 diopter cylinders x 80°, Near vision with addition of + 2.50 DS -20/100).
Figure 2: Preoperative picture showing spontaneous absorption of cataract, anterior capsular defect, and anterior chamber reaction at the angle

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Figure 3: Postoperative picture showing posterior chamber intraocular lens in situ, mildly decentered but stable. A residual membranous cataract was visible and anterior chamber reaction was absent

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  Discussion Top

No specific gender predilection in HSS is reported.[4],[5] The patient, previously undiagnosed with HSS, had maximum features of HSS except microphthalmia. In addition, the life-threatening manifestations of the syndrome, such as respiratory difficulties and cardiac defects, were however absent in this patient.[6] Spontaneous absorption of cataract in patients with HSS is known.[6],[7],[8],[9] Other commonly noticed ocular findings include nystagmus (32-45%), strabismus (33-37%), blue sclera (22-31%), sparse eyelashes and eyebrows (29%), fundus anomalies (18-22%), conjunctival defects (11%), cornea abnormalities (9-14%), and down-slanting palpebral fissures (12-13%).[4] Glaucoma in patients with HSS is rare.[7],[10] Glaucoma may be a result of developmental anomalies or due to complication or sequel of intraocular inflammation. Glaucoma usually occurs after the lens has been needled or has undergone a spontaneous rupture and may result in a granulomatous anterior uveitis.[10] However, the evidence of intraocular inflammation in HSS is strong and where glaucoma has occurred, background signs of uveitis or its sequel invariably exist. Lens particle glaucoma differs from phacolytic glaucoma by the presence of a macroscopically visible capsular rupture with the release of cortical matter into the AC, which induces an immunologic response leading to blockage of the trabecular meshwork by inflammatory cells and lens proteins or lenticular fluid. Spontaneous anterior lens capsule rupture without a lens injury has been described.

In this case, the left eye was pseudophakic yet amblyopic. The other eye was delayed in its timely management, hence developed complications. Therefore it appears preferable to operate on the cataracts OU of these patients as soon as possible, thus avoiding spontaneous cataract absorption and suppression amblyopia, hence improving the prognosis.[4],[5],[6],[7],[8],[9],[10] Postoperative reviews must be frequent and early amblyopia therapy may prove beneficial for rehabilitation. A delayed intervention in the hope that spontaneous cataract absorption will occur in a difficult microphthalmic eye only worsens the prognosis.[10] Therefore, it is wise to prime these patients for a timely surgery of cataract to prevent sight-threatening sequel and complications.

  Conclusion Top

Spontaneous anterior capsular rupture and lens particle glaucoma may complicate HS syndrome. This is one such report. We emphasize on early cataract surgery on HS syndrome.

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Conflicts of interest

There are no conflicts of interest.

  References Top

Kirzioğlu Z, Ceyhan D. Hallermann-Streiff syndrome: A case report from Turkey. Med Oral Patol Oral Cir Bucal 2009;14:E236-8.  Back to cited text no. 1
Shiomi T, Guilleminault C, Izumi H, Yamada S, Murata K, Kobayashi T. Obstructive sleep apnoea in a puerperal patient with Hallermann-Streiff syndrome. Eur Respir J 1999;14:974-7.  Back to cited text no. 2
Tuna EB, Sulun T, Rosti O, El Abdallah F, Kayserili H, Aktoren O. Craniodentofacial manifestations in Hallermann-Streiff syndrome. Cranio 2009;27:33-8.  Back to cited text no. 3
Cohen MM Jr. Hallermann-Streiff syndrome: A review. Am J Med Genet 1991;41:488-99.  Back to cited text no. 4
Francois J. A new syndrome; dyscephalia with bird face and dental anomalies, nanism, hypotrichosis, cutaneous atrophy, microphthalmia, and congenital cataract. AMA Arch Ophthalmol 1958;60:842-62.  Back to cited text no. 5
David LR, Finlon M, Genecov D, Argenta LC. Hallermann-Streiff syndrome: Experience with 15 patients and review of the literature. J Craniofac Surg 1999;10:160-8.  Back to cited text no. 6
Shen W, Dai M, Su Y, Zhang Q, Li H. Hallermann-Streiff syndrome with uncommon ocular features, ultrasound biomicroscopy and optical coherence tomography findings: A case report. Medicine (Baltimore) 2019;98):e18272.  Back to cited text no. 7
Rohrbach JM, Djelebova T, Schulze Schwering MJ, Schlote T. Hallermann-Streiff-Syndrom: Sollte die Spontanresorption der getrübten Linse abgewartet werden? [Hallermann-Streiff syndrome: Should spontaneous resorption of the lens opacity be awaited?]. Klin Monbl Augenheilkd 2000;216:172-6. German.  Back to cited text no. 8
Soriano JM, Funk J. Spontane beidseitige Linsenresorption bei einem Fall von Hallermann-Streiff-syndrom [Spontaneous bilateral lens resorption in a case of Hallermann-Streiff syndrome]. Klin Monbl Augenheilkd 1991;199:195-8. German.  Back to cited text no. 9
Hopkins DJ, Horan EC. Glaucoma in the Hallermann-Streiff syndrome. Br J Ophthalmol 1970;54:416-22.  Back to cited text no. 10


  [Figure 1], [Figure 2], [Figure 3]


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