Blepharochalasis

Definition of blepharochalasis

Blepharochalasis (ICD-10 H02.3) is defined as an idiopathic, recurrent inflammation and swelling of the eyelids, typically affecting the upper eyelids and leading to progressive atrophy of the eyelid skin, subcutaneous tissue and orbital septum. This results in thin, wrinkled, „cigarette-paper-like“ skin and, potentially, prolapse of orbital fat or the lacrimal gland. The condition can occur unilaterally or bilaterally and often begins during puberty or in young adulthood. Although it is sometimes confused with the term „drooping eyelids“ (dermatochalasis), it is a distinct condition with a different pathophysiology.

Etymology of blepharochalasis

The term „blepharochalasis“ is derived from Greek: „blépharon“ (βλέφαρον) means „eyelid“ and „chálasis“ (χάλασις) means „sagging“ or ‘loosening’. This aptly describes the clinical presentation of slackened eyelid skin resulting from the condition.

Synonyms and English translation of blepharochalasis

Common, though not always precise, synonyms or related terms include:

• Eyelid drooping (not specific)
• Blepharochalasis
• Sometimes incorrectly referred to as „drooping eyelids“ in everyday language, although a clear distinction must be made from dermatochalasis.

English translation

The common English term is blepharochalasis or blepharochalasis syndrome.

Causes and aetiology of blepharochalasis

The exact aetiology of blepharochalasis is often idiopathic (unknown). However, various factors and associations are being discussed:

• Recurrent, painless eyelid oedema: These episodes of swelling are a key characteristic and lead to tissue damage. The causes of the oedema are often unclear, although stress or hormonal fluctuations (e.g. during puberty or menstruation) may play a role.
• Genetic predisposition: A family history of the condition has been reported in some cases, suggesting a hereditary component.
• Association with Ascher syndrome: Blepharochalasis is a key feature of the rare Ascher syndrome, which is also characterised by a cleft lip (usually the upper lip) and an euthyroid goitre.
• Immunological factors: There is evidence to suggest a possible immunological involvement, as histopathological studies have revealed IgA deposits and perivascular inflammatory cells.
• Degenerative processes: In the long term, this leads to degeneration and atrophy of the elastic and collagenous connective tissue in the eyelid skin.
• Possible triggers: Hormonal changes, infections, trauma or allergies are thought to be potential triggers of oedema episodes, but this has not been conclusively proven.

Symptoms of blepharochalasis

The clinical manifestations of blepharochalasis typically develop over a period of years and are characterised by two phases: an active phase with recurrent oedema and a subsequent atrophic phase.

Diagnosis of blepharochalasis

The diagnosis of blepharochalasis is primarily clinical The diagnosis is made on the basis of the characteristic clinical history (recurrent, painless eyelid oedema, often beginning at a young age) and the typical physical examination findings of atrophic eyelid skin. Key diagnostic steps include:

• Medical history: A detailed assessment of the patient’s medical history regarding the onset, frequency and duration of eyelid swelling, as well as family medical history.
• Inspection of the eyelids: Assessment of skin texture (thinness, wrinkling, colour), eyelid position (ptosis), and the presence of excess skin, fat prolapse or lacrimal gland prolapse.
• Checking the position of the eyelid margins: To distinguish it from true ptosis.
• Visual field testing (perimetry): To assess any potential visual field restrictions caused by drooping eyelid skin.
• Ruling out other causes: Systemic conditions that may lead to eyelid oedema (e.g. kidney or thyroid disorders) must be ruled out.
• Photographic record: For monitoring the course of the condition and planning therapeutic measures. A biopsy is not usually necessary, but in unclear cases it may reveal typical histological changes (loss of elastic fibres, thinning of the dermis, lymphocytic infiltrates).

Therapy and treatment of blepharochalasis

The treatment of blepharochalasis depends on the stage and the extent of the functional or cosmetic impairment. There is no known causal treatment that prevents recurrent oedema.

• Conservative measures: During the acute phase of oedema, cooling compresses can provide relief, but do not affect the long-term outcome. Lymphatic drainage is a subject of debate and may not be effective, or may even be harmful, in cases of thin, atrophic skin.
• Surgical treatment (blepharoplasty): Definitive treatment is usually carried out surgically once the condition has entered a stable, atrophic phase and acute swelling no longer occurs. The aim of the operation (a form of blepharoplasty) is to remove the excess, atrophic skin (excision). Where necessary, prolapsed orbital fat may also be removed or repositioned. In cases of accompanying ptosis, correction of the levator muscle (levator aponeurosis) or a tarsoconjunctival myulectomy (Müller’s muscle resection) may be required.

Surgical procedures should be carried out by an experienced eyelid surgeon (oculoplastic surgeon) in order to achieve the best possible results and avoid complications.