Beware, Winter: A teenager develops relatively acute unilateral eye swelling, which confuses the treating physicians. Was a snowball fight to blame?
Welcome to a wintry casuistry from the latifundia of ophthalmology. A 16-year-old boy presented to an ophthalmologist with acute right-sided periorbital swelling. He reported that a snowball had hit him in this area of the face a few days earlier.
Only after three weeks MRI in the emergency room
The ophthalmologist recommended waiting and applying warm compresses. When that didn’t help, an unclear infection was suspected Doxycycline– Treatment initiated. The whole thing dragged on for almost three weeks. A clear development developed Proptosis. Eventually, the patient reported double vision, pain in the area of swelling increased, and when vision in the affected eye became progressively blurred, the decision was finally made to go to a hospital emergency department.
There, the admitting physicians were sufficiently concerned to initiate an MRI scan of the head. This showed a homogeneous mass in the Orbit, more precisely in the intraconal space between the eyeball and the intraorbital muscles of the eye. The mass was severely hyperintense on T1 imaging but showed no fat saturation. The MRI image led to an emergency referral to the eye clinic.
Pediatric radiologist: epidermoid cyst!
Even in the eye clinic, the teenager and his strange MRI image were initially not very clever. So further diagnostics: The visual acuity on the left was okay according to age, on the right it was only about a third, matching what the patient described. The pupillary reaction was normal on both sides, but not the intraocular pressure, which was 17 mmHg on the left side and 24 mmHg on the right side. The proptosis was measured and quantified to 5 mm. the subjunctive were inflamed red, the Optic nerve papilla severely edematous (grade IV) and the Choroidea showed clear folds.
All of this still didn’t really help, so an ultrasound examination was initiated. The intraconal mass emerged as an approximately 2.0 x 2.5 cm non-vascularized, hypoechoic structure. This image, along with the MRI, was then sent to the pediatric radiologist on duty, who made a presumptive diagnosis of an epidermoid cyst. What you have as a child behind the eye.
Biopsy: Pustekuchen!
At this point, something finally happened that no one likes to do with the eye, but there was no alternative – a biopsy was performed. Short interim assessment: The MRI did not give any convincing indications of an inflammatory process, so that a conceivable emergency steroid therapy for decompression did not really seem indicated. And neither MRI nor ultrasound looked like neuroblastoma or any other malignant childhood tumor. Ergo: Reinstabung, after all, there was now – see intraocular pressure, see papilledema, see choroid folds – acute danger of irreversible damage to the optic nerve or Retina.
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What the surgeons found was a bloody fluid that could also be suctioned out. No “mass”, no capsule. In order not to be left empty-handed after the biopsy, the surrounding tissue was biopsied. A first success was that the disc edema decreased after the biopsy, the intraocular pressure normalized and the visual acuity developed towards the normal level again. Doctors were now fairly certain the optic nerve was still intact, but still had no diagnosis. As is so often the case, the pathologist supplied them. In fact, what the MRI had suggested was a dermatoid cyst turned out to be a hemorrhage. However, this was not a normal traumatic bleeding – it had been a month since the snowball had been thrown.
The pathologist diagnosed a Lymphangiom, which the colleagues had not thought of before because the MRI image did not look like a lymphangioma, in which multicystic structures are typically expected, which are usually characterized by several liquid-liquid levels. A closer look at the MRI retrospectively showed indications of a certain compartmentalization of the “mass”, but it was definitely not a clear MRI finding. And that was probably due to the snowball, which probably led to bleeding into the lymphangioma, which in turn blurred the MRI findings.
Conclusion: just in time
Lymphangiomas in children are not uncommon, emphasized the authors of the case report, which they published in the specialist journal JAMA Ophthalmology have published. About every twenty-fifth intraorbital lesion in under 20-year-olds is a lymphangioma. There are therapeutic options in the early stages, but only an operation helped in the case of the child who presented here. Six months after the operation, the boy was largely recovered. However, there were slight double visions, so that in the course of the Strabismus-Surgery becomes necessary.
Image source: Pauline Bernfeld, Unsplash