Young patient experiences bilateral fatty infiltrative ptosis
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Fatty infiltration of the levator muscle has been studied widely as a contributing factor in the pathogenesis of congenital, acquired and involutional ptosis.
In 1986, Cahill and colleagues first reported combined fatty degeneration of Müller’s muscle and the anterior levator muscle. This can act as a common differential for other forms of ptosis in young individuals. We present a case report of bilateral fatty infiltrative ptosis in a young patient and the way it was managed surgically.
Clinical presentation
A 26-year-old woman presented to us with slow and progressive drooping of both upper lids over a period of 4 years. On examination, bilateral ptosis was apparent (more severe in the right eye) with frontalis overaction and constitutional inferior scleral show. Both lids showed fullness and negative lid transparency sign. For the right upper lid, marginal reflex distance 1 (MRD1) was 0.5 mm, marginal reflex distance 2 (MRD2) was 9 mm, vertical fissure height (VFH) was 9 mm, and levator palpebrae superioris (LPS) action was 11 mm of lid excursion. For the left upper lid, MRD1 was 1 mm, MRD2 was 9 mm, VFH was 10 mm, and LPS action was 15 mm (Figure 1).
Source: Dhivya Ashok Kumar, MD, FRCS, FICO, FAICO, Amar Agarwal, MS, FRCS, FRCOphth, and Shana Sood, MBBS, DNB
Marked inferior scleral show was present, but lagophthalmos was absent, and good Bell’s phenomenon was noted in both eyes, with no synkinesis or lid twitch noted. Both pupils were isochoric with normal light and consensual reflex, and extraocular movements were free and full in both eyes. Phenylephrine test showed no improvement in ptosis in either eye, suggestive of absent/poor Müller’s muscle function. No fatigability was present with negative ice pack test. Assessment of old family photographs showed bilateral ptosis manifesting only in the last few years; it was absent in childhood. The patient’s thyroid profile was normal.
Intraoperative observation and histology
The patient was advised to undergo bilateral LPS plication, right eye followed by left eye. Intraoperative dissection revealed yellow fatty lobules dispersed evenly across the Müller’s muscle projection area and on the levator muscle fibers; excision of this fatty tissue was carried out along with LPS plication. Histopathological examination revealed mature adipocytes interspersed among skeletal muscle fibers, suggestive of fatty infiltration of the levator muscle. There were linear fragments of tissue showing skeletal muscle fibers, with interspersed fibrocollagenous stroma showing sparse lymphocytic infiltration, fibroblastic proliferation and foci of lobule of mature adipocytes. The patient responded well to LPS plication with a good cosmetic outcome and no functional deficit or complications on follow-up (Figure 2).
Infiltrative ptosis
Severity of ptosis has been inversely related to the amount of muscle fibers at the time of surgery, and levator function has been inversely related to the amount of fat infiltration. Atrophy of the muscle is reported to be more severe in children older than 4 years with congenital ptosis as compared with children younger than 4 years, suggesting a progressive pathology besides congenital dysgenesis. Typical features in this group of patients include moderate to severe ptosis with good levator function and normal position of lid crease, with patients responding well to levator resection. Kataev and colleagues reported clinical features in a group of 11 patients with histopathological reports suggestive of smooth myocytes against a background of fatty infiltration suggestive of isolated fatty infiltration of Müller’s muscle (upper tarsal smooth muscle). The Kataev study highlighted the phenomenon of acquired ptosis due to fatty degeneration of Müller’s muscle.
Additional clinical features reported by Kataev and colleagues included negative lid transparency and visibly thickened Müller’s muscle on upper lid eversion. Ultrasound biomicroscopy showed thickened tarsoconjunctival complex with marked hypoechogenic reflex as compared with a normal eye. Clinical suspicion in our case arose from the patient’s appearance of severe ptosis with good levator function but fullness of lids with negative lid transparency and poor/absent Müller’s function indicated by no response in ptosis with application of topical phenylephrine drops. Moreover, bulky upper lids and the acquired nature of the ptosis made us do a biopsy of the muscle. Planned LPS plication was carried out in our case; however, further study is needed with respect to the role of isolated excision of Müller’s muscle, the component of mechanical ptosis due to excessive fatty infiltration and the advantage of early intervention.
Important differential diagnoses to be considered in such cases are orbital fat prolapse and lipoma of the upper eyelid. Orbital fat prolapse can be differentiated by elderly age group and reducibility along with normal lid position or involutional ptosis. Lipomas are slow-growing, benign neoplasms of mature adipocytes. They rarely present in the eyelid as encapsulated (lobulated) or non-encapsulated (infiltrative). They have been reported to occur in the pre-septal, post-septal and intramuscular region. The usual presentation is mechanical ptosis produced by a slow, progressive, painless mass on the eyelid.
Summary
Clinical examination of our case showed moderate to severe ptosis with good LPS function, fullness of the lids over the Müller’s muscle projection area, negative lid transparency and no response to phenylephrine. This report highlights the presentation of acquired bilateral ptosis due to isolated fatty degeneration of the levator muscle, which can be managed surgically.
- References:
- Cahill KV, et al. Ophthalmic Plast Reconstr Surg. 1986;doi:10.1097/00002341-198601070-00008.
- Grace Lee N, et al. Digit J Ophthalmol. 2015;doi:10.5693/djo.01.2015.05.001.
- Kataev MG, et al. Vestn Oftalmol. 2019;doi:10.17116/oftalma201913502148.
- Thyparampil P, et al. Orbit. 2012;doi:10.3109/01676830.2012.681099.
- For more information:
- Amar Agarwal, MS, FRCS, FRCOphth, director of Dr. Agarwal’s Eye Hospital and Eye Research Centre, is the author of several books published by SLACK Books, sister company of Healio publisher Ocular Surgery News, including Phaco Nightmares: Conquering Cataract Catastrophes, Bimanual Phaco: Mastering the Phakonit/MICS Technique, Dry Eye: A Practical Guide to Ocular Surface Disorders and Stem Cell Surgery and Presbyopia: A Surgical Textbook. He can be reached at 19 Cathedral Road, Chennai 600 086, India; email: aehl19c@gmail.com; website: www.dragarwal.com.
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