Warsaw Oculoplastics Congress 2025
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Clinical Update: Periocular & Lacrimal Conditions
Malignancy, Vascular Lesions, Mucoceles, and Necrotising Fasciitis
Mr Nabil El-Hindy FRCOphth
Warsaw 12th -13 September 2025
A concise, evidence-focused summary of recent research, trials and important drug interactions for oculoplastic surgeons and trainees
Relax and immerse yourself in the learning experience - all slides will be available for download after the session.
We encourage questions, discussions, and sharing of your own clinical experiences throughout the presentation. Let's explore these fascinating periocular cases together!
Dr. Nabil El-Hindy
Dr. Radoslaw Rozycki
Case Index
This presentation covers six key periocular conditions with detailed case presentations, clinical updates, and therapeutic considerations relevant to oculoplastic practice.
1
The most common periocular malignancy, primarily affecting elderly and fair-skinned populations with UV exposure history
2
A rare and aggressive lacrimal sac malignancy often mimicking chronic dacryocystitis with delayed diagnosis
3
An uncommon but aggressive neuroendocrine tumour with potential for early metastasis
1
Common vascular anomaly in infancy with potential for amblyopia and visual axis obstruction
2
Expansile lesions of the paranasal sinuses with orbital manifestations requiring collaborative management
3
Rare but potentially devastating soft tissue infection requiring prompt recognition and intervention
Each case presentation includes epidemiology, clinical presentation, management approaches, and critical drug interactions to consider in treatment planning.
Navigate through the presentation by selecting a specific case, or proceed sequentially through all cases.
Comparative Prevalence
Basal cell carcinoma represents the vast majority of eyelid malignancies, whilst transitional cell carcinoma of the lacrimal sac is exceptionally rare. Periocular haemangiomas occur in approximately 1 in 1,586 births (calculated as ~63 per 100,000). Fronto-ethmoidal mucoceles remain uncommon but represent the most frequent sinus pathology causing orbital symptoms. Note: Log scale used for visualisation purposes due to significant prevalence differences.
Case 1:
We begin with the most frequently encountered periocular malignancy. This section details a typical presentation of basal cell carcinoma (BCC) affecting the eyelid, highlighting key clinical features and diagnostic considerations. Understanding its characteristic appearance is crucial for early detection and optimal management outcomes.
Case 1: Basal Cell Carcinoma of the Eyelids
Discussion Points
What specific clinical features in these images lead you towards a diagnosis of Basal Cell Carcinoma?
What key questions would you ask the patient regarding their medical history and sun exposure?
What are the immediate next steps in the diagnostic and management pathway?
Case 1: Basal Cell Carcinoma of the Eyelids
Demographics & Epidemiology
Basal cell carcinoma (BCC) represents the most common periocular malignancy, accounting for 80-95% of all eyelid skin cancers. The peak incidence occurs in older adults (>60 years), with significantly higher rates observed in fair-skinned populations and those with substantial ultraviolet radiation exposure history. The incidence is approximately 22 per 100,000 persons per year, though this varies considerably by geographic region, with Australia and New Zealand reporting significantly higher rates than Northern European countries.
Risk factors include:
  • Cumulative UV exposure (occupational and recreational)
  • Fair skin, light eyes, and blonde/red hair (Fitzpatrick types I-II)
  • History of sunburns, especially in childhood
  • Immunosuppression (transplant recipients, HIV)
  • Previous radiotherapy to the head and neck region
  • Genetic predisposition (e.g., Gorlin syndrome, xeroderma pigmentosum)
Clinical Presentation of Eyelid BCC
Eyelid BCC typically presents as a slowly enlarging lesion with characteristic features that vary by subtype:
Nodular BCC (most common)
  • Pearly, translucent nodule with rolled borders
  • Prominent telangiectatic vessels on surface
  • Central depression or ulceration ("rodent ulcer")
  • Madarosis (loss of eyelashes) when margin involved
Morphoeic/Sclerosing BCC
  • Scar-like, flat or slightly depressed
  • Indistinct borders making clinical delineation difficult
  • Often more aggressive with subclinical spread
  • Higher recurrence rates post-excision
Special anatomical concerns include:
  • Medial canthal lesions risk orbital/lacrimal drainage system invasion
  • Lower eyelid involvement predominates (≈50-60%)
  • Upper eyelid (≈15-20%), medial canthus (≈25-30%)
  • Lateral canthus involvement least common (≈5-10%)
Management of Eyelid BCC
Surgical Management (Gold Standard)
Margin-controlled excision remains the mainstay of treatment:
  • Mohs micrographic surgery - highest cure rates (97-99%) with tissue preservation; preferred for high-risk locations (medial canthus)
  • Frozen section margin control - alternative when Mohs unavailable
  • Standard excision with 4mm margins - acceptable for well-defined, small BCCs in non-critical areas
  • Reconstructive considerations include functional and aesthetic outcomes
Non-surgical Options
Reserved for specific scenarios:
  • Radiotherapy - elderly/inoperable patients, adjuvant for perineural invasion
  • Topical imiquimod/5-FU - superficial BCCs only, away from eyelid margin
  • Cryotherapy - very small, superficial lesions in non-critical locations
  • Photodynamic therapy - limited role in periocular region
Recent evidence demonstrates excellent outcomes with primary Mohs surgery for periocular BCCs, with 5-year recurrence rates of <3% even for morphoeic subtypes. The functional and aesthetic benefits of tissue-sparing techniques are particularly important in the periocular region where tissue preservation is critical.
Advanced and Recurrent Eyelid BCC
Hedgehog Pathway Inhibitors
Breakthrough therapy for locally advanced or metastatic BCC:
  • Vismodegib (Erivedge): First-in-class SMO inhibitor
  • Sonidegib (Odomzo): Second-generation SMO inhibitor
  • Response rates: 30-60% for locally advanced BCC
  • Duration of therapy remains controversial (typically until maximum response or intolerable toxicity)
Emerging role as neoadjuvant therapy to reduce surgical morbidity in complex periocular cases (orbital involvement, extensive medial canthal invasion).
Side Effects & Drug Interactions
Key considerations with Hedgehog inhibitors:
  • Muscle cramps (70-75%)
  • Dysgeusia/ageusia (50-60%)
  • Alopecia (40-65%)
  • Weight loss (25-50%)
  • Fatigue (30-40%)
  • Teratogenic - contraception essential
Important interactions:
  • CYP3A4 substrates/inhibitors - dose adjustments needed
  • QT-prolonging medications - caution and monitoring required
  • P-glycoprotein modulators - altered bioavailability
Case 1: BCC Clinical Photographs
Key Learning Points - Eyelid BCC
Early Identification
Recognise subtle signs including localised madarosis, telangiectasia, and pearly nodules, especially in elderly fair-skinned patients
Surgical Planning
Margin-controlled excision techniques (Mohs or frozen sections) are essential for periocular lesions, particularly medial canthal BCCs
Advanced Disease
Consider Hedgehog pathway inhibitors for locally advanced, recurrent, or inoperable cases with careful monitoring for adverse effects
Case Presentation
Patient Profile
  • 47-year-old, Caucasian, Female
  • Referred February 2017 (2WW)
  • Left medial canthal mass present for 4-5 months
  • 1cm painless firm non-mobile mass above MCT
Initial Imaging
  • CT showed 14 mm well-circumscribed, homogenous soft tissue mass
  • Adjacent to insertion of left medial rectus
  • Radiologically appeared indolent
Radiological Findings
The imaging studies revealed a well-defined mass in the region of the lacrimal sac without evidence of bony erosion or invasion into surrounding structures. These findings were initially interpreted as suggesting a benign process, highlighting the challenge in radiologically diagnosing this rare entity.
Diagnostic Journey
Initial Plan
Patient was listed for anterior orbitotomy and excision
Procedure was delayed due to patient getting married
Findings
Histology: High grade Transitional Cell Carcinoma most likely from superior lacrimal sac (second opinion from Sheffield)
Further Imaging: MRI head and CT CAP showed no extension or metastases
Treatment Approach
Head/Neck MDT Discussion
  • Recommended excision with clear margins
  • Goal to avoid unnecessary disfiguring surgery
Surgical Intervention
  • En-bloc excision of upper/lower/common canaliculi
  • Removal of nasolacrimal sac and duct
  • Injection of Interferon alpha 2a into local tissues
  • Reconstruction with local advancement flaps
Follow-up
  • Uneventful post-operative recovery
  • Serial MRIs showed no recurrence (last August 2019)
Post-Operative Results
The patient experienced an uneventful post-operative recovery with good cosmetic and functional outcomes. The surgical approach successfully removed the tumor while minimizing facial disfigurement, demonstrating the value of the MDT-guided treatment plan.
Post-Operative Results
Case 2: Transitional Cell Carcinoma of the Lacrimal Sac
Demographics & Epidemiology
Transitional cell carcinoma (TCC) of the lacrimal sac represents an exceptionally rare but aggressive malignancy. Unlike the more common BCC, it arises from the lacrimal drainage system epithelium rather than cutaneous tissue. The epidemiological profile includes:
  • Middle-aged to older adults (peak 50-70 years)
  • No clear gender predilection in most series (slight female predominance in some reports)
  • No established racial or ethnic predisposition
  • Extremely rare incidence, with fewer than 55 well-documented cases in the world literature
  • Represents approximately 15% of malignant epithelial lacrimal sac tumours
Risk factors remain poorly defined due to rarity, but may include:
  • Chronic inflammation (e.g., long-standing dacryocystitis)
  • Potential association with human papillomavirus (HPV) infection (similar to urothelial TCC)
  • Smoking has been suggested but not definitively established
Clinical Presentation of Lacrimal Sac TCC
The presentation of lacrimal sac TCC is often insidious and easily misdiagnosed as benign dacryocystitis or nasolacrimal duct obstruction. Key clinical features include:
  • Persistent medial canthal mass or swelling (90-95%)
  • Epiphora (excessive tearing) from lacrimal outflow obstruction (85-90%)
  • History of recurrent dacryocystitis unresponsive to conventional treatment
  • Bloody discharge or epistaxis (50-60%) - a critical red flag
  • Painless in early stages (distinguishing from acute infectious dacryocystitis)
  • Progressive firm to hard consistency of the lacrimal sac region
Delayed diagnosis is unfortunately common, with an average delay of 10-12 months from symptom onset to definitive diagnosis. This significantly impacts prognosis, as invasion of adjacent structures (orbit, paranasal sinuses, nasal cavity) often occurs by the time of diagnosis.
Diagnostic red flags for lacrimal sac malignancy:
  • Bloody reflux on lacrimal irrigation
  • Firm, non-compressible lacrimal sac mass
  • Rapid progression of symptoms
  • Unilateral presentation in older adults
  • Failure to respond to standard treatment for dacryocystitis
Imaging and Diagnosis of Lacrimal Sac TCC
CT Imaging
First-line investigation for anatomical assessment:
  • Soft tissue mass in lacrimal sac fossa
  • Bony erosion of lacrimal crest or maxilla (40-50%)
  • Extension into nasal cavity or paranasal sinuses
  • Orbital invasion in advanced cases
MRI Features
Superior soft tissue contrast for extent assessment:
  • Intermediate T1 and T2 signal intensity
  • Heterogeneous contrast enhancement
  • Perineural spread along infraorbital or anterior ethmoidal nerves
  • Distinguishes tumour from inflammatory processes
Diagnostic Biopsy
Essential for definitive diagnosis:
  • Direct incisional biopsy (preferred)
  • Avoid routine dacryocystorhinostomy (DCR) if malignancy suspected
  • Cytological examination of lacrimal irrigation fluid (lower sensitivity)
  • Immunohistochemistry: CK7+/CK20-, p63+, GATA3+
Complete evaluation should include regional lymph node assessment (submandibular, preauricular, cervical) and systemic staging with consideration of PET-CT in advanced cases to identify distant metastases, particularly given the propensity for haematogenous spread.
Management of Lacrimal Sac TCC
Surgical Approach
The cornerstone of treatment is complete surgical excision:
  • En bloc resection of lacrimal sac (dacryocystectomy)
  • Wide margins including lacrimal fossa bone
  • Medial maxillectomy for extensive local disease
  • Orbital exenteration for cases with orbital invasion
  • Sentinel lymph node biopsy or elective neck dissection in high-grade tumours
Recent evidence supports organ-preservation approaches with advanced reconstructive techniques for selected cases without extensive orbital invasion, potentially preserving visual function while maintaining oncological principles.
Adjuvant Therapy
Multimodal treatment improves outcomes in high-risk disease:
  • Adjuvant radiotherapy for high-grade, positive margins, or perineural invasion (54-66 Gy)
  • Chemotherapy regimens follow urothelial carcinoma protocols:
  • Platinum-based combinations (cisplatin/gemcitabine)
  • Taxane regimens for platinum-ineligible patients
  • Emerging role for immune checkpoint inhibitors (pembrolizumab, atezolizumab) in advanced disease
Five-year survival rates remain poor (30-50%) due to late diagnosis and aggressive biological behaviour. Early diagnosis with prompt multidisciplinary management offers the best chance for survival.
Drug Interactions in Lacrimal Sac TCC Management
When managing patients with lacrimal sac TCC, several important drug interactions require careful consideration:
Platinum Compounds (Cisplatin, Carboplatin)
  • Nephrotoxicity enhanced by concurrent NSAIDs, aminoglycosides, and loop diuretics
  • Ototoxicity potentiated by aminoglycosides and loop diuretics
  • Renal function monitoring essential; dosage adjustments required for CrCl <60 ml/min
  • Hydration protocols mandatory to mitigate nephrotoxicity
Gemcitabine
  • Increased myelosuppression with concurrent radiotherapy
  • Enhanced toxicity with capecitabine co-administration
  • Avoid live vaccines during treatment
  • Dose reduction required in hepatic and renal impairment
Immune Checkpoint Inhibitors
  • Systemic corticosteroids may reduce efficacy (avoid unless managing immune-related adverse events)
  • Potential for enhanced autoimmune effects with other immunomodulatory drugs
  • Increased risk of ocular inflammatory side effects requiring ophthalmological monitoring
  • Thyroid function monitoring required due to risk of thyroid dysfunction
Case 3:
Merkel cell carcinoma (MCC) is a rare, highly aggressive neuroendocrine tumour of the skin. Though predominantly found in sun-exposed areas, its appearance on the eyelid poses a unique set of diagnostic and therapeutic challenges, often mimicking more benign conditions.
Discussion Points:
  • Given its rarity, what clinical red flags on the eyelid should prompt suspicion for Merkel cell carcinoma over other common eyelid lesions?
  • What immediate diagnostic steps are essential when MCC is suspected, considering its aggressive biological behaviour?
  • How does the anatomical location of the eyelid influence the surgical and reconstructive approaches for this type of malignancy?
  • Beyond surgery, what adjuvant therapies might be considered for eyelid MCC, and why is multidisciplinary management crucial?
Case 3: Merkel Cell Carcinoma of the Eyelid
Demographics & Epidemiology
Merkel cell carcinoma (MCC) is an uncommon but highly aggressive neuroendocrine skin tumour with a propensity for local recurrence and early metastasis. The epidemiological profile includes:
  • Predominantly affects older adults (>70 years)
  • Incidence of 0.1-1.6 per 100,000 persons per year overall
  • Periocular involvement in approximately 5-10% of cases
  • Higher incidence in fair-skinned populations (Caucasians > other ethnicities)
  • Male predominance (approx. 1.5:1 male:female ratio)
Key risk factors include:
  • Immunosuppression (5-10× increased risk in transplant recipients, HIV patients)
  • UV exposure history (80% of cases on sun-exposed skin)
  • Merkel cell polyomavirus (MCPyV) infection (present in 80% of tumours)
  • Advanced age (immune senescence)
  • Prior radiation therapy to the affected region
Clinical Presentation of Eyelid MCC
MCC typically presents with a rapidly growing, painless lesion on the eyelid or periocular region. The classic presentation is summarised by the acronym AEIOU:
  • Asymptomatic (non-tender)
  • Expanding rapidly (often doubling in size within 2-3 months)
  • Immunosuppression
  • Older than 50 years
  • UV-exposed site
Specific clinical features include:
  • Firm, dome-shaped nodule
  • Red-violet or skin-coloured appearance
  • Shiny, telangiectatic surface
  • Usually non-ulcerated in early stages
  • Eyelid margin or tarsal involvement common
Diagnostic challenges:
  • Frequently misdiagnosed as chalazion or pyogenic granuloma
  • Delays in diagnosis common (mean 3-5 months)
  • Regional lymph node metastasis present in 20-30% at initial diagnosis
  • Occult micrometastases in clinically node-negative patients (30-40%)
  • High risk for distant metastasis (lungs, liver, bone, brain)
The rapid growth and aggressive behaviour distinguish MCC from the more indolent BCC, though clinical distinction can be challenging without histopathological confirmation.
Histopathology and Diagnostic Workup
Histopathological Features
Distinctive microscopic appearance:
  • Small round blue cells with scant cytoplasm
  • Vesicular nuclei with dispersed chromatin
  • High mitotic index (>5 per HPF)
  • Tumour necrosis common
  • Infiltrative growth pattern
  • Lymphovascular invasion in 30-60%
Immunohistochemistry
Critical for definitive diagnosis:
  • CK20: positive (paranuclear dot pattern)
  • Neuroendocrine markers: chromogranin, synaptophysin
  • CD56: typically positive
  • TTF-1: negative (distinguishes from small cell lung carcinoma)
  • MCPyV large T-antigen: positive in virus-associated cases
Staging Evaluation
Comprehensive assessment required:
  • Sentinel lymph node biopsy (SLNB) - standard of care
  • CT or PET/CT for regional/distant disease
  • MRI for perineural invasion assessment
  • Serum MCPyV antibody titres (emerging biomarker)
  • 8th edition AJCC staging system for cutaneous MCC
Recent advances in pathological assessment include molecular profiling (MCPyV status, tumour mutational burden) and immune microenvironment characterisation (PD-L1 expression, tumour-infiltrating lymphocytes), which increasingly guide treatment decisions, particularly regarding immunotherapy candidacy.
Management of Eyelid MCC
Surgical Management
Primary surgical approach includes:
  • Wide local excision with 1-2 cm margins when feasible
  • Mohs micrographic surgery with immunohistochemistry for margin control
  • Sentinel lymph node biopsy (SLNB) mandatory regardless of clinical node status
  • Complete lymph node dissection for positive SLNB or clinically positive nodes
  • Reconstructive considerations particularly challenging in periocular region
Special periocular considerations:
  • Tissue-sparing approaches may be necessary for functional preservation
  • Frozen section control of surgical margins
  • Careful assessment of deep orbital involvement
Adjuvant Therapy
Multimodal approach for optimal outcomes:
  • Adjuvant radiotherapy to primary site (50-60 Gy)
  • Reduces local recurrence by 60-70%
  • Consider for all periocular MCCs due to anatomical constraints on margins
  • Nodal basin irradiation for:
  • Positive SLNB without complete lymphadenectomy
  • Extracapsular extension
  • Multiple positive nodes
  • Chemotherapy largely abandoned as adjuvant therapy (no survival benefit)
Advanced and Metastatic Eyelid MCC
Paradigm Shift: Immune Checkpoint Inhibitors
Recent years have witnessed a revolutionary change in the management of advanced MCC with the introduction of immune checkpoint inhibitors (ICIs). Current evidence supports:
First-line Therapy
Avelumab (anti-PD-L1): FDA/EMA approved with 50-70% response rates in treatment-naïve patients; pembrolizumab and nivolumab show similar efficacy
Response Patterns
Durable responses in 65-70% of responders; MCPyV-positive tumours may show higher response rates; median PFS 9-12 months
Ocular Considerations
Risk of immune-related ocular adverse events (1-3%) including uveitis, dry eye, and episcleritis; baseline and periodic ophthalmological assessment recommended
For patients ineligible for immunotherapy or with primary/secondary resistance, second-line options include platinum-based chemotherapy (response rates 30-40%, but brief duration) or enrolment in clinical trials investigating novel approaches such as adoptive T-cell therapy and MCPyV-directed treatments.
Case 4:
A 6-month-old female infant presented with a rapidly growing periocular lesion:
  • Noted at 2 weeks of age, initially as a small red mark.
  • Rapid expansion over 5 months, now a 2x3 cm bright red-blue mass.
  • Located on the left upper eyelid, causing significant mechanical ptosis.
  • Interfering with visual axis, raising concern for amblyopia.
  • No pain, discharge, or signs of inflammation.
Case 4: Periocular Capillary Haemangioma
Demographics & Epidemiology
Infantile haemangiomas (IH) represent the most common benign vascular tumours of infancy, with periocular involvement occurring in a significant subset. Key epidemiological features include:
  • Overall prevalence of infantile haemangiomas: 4-5% of all infants
  • Periocular involvement in approximately 1 in 1,586 births
  • Female predominance (2-3:1 female:male ratio)
  • Higher incidence in:
  • Premature infants (<37 weeks gestation)
  • Low birth weight (<2.5 kg)
  • Multiple gestations
  • Advanced maternal age
  • In vitro fertilisation pregnancies
Periocular haemangiomas account for approximately 10% of all infantile haemangiomas, with the upper eyelid being the most common location (60-80%), followed by the lower eyelid and orbital involvement.
Natural History and Clinical Presentation
Natural History
The typical lifecycle of infantile haemangiomas includes three phases:
  1. Proliferative phase (0-12 months):
  • Rapid growth (80% of growth complete by 3-4 months)
  • Brightest red coloration
  • Firm consistency
  1. Plateau phase (12-18 months):
  • Growth stabilisation
  • Beginning colour changes (dulling of red)
  1. Involutional phase (1.5-7+ years):
  • Gradual regression (10-20% per year)
  • Central greying or lightening
  • Softening of the lesion
Complete spontaneous resolution occurs in 60-70% by age 7, with residual changes (fibrofatty tissue, skin laxity, telangiectasia) common in larger lesions.
Clinical Presentation
Periocular haemangiomas present with distinctive features based on depth:
  • Superficial haemangiomas:
  • Bright red "strawberry" appearance
  • Well-demarcated, slightly raised
  • Blanches with pressure
  • Deep haemangiomas:
  • Bluish or skin-coloured swelling
  • Less distinct borders
  • Firmer consistency
  • Mixed haemangiomas:
  • Combined features of both types
  • Most common presentation
Visual Complications of Periocular Haemangiomas
Amblyopia
The most significant concern, occurring in 43-60% of untreated cases:
  • Deprivation amblyopia from ptosis and visual axis obstruction
  • Refractive amblyopia from induced astigmatism
  • Anisometropic amblyopia from pressure effects on the globe
Refractive Error
Direct mechanical effects on the developing globe:
  • Astigmatism (most common) - typically with-the-rule
  • Myopia from axial length changes
  • Mean astigmatism of 2.5-3.0D in affected eyes
Strabismus
Muscle and mechanical effects:
  • Restrictive strabismus from direct pressure
  • Sensory strabismus secondary to visual deprivation
  • Deep orbital involvement affecting extraocular muscle function
Other Complications
Additional concerns requiring intervention:
  • Exposure keratopathy from eyelid retraction
  • Proptosis from orbital involvement
  • Optic nerve compression (rare)
  • Corneal deformation
The critical period for amblyopia development (first 6-12 months of life) coincides with the proliferative phase of haemangiomas, creating an urgent treatment window. Visual rehabilitation measures, including patching and optical correction, should be initiated concurrently with haemangioma treatment.
Management of Periocular Haemangiomas
Indications for Treatment
While many infantile haemangiomas can be observed, periocular lesions often require intervention due to:
  • Risk of amblyopia during critical visual development
  • Significant astigmatism (>1.5D)
  • Visual axis obstruction
  • Proptosis >2mm
  • Documented progression of refractive error
  • Exposure keratopathy
First-Line Therapy: Beta-Blockers
Revolutionary change in management since 2008:
  • Oral propranolol: 2-3 mg/kg/day in divided doses
  • Response rates >90% with significant reduction in size
  • Rapid effect (colour change within 24-48 hours)
  • Treatment duration typically 6-12 months
  • Monitoring required for heart rate, blood pressure, and glucose
  • Topical timolol maleate 0.5%: For superficial, smaller lesions
  • Applied twice daily
  • Less effective than oral therapy but minimal systemic absorption
  • Useful for thin eyelid lesions or maintenance therapy
Alternative Treatments
Reserved for beta-blocker contraindications or inadequate response:
  • Intralesional corticosteroids
  • Triamcinolone + betamethasone
  • Risk of eyelid depigmentation, fat atrophy
  • Systemic corticosteroids (largely abandoned)
  • Pulsed dye laser for superficial residual vessels
  • Surgical excision for selected cases with:
  • Fibro-fatty residua after involution
  • Focal lesions in non-critical locations
  • Failed medical management with vision threat
Drug Interactions and Safety Considerations
Propranolol Safety Profile
Key monitoring parameters and precautions:
  • Heart rate and blood pressure monitoring (baseline and during dose escalation)
  • Blood glucose monitoring (risk of masking hypoglycaemia symptoms)
  • Respiratory assessment (contraindicated in bronchospastic disease)
  • Sleep disturbances and nightmares (10-15%)
  • Initiation preferably in controlled setting for high-risk infants
Contraindications
Absolute and relative contraindications:
  • Bradycardia or heart block
  • Hypotension
  • Bronchial asthma or reactive airway disease
  • Heart failure or cardiogenic shock
  • Hypoglycaemia or diabetes
  • PHACES syndrome with arterial anomalies (relative)
Drug Interactions
Important interactions to consider:
  • Other beta-blockers (additive effects)
  • Calcium channel blockers (hypotension, AV block)
  • Hypoglycaemic agents (masks symptoms, potentiates effect)
  • CYP2D6 inhibitors (fluoxetine, paroxetine) increase propranolol levels
  • Anaesthetic agents (potentiates hypotension)
Recent long-term follow-up studies have demonstrated no negative effects on growth, development, or cardiovascular function in children treated with propranolol for haemangiomas. The excellent safety profile and efficacy have established beta-blockers as the definitive first-line therapy.
Case 5: Fronto-ethmoidal Mucoceles
Demographics & Epidemiology
Paranasal sinus mucoceles are benign, expansile, mucus-containing lesions that develop when sinus ostia become obstructed, leading to mucus retention and gradual expansion. Fronto-ethmoidal mucoceles represent the most common subtype with orbital manifestations. Key epidemiological features include:
  • Peak incidence in middle-aged adults (40-60 years)
  • Slight male predominance (1.2-1.5:1 male:female ratio)
  • Distribution by sinus location:
  • Frontal sinus: 60-65%
  • Ethmoid sinuses: 20-25%
  • Maxillary sinus: 10-15%
  • Sphenoid sinus: 1-2%
  • Frontal and ethmoid mucoceles most likely to cause orbital symptoms due to anatomical proximity
Predisposing factors include:
  • Chronic rhinosinusitis (most common)
  • Previous sinus surgery (especially with scarring of ostia)
  • Facial trauma with sinus outflow obstruction
  • Allergic fungal sinusitis
  • Anatomical variations (concha bullosa, septal deviation)
  • Benign sinus tumours (osteomas, papillomas)
Pathophysiology and Clinical Presentation
Pathophysiology
The development of mucoceles follows a distinct sequence:
  1. Obstruction of sinus ostium (from inflammation, scarring, mass)
  1. Continued mucus production by respiratory epithelium
  1. Accumulation of mucus and expansion of the sinus cavity
  1. Pressure-induced bone remodelling and erosion
  1. Extension into adjacent structures (orbit, anterior cranial fossa)
Histologically, mucoceles are lined by respiratory epithelium and contain sterile mucus unless secondarily infected (mucopyocele). The expansile nature results from inflammatory mediators (IL-1, TNF-α, PGE2) that activate osteoclastic bone resorption, allowing progressive growth over years.
Clinical Presentation
Fronto-ethmoidal mucoceles with orbital involvement typically present with:
  • Gradual, painless onset of symptoms (months to years)
  • Unilateral proptosis (90-95%), typically non-axial, infero-lateral displacement
  • Periorbital swelling (80-85%)
  • Diplopia (50-60%) from extraocular muscle displacement
  • Palpable mass in superomedial orbit (55-65%)
  • Visual disturbance (30-40%)
  • Frontal headache (40-50%)
  • Epiphora from lacrimal drainage compression
Diagnostic Imaging
CT Findings
Primary imaging modality providing excellent bony detail:
  • Homogeneous, non-enhancing expansile mass
  • Characteristic bone remodelling and thinning
  • Smooth margins with scalloped appearance
  • Variable density depending on protein content
  • Complete opacification of affected sinus
MRI Characteristics
Complementary to CT, superior for soft tissue evaluation:
  • T1: variable signal (low to high) depending on protein content
  • T2: typically hyperintense unless very concentrated
  • Rim enhancement with gadolinium (inflamed mucosa)
  • No internal enhancement (distinguishes from neoplasms)
  • Superior for intracranial extension assessment
Nasal Endoscopy
Essential for surgical planning and assessment:
  • May reveal bulging of sinus wall into nasal cavity
  • Helps identify anatomical variations
  • Evaluates nasal polyps or other obstructive lesions
  • Guides approach for endoscopic marsupialisation
  • Critical for postoperative monitoring
Differential diagnosis based on imaging includes: frontal sinus osteoma, dermoid cyst, lacrimal gland tumours, orbital lymphoma, metastatic disease, and meningioma. The characteristic bony changes and signal characteristics typically allow definitive diagnosis without biopsy.
Management of Fronto-ethmoidal Mucoceles
Surgical Approaches
Definitive treatment requires surgical intervention, with the approach dictated by location and extent:
  • Endoscopic marsupialisation (preferred first-line):
  • Wide opening of mucocele into nasal cavity
  • Preservation of mucosa to prevent recurrence
  • Minimal morbidity and rapid recovery
  • Success rates >90% with experienced surgeons
  • Combined approaches (for complex cases):
  • Endoscopic + small external incision (Lynch, brow)
  • Useful for lateral frontal sinus disease
  • Better visualization of complex anatomy
  • Traditional external approaches (now limited use):
  • Lynch incision or osteoplastic flap
  • Reserved for recurrent, complex, or inaccessible mucoceles
  • Higher morbidity and cosmetic concerns
Perioperative Management
Comprehensive care includes:
  • Preoperative:
  • Control of rhinosinusitis with antibiotics and steroids
  • CT navigation protocol for complex cases
  • Ophthalmological assessment of visual function
  • Intraoperative:
  • Wide marsupialisation of mucocele cavity
  • Preservation of mucosa when possible
  • Identification and enlargement of natural drainage pathways
  • Careful handling of orbital contents if exposed
  • Postoperative:
  • Saline irrigations to maintain patency
  • Topical intranasal steroids
  • Endoscopic debridement and surveillance
  • Long-term follow-up to detect recurrence
Oculoplastic Considerations and Collaborative Care
Oculoplastic Assessment
Critical role in orbital manifestations:
  • Detailed evaluation of proptosis and globe displacement
  • Quantification of extraocular muscle function
  • Assessment of optic nerve function (visual acuity, colour vision, RAPD)
  • Evaluation of lacrimal drainage system involvement
  • Documentation of lid position and function
Multidisciplinary Approach
Optimal management through collaboration:
  • Rhinologist/ENT: primary surgical management
  • Oculoplastic surgeon: orbital decompression if needed
  • Neuro-ophthalmologist: visual function monitoring
  • Neurosurgeon: cases with intracranial extension
  • Radiologist: detailed imaging interpretation
Outcome Monitoring
Long-term follow-up essential:
  • Resolution of proptosis (typically 80-90% within 1-3 months)
  • Improvement in ocular motility (may be incomplete)
  • Visual function recovery (dependent on duration and degree of compression)
  • Endoscopic surveillance for recurrence (5-10% risk)
  • Periodic imaging in complex cases
The multidisciplinary approach is particularly important in mucoceles with substantial orbital involvement, where coordinated care between ENT and oculoplastic surgeons ensures optimal functional and aesthetic outcomes. Recent advances in image-guided navigation systems have improved surgical precision and safety, particularly in cases with distorted anatomy or revision surgery.
Case 6:
Clinical Presentation
A 55-year-old male presented to the emergency department with a rapidly progressing, painful swelling of his right periorbital region. Key presenting features included:
  • Acute onset of severe, rapidly worsening right upper and lower eyelid oedema and erythema.
  • Intense pain described as out of proportion to the visible inflammation.
  • Associated systemic symptoms including fever (39°C), chills, and malaise.
  • On examination, the skin of the eyelids was tense, dusky red, and exhibited areas of violaceous (purplish) discoloration.
  • Palpation revealed a subtle crepitus (crackling sensation) indicating subcutaneous gas.
  • Visual acuity and extraocular movements were initially normal, though swelling made assessment challenging.
  • No history of recent trauma, insect bite, or surgical intervention in the periorbital area was reported.
Beneath the Eyelid
Post Theatre Images
Case 6: Necrotizing Fasciitis of the Eyelids
Demographics & Epidemiology
Necrotizing fasciitis (NF) is a rare but potentially devastating soft tissue infection characterised by rapidly progressive necrosis of the superficial fascia and subcutaneous tissues. Periorbital involvement is exceptionally rare but carries significant morbidity and mortality. Key epidemiological features include:
  • Overall incidence of NF: approximately 3.5 cases per 100,000 population
  • Periorbital/orbital NF: less than 100 well-documented cases in literature
  • No significant gender predilection
  • Higher incidence in:
  • Older adults (peak 50-70 years)
  • Immunocompromised patients
  • Diabetics (4-5× increased risk)
  • Alcoholics
  • Patients with peripheral vascular disease
The head and neck region accounts for only 8-10% of all NF cases, with periorbital involvement representing a small subset. Despite its rarity, recognition is critical due to the high mortality rate (25-30% overall) and risk of vision loss.
Microbiology and Pathophysiology
Microbial Aetiology
NF is classified based on microbiological findings:
  • Type I (polymicrobial):
  • Mixed aerobic and anaerobic bacteria
  • Commonly includes Streptococci, Enterobacteriaceae, anaerobes
  • Most common in periorbital region (60-70%)
  • Type II (monomicrobial):
  • Group A Streptococcus (S. pyogenes) predominant
  • Often with Staphylococcus aureus co-infection
  • Associated with "streptococcal toxic shock syndrome"
  • Type III (marine-related):
  • Vibrio species (rare in periorbital region)
  • Associated with seawater exposure
Community-acquired methicillin-resistant S. aureus (CA-MRSA) has emerged as an increasingly important pathogen in recent years, particularly in type II infections.
Pathophysiology
The characteristic fulminant progression results from:
  1. Bacterial entry via trauma, surgery, or contiguous spread
  1. Rapid bacterial proliferation along fascial planes
  1. Production of exotoxins (particularly Group A Strep)
  • Streptococcal pyrogenic exotoxins (SPEs)
  • Streptolysin O
  • M proteins
  1. Fascial necrosis from direct bacterial action and thrombosis of nutrient vessels
  1. Systemic toxicity from cytokine storm and superantigen effects
Clinical Presentation and Diagnosis
Early Signs (0-24 hours)
Often mistaken for cellulitis or preseptal infection:
  • Rapidly progressive erythema and oedema
  • Pain disproportionate to physical findings
  • Tense, shiny skin with indistinct borders
  • Low-grade fever
  • Mild tachycardia
Intermediate Signs (24-72 hours)
Progressive deterioration with characteristic findings:
  • Dusky or violaceous skin discoloration
  • Bullae formation with serous fluid
  • Decreased pain (nerve destruction)
  • Skin anaesthesia in affected areas
  • Marked systemic toxicity
Late Signs (>72 hours)
Fulminant phase with tissue necrosis:
  • Frank cutaneous necrosis with eschar formation
  • Crepitus from gas-forming organisms
  • Purulent discharge
  • Haemorrhagic bullae
  • Septic shock with multi-organ dysfunction
Diagnosis is primarily clinical, with a high index of suspicion critical for early intervention. The LRINEC score (Laboratory Risk Indicator for Necrotizing Fasciitis) using CRP, WBC, haemoglobin, sodium, creatinine, and glucose can aid risk stratification, though its sensitivity in periorbital NF is not well-established. Definitive diagnosis requires surgical exploration with "finger test" demonstrating lack of resistance to blunt dissection along fascial planes.
Management of Periorbital Necrotizing Fasciitis
Immediate Interventions
Treatment must be aggressive and multifaceted:
  1. Broad-spectrum IV antibiotics (immediate initiation):
  • Triple therapy initially: β-lactam/β-lactamase inhibitor + clindamycin + vancomycin
  • Clindamycin crucial for toxin suppression
  • Adjust based on culture results and clinical response
  • Typical duration: 2-3 weeks
  1. Urgent surgical debridement (cornerstone of management):
  • Wide excision of all necrotic tissue
  • Exploration along fascial planes until viable tissue encountered
  • Serial debridements often necessary (every 24-48 hours)
  • Preservation of vital structures when possible
  1. Intensive supportive care:
  • Aggressive fluid resuscitation
  • Vasopressor support if needed
  • Nutritional support
  • Pain management
Adjunctive Therapies
Additional measures to consider:
  • Hyperbaric oxygen therapy:
  • 2.0-2.5 ATA for 90 minutes, 2-3 times daily initially
  • Inhibits anaerobic growth
  • Enhances neutrophil function
  • Promotes fibroblast activity
  • Evidence primarily from case series
  • Intravenous immunoglobulin (IVIG):
  • Neutralises streptococcal superantigens
  • Consideration in streptococcal toxic shock syndrome
  • Typically 1g/kg on day 1, then 0.5g/kg for 2 days
  • Negative pressure wound therapy:
  • After initial debridement and infection control
  • Accelerates granulation tissue formation
  • Use with caution near vital structures
Oculoplastic Considerations and Reconstruction
Acute Phase Considerations
Critical issues in periorbital involvement:
  • Corneal protection with temporary tarsorrhaphy if eyelid closure compromised
  • Lubrication and moisture chambers for exposure
  • Monitoring for orbital compartment syndrome
  • Assessment of optic nerve function
  • Judicious debridement near the lacrimal system, levator, and canthal tendons
Reconstructive Planning
Approach to periorbital reconstruction:
  • Delayed reconstruction after infection resolution (typically 3-6 weeks)
  • Staged approach often necessary
  • Consideration of functional priorities (eyelid closure, lacrimal drainage)
  • Flap rather than graft coverage for exposed bone or tendon
  • Regional flaps (temporal, forehead) preferred over distant tissue transfer
Long-term Management
Ongoing care requirements:
  • Management of cicatricial ectropion or lagophthalmos
  • Secondary procedures for contour abnormalities
  • Addressing lacrimal system dysfunction
  • Psychological support for facial disfigurement
  • Long-term follow-up for contracture development
The reconstructive challenge in post-NF defects is substantial, requiring a balance between functional and aesthetic outcomes. Modern approaches emphasise tissue preservation during debridement when possible, though never at the expense of infection control. Multidisciplinary collaboration between oculoplastic surgeons, infectious disease specialists, and intensive care teams is essential for optimal outcomes.
Summary and Key Learning Points
Malignancies
  • Eyelid BCC remains the most common periocular malignancy, with Hedgehog pathway inhibitors representing a significant advance for advanced/unresectable disease
  • Lacrimal sac TCC requires high clinical suspicion with bloody epiphora and medial canthal mass; early en bloc resection is critical
  • Merkel cell carcinoma demands aggressive management with wide excision, SLNB, and consideration of immunotherapy for advanced disease
Vascular & Inflammatory Conditions
  • Periocular infantile haemangiomas require prompt treatment to prevent amblyopia, with propranolol as established first-line therapy
  • Fronto-ethmoidal mucoceles present with gradually progressive proptosis and globe displacement; endoscopic marsupialisation offers high success rates with minimal morbidity
  • Periorbital necrotizing fasciitis represents a surgical emergency requiring immediate broad-spectrum antibiotics and aggressive debridement
Critical Drug Interactions
  • Hedgehog inhibitors: CYP3A4 interactions, teratogenicity, QT prolongation
  • Propranolol: monitoring HR/BP/glucose, caution with asthma and concurrent beta-blockers
  • Immune checkpoint inhibitors: risk of immune-related adverse events including ocular inflammation
  • Clindamycin in NF: essential for toxin suppression but risk of C. difficile colitis
This clinical update has provided an evidence-focused review of six important periocular conditions requiring oculoplastic surgical expertise. Multidisciplinary collaboration remains essential, particularly for complex cases involving orbital structures and adjacent specialties. Continued advances in medical and surgical management offer improved outcomes for these challenging conditions.
Thank you for joining this clinical journey through complex periocular conditions!
Dr. Nabil El-Hindy FRCOphth