WCPOS V 2024 Plenary Highlights Becoming a Superhero Amidst Pediatric Orbital Diseases

Orbital considerations are well trodden ground for pediatric ophthalmologists, and proper management might not only be decided whether a patient lives a life of clear vision. Orbital diseases, including tumors, can often be a matter of life and death. 

This turns ordinary ophthalmologists into life- and sight-saving superheroes, according to Dr. Celeste Mansilla of Argentina. And like any superhero, superpowers must be augmented by the knowledge of how to use them. This was the topic of the first plenary session of the 5th World Congress of Paediatric Ophthalmology and Strabismus (WCPOS V 2024) in Kuala Lumpur, Malaysia, where experts from around the world gathered to share their expertise on the world’s myriad of orbital maladies. 

When orbital dermoids go deep

Orbital dermoids were first on the list in a pearl-packed presentation from Dr. Bipasha Mukherjee (India). Her time at the podium saw a journey through the various kinds of cysts, including a case study-guided exposition on how to recognize, diagnose and treat each one. 

“Superficial dermoids are very easy to recognize and manage,” said Dr. Mukherjee. “But it is the deep dermoids which often cause the problems.”

Clinical presentations of these can include proptosis, ptosis, diplopia, a squint and orbitocutaneous fistula. Many can even lead to optic nerve compression if undetected, as they grow over time.

Dr. Mukherjee noted that diagnosis is often done by CT scans, but an MRI must be used in cases where dermoids go particularly deep or threaten underlying ocular structures.

“The management of choice is complete surgical excision,” Dr. Mukherjee explained. These cysts must be removed along with the periosteum, and can be irrigated to reduce the size. In the case of an intraoperative rupture, Dr. Mukherjee recommends removing the cyst wall completely and irrigating with saline. 

The so-called dumbbell orbital cysts present additional challenges. For Dr. Mukherjee, the key here is complete removal of all components on both sides of the bony canal. The consequences for not doing so include inflammation, recurrence, abscess formation or new orbitocutaneous fistula appearing. 

In the end, Dr. Mukherjee stressed that these dermoids are typically routine, with excellent prognoses—especially if complete removal of all dermoid components can be achieved. 

Avoiding orbital cellulitis overdiagnosis

Are we going overboard with orbital cellulitis overdiagnosis in children? Dr. Katya Tambe (United Kingdom) believes so, and she provided an overview of the sinister syndromes that mimic orbital cellulitis—as well as giving tips for how to avoid falling into this potentially sight-robbing trap.

Dr. Tambe began with a disturbing statistic. A 2016 study found that 50% of cases of idiopathic orbital inflammation (IOI) in children were misdiagnosed as orbital cellulitis. And according to Dr. Tambe, this is not without its consequences.¹

In one case, a biopsy of a lesion masquerading as orbital cellulitis revealed Langerhan’s Cell Histiocytosis, a serious rare disease requiring immediate attention and consultation of a spate of subspecialists from hematology to radiology.

The case presentations went on, and ranged from treatable IOI causes with excellent prognoses like sclerosing orbital inflammation to downright scary ones such as IgG4 related disease and rhabdomyosarcoma. 

In the end, however, Dr. Tambe sees positive changes down the road for these often tragic misdiagnoses subsiding. “There’s a lot coming in the future,” she said. “AI, deep learning, miRNA and more—these can help us understand these inflammatory conditions and will probably help us make diagnoses earlier and treat these children faster.”

Proptosis and tumors

To this point, proptosis has been a common symptom featured in the other two talks. But for Dr. Celeste Mansilla, it was the main event—and protecting and managing the cornea during proptosis can turn ordinary pediatric ophthalmologists into superheroes. 

For Dr. Mansilla, not only is proptosis an important early indicator for orbital disease, but it can also be a significant threat to pediatric sight or worse. Speed is paramount in treatment. “We have to be as quick as the Flash doing diagnostics and starting treatment,” she said, invoking the superhero theme of her presentation. “While we perform the tomography, wait for the biopsy and the pathology results, a malignant tumor continues to grow and grow.”

As soon as blinking is not present, Dr. Mansilla emphasizes that it is time for action. “We have to start using drops and gels intensively, and intensively means it is not once every two hours. It’s more, because we blink twenty times in a minute,” she said. 

She proposed other solutions, including duoderm patches, moisture chamber goggles and amniotic membrane patches. In extreme cases, a temporary tarsorrhaphy can be employed. 

These solutions all exist along a continuum of severity, but the important thing is to take action with whatever is around—and to realize that these measures are temporary until the condition is diagnosed and treatment is begun. “After we do this, we are capable of not only saving lives, but keeping the cornea transparent. And this is what a superhero does,” she said in conclusion to applause.

Dr. Manoj Parulekar (United Kingdom) built on what Dr. Mansilla said with a wide-ranging and comprehensive tour through the many different types of tumors and what can be done about them. 

Though his talk covered a lot of ground—from rhabdomyosarcoma to teratoma, gliomas, leukemia and more, some top tips emerged. Just as for Dr. Mansilla, early action is key, and sorting malignant cases from benign ones is critical.

MRI imaging is the imaging of choice, but with a key notation. “Diffusion weighted imaging is quite useful because this really indicates the water content and decreased intercellular spaces,” said Dr. Parulekar, noting that restriction of a lesion can be—but not always—indicative of malignancy. 

He concluded his talk with more pearls, including paying attention to rapidity of onset to identify serious disease and prevent hemorrhaging, and differentiating bony enlargement from soft tissue and global enlargement. 

But there is no substitute in the end for keeping it old fashioned for Dr. Parulekar. “Although imaging is important, nothing can supplant a careful clinical examination,” he concluded. 

Structural considerations 

The plenary session ended with two talks on structural considerations in orbital disease. Dr. May May Choo (Malaysia) started by describing her multidisciplinary approach to managing craniofacial deformities at the University of Malaya. 

The role she outlined for ophthalmologists is critical, largely revolving around informing management decisions. For Dr. Choo, the ophthalmologist is responsible for sharing baseline ophthalmic findings, detecting immediate danger to the visual system and helping to monitor the patient closely after the surgery. 

The keys to look out for in the examination for Dr. Choo are as follows: proptosis, exposure keratopathy, pupillary light reflex, visual acuity (cycloplegic refraction), fundus examination for optic disc compromise, and IOP to try and detect damage to the optic nerve. 

Dr. Blanche Lim (Singapore) finished with an exhaustive guide to reconstructing and caring for the pediatric socket. After an initial case study, she laid out her priority considerations for reconstruction of the pediatric socket: preoperative considerations, socket volume, forniceal volume and viability, the growth of the child, and postoperative aftercare.

From cost to where to draw extra tissue from and cultural considerations about removal of the globe, there’s much to keep in mind for Dr. Lim. But she sees an ophthalmic landscape shifting for the better in reconstructing the socket, and this can only mean better patient care in the future.

Reference

1. Spindle J, Tang SX, Davies B, et al. Pediatric Idiopathic Orbital Inflammation: Clinical Features of 30 Cases. Ophthalmic Plast Reconstr Surg. 2016;32(4):270-274.

Editor’s Note: Reporting for this article occurred at the 5th World Congress of Paediatric Ophthalmology & Strabismus (WCPOS V 2024) from 11-13 July in Kuala Lumpur, Malaysia.