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Blindness: a curable complication of acute sinusitis

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(Saudi Journal of Oto-Phino-Larynfflloffl Head and Neck 6urgery Volume 9 No. 1, January 2007 Blindness: a curable complication of acute sinusitis Abdulrahman ffagr MBBS. FRCS (C) Abstract We report a case
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(Saudi Journal of Oto-Phino-Larynfflloffl Head and Neck 6urgery Volume 9 No. 1, January 2007 Blindness: a curable complication of acute sinusitis Abdulrahman ffagr MBBS. FRCS (C) Abstract We report a case of a 9 year-old child, daughter of a drug addict, who presented with left nasal discharge and found to have an absent light reflex and a frozen eye. CT Scan of the sinuses showed bilateral pan-sinusitis, with extension of infection in the left side from the ethmoid to orbit forming a sub-periosteal abscess with optic nerve compression as well as bilateral frontal epidural empyema. She underwent sinus and brain surgery to drain the pus with eventful post-operative course. Eventually, her vision recovered completely. Key words: blindness, sinusitis, sub-periosteal abscess,proptois. Introduction Orbital infection has long been associated with sinusitis, and is common in both children and adolescents (1). Orbital complaints may be the first sign of sinus infection in children (2). It is estimated that approximately 70% of all cases of acute orbital inflammation is due to sinusitis (3). Symptoms may range from visual dysfunction (diplopia, blurriness, scotomas) to marked proptosis, eyelid edema or cellulitis, chemosis, and severe restriction of extraocular movements. Headaches, orbital pain, facial pain, and pain in the upper jaw are also common complaints. Acute sinusitis is typically a mild disease in children, often resolving without any antibiotic treatment (4). Yet about 1-2% of all paranasal sinus infections lead to orbital or intracranial complications (5). Extraocular involvement in acute sinusitis includes osteitis, cavernous sinus thrombosis, meningitis and subdural, epidural or cerebral abscess formation (2). In the pre-antibiotic era, 17% of patients with orbital cellulitis died of meningitis (1). Children with recognized complication of acute sinusitis must be hospitalized, whatever the severity, with treatment initialized immediately. Which patients will regain their vision after orbital cellulitis secondary to sinusitis and how this may be determined is not yet known. Case reports have mostly documented irreversible loss of vision, yet there are isolated reports of reversal of blindness. Today orbital infections are typically defined in relation to the orbital septum, which is the anterior extension of the orbital periosteum (6). Inflammation anterior to the septum, or preseptal cellulitis. is common in young children, in whom it is usually associated with Hemophilus influenzae septicemia. Nevertheless, penetrating trauma or secondary infection of skin lesions are also common etiologies of periorbital cellulitis. This category includes inflammatory edema, which occurs when infection is still confined to the sinuses. Edema and tenderness are noted adjacent to the affected sinuses (2). Lid edema with no signs of gaze restriction nor proptosis is generally characteristic of preseptal cellulitis. The most widely accepted staging of orbital involvement in sinusitis was proposed by Chandler(7) in 1970: inflammatory orbital edema and orbital cellulitis, followed by subperiosteal abscess, orbital abscess, and cavernous sinus thrombosis in increasing severity of symptoms and prognosis. We report a case of curable complication of acute sinusitis. Case History We present a 9 year-old, daughter of a drug addict, who was in her usual state of health until 6 days prior to admission when mild erythema of both upper & lower left eyelids were noted. The child claimed she had been hit on the face by a classmate. Nausea, vomiting and fever, though intermittent, began the next day. One day later, pain & non-erythematous swelling of the left eye was noted, worsening progressively. On the day before admission, the child had been vomiting severely, but there was no diarrhea. The only medication she had taken was paracetamol. The social situation was such that the child had apparently been taken to a clinic twice, as per her primary caregiver's instructions, once 3 days Assistant Professor and Consultant Department of Otolaryngology King Abdulaziz University Hospital - Riyadh, Saudi Arabia. 30 Hagr; Blindness after acute sinusitis before presentation, where the family was told not to worry and sent home, and the second instance, on the day before admission, when they were referred to the hospital and the family delayed seeking further medical attention. In the emergency room, the child was drowsy, lethargic and disoriented, with temperature 38.6 C. Her pulse was lll\minute, and blood pressure at 136/79. Poor hygiene and lack of cleanliness were commented upon, as was the child's diminutiveness. The child complained of headache, severe left eye pain and diplopia. Discharge was noted from the left nostril. Left light reflex was absent, but there was no afferent pupillary defect noted. Extra-ocular movement was limited on the left: the eye was found to be more or less fixed, with only minimal movement vertically. Severe massive proptosis, with eye edema and conjunctival chemosis led to the diagnosis of left orbital cellulites vs. abscess with possibility of intracranial involvement. Complete septic workup was done including eye and nose swabs as well as blood cultures. Brain and sinus CT showed bilateral pansinusitis, with extension of infection in the left side from ethmoid to orbit forming a subperiosteal abscess with optic nerve compression (Figure 1). Left eye proptosis with evidence of preorbital and retro-ocular cellulites was noted. A bilateral frontal epidural empyema was noted (Figure 2), with air pocket on the right side, along with a subdural fluid collection with mass effect along the anterior left side of the interhemispheric fissure. The patient was immediately begun on IV Ceftriaxone, Metronidazole, and Penicillin G. An emergency bilateral endoscopic sinus surgery was peformed, with left orbital decompression (drainage of subperiosteal orbital abscess). Thick yellow pus was seen flowing from left the orbit through the lamina papyracea, the flow of which increased with orbital massage. Endoscopic sinus surgery was followed immediately by bicoronal craniotomy and drainage of the frontal subdural/epidural abscesses. A large amount of pus was drained when periosteum was dissected off left orbital roof. Subsequently a large bilateral epidural and left frontal purulent subdural collections were observed. Thick yellow fibrinous pus was seen upon opening the dura. Parenchymatous invasion was observed, with subpial and intraparenchymal superficial fluid collection, as well as a large parafalcial pus collection on left side. Multiple biopsies were taken to exclude rhabdomyosarcoma. Pathological examination showed only signs of acute and chronic inflammation. Preoperatively Eye and nose swabs cultures grew Corynebacterium species. Streptococcus viridans, and Moraxella catarrhalis while blood cultures proved to be negative. Intra-operative swabs grew gram positive cocci and chains were cultured from intracranial pus, with Streptococcus Milleri being most abundant, along with Streptococcus Constcllatus while cultures of left infected nasal mucosa samples grew Corynebacterium and Streptococcus abiotrophia, which led to suspicion of nutritional deficiency, as well as many anaerobic Gram negative rods. For the first 3 days, the child was somnolent, and extremely irritable when awakened. She was febrile (38 C), with the temperature peaking by 2nd postoperative day. Periorbital swelling seemed to gradually decrease, but erythema and proptosis remained. There was conjectival prolapse in the left eye, but no corneal scarring. Pupils were equal and reactive, and no afferent pupillary defect was noted. Visual acuity was evaluated with difficulty, and estimated to be at least 20/800 in the left eye, with some perseveration noted. After a partial complex seizure, the patient was put on Phenytoin load, with no complications. EEG was abnormal over both hemispheres, but not overtly epileptiform. The patient was noted to be moaning, with extensor posturing of the left extremities. Brain CT scan at this point showed some refilling of the subdural abscesses over the left frontal lobe convexity. A left frontoparietal epidural abscess, with slightly increased peripheral enhancement, was creating a rightward midline shift. There was expansion of left-sided parafalcine collection. Repeated craniotomy and evacuation of the left subdural parafalcial empyemae was performed, revealing a large (15-20cc) collection of pus. The next day, the child had a 30-minute partial complex seizure, evolving into status epilepticus, and was transferred to intensive care. Tonic-clonic movements of right arm and both legs were noted. The patient was treated with multiple Ativan, Phenytoin, and Phenobarbital. Her Glasgow Coma Score (GCS) progressed from 5 that day to 15 two days later. The patient's course in the 1CU included many desaturation episodes, an episode of Syndrome of Inappropriate Antidiuretic Hormone Secretion (SI- ADH), and intermittent moderately severe vomiting. The child had initially been found to be angry, aggressive & seemed depressed. But her mood slowly improved as did her vision. On the 10th day post-admission, the patient was stable enough to be transferred to the wards. Anemia and chronic malnutrition, with poor post-operative intake, were 31 csaudi Journal ol Ot.o-Phino-Laryi\g,olog)' Mead and Xcck (Surgery Vo\ume9 No.\, January 2007 noted and she was begun on a five-day course of total parenteral nutrition (TPN). Work-up for immune compromise was negative. Physiotherapists briefly followed the child for weakness and loss of balance during ambulation. Two weeks post-operatively, brain CTscan revealed a residual subdural hypodense collection at the left frontoparietal and parafalcine level. Left ethmoid was seen to be normal. Visual acuity was 20/20 on the right, 20/200 on the left. One week later, acuity on the right side was, 20/20 and 20/25 on the left, with persistent left proptosis and normal fundi. No afferent pupillary defects, diplopia, visual field defects, or corneal abrasions were detected. Extraocular movements were full. Five weeks after the first presentation, CT scan of the brain and para-nasal sinuses showed no signs of infection. One week later, the child was sent home on seizure medications after ending her course of antibiotics. Vision tests showed no change. and has been \den\\fted as a pathway \v\fec\.\on. Anterior and posterior ethmoidal foramina, which communicate with the orbit, are another reason why orbital cellulitis commonly arises from infected ethmoid cells. Generally-speaking, it is recognized that both ethmoid and maxillary sinuses, which are present from birth, are of greater clinical importance in the pediatric population than the frontal and sphenoid sinuses, which only develop late in childhood (2). Figure 1 : CT scan showing a periosteal abscess and optic nerve compression Discussion Postseptal orbital cellulitis can present with ophthalmoplegia and loss of vision. Local contiguous spread of infection is the most common cause of postseptal cellulites. Orbital cellulitis presents when bacteria infiltrate the orbital fat. However, subperiosteal inflammation leading to abscess is the most common manifestation of postseptal cellulitis, either via dehiscent lamina papyracea or from septic thrombophlebitis. Because the periorbital tissues are so loosely adherent to the underlying bone, they are easily elevated by purulent collections and form subperiosteal abscesses. Intraorbital suppuration may then ensue. In any case, extraocular motion is progressively impaired, leading to eventual immobility of the globe. Proptosis is severe and blindness may be an unfortunate consequence. Cavernous sinus thrombosis, the most severe form of postseptal cellulitis, results from extension of infection via the valveless veins connecting the orbit with the cavernous sinus. Clinical signs include bilateral ophthalmoplegia and rapidly progressing visual loss. Signs of meningitis and fluctuating behaviour may also be present. Orbital cellulitis is a result of direct extension of infection from paranasal sinusitis through neurovascular foramina, congenital or acquired bony openings, and valveless venous conduits (4). The paranasal sinuses, once fully developed, surround over half the circumference of the orbit, separated from it only by thin bone. The lamina papyracea, the aptly named wafer thin partition between ethmoid sinus and orbit, commonly exhibits congenital dehiscences (8) Figure 2 : Bilateral frontal epidural empyema withair pocket and subdural fluid collection In Hawkins and Clark's series 1977 (2), the affected children followed abi-modal distribution, with most of the patients being either under 4 years of age, or between years. Most were male. In every instance, more than one sinus was involved. There are conflicting reports as to seasonal variation (9). The most commonly reported pathogens involved in the pediatric group include, in varying order. Staphylococcus aureus, Streptococcus pneumoniae, and Hemophilus influenzae (2). In one study, organisms involved were found to be atypical of acute sinusitis, such as Staphylococcus aureus and Staphylococcus epidermidis (5). In adults, both aerobes and anaerobes may be implicated (3). This occurs in 4% of all acute orbits, according to one study, while purely anaerobic isolates occur in 9% (1). In Fenton's se- 32 Hagr; Blindness after acute sinusitis ries, 10 of 16 patients with sinogenic brain abscess, most of which were young males, were found to have Streptococcus Milleri. The main concern in choosing appropriate treatment lies with complications of blindness. It is estimated that 10% of sinusitis cases with orbital involvement will be associated with temporary vision loss resolving within 2-6 weeks (10), with or without intensive treatment (11). In Singh's study 1995(9), intravenous antibiotics and surgical drainage for anything but mild cases of periorbital cellulitis was sufficient to restore normal vision to the whole cohort within 1 month (90% within 10 days). Severe irreversible visual loss has been documented with orbital cellulitis and subperiosteal abscesses since 1893 (6). Before the advent of antibiotics, up to 20% of patients with postseptal cellulitis became blind. Even today, permanent visual loss secondary to orbital inflammation is not uncommon, and remains quite preventable (6). Current reported incidence of irreversible blindness ranges from 3-15% (5,6,8,12). Etiological factors include vascular compromise of the retina or the optic nerve, inflammatory optic neuropathy as a reaction to nearby infection, and optic neuropathy secondary to compression from edema or bowing of ethmoidal air cells (8,6). Septic vasculitis and increased intraorbital pressure have also been implicated. Pressure ischemia is common with subperiosteal abscess, which can arise suddenly, producing acute changes in intraocular pressure. Slavin suggests that acute total blindness may be more vascular in causation, whereas subtotal visual losses that are frequently reversible may signal infiltrative or compressive optic neuropathy. After all, the retina and optic nerve can tolerate ischemia for only a matter hours (4). While timely relief of intraocular pressure may restore vision, retinal damage may be too far along to allow for recovery. As Postma, Chule and Nunzek note 1995(11), irreversible injury to the optic nerve may occur before gross intraorbital pathology develops. It is therefore in the patient's best interest to treat and to treat early. Any orbital involvement signals sinusitis severe enough to warrant aggressive treatment, not only to reverse blindness in the affected eye, but also to prevent extension of disease into the other. Early treatment of periorbital cellulitis is required to prevent disease progression to blindness, intracranial complications, and even death (3). Aggressive treatment and emergent surgical drainage usually becomes indicated when extraocular movements are compromised and proptosis appears. As Hawkins and Clark 1977 (2) point out, visual acuity is difficult to assess in a sick child with swollen eyes. Furthermore, treatment should be instituted in periorbital cellulitis long before visual acuity becomes a problem, at which time the loss is invariably irreversible. The same caveat applies for pupillary reactivity. Progression of visual and orbital symptoms is always an indication for aggressive therapy, as is poor initial presentation. Criteria for immediate drainage are controversial. Traditionally, surgical options were elected if no improvement was seen with intravenous antibiotic therapy (1,3,8). Among Jabor and 1997 Patt's 1991 (5,6)suggested indications for surgery are: proven intracranial involvement, severe decrease in visual acuity initially or severe initial orbital compromise with ipsilateral sinusitis, near-total ophthalmoplegia, worsening visual acuity while on medical treatment, worsening ocular examination while on medical treatment, and failure to show clinical improvement after 48 hours of medical treatment. Para-nasal CT scanning in itself may produce delays in diagnosis and treatment. If films are incorrectly ordered or inadequate for proper sinus assessment by otolaryngology consultants, time is lost as further views are taken (13). In Patt's report (6) 10 patients with postseptal orbital abscess not seen on CT, permanent unilateral blindness was the end result in 4 cases, seemingly attributable to missed diagnoses on CT. In El-Silimy's series (1), 2 of 4 subperiosteal abscess were not detected on CT scan, but no patient suffered permanent visual complications. Age may be another consideration when deciding when and how to treat postseptal abscesses. It has been shown that most children under the age of 9 would respond to medical management alone, while those over 15 would definitively require surgical drainage of a subperiosteal abscess (5). It is thought that the difference in response may be due to agerelated anatomical considerations. Even then, these measures may often be too late. Patt et al (6) reported a case of a 15-year-old boy with a 'massively' swollen left eye that precluded visual acuity testing. Proptosis, chemosis, severe gaze restriction, and light-perception-only visual acuity that later progressed to complete blindness.. No improvement was seen after 48 hours of intravenous antibiotic treatment, when an afferent pupillary defect was noted. An external ethmoidectomy with drainage of subperiosteal abscess at this point failed to restore vision. El-Sayed and Al-Muhaimed 1993(14) reported two cases. In the first case, unilateral periorbital swelling over one day and acutely decreasing vision was 33 (Saudi Journal of OtaRhinaLaryngology Head and Neck (Surgery Volume 9 No. 1, January 2007 seen. Vision was limited to hand movement.the child regained vision completely with medical treatment. Their second case has unilateral amaurosis. Surgical exploration and drainage was done and patient did well by the 5th day. Banfield and Daya's 1996(15) reported patient with right eye proptosis and diminished visual acuity. Patient underwent partial ethmoidectomy with drainage of an orbital abscess. Complete return of vision occurred within 48 hours. Jarrett and Gutman (16) described a case with unilateral visual loss to hand movements only. Visual acuity recovered completely within 48 hours after intravenous therapy was instituted. Postma, Chole and Nunzak (11) reported acute sphenoiditis with acute near-total loss of vi- sion bilaterally, despite antibiotic coverage. Within 48 hours of aggressive surgical and medical treatment, the patient's vision returned to 20/20. Unfortunately, acute sinusitis cases with orbital involvement are commonly encountered late in their course by otolaryngologists. Timely diagnosis, with surgical drain
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