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At least 109 records · Page 6Linked to original sources
PubMed1991

New observations on midline defects: coincidence of anophthalmos, microphthalmos and cryptophthalmos with hypothalamic disorders.

Four children with severe congenital eye anomalies are described of which three had related symptoms. Two had bilateral anophthalmia, the optic nerves not detectable by computed cranial tomography and magnetic resonance imaging, and the third child had bilateral microphthalmia and coloboma iridis. The fourth patient had bilateral cryptophthalmia as part of Fraser syndrome. All four patients were of small stature. In three of them growth hormone deficiency was demonstrated which was of hypothalamic origin as shown by growth hormone releasing hormone tests. In the fourth child hypogonadotropic hypogonadism and tertiary thyroid deficiency were diagnosed which responded well to thyroxine treatment. Pathogenetically the described disorders are due to congenital defects of midline structures as a common "developmental field".

Abnormalities, Multiple↗
PubMed2000

Reliability of intraocular lens power calculation after cataract surgery in patients with relative anterior microphthalmos.

BACKGROUND: Patients with relative anterior microphthalmus (RAM) are characterized by special anatomical features (corneal diameters <11 mm, anterior chamber depth <2 mm and normal axial length) that sometimes make intraocular lens (IOL) power calculation difficult. PATIENTS AND METHODS: Seventy-five patients aged 75.6+/-10.3 years with RAM were evaluated for preoperative target refraction and postoperative refraction after cataract surgery. We used biometric formulas as modified by Haigis for IOL power calculation. RESULTS: The average IOL power implanted was 25.2+/-2.8 dptr (one-piece all-PMMA IOL; range 19-31 dptr). The preoperatively calculated target refraction was -0.71+/-1.43 dptr (range -4.3 to +2.2 dptr). The average postoperative spherical equivalent was -0.41+/-1.50 dptr (range -5.5 to +6.0 dptr). The mean difference between target and end refraction was -0.30+/-1.54 dptr Fifty-seven percent of cases did not differ by more than 1 dptr from target refraction, 81.3% by not more than 2 dptr, and 94.7% by not more than 3 dptr; only 5.3% of cases deviated by more than 3 dptr. There was no correlation of the amount of deviation between target and postoperative refraction with axial length, corneal diameter and anterior chamber depth. There was, however, a significant correlation between target refraction and amount of deviation of spherical equivalent. The largest differences were found with target refractions greater than +2 dptr. CONCLUSIONS: In spite of the special anatomical conditions in patients with RAM the biometric formulas as modified by Haigis produced reliable IOL power calculations. The best accuracy was achieved when aiming at a target refraction in the range of +/-2 dptr.

Adult↗
PubMed2001

Abnormal development of the lesser wing of the sphenoid with microphthalmos and microcephaly.

We report two patients with abnormal development of the lesser wing of the sphenoid bone, globe, optic nerve and cerebral hemisphere without stigmata of neurofibromatosis type 1. The lesser wing of the sphenoid bone was abnormally formed and was not ossified ipsilateral to the dysmorphic eye and underdeveloped cerebral hemisphere. Maldevelopment of the sphenoid wing may interfere with the normal closure of the optic vesicle and normal growth of encephalic structures, possibly by disturbing developmental tissue interactions. These patients may exhibit a type of restricted primary sphenoid dysplasia, while the sphenoid dysplasia of neurofibromatosis type 1 may be secondary to orbital or ocular neurofibromas and other factors associated with that disease.

Child↗
PubMed1985

Juvenile iridoschisis and microphthalmos.

Spontaneous iridoschisis and corneal edema developed in the microphthalmic eye of an 8-year-old. There was no history of trauma, inflammation, or congenital iris abnormality.

Cell Count↗
PubMed2001

Piggyback foldable intraocular lens implantation in patients with microphthalmos.

PURPOSE: To evaluate the clinical results of phacoemulsification and implantation of 2 foldable acrylic intraocular lenses (IOLs) in microphthalmic eyes. SETTING: University of Tokyo School of Medicine, Tokyo, Eguchi Eye Hospital, Hakodate, and Osaka Rosai Hospital, Osaka, Japan. METHODS: This study comprised 5 eyes of 3 patients whose manifest spherical equivalent was +10.5 to +18.0 diopters (D) and axial length was 15.79 to 16.82 mm. After phacoemulsification, 2 foldable acrylic IOLs with a mean power of +43.0 D +/- 9.5 (SD) (range +32.0 to +55.0 D) were implanted in the capsular bag. RESULTS: There were no significant intraoperative complications, and there was significant improvement in uncorrected and best corrected visual acuities after surgery. The spherical equivalent decreased significantly; however, the postoperative refraction was considerably more hyperopic than predicted in all eyes. Except for posterior synechias in 2 eyes and slight interlenticular opacification in 2 eyes, no major postoperative complications occurred during the mean follow-up of 14.8 months. CONCLUSIONS: Implanting 2 piggyback IOLs was beneficial in eyes with an extremely short axial length. However, the current system of lens power calculation markedly underestimates the required lens power, resulting in a hyperopic refractive error after surgery. The benefits of in-the-bag placement of 2 foldable lenses are questionable.

Acrylic Resins↗
PubMed1981

[Oculo-cerebral dysplasia: aplasia of the optic nerve with familial microphthalmos and cryptophthalmus. Clinical and computer tomography study].

The authors report on two siblings with severe malformations of the eyes. The 16-year-old girl has bilateral microphthalmus with aplasia of the right optic nerve and a Dandy-Walker cyst of the cerebellum. The right eye has no optic disk or retinal vessels. Her 13-year-old brother has unilateral aplasia of the optic nerve with cryptophthalmus and contralateral microphthalmus. In a computer tomography examination no optic nerve could be demonstrated on the left. The presence of an oculocerebral dysplasia in a brother and sister with healthy parents suggest an autosomal-recessive mode of inheritance of the aplasia of the optic nerve with microphthalmus and/or cryptophthalmus.

Adolescent↗
PubMed2005

Heminasal proboscis with associated microphthalmos and encephalocele.

Heminasal proboscis is a rare congenital malformation that presents complex management issues when associated with other craniofacial abnormalities. A newborn male, known to have a facial mass on prenatal ultrasonography, was delivered by planned induction at 37 weeks' gestation. He was intubated immediately because of anticipated respiratory difficulty. He had a right-sided proboscis, pedicled on the right medial canthal region. Additional airway evaluation identified a piriform aperture and choanal stenosis. Magnetic resonance imaging confirmed a hypoplastic orbit with aplastic optic nerve and a naso-orbito-ethmoidal encephalocele. Right upper and lower eyelid colobomas were present. The airway was initially stabilized with a tracheostomy. Beginning at the age of 10 weeks, he underwent several staged procedures to excise the encephalocele and to reconstruct the nose, orbit, eyelid, and lacrimal drainage system. The encephalocele was exposed via a bicoronal approach and frontal craniotomy. The dural defect was repaired with a pericranial flap. Nasal reconstruction provided an adequate nasal passage and tip after three staged procedures. Creation of a conjunctival flap prepared the right eye for later insertion of a prosthetic shell. The patient remained successfully decannulated 3 years after his last reconstructive procedure. Identification of all associated anomalies is important before initiating surgical repair. The presence of coexisting craniofacial abnormalities requires a multispecialty approach and coordination of surgical procedures.

Encephalocele↗