Finally, 40% of patients with biopsy-proven orbital inflammation were classified as IgG4-RD, with typical histological features, but without specific clinical or radiological findings.
Fifty-three eyelids with severe ptosis were treated by frontalis suspension with temporalis fascia. The surgical indications were limited to cases in which there was absent or minimal levator function. The authors used this as a primary modality in patients with congenital ptosis, blepharophimosis syndrome, Marcus Gunn jaw winking syndrome, severe progressive external ophthalmoplegia, severe blepharospasm and post-traumatic ptosis. Temporalis suspension was performed as a secondary procedure after failure of various other procedures, including levator resection, and suspension with PTFE (Goretex), Mersilene, or fascia lata. Results after an average followup period of six months (two to 14 months) were good in 51 cases ; in two cases release of the suspension and recurrence of ptosis were treated by re-suspension with fascia lata.Temporalis fascia has been used in the correction of ectropion, lagophthalmos in facial nerve palsy and in reconstruction of post-traumatic eyelid defects. It has three major advantages: (1) it is autogenous and hence better tolerated than synthetic materials such as Goretex or Mersilene;(2) it is easily harvested, requiring only one operative field, under local anesthesia; (3) it yields minimal post-operative morbidity (no interference with ambulation, shorter convalescence). The disadvantage of this procedure is the fragility of temporalis fascia as compared to fascia lata, which limits its use to patients over six years old.
To our knowledge, only 10 cases of Joubert syndrome have been published so far. In this paper, we describe the clinical, radiological (computerized tomography) and polygraphic findings in an additional patient. The female presented here is the product of consanguineous parents and a sibling of a previously reported infant. In addition to the well-known episodic tachypnea in an awake state, representing the clinical hallmark of this syndrome, this child also had bouts of tachypnea while asleep. Interestingly enough, these were confined only to non-REM sleep.
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