Introduction
The human orbits are symmetrically paired structures separated by the nasal cavity and paranasal sinuses [1]. Seven bones of the craniofacial regions contribute to form the orbital cavity, namely frontal, sphenoid, zygomatic, palatine, ethmoid, and lacrimal bones [2]. It is pyramidal with the apex directed posteriorly and is anatomically complex due to the interactions between bony, vascular, cranial nerves, and other neural structures [1]. Certain clinical conditions such as infections, tumors, and trauma can compromise the orbital apex leading to a constellation of recognizable clinical features termed “orbital apex syndrome” [1,2]. This syndrome is characterized by signs and symptoms related to vital structures located in the orbital apex which include ophthalmoplegia (due to involvement of oculomotor, trochlear, and abducens) blindness (due to optic nerve involvement) and loss of sensation in the frontal and preorbital region (due to involvement of the ophthalmic division of trigeminal nerve) [2,3].
There have been several reports of orbital apex syndrome secondary to oro-dental infections but to the best of our knowledge, no previous case has been reported implicating noma in the literature. We report a case of orbital apex syndrome secondary to noma destruction highlighting the important aspect of management.
Case report
A case of a 7-year-old female child was referred from Primary Health Care Center to our hospital with chief complaints of ulceration on the left cheek region and left orbital pain with reduced vision in the left eye has been presented after obtaining consent from the patient’s caregiver. The onset of symptoms dated 13 days before presentation, with pain, cheek swelling, fever, and loss of appetite. There was a history of Malaria just before the onset of the symptoms and no history of noma in the family.
Clinical examination revealed a stable patient, who was not in any obvious distress, pale, acyanosed, and anicteric. There was a cone-shaped buccal defect involving the left outer and inner cheek walls (Fig. 1, Right) with extension to the dentoalveolar segment, the maxilla, and the posterior palatal region (Fig. 1, Left). The healing outer cheek defect measures 3 × 3 cm in diameter. Ophthalmological examination revealed no perception of light, complete ptosis, ophthalmoplegia, hypoesthesia involving the preorbital region, loss of corneal sensation, and absolute pupillary defect on the affected eye. There was loss of preorbital in the frontal and preorbital region, and no pupilar response to light. Fundoscopy revealed primary optic atrophy, however, no choroidal folds were seen. Figure 2 shows complete ptosis on the left eye (right) and frozen globe by the absence of mobility in all directions of gaze (left).
Figure 1.
Clinical photograph showing buccal defect involving both the left outer and inner cheek walls (right) with extension to the dentoalveolar segment, the maxilla, and posterior palatal (left).
Laboratory investigations revealed a white blood cell (WBC) count of 12.30 × 109/l, hemoglobin of 6.6 g/dl, Genotype AS, and a blood group B, rhesus D positive. There were negative serological markers (retroviral screening and hepatitis B surface antigens). The patient has a total protein of 5.0 g/dl and albumin of 2.2 g/dl. The patient had a mid-upper arm circumference of 13.6 and a Z score of −1 standard deviation (SD) calculated from the patient’s weight of 15 kg and height of 104 cm. Anemia and malnutrition were identified as a comorbidities in this patient. The Coronal reformatted craniofacial computed tomography (CT scan) of the patient (bone window) showed lytic destruction of the left side alveolar process of the maxillary bone and walls of the left maxillary sinus and orbital floor with collection in the ipsilateral maxillary and ethmoidal sinuses (Fig. 3). Axial CT scan image of the patient also showed irregular enhancing hyperdensity in the left orbital apex obscuring the optic nerve and the distal aspect of the lateral and medial recti muscles suggestive of inflammatory changes, associated proptosis of the ipsilateral eye globe also demonstrated (Fig. 4).
A diagnosis of orbital apex syndrome secondary destruction was made based on the above clinical findings. The patient was treated with intravenous antibiotics (Amoxycillin-clavulanate) (Augmentin) 30 mg/kg every 12 hours for 2 weeks), high calorie and high protein diet, intravenous fluid, analgesics (IV Paracetamol (Intemol) 0.75 mg/kg 8 hourly for 5 days). In addition, wound debridement and dressing using povidone-iodine was done. Sequestrectomy was done to remove the necrotic bone (Fig. 5). The patient was followed up until resolution of the condition. However, the ophthalmological features remain persistent. Following the resolution of the acute phase, the patient was discharged home in satisfactory condition. Follow up was uneventful and awaiting surgical reconstruction of the defect at a later date.
Figure 2.
Clinical photographs showing complete ptosis on the left eye (right) and the presence of a fixed eyeball with all the directions of gaze (left).
Figure 3.
Coronal reformatted craniofacial CT scan of the patient (bone window) showing lytic destruction of the left side alveolar process of the maxillary bone and walls of the left maxillary sinus and orbital floor with collection in the ipsilateral maxillary and ethmoidal sinuses.
Discussion
Orbital apex syndrome could be caused by several etiologies such as infection, inflammation, thrombosis, neoplasia, trauma, and even iatrogenic diseases [3]. This case report was secondary to the noma destructive process that extends to the orbital apex. Noma could rapidly spread to destroy the soft tissues and hard tissues of the face and may include vital structures such as nerves [4]. Orbital apex syndrome secondary to infections could arise from various structures in the head and neck region. It most often is associated with invasive fungal sinusitis, aggressive bacterial sinusitis as well as bacterial orbital cellulitis [5]. Although very rare, the odontogenic infection has been reported to cause orbital apex syndrome [6]. Spread of infections from periapical abscess, maxillary osteomyelitis of odontogenic origin could occur to the maxillary sinus and other sinuses through the infected dental fossa, then, to the orbit and surrounding areas, involving the extraocular muscles and optic nerve, and possibly to the cavernous sinus [5,6].
Figure 4.
Axial CT scan image of the patient showing irregular enhancing hyperdensity in the left orbital apex obscuring the optic nerve and the distal aspect of the lateral and medial recti muscles suggestive of inflammatory changes, associated proptosis of the ipsilateral eye globe also demonstrated.
Previous studies have shown diabetes mellitus as the most common predisposing factor in the spread of infection to cause orbital syndrome [5,7]. Malnutrition and anemia have been the possible predisposing factors in this current case report.
The stamping feature of orbital apex syndrome is blurred or loss of vision due to optic nerve involvement and ophthalmoplegia secondary to involvement of cranial nerves III, IV, and VI [6]. In our study, the clinically identifiable features were ophthalmoplegia, loss of vision, ptosis, and hypoesthesia. Differentiation between orbital apex syndrome and other clinical entities such as superior orbital fissure syndrome, Tolosa-Hunt syndrome, and cavernous sinus syndrome might be difficult. In general, it might be significant to point out that optic nerve involvement along with any combination of oculomotor, trochlear, abducens, and ophthalmic division of trigeminal nerve with or without maxillary division involvement points to orbital apex syndrome, whereas the same picture excluding involvement of the optic nerve should point towards superior orbital fissure syndrome [7]. Cavernous sinus syndrome/thrombosis can be diagnosed by considering all the features of superior orbital fissure syndrome along with the involvement of the maxillary division of the trigeminal nerve [8]. Chemosis, periorbital swelling, and rapid involvement of the contralateral eye are likely findings in cavernous sinus thrombosis.
Figure 5.
Clinical photograph showing the operation site and the sequestrum removed.
The main focus of the surgeon is to locate the lesion and identify the etiological factor. Thorough and careful clinical examination is of utmost importance [9]. Ophthalmological evaluations must be done to establish ophthalmoplegia, vision status, and ptosis [7]. For accurate diagnosis of orbital apex syndrome, neuroimaging modalities are necessary [10]. Magnetic resonance imaging (MRI) is the best neuroimaging modality with a CT scan as a second option when an MRI facility is unavailable [7]. Due to the unavailability of MRI in our clime, CT was done and it showed lytic destruction of the alveolar process of the maxilla and walls of the maxillary sinus and orbital floor with a collection of inflammatory exudates in the ipsilateral maxillary and ethmoidal sinuses. It has demonstrated enhancing hyperdensity in the affected orbital apex that obscures the optic nerve and the distal aspect of the lateral and medial recti muscles suggestive of inflammatory changes associated with proptosis of the ipsilateral eye globe also demonstrated. CT scan also demonstrated proptosis in the affected eye.
The treatment of orbital apex syndrome is directed towards the underline condition [11]. Our patient received antibiotics and had serial debridement and medical and nutritional supportive treatment. A sequestrectomy was done under general anesthesia and the patient was monitored closely with the hope for symptom improvement in the near future.
Conclusion
Noma is a rapidly progressive disease that is capable of destroying oral and surrounding soft, and hard tissues with serious functional and cosmetic complications. Orbital apex syndrome was seen as a complication in this case report. This reinstates the need for early detection and treatment of noma.
Conflict of interest
None declared.
Funding
Nil.
