Managing Paediatric Eye Health with Ocular Plastics

People commonly believe that dealing with paediatric patients and their nervous parents is difficult. To the contrary, this part of my practice is often the most enjoyable and satisfying. I believe that all health care professionals can enjoy working with paediatric patients, as long as they appreciate two points:

1. A parent who may appear overbearing is only trying to be the best advocate for their child.
2. When a child won’t allow you to examine them, it’s best to diffuse the situation by not examining them. Instead, give them a small and age-appropriate token gift and keep talking to the parent (I keep a box of trinkets and stickers for the child to choose from, Figure 1), then engage them with play-based interaction. This will give you the opportunity to observe and glean important signs, such as levator function and lid crease as the child fixes and follows an interesting toy. If, after a couple of minutes the child still won’t allow you to examine them, it is important not to persist. Finish the consult and rebook for another time. Most children will have more confidence in you and allow you to examine them at the second meeting.

The incorporation of a nasal endoscope into the management of childhood epiphora is the most significant advance in recent years

CONGENITAL NASOLACRIMAL OBSTRUCTIONS

Congenital Nasolacrimal Duct Obstruction 

Congenital nasolacrimal duct obstruction (CNLDO) is extremely common, affecting 11.3% –20% of newborns.¹ 

Parents will report epiphora and recurrent ‘conjunctivitis’ with mucus discharge, and sometimes, dermatitis from constantly tearmoist cheeks. The signs on examination are a high tear meniscus, delayed fluorescein dye disappearance, mucus clumping of lashes, and less commonly, a mucocele (mucus filled lacrimal sac), mucus regurgitation on lacrimal sac pressure, or dacryocystitis (Figure 2).

Before making a diagnosis of CNLDO, other causes of childhood tearing need to be considered, such as epiblepharon, uveitis, keratitis or glaucoma. It is relatively simple to differentiate these with a delayed fluorescein dye disappearance test, which has a 90% sensitivity and 100% specificity.²

The treatment pathway for CNLDO depends on the presence or absence of a lacrimal sac mucocele (Figure 3 and 4). Given that 96% of cases will resolve spontaneously in the first 12 months of life, and 45% of cases that are still tearing at 12 months of age will resolve spontaneously (assuming no mucocele), the mainstay of treatment is a ‘wait and see’ approach with lacrimal sac massage.³ 

Lacrimal sac massage is best performed when the child is drowsy after feeding. For greatest effect, the pulp of the index finger is placed posterior to the anterior lacrimal crest, so that pressure is applied to the sac and not the bone itself. Downward pressure is exerted towards the alar of the nose to push any mucus trapped within the sac down the duct, perforating the membrane at the distal end of the duct (Valve of Hasner).

If spontaneous resolution hasn’t occurred by 16–18 months of age, interventional treatment is indicated. For severe symptoms, such as frequently recurrent conjunctivitis, copious mucus discharge, and epiphora associated dermatitis of the cheeks, earlier intervention is offered to the parents.

Traditionally, a progression of procedures is advocated, beginning with a probe and syringing. If this fails, lacrimal duct stenting (-/+balloon dilation of the lacrimal duct) is performed and finally, should this fail, we progress to dacryocystorhinostomy (DCR).

The incorporation of a nasal endoscope into the management of childhood epiphora is the most significant advance in recent years. Endoscopic examination at the initial general anaesthetic allows the lacrimal surgeon to assess the most appropriate procedure, rather than taking a one size fits all approach which can result in the need for repeat general anaesthetics and multiple procedures.⁴ For example, while probing alone is an effective treatment for a simple membrane obstruction of the distal duct (Valve of Hasner), some children have a congenital absence of the valve. For these patients, blindly pushing the probe through, or stenting a small probegenerated opening, will create a small hole in the mucosa that will spontaneously close or be inadequate post-stent removal. Instead, a mucosal window should be fashioned under direct endoscopic vision, using sharp dissection, with stents placed through the newly created opening. This approach will re-establish the natural outflow via the duct, and avoid the need for DCR, which alters the natural pathway by bypassing the nasolacrimal duct and requiring bone removal. DCR should only be performed in cases of bony obstruction or narrowing of the nasolacrimal duct.

The ability to assess and successfully treat intranasal pathologies that are causal in the CNLDO under direct vision via a nasal endoscope reduces the risk of scarring of the nasal mucosa and the creation of false passages.⁵ Unnecessary trauma to the inferior meatus of the nose (into which the distal nasolacrimal duct opens) may be irreparable and necessitate a DCR, whereas an initial stenting, done atraumatically, can cure the CNLDO.

Mucocele/Dacryocystocele 

Only 0.1–0.3% of CNLDO cases present at birth or soon after with a mucocele of the lacrimal sac, which is often called a dacryocystocele in this age group.⁶ Dacryocystoceles are associated with an unacceptably high incidence of respiratory distress and dacryocystitis.

The incidence of dacryocystitis in these patients can be over 70% if left untreated.⁷

Of dacryocystocele cases, 50.6% are associated with an intranasal cyst, with 19.6% suffering respiratory distress6 (Figure 5). Babies under the age of six weeks, and in some cases up to six months, are obligate trans-nasal breathers, except when crying, to aid with breast feeding.⁸ Additionally, 80% of airflow is through the lower third of the nose with the narrowest point being the internal nasal valve. This is in the plane of the Valve of Hasner, which is where the cyst is positioned, being the lower end of the nasolacrimal duct. The nasal septum in neonates is soft and malleable so even a unilateral cyst can cause bowing of the septum, affecting bilateral airflow. Unilateral intranasal cysts have a 10% association with respiratory distress.⁹ 

When presented with an infant with a lacrimal mucocele, it is most important to exclude an intranasal cyst that can potentially result in respiratory distress. Management must start with an intranasal examination, performed by the treating person if they are very comfortable with nasal endoscopy, or by an ear, nose and throat surgeon. In the case of an intranasal cyst, urgent de-roofing is undertaken in theatre to resolve the CNLDO and intranasal cyst.

If there is no intranasal cyst, a fourweek course of lacrimal sac massage should be followed, with an endoscopic examination under anaesthesia should the dacryocystocele not have resolved. During the same anaesthetic the patient will be treated as required, usually with a probe alone, however primary stenting may occasionally be necessary.

PTOSIS

Retrospective studies report around 0.079% incidence of congenital ptosis in children.¹⁰ The ptosis can be unilateral or bilateral. Additionally, it may appear unilateral, but in reality, be bilateral with significant asymmetry. If this last scenario is not suspected, and surgery proceeds to treat the more obvious side, the procedure may unmask or cause an apparent increase in the ptosis of the contralateral upper eyelid post-operatively (Figure 6).

Ptosis Classification: 

Myogenic 

Myogenic ptosis is the most common form of ptosis and is caused by embryonic dysgenesis of the muscle.

Because the muscle is infiltrated with a fibrofatty tissue, clinically we see reduced excursion of the muscle with poor contraction and relaxation; poorly formed lid crease; lid hang up on downgaze; lagophthalmos and frontalis muscle overaction. Five per cent of cases can be associated with superior rectus weakness,11 known as ‘double elevator palsy’.

Neurogenic 

Neurogenic ptosis is rare and presents as Marcus Gunn jaw-winking ptosis, congenital Horner’s Syndrome or III nerve palsy.

Marcus Gunn Jaw-Winking Ptosis 

This is caused by a misdirection of the nerve fibres, with an abnormal connection between the levator muscle and the motor division of the fifth cranial nerve that innervates the external pterygoid muscle (jaw muscle) so the ptotic lid lifts on movement of the jaw, e.g., chewing. This can occur in 2–13% of congenital ptosis cases.¹²

Congenital Horner’s Syndrome 

This is caused by a developmental interruption of the sympathetic fibres, resulting in a mild ptosis, miosis, facial anhidrosis (no sweating) and often a lighter iris.

III Nerve Palsy 

In the presence of a complete III palsy, there is ptosis, a dilated pupil and a down and out eye.

Both III nerve palsy and congenital Horner’s Syndrome need to be investigated with MRI imaging, looking for intracranial lesions.

Aponeurotic 

Aponeurotic ptosis is quite uncommon congenitally, and occasionally occurs secondary to birth trauma.

Ptosis Surgery 

Ptosis surgery is generally timed to take place at the age of four to five-years, just before the child enters preschool and meets a new cohort of friends. Amblyopia is the most feared complication of paediatric ptosis, resulting in permanent loss of vision in up to 20% of patients.¹³ Amblyopia, caused by visual obstruction or astigmatism due to the low lid position, results in earlier surgery being offered. Another indication to operate early is a constant backward head tilt to maintain a clear visual axis as this can adversely affect posture and mobility milestones. Being ostracised in the day care environment is another reason to proceed to surgery earlier in the absence of amblyopia or motor delay.

The form of surgery chosen depends on levator function. This is assessed by the excursion of the lid in up and downgaze, the velocity of that movement (slower = weaker) and presence or absence of a lid crease (absent = poor):

1. If levator function is good (>8mm) a levator advancement is usually adequate.
2. If levator function is moderate (5–8mm) then a levator resection is advocated, however I advocate performing a combined muller and levator advancement, which I find more effective and physiologic than levator resection alone.
3. When levator function is very poor (<4mm), a silicone sling is threaded from the tarsal plate up into the brow to recruit the brow/frontalis muscle complex to more effectively raise the lid (Figure 7). While autogenous fascia lata (a fibrous band in the lateral upper thigh) was traditionally used for slings, this is now much less common as it necessitates a second operative site (leg for harvesting) or donor tissue, which I prefer not to use.

It is particularly challenging to get excellent symmetry for height, contour and lid crease reconstruction when performing ptosis surgery (Figure 8). Most children under the age of 10 will require a general anaesthetic for the procedure, and so the degree of muscle advancement or tightening of the silicone sling is based on measurements taken at the preoperative examination then modified, to a large extent, by the surgeon’s prior experience.

CHALAZIA

Chalazia is one of the most common paediatric ocular plastic presentations. It occurs when a meibomian gland blocks and the lining of the gland is disrupted, exposing the surrounding tissue to oily secretions and inciting a foreign body inflammatory reaction. When the reaction is contained within the tarsal plate, a well-defined mass is palpable with variable inflammation of the overlying skin. If the distal part of the gland is involved, the overlying lid margin is raised, swollen and red (marginal chalazion, Figure 9). If the oily secretions enter the skin/muscle layer of the eyelid, the inflammatory reaction intensifies and can cause ulceration of the skin. While chalazia can look very red and angry, they cause minimal discomfort and only become acutely tender with secondary infection. At this point they need to be treated with oral antibiotics, as topical antibiotics are inadequate. Under-treated infections can result in periorbital cellulitis.

In the first instance, chalazia are treated conservatively, if the child is older or cooperative enough, heat packs are applied. The parents are asked to aim for a fiveminute period of heat contact, with an adequate heat sink, such as a small wheat bag. Parents are instructed to follow this with firm compression of the eyelid.

Chalazia will often resolve spontaneously, and it is only if they don’t resolve over months, or if they are migrating into the skin/muscle layer of the lid causing intense inflammation and ulceration, that surgical drainage is indicated (Figure 10). Depending on the patient’s personality, a general anaesthetic will usually be required for those under the age of eight. For this reason, I usually have a higher threshold to proceeding to curettage in children compared with adults.

Parents often ask about how to reduce the chance of recurrence. There are antibiotics as well as complementary remedies with Omega-3 which exhibit anti-metalloproteinase actions, potentially improving meibomian secretions.¹⁴ Traditionally, ocular rosacea and meibomian gland dysfunction is treated with oral tetracycline or doxycycline, however these can accumulate in dental enamel, nails and bones in children, and so are best avoided. In severe cases however, a one-to-two-week course of Augmentin duo or erythromycin will often be of benefit.^15,16 

EPIBLEPHARON

Epiblepharon (Figures 11 and 12) is typically a congenital condition where an absence of a lid crease allows slippage of the anterior skin/muscle layer over the posterior layer, especially in downgaze, causing skin and lashes to touch the cornea. This differs from entropion where the entire lid turns in. While unusual in Caucasian children, the incidence is up to 12% of Asian children.¹⁷ It is typically bilateral, although often asymmetric and involving the medial two thirds of the lower lid. It occasionally occurs in the upper lid as well.

Parents say their child squints or closes their eyelids in sunlight, has epiphora, frequently blinks and rubs their eyes, and experiences ocular irritation, especially in downgaze.

On examination, the lid crease is poorly formed or absent in downgaze; the lashes have an upward tilt in primary gaze and on downgaze are pushed further posteriorly, increasing lash to corneal touch.

Multiple surgeries are described, with the principal being to excise a narrow strip of pretarsal skin and orbicularis, and reform a lid crease.

DERMOID

Dermoid cysts (Figures 13 and 14) are the most common benign periorbital tumour in the paediatric population.¹⁸ Dermoid cysts arise from misplaced ectodermal cells (skin precursors) during early embryonic development and are thus considered a developmental choristoma. They represent 35–47% of orbital paediatric lesions and 89% of all orbital cystic lesions.¹⁹ The majority are associated with the frontozygomatic (superolateral orbital rim) and, less commonly, the fronto-ethmoidal sutures (superomedial orbit). Uncommonly, these anterior dermoids can have a ‘dumbbell’ configuration with a contiguous posterior bulb extending deeper into the orbit. Rarely, they present as an isolated lesion deep within the orbit, remaining asymptomatic until sometimes rupturing late in adult life to cause an intense orbital inflammatory reaction that can be vision threating.

Dermoids usually come to the parent’s attention within the first few months of life and tend to grow slowly. Parents are often unsure of whether the growth is in line with, or faster than the child’s general growth rate. While the rate of spontaneous rupture in childhood is low at five in 115 cases,¹⁸ the risk of spontaneous rupture rises if the cyst is left into adulthood, allowing it to increase in size. The keratin-based contents of the cyst incite a severe inflammatory response in the surrounding tissue, which is best avoided.

Treatment is excision via a simple lid crease incision as a day surgery procedure.

CAPILLARY HAEMANGIOMA

Capillary haemangiomas (Figures 15 and 16) are the most common benign tumour in infants, with a 4–5% prevalence, a preponderance for females, and distribution on the face.²⁰ They are thought to be secondary to an embolism of placental tissue in utero.

Superficial hemangiomas are red and protuberant, with well-defined edges and they blanch on compression. They are sometimes more deeply situated and can even be deep within the orbit.

Capillary haemangiomas can be unnoticeable at birth and grow in the first few months of life. They tend to grow rapidly in the first year of life before starting to involute, with 90% of lesions regressing by the age of nine.²⁰ 

The superficial lesions are an aesthetic concern for parents, but if large enough can cause occlusive or anisometropic amblyopia, requiring treatment. The deeper lesions can cause proptosis, diplopia and even optic neuropathy and should also be treated.

The mainstay of treatment in the past was oral and/or intralesional injections of steroids. While these were effective, systemic steroids are associated with considerable side effects. Additionally, intralesional steroids – especially for deeper orbit lesions – were technically challenging and associated with more severe side effects, including central retinal artery occlusion. In 2008, a French cardiologist noted that children taking oral propranolol for cardiac conditions underwent incidental regression of concomitant haemangiomas.²¹ 

Regression of infantile haemangiomas occurs in 94% of patients treated with oral propranolol,²² and it is certainly rewarding to be able to comfort a distressed parent with this information. Referral to a paediatrician for propranolol treatment is appropriate as some patients will suffer blood pressure or blood sugar changes, principally at the initiation of treatment. The sooner propranolol is instituted the better, as the response rate reduces with age. Thankfully, surgical excision is rarely required, as these lesions are poorly encapsulated and have a very high blood flow, which makes it a high-risk procedure in infants with low circulating blood volumes.

PERIORBITAL AND ORBITAL CELLULITIS

Significant periorbital infections are treated as inpatients and so are beyond the scope of this article. However, it is important to discuss periorbital cellulitis in order to improve the early management of these patients so that we can reduce the likelihood of progression to hospitalisation for IV antibiotics and surgical intervention.

Observational studies have shown that 5–10% of upper respiratory infections are complicated by acute bacterial sinusitis,^23,24 which is the principal cause of periorbital cellulitis.

We classify periorbital cellulitis into preseptal and orbital, depending on where we believe the extent of the infection is in relation to the orbital septum. The orbital septum is a fibrous sheet that separates the orbicularis muscle anteriorly from the orbital fat situated immediately posterior to the septum. The septum starts just shy of the edge of the tarsal plates in the upper and lower lids and ends at its insertion onto the bone of the anterior orbital rim.

In preseptal cellulitis, the inflammation is ‘outside’ the orbit, manifesting inflammatory signs of the skin/orbicularis layer. There is redness, heat and oedema, but the eye is white, with no orbit signs of chemosis, proptosis or ocular rotation restriction. If the retroseptal tissues of the orbit become involved, there is a progression of orbital signs from ocular erythema/chemosis, proptosis, restriction of ocular rotations, through to optic nerve dysfunction. Orbital infection, if undertreated, will progress to abscess formation and finally intracranial extension with abscess formation and cavernous sinus thrombosis. In patients with intracranial extension, the incidence rates for loss of vision and death are 3–11% and 1–2.5%, respectively.^25,26 Prior to antibiotic therapy, 17% of these patients died with meningitis and 20% developed blindness.²⁷

Although preseptal cellulitis can be caused by a skin-based infection, such as trauma or an infected chalazion, it often arises with ethmoid sinus infection and is the most common complication of acute bacterial sinusitis in children, representing 80–90% of all extracranial complications of sinusitis.²⁸ 

If preseptal cellulitis is inadequately managed, it will proceed to orbital cellulitis as outlined in the widely accepted Chandler Classification.29 Understanding preseptal cellulitis and treating it aggressively by starting oral antibiotics early is essential. Draw a line with a skin marker around the border of the skin erythema and review after 24 hours (Figure 17). Any progression on antibiotics warrants referral to hospital for IV antibiotics and further assessment by the paediatric; ear, nose and throat; and ophthalmology teams. It is important to note that 74–85% of all complications of acute sinusitis are orbital.³⁰

Associate Professor Geoff Wilcsek is an adult and paediatric ophthalmic plastic and reconstructive surgeon, with a specific interest in endoscopic approaches to the lacrimal system and orbit. He is a Fellow of the Royal Australian and New Zealand College of Ophthalmology and a member of the Australian and New Zealand Society of Ophthalmic Plastic and Reconstructive Surgeons and American Society of Ophthalmic Plastic and Reconstructive Surgeons. After completing training as an ophthalmologist at the Prince of Wales hospital, Assoc/Prof Wilcsek completed two fellowships in Ocular Plastics and Orbit Surgery – the first at the Columbia Presbyterian Hospital, Manhattan, New York and the second at the Royal Manchester Eye Hospital, Manchester, England. Assoc/Prof Wilcsek is a consultant ophthalmic plastic surgeon at the Prince of Wales Hospital and Sydney Children’s Hospitals and a member of the Skull Base Unit, Macquarie University Hospital. He has authored and co-authored over 60 scientific articles in peer reviewed journals and four book chapters.

References 

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