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mary 's hospital complaining of ocular discomfort due to cilia that had been touching the corneas of both eyes for several years .
he had bird - like facies , a pinched nose , hypotrichosis of the scalp , mandibular hypoplasia with forward displacement of the temporomandibular joints , a small mouth with abnormal dentition , and proportional short stature ( about 150 cm in height ) .
his general appearance led us to diagnose him with hallermann - streiff syndrome ( fig .
his right visual acuity was 0.04 ( 0.1  -2.75 ds = -3.0 dc a  90 ) by snellen chart , and his left visual acuity allowed him to count fingers at 20 cm ( uncorrected  -2.5 ds = -1.25 dc a  90 ) .
keratometry measurements were k1 = 57.00 / k2 = 58.75 in the right eye and k1 = 54.50 / k2 = 55.75 in the left eye .
his axial length was 23.77 mm in the right eye and 23.69 in the left eye .
ophthalmic features included sparse eyelashes and eyebrows , microcornea ( cornea diameter of right eye vertical / horizontal ( v / h ) = 9.25/8.75 mm , left eye ( v / h ) = 9.0/8.75 mm ) , nystagmus , right lower eyelid entropion , and bilateral upper eyelid entropion with aponeurotic blepharoptosis .
right marginal reflex distance 1 ( mrd1 , distance from upper eyelid margin - corneal reflex ) was 1 mm , left mrd1 was 0 mm , and levator palpebrae function was 10 mm in both eyes .
we noted lid drooping at his inferior gaze and an overactive frontalis muscle compensating for weak levator function , especially on the right side .
there was esodeviation of the eyeball of more than 100 prism diopters at near and distance .
the patient had never undergone cataract surgery , but he was aphakic in both eyes .
the patient refused to undergo dna analysis or imaging evaluation for other associated systemic findings , such as spina bifida , scoliosis , lordosis , respiratory disorders , and dental abnormalities .
the capsulopalpebral fascia was repaired to treat right lower eyelid entropion , but an additional quickert suture was needed to prevent recurrence .
upper eyelid blepharoplasty and levator palpebrae repair were performed for correction of aponeurotic blepharoptosis and dermatochalasis .
we noted very thin eyelid skin and tarsal plates , fatty infiltration in front of the tarsal plates , and severe fat tissue replacement of the levator palpebrae .
three months after the eyelid surgeries , the right medial rectus was recessed 7.5 mm , the left medial rectus was recessed 7.25 mm , and the left lateral rectus muscle was resected 8.0 mm under general anesthesia with endotracheal intubation ( fig .
the patient had persistent esodeviation of about 20 prism diopters but had no other lid complications at his last visit .
most cases seem to be sporadic , but some genetic abnormalities and family histories have been reported .
hss patients exhibit many abnormalities involving various systems : craniofacial , mandibular , and dental anomalies with or without musculoskeletal , cardiac , or respiratory problems and mental retardation [ 1 - 3,7 - 10 ] .
diagnosis is based on clinical findings like dyscephaly with bird - like facies , abnormal dentition , hypotrichosis , atrophy of the skin ( especially on the nose ) , congenital cataracts , bilateral microphthalmia , and proportionate dwarfism .
ocular abnormalities are a major problem , with the most common ocular features being microphthalmia and cataracts , which are present in 90% of hss patients [ 1,2,6 - 8 ] .
seven cases of hss have been reported in korea : 2 by pediatricians and 5 by ophthalmologists [ 9,12 - 17 ] .
although congenital cataracts are one of the most common characteristics of hss , two previously reported patients had spontaneous aphakia . the patient in this report had no notable family history , cardiac or respiratory disorders , or mental retardation .
table 1 shows the ophthalmic manifestations that have been reported in patients with hss , as well as the positive findings in this case [ 3 - 11 ] .
the patient had normal range of axial length , but did have microcornea , which is uncommon in hss .
he was also aphakic , despite the fact that he had never undergone cataract surgery .
the lenses may have been absorbed spontaneously after birth as he advanced in age , which sometimes occurs in the setting of hss .
other ophthalmic findings included nystagmus , intraocular hypertension , sparse eyelashes and eyebrows , esotropia , and eyelid problems : blepharoptosis and entropion . to our knowledge
we performed entropion and blepharoptosis repair after administering a local anesthetic injection . during eyelid surgery
the patient 's precise esodeviation could not be determined because of his nystagmus , but it was certainly over 100 prism diopters . considering the preoperative extraocular muscle limitations , we chose 3 muscles to reposition no more than 8 mm . because of poor visual acuity , the patient had no diplopia before or after surgery .
david et al .   reported that general anesthesia with endotracheal intubation was difficult and risky in hss , even in its relatively mild form .
many cases of respiratory distress have been reported , especially in the postoperative period . in the current case , we prepared by consulting the anesthesia team before strabismus surgery .
in conclusion , we present a case of successful surgical repair for hss in a patient who had no complications but who had uncommon ophthalmic features of strabismus , entropion , and blepharoptosis .
we report a case of surgical treatment for hallermann - streiff syndrome in a patient with ocular manifestations of esotropia , entropion , and blepharoptosis . 
 a 54-year - old man visited yeouido st . 
 mary 's hospital complaining of ocular discomfort due to cilia touching the corneas of both eyes for several years . 
 he had a bird - like face , pinched nose , hypotrichosis of the scalp , mandibular hypoplasia with forward displacement of the temporomandibular joints , a small mouth , and proportional short stature . 
 his ophthalmic features included sparse eyelashes and eyebrows , microphthalmia , nystagmus , lower lid entropion in the right eye , and upper lid entropion with blepharoptosis in both eyes . 
 there was esodeviation of the eyeball of more than 100 prism diopters at near and distance , and there were limitations in ocular movement on lateral gaze . 
 the capsulopalpebral fascia was repaired to treat the right lower lid entropion , but an additional quickert suture was required to prevent recurrence . 
 blepharoplasty and levator palpebrae repair were performed for blepharoptosis and dermatochalasis . 
 three months after lid surgery , the right medial rectus muscle was recessed 7.5 mm , the left medial rectus was recessed 7.25 mm , and the left lateral rectus muscle was resected 8.0 mm .