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A retrospective analysis of 20 cases of orbital fistula in Shanghai showed the causes to be trauma with foreign-body retention, osteomyelitis, mucocele, and dermoid cyst. Half the patients were children younger than 10 years old. Cicatricial ectropion, ptosis, and extraocular motility disturbance constituted the common clinical findings. Treatment according to the various causes included surgical removal of the foreign body, oral administration of antibiotics combined with local irrigation, radical extraction of all the epithelium lining the fistula, and excision of the fistula.
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PMID:Orbital fistula. Causes and treatment of 20 cases. 663 28

An eight-month-old girl presented with congenital exotropia and latent nystagmus. Further evaluation revealed congenital ptosis of the left eye and restriction of the elevation, depression and adduction of the left eye. A diagnosis of congenital oculomotor palsy was made. At the age of three months she had been examined by the neurologist because of retarded psychomotor development. All laboratory investigations were normal. At the age of eight months, a CT scan of the brain and orbit was found to be normal. The patient was treated for amblyopia. At the age of five, strabismus surgery was performed, and a large fibrous tumor encapsulating the superior and lateral rectus muscle was found. A biopsy was taken and pathology showed fibrous tissue containing a hair. Based on the clinical history, the diagnosis of a perinatally ruptured orbital dermoid cyst was made. Review of the previous CT and an additional CT showed enlargement of the left lateral orbital wall with a notch in the lateral wall, indicative of a dermoid cyst.
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PMID:Perinatally ruptured dermoid cyst presenting as congenital oculomotor palsy. 1085 51

A 62-year-old lady presented with a six-month history of swelling of the left upper eyelid, resulting in mild mechanical ptosis. Clinical assessment suggested a provisional diagnosis of dermoid cyst. The lesion was excised and histology revealed nodular deposits of amorphous eosinophilic material surrounded by lymphocytes and plasma cells. Special histochemistry and immunoperoxidase stain results showed deposition of amyloid, non-AA type. The lesion recurred 6 months later.
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PMID:Primary localised deep cutaneous amyloidosis of the eyelid. 1719 94

Phacomatosis pigmentokeratotica (PP) is a mosaic disorder that represents a distinct epidermal naevus syndrome. Its defining features are an epidermal naevus that is usually of the sebaceous type and a speckled lentiginous naevus arranged in a chequerboard pattern. In addition, there are neurological, ophthalmological and skeletal abnormalities, including limb hemiatrophy with muscular weakness, ptosis, seizures and ipsilateral segmental hyperaesthesia and hyperhidrosis. We report a 44-year-old man with an extensive epidermal naevus and an ipsilateral speckled lentiginous naevus. He also had ipsilateral right leg hypertrophy and a suprasellar dermoid cyst with associated neurological abnormalities. We propose that this case represents an unusual example of PP.
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PMID:Phacomatosis pigmentokeratotica associated with a suprasellar dermoid cyst and leg hypertrophy. 1786 92

Dermoid cysts are choristomas (a mass of histologically normal tissue in an abnormal location), which originate from aberrant primordial tissue and are often evident soon after birth. It may occur anywhere in the body. Dermoid cysts account for about 3-9% of all orbital masses and 0.04-0.6% of primary orbital tumors. The frequent site of origin is the superotemporal quadrant of orbit. Depending on the location, size, and associated abnormalities of the cyst, the patient may have proptosis, diplopia, and restriction of eye movements. Monocular elevation deficiency (MED) is the inability to elevate one eye in abduction, adduction, or from primary position. We report this case of orbital dermoid in an adult female presenting as a monocular elevation deficiency with unilateral ptosis of right eye which was investigated and managed with good results.
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PMID:Orbital dermoid mimicking a monocular elevation deficiency. 2299 70

The aim of this study was to investigate the types and presentation pattern of congenital defects of the eye and adnexia in our center. This is a retrospective review of congenital defects of the eye and adnexia over a 20-month period at a tertiary referral center in Lagos, Nigeria. Records were analyzed for age at presentation, laterality, gender, vision assessment, and type(s) of abnormality. Out of 412 pediatric patients, 40 (9.7 %) were seen to have congenital abnormality of the eye and/or its adnexia during the study period. There were 17 (42.5 %) males. Twelve patients (30.0 %) presented with involvement of the right eyes, nine (22.5 %) with left eyes, while 19 (47.5 %) had bilateral involvement. Twenty-eight patients (70.0 %) were aged 1 year or less at the time of presentation. A total of 69 entities were recognized as some children had two or more malformations. The common congenital defects identified were cataract (39.1 %), ptosis (17.4 %), glaucoma (8.7 %), and cornea opacity (7.2 %). Other less common congenital defects include: microphthalmos, anophthalmos, coloboma (lid and iris), dermoid cyst, and aniridia. All of the patients with available visual acuity documentation had visual impairment. A high proportion of the patients were lost to follow-up. Cataract, ptosis, and glaucoma were the commonest congenital defects of the eye and adnexia in our center and were associated with visual impairments. The significant loss of the patients during follow-up needs urgent investigation and attention to forestall this trend.
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PMID:Spectrum of congenital defects of the eye and its adnexia in the pediatric age group; experience at a tertiary facility in Nigeria. 2474 44

Ectopic cilia are extremely rare congenital anomalies in which eyelash follicles appear in an abnormal place on the eyelid, most typically on the lateral quadrant of the anterior surface of the upper eyelid. In the majority of cases, simple surgical excision of ectopic cilia is indicated because of its cosmetic aspect. There is usually no associated medical co-morbidity with this anomaly. The authors report an unusual case of ectopic cilia associated with an orbital dermoid cyst and sinus tract. A 3-year-old boy was initially diagnosed with ectopic cilia on the left upper eyelid. There was no history of inflammation or swelling of the eyelid. An ophthalmological examination revealed only 1 mm of ptosis; no proptosis, inferior displacement, or palpable orbital mass was present. During surgical excision of the ectopic cilia, a thin sinus tract was identified, leading posteriorly to the orbit. Magnetic resonance imaging performed after the excision showed a supraorbital extraconal mass just below the roof of the left orbit. A supraorbital 2-piece craniotomy was performed with total extirpation of the dermoid cyst. The cyst was removed en bloc without damage to the extraocular muscles, but the sinus tract could no longer be identified. Follow-up MRI was performed 6 months after surgery and showed no evidence of recurrence. A follow-up ophthalmological examination showed no signs of inferior displacement or proptosis. To the best of the authors' knowledge, this case is the first reported instance of ectopic cilia associated with a dermoid cyst and sinus tract in which no typical clinical signs and symptoms of possible orbital pathology were present. This case highlights the value of radiological examination in all cases of ectopic cilia prior to surgical excision.
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PMID:Ectopic cilia associated with an orbital dermoid cyst and sinus tract: case report. 2597 33