Archives of Medicine and Health Sciences

CASE REPORT
Year
: 2017  |  Volume : 5  |  Issue : 2  |  Page : 242--244

Visual rehabilitation with secondary intraocular lens implantation in a case of hallermann–streiff syndrome


S Rashmi1, Praveen S Alavandi2,  
1 Department of Ophthalmology, Yenepoya Medical College, Yenepoya University, Mangalore, Karnataka, India
2 Agarwal's Eye Hospital, Kuvempunagar, Mysore, Karnataka, India

Correspondence Address:
S Rashmi
Department of Ophthalmology, Yenepoya Medical College, Yenepoya University, Deralakatte, Mangalore - 575 018, Karnataka
India

Abstract

Hallermann–Streiff syndrome (HSS) is a rare congenital disorder with multiple abnormalities mainly affecting face, skull, hair, eyes, and teeth. We report a case of an 18-year-old Indian girl with HSS having typical physical features and ophthalmological manifestations. She had been operated for bilateral congenital cataract in her infancy, and since then, she had been aphakic in both eyes. She had poor vision in the right eye and irreversible blindness in the left eye. She underwent scleral-fixated (glued) intraocular lens implantation in the right eye with good visual recovery.



How to cite this article:
Rashmi S, Alavandi PS. Visual rehabilitation with secondary intraocular lens implantation in a case of hallermann–streiff syndrome.Arch Med Health Sci 2017;5:242-244


How to cite this URL:
Rashmi S, Alavandi PS. Visual rehabilitation with secondary intraocular lens implantation in a case of hallermann–streiff syndrome. Arch Med Health Sci [serial online] 2017 [cited 2022 May 20 ];5:242-244
Available from: https://www.amhsjournal.org/text.asp?2017/5/2/242/220822


Full Text



 Introduction



Hallermann–Streiff syndrome (HSS) is synonymous with Francois dyscephalic syndrome, Aubry syndrome, Ullrich-Fremerey-Dohna syndrome, oculomandibulofacial syndrome, or oculomandibulodyscephaly with hypotrichosis.

It is a syndrome with multiple congenital abnormalities, which affects face, skull, hair, eyes, teeth, and overall growth and development. It affects both males and females in all ethnic groups. Over 150 cases of HSS have been reported worldwide.[1]

The potential causes of this syndrome include an asymmetric second branchial arch defect that arises during the 5th or 6th gestational week, maternal viral infection, toxin exposure, and paternal age. Most cases are sporadic. An autosomal dominant inheritance with variable expression or a new mutation has been mentioned, but some reports have suggested the possibility of autosomal recessive inheritance.[2]

General features

Patients generally present with small bird-like facies, a beak-shaped nose which is pinched and tapering at the tip. The skull is brachycephalic with frontal bossing. They have a small chin, underdeveloped jaw, and a small mouth. Hair is usually sparse particularly that of scalp, eyebrows, and eyelashes (hypotrichosis). Skin shows atrophy. They may have natal teeth, and dental anomalies are common. Short stature is seen in about half of the individuals with HSS (proportionate dwarfism). There may be musculoskeletal and cardiac abnormalities. Most individuals have normal intelligence; however, 15%–30% of individuals have some degree of mental retardation. A narrow upper airway, small chin, and shape of the skull can pose a risk during intubation and general anesthesia. They may suffer sleep apnea and repeated respiratory infections which can be fatal.[1],[2],[3]

Ophthalmological features

Ocular abnormalities are a major problem with the most common ocular features being microphthalmia and cataracts which are present in 90% of HSS patients.[1] Congenital cataracts are one of the most common characteristics of HSS.[1] Cataract may be membranous or may have spontaneous absorption leading to aphakia. Other ophthalmic features include nystagmus, strabismus, blue sclera, and microcornea. Adnexal abnormalities include sparse eyelashes and eyebrows, skin atrophy, hypoplasia of lacrimal puncta, eyelid abnormalities (entropion, ptosis, and lower eyelid coloboma), and downslanting palpebral fissure. Some other findings which may be present are ocular hypertension, glaucoma, pale optic disc, disc coloboma, choroidal atrophy, macular degeneration, iris atrophy, aniridia, and corneal stromal opacities.[1],[2]

The differential diagnoses of this condition include oculodentodigital dysplasia, mandibulofacial dysostosis, cleidocranial dysostosis, progeria, and other progeroid syndromes.[2]

HSS is a rare syndrome although it is a known entity. Cataract surgery with intraocular lens (IOL) implantation can be a challenging issue in these patients, and there is very little literature available regarding this. This particular case report provides evidence that glued IOL implantation can be safe, if done carefully. Hence, it can be considered as an effective option for visual rehabilitation in these patients.

 Case Report



An 18-year-old Indian female patient presented with poor vision in both eyes. She was born after an uncomplicated full-term pregnancy of a nonconsanguineous marriage. She was diagnosed to have congenital cataract at the age of 1 month, and lensectomy was done in both eyes. She had been wearing aphakic glasses since childhood. Her general appearance and ophthalmological findings led us to diagnose her with HSS [Figure 1] and [Figure 2]. There was no notable family history. She had an average height (160 cm) and had mild mental retardation. She had microphthalmos, microcornea, nystagmus, and bilateral surgical aphakia. Corneal diameter in the right eye was 9.5 mm and in the left eye was 9 mm. Uncorrected visual acuity in the right eye was only counting finger at 3 m. Even with her very thick and heavy plus 16 Diopters glasses, she could hardly read the 6/60 line in the Snellen chart. The left eye was totally blind (no perception of light, glaucomatous optic atrophy, exotropia, and no fixation). Secondary IOL implantation was planned in the right eye. Biometry showed short axial length (18.76 mm) with normal anterior chamber depth (4.34 mm). Corneal curvature measured 50 Diopters both horizontally and vertically. IOL power calculated was plus 30 Diopters. There was no posterior capsular support. She underwent glued IOL implantation under peribulbar anesthesia. Glued IOL implantation is a technique wherein posterior chamber IOL is implanted with the use of fibrin glue.[4]{Figure 1}{Figure 2}

Two partial-thickness scleral flaps were constructed exactly 180° diagonally apart followed by sclerotomy 1 mm from the limbus. Thin sclera should be kept in mind while choosing the positioning of flaps. An anterior chamber maintainer was used. Anterior vitrectomy was done. Sensar 3-piece acrylic foldable IOL was injected through clear corneal incision. First, the leading haptic and subsequently the trailing haptic were externalized using an end gripping 23G microrhexis forceps (MicroSurgical technology, USA). Both haptic tips were tucked into the intralamellar scleral tunnel made with a 26G needle in the bed of scleral flaps. The sclerotomies were sutured, and the scleral flaps were glued over using fibrin glue (Tisseel, Baxter, USA). Conjunctiva over sclera flaps was closed with 10-0 monofilament nylon. Corneal wound was hydrated and closed after removing the anterior chamber maintainer. At the end of the surgery, well-centered scleral-fixated (glued) IOL was achieved.

Postoperative course was uneventful. Postoperative intraocular pressure was 12 mmHg (measured with Goldmann applanation tonometry). Her vision improved to 6/24 (uncorrected visual acuity) at the end of 6 weeks. Near vision was N8 with a correction of +2.5 Diopter. At 1-year follow-up, vision remained the same; IOL was well centered, stable with no pseudophacodonesis; haptics were in position with no extrusion. No evidence of secondary glaucoma was noted in the operated eye.

 Discussion



A multidisciplinary approach may be required to tackle the multiple disorders of HSS patients.[3],[5],[6],[7] Ophthalmologists have a major role to play as the patients present with multiple problems related to eyes, the majority being congenital cataract or aphakia in later life (either due to spontaneous absorption of lens or surgical removal of cataractous lens).[2],[3],[8],[9] Special attention should be paid during surgical treatment. Careful anesthetic management is needed.[1],[6] IOL sizing as well as high plus power IOL could be an issue in small eyes. Shen et al. have reported piggyback IOL in a patient with HSS.[2]

The short eye axis and thin sclera need cautious handling during surgery. They pose a risk of postoperative hypotony, exudative retinal detachment, and choroidal detachment. The cases with exudative retinal detachment after operating cataract in HSS patients have been reported.[10] Surgeons should keep in mind sclerectomy if needed. A vitreoretinal setting should be available.

As with any surgery, glued IOL surgery has its own complications, and the surgeon should be careful in handling them. The surgery, if done well, can give excellent results.[11] Advances in surgical techniques such as scleral tucking of the haptics increase the stability of the IOL tremendously, and also, consistent vault is maintained between the iris and the IOL which is one of the factors for less postoperative uveitis and pigment dispersion. In a retrospective analysis of the patients who underwent glued IOL implantation, there was no postoperative secondary glaucoma in any of the eyes.[4]

Scleral-fixated (glued) IOL is a safe and effective option for providing visual rehabilitation in patients with HSS. Good visual outcome was achieved in our patient, which relieved her from aphakic glasses and significantly improved her quality of life. However, careful preoperative planning, meticulous surgery, and close postoperative follow-up are needed.

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.

Financial support and sponsorship

Nil.

Conflicts of interest

There are no conflicts of interest.

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