An unusual case of total ophthalmoplegia

An eight-year-old male child presented with drooping of the left eyelid with a history of penetrating injury of hard palate by an iron spoon seven days ago, which had already been removed by the neurosurgeon as the computed tomography scan revealed a spoon in the left posterior ethmoid and sphenoid bone penetrating into the middle cranial fossa. On examination, visual acuity was 20/20 in each eye and left eye showed total ophthalmoplegia. Oral cavity revealed a hole in the left lateral part of the hard palate. We managed the case with tapering dose of systemic prednisolone. The total ophthalmoplegia was markedly improved in one month. Cases of foreign bodies in the orbit with intracranial extension are not unusual, but the path this foreign body traveled through the hard palate without affecting the optic nerve, internal carotid artery or cavernous sinus makes an interesting variation. Primitive neuroectodermal tumor (PNET) is a small round cell malignant tumor of neuroectodermal origin. Most of the PNETs occur in the central nervous system (CNS). PNETs recognized outside of CNS are diagnosed as peripheral PNET (pPNET). This tumor which expresses MIC-2 gene (CD99) seems to be least aggressive after complete tumor resection. We describe a rare case of PNET in a young girl.

An eight-year-old male child presented with drooping of the left eyelid with a history of penetrating injury of hard palate by an iron spoon seven days ago, which had already been removed by the neurosurgeon as the computed tomography scan revealed a spoon in the left posterior ethmoid and sphenoid bone penetrating into the middle cranial fossa. On examination, visual acuity was 20/20 in each eye and left eye showed total ophthalmoplegia. Oral cavity revealed a hole in the left lateral part of the hard palate. We managed the case with tapering dose of systemic prednisolone. The total ophthalmoplegia was markedly improved in one month. Cases of foreign bodies in the orbit with intracranial extension are not unusual, but the path this foreign body traveled through the hard palate without affecting the optic nerve, internal carotid artery or cavernous sinus makes an interesting variation. Cases of unexpected foreign body in the brain penetrating through the orbit presenting as total ophthalmoplegia have been reported in the literature. [ ¹ -3] However, a foreign body penetrating through the hard palate into the brain presenting as total ophthalmoplegia has never been reported as per our knowledge and thorough Medline search. The material of the penetrating foreign body was wood in the previous reported cases whereas in our case, we found it to be an iron spoon.

Case Report
An eight-year-old male child attended the outpatient department, complaining of drooping of the left eyelid [ Fig.  1] with a history of penetrating injury of the hard palate by an iron spoon seven days back. The injury had occurred while the child had fallen down with the spoon in his mouth. The iron spoon had already been removed by the neurosurgeon under general anesthesia elsewhere before the child presented to us. The X-ray and computed tomography (CT) scan revealed a radio-opaque spoon perforating the hard palate, passing through the left posterior ethmoid and sphenoid bone reaching into left middle cranial fossa [ Fig. 2]. The patient did not complain of any fever, chill or rigor. There was no history of seizures or loss of consciousness. However, bleeding from the nose was reported by the patient. The drooping of eyelid was present before removal of the iron spoon as told by the child's father. This was confirmed by the discharge report of the neurosurgeon, mentioning the presence of ptosis of the left eye and total ophthalmoplegia before removal of the foreign body.
On local examination, the left eye showed complete ptosis with levator function of 2 mm [ Fig. 1]. Orbital margins were normal. Extra-ocular movements were absent in all directions of gaze [ Fig. 1]. The lid and conjunctiva were normal. Corneal sensation was impaired. The pupil was dilated and fixed. Both direct and consensual light reflex were absent in the left eye and were intact in the right eye. Other anterior segment and posterior segment details were within normal limits. The visual acuity in each eye was 20/20. Color vision of both eyes was intact. The cranial nerve examination of left eye showed third, fourth and sixth nerve palsy along with the involvement of the ophthalmic division of trigeminal nerve.
On examination of the oral cavity, a small full thickness hole of size 1.5 mm×1 mm in the left lateral part of hard palate was seen [ Fig. 3]. Hemoglobin, differential count, total leucocyte count and fasting blood sugar were done which were within normal limits.
As the patient came to us with total ophthalmoplegia, we managed the case as left superior orbital fissure syndrome following trauma and started systemic prednisolone empirically with a dose of 1 mg/kg body weight and tapered over a period of one month. Marked improvement of symptoms with closure of the hole in the hard palate was seen in one month follow-up [ Fig. 4].

Discussion
This case presents the clinical course of a child who sustained injury with an iron spoon. Cases of retained foreign bodies in the orbit with intracranial extension are not so unusual, [1][2][3] but the path which this foreign body traveled makes an interesting one. After having pierced the hard palate, it passed through the posterior ethmoid and sphenoid bone to enter the cranium, narrowly missing the carotid artery and optic nerve. The development of ptosis, ophthalmoplegia and impaired corneal sensation could be explained as direct injury to structures exiting through the superior orbital fissure. The superior orbital fissure syndrome is a rare complication of craniomaxillo facial trauma. The neurological symptoms are generally due to reversible neuropathy caused by edema, contusion and compression of nerves. [4] Improvement of neurological symptoms following post-traumatic superior orbital fissure syndrome have been reported following mega dose of corticosteroid. [5,6] Some authors advocate emergency optic nerve decompression as another treatment modality. However, the study of Sabri et al. finds satisfactory results and no complications attributable to mega doses of corticosteroids. [5] Marked improvement of ophthalmoplegia following steroid administration in our case justifies the diagnosis, as the improvement is due to resolution of edema and contusion caused by the trauma. Here a question arises about the cause of the injury, whether directly due to the foreign body or during the surgery for the removal. However, the history and the data in the discharge report clarified that the impairment was present before the surgery. The absence of cavernous sinus thrombosis, optic nerve injury or injury to internal carotid artery, despite the close proximity of this foreign body highlights this rare case. 5. Sabri A, Tugay S, Eral K.Megadose corticosteroid treatment for superior orbital fissure and orbital apex syndromes. Annals of  is seen in young adults and adolescents and has no sex predilection. Primary orbital pPNET is extremely rare and only nine cases have been reported previously. [1][2][3][4][5][6][7][8][9] pPNET shows characteristic small round cell tumor with rosette or pseudo-rosette, positive immunohistochemistry (IHC) and in some cases there are ultrastructural findings of neurosecretory granules. This report presents the clinical, radiological and histopathologic features of a left orbital mass in a young girl. IHC confirmed the diagnosis of pPNET. This rare tumor of the orbit should be considered in the differential diagnosis of hypercellular small round cell tumor of the orbit.

Case Report
An eight-year-old female child was brought to our outpatient department for progressing protrusion of the left eye ball for two months. There was associated diplopia with restriction of ocular movements superiorly and laterally. There was minimal ptosis and she had ocular pain over a period of four weeks. There was no history of injury, fever, chronic cough or other systemic symptoms. Her ophthalmic examination did not reveal any additional pathology other than a localized mass in the anterior orbit. On examination, a tender, firm, fixed, non-pulsating globular mass (4 x 3 cm) with irregular surface was felt in the supero-temporal quadrant of the left orbit. Ocular examination was otherwise normal. Computed tomography (CT) scans of the orbit and brain depicted a soft tissue enhanced irregular mass located in the supero-temporal part of the left orbit without evidence of any bone defect or erosion [ Fig. 1]. Complete hemogram, including general blood picture, erythrocyte sedimentation rate and X-ray chest were normal. Human immunovirus and HBsAg screening tests were negative. Anterior orbitotomy of the left orbit was done under general anesthesia and en block excisional biopsy of the tumor was performed. Grossly, the tumor was well encapsulated and measured 3.2 x 2.6 x 1.5 cm and weighed 12 g.
The 10% neutral buffered formalin-fixed tissue fragment was white and firm. It was embedded in paraffin and sections were stained with hematoxylin-eosin. Histopathological examination of the tumor revealed round cells having eosinophilic cytoplasm and round nuclei. The cells were arranged in sheets and cords with thin sclerotic stroma. Few pseudo-rosettes which were viable tumor cells around the blood vessels were seen. There was no evidence of necrosis and hemorrhage. [ Fig. 2A] Sections were studied by IHC for neuron-specific enolase (NSE) (ab "BBS/NC/VI-HI4), leukocyte common antigen (LCA) CD45 (ab "2BII+PD 7/26"), S-100 protein (ab "S1/61/69"), Desmin (ab "D-33") and MIC-2 (ab "12E7") using avidin-biotin complex technique. These antibodies were applied synchronously with appropriate positive control slides. In our case, IHC was positive for MIC-2 gene (CD45-ve, S-100-ve, Desmin -ve, NSE-ve) [ Fig. 2 B]. On the basis of these findings, a diagnosis of primary pPNET of the orbit was made. The patient's diplopia and pain resolved one week after the surgery. Complete systemic evaluation was carried out which was normal. Patient is being followed up every three months and orbital CT scans are repeated on every visit without any sign of recurrence. Patient is under close observation of oncologist and there are no signs of recurrence for the last six months.