A DIABETIC PATIENT WITH SUDDEN PERIORBITAL SWELLING AND PROPTOSIS
Dr Mohammad Yousuf Rathor[a] and Professor Humairah Samad Cheung[b]
CASE PRESENTATION
A 45-year old Malay teacher in a local school, a known diabetic, was admitted to our hospital on October 2001 with a history of sudden onset of left periorbital swelling and eye protrusion developing over two days. In addition, he complained of low grade fever and intermittent headache for the past 3 weeks. There was no history of preceding trauma or fall. There was no history of nausea, vomiting or seizures. No infection of the paranasal sinuses, middle ear, facial skin or oropharynx. His diabetic control was found to be very erratic due to poor compliance to his medications. His past medical and surgical history were unremarkable
He was febrile at 39.5ºC on the day of admission, with a regular pulse rate of 105 beats/min, and a BP of 120/80. His body mass index was calculated at 28. Clinical examination showed redness in the left eye; conjunctival congestion and chemosis. There was obvious left eye proptosis with periorbital swelling and partial ptosis of the left upper eye lid (Figure 1). External ocular movements were restricted in all directions. No bruit or pulsation was present over the eyeballs. There was no corneal ulceration. The pupil size was equal bilaterally and both pupils were reactive to light. Fundoscopic examination was normal. His general systemic review was unremarkable.
Initial investigations revealed the following results: Capillary blood glucose was 16.3 mmol/L, white blood cell count was 18.3x109/l, hemoglobin of 16.7 g/dl, platelets 262x109/l, serum sodium 129 mmol/L, potassium 6.0 mmol/L, blood urea 20 mmol/LS, creatinine 190 micro mol/L, ESR 89 mm/1hr, CRP 96.0 mg/L, HB A1C was 11%. His CXR was normal.
A provisional diagnosis of orbital abscess was made. A bacterial aetiology was considered likely, taking into account the white cell response. With the background of fever for three weeks and an uncontrolled diabetic state, treatment for probable fungal infection was also commenced. Intravenous Ceftazidime 2gm stat and 2gm 8-hourly, IV Metronidazole 500mg 8-hourly, and IV Fluconazole 400mg daily were commenced.
Further investigation included a CT scan of the brain and orbits shortly after admission. This showed proptosis of the left eyeball associated with left lachrymal gland enlargement (Figure 2a & b) and left superior rectus thickening. There was no evidence of any pus collection. Blood cultures grew Pseudomonas pseudomallei sensitive to Ceftazidime and co-amoxiclav amongst others.
Figure 1: Left periorbital swelling and chemosis at patient’s initial presentation.
Figure 2a: CT scan following admission, showing proptosis of the left eyeball
Figure 2b: There is associated enlargement of the left lachrymal gland.
On the 4th hospital day he developed an abscess on the left side of his fore head (Figure 3), which was drained. Pus sent for culture and sensitivity reaffirmed the isolation of Pseudomonas pseudomallei. The patient’s antibiotic regime was changed to IV ciprofloxacin and IV Ceftazidime, to both of which the organism was sensitive.
Figure 3: Close-up view of the patient’s left eye shortly after pus was drained from the abscess.
A follow up CT scan of the brain and neck a week later (Figures 4, 5, 6) showed fluid collections in the left frontal and ethmoid sinuses (Figure 4), and left orbit (Figure 5) extending intra-cranially to the subdural spaces (Figure 6) over the frontal convexity and along the falx. There was also marked swelling of his left parotid gland (Figure 7) and left side of his face. The swelling increased further and he remained pyrexial on the seventh day despite dual antibiotic therapy. His vital signs however remained stable. On the eighth day he was referred to the Neurosurgical Institute in the capital Kuala Lumpur for neurosurgical intervention. He developed septicaemic shock a day later and was ventilated for four days before he succumbed to widespread sepsis on day 14.
Figure 4: Follow-up axial CT shows fluid in the left frontal and ethmoid sinuses
with swelling of the Lt lachrymal gland (arrow) and the left temporalis muscle.
Figure 5: Coronal CT with the patient lying prone shows fluid
in the left frontal sinus, and in the left orbit and upper eyelid
(arrows), with depression of the left eyeball.
Figure 6: Fluid is present in the subdural spaces on either side
of the falx, suggesting the presence of subdural empyema.
Figure 7: Axial contrast-enhanced CT of the neck showed asymmetrical
swelling of the left parotid gland (asterisk) and the left masseter muscle.
DISCUSSION
Melioidosis is the name given to an infection caused by Burkholderia (formerly Pseudomonas) pseudomallei . Besides humans many animal species are susceptible to the infection, and these animals include sheep, goats, horses, pigs, dogs and cats.
Meliodosis is endemic in South-East Asian countries such as Malaysia, Thailand, Vietnam, and Burma and in northern Australia, Africa, and central and South America[1]. Sporadic cases occur in temperate zones; however, almost all cases are imported from endemic countries due to globalisation and increased world travel[2]. It is a common cause of septicemia in rural Thailand, especially in rice farmers during the rainy season6.
The bacterium is an environmental saprophyte found in soil, ponds and shallow water (e.g. rice paddy fields). Infection is usually acquired through inoculation, occasionally aspiration or inhalation. Person-to person spread is very rare. Two cases of sexual transmission have been reported; in both the source patients had chronic prostatitis. Infection resulting from laboratory exposure has also been documented. Disease is commonest in males aged 40-60 years. The incubation period is variable and may be as short as 2-3 days, or as long as several months to decades. The likelihood of disease developing depends on a balance between the host immune system, the inoculum and presumably the intrinsic virulence of the infecting strain. Risk Factors in Melioidosis include an immunocompromised state as is shown by the preponderance of the infection amongst patients with diabetes, chronic renal and lung diseases, HIV infection and in alcoholics[3], [4]. Travel to the tropics is also an important predisposing factor as sporadic cases have been reported in many temperate areas from travelers who have been to these regions[5].
The clinical spectrum of meliodosis ranges from an acute fulminant septicemia, a subacute illness, chronic infection or a sub-clinical disease[6]. Overall, 60% of cases have a community-acquired sepsis syndrome with a high mortality rate[7], although some have sub-acute presentation. The remaining 40% have localized infections, in any organ, but particularly the lung, liver and spleen, prostate, subcutaneous tissues, bones and joints and the parotid in children. Lymphadenopathy is not uncommon, and histology can be granulomatous. Chronic foci often become granulomatous. Any organ may be affected, hence called “the great imitator”. The lungs are most commonly involved and it may cause an acute fulminant pneumonia, an indolent cavitatory disease with lung abscesses or empyema[8]. A case series of 50 patients from Malaysia showed a preponderance of lung involvement and skin and soft tissue abscesses9. Chest radiographic features may resemble pulmonary tuberculosis. Metastatic abscesses are common at any site.
Our patient demonstrates some of the recognized features of meliodosis, such as septicaemia, and involvement of the subcutaneous tissues of the left face and neck. Lung involvement was not a prominent feature in this case. The CT scans documented the presence of periorbital cellulitis and abscesses, and the presence of infection in the subdural spaces. Despite treatment with appropriate antibiotics to which the organism is sensitive, the infection evolved rapidly with a fatal outcome. His uncontrolled diabetic status was a major contributory factor.
The diagnosis of melioidosis must be considered in any patient presenting with sepsis, cellulites and abscesses, especially if they are diabetic. Cultures from sterile sites (e.g. blood, pus, urine) and in selective media increase the isolation rate of the organism. Physicians need to alert microbiologists as to the likelihood of melioidosis. Numerous serological tests for both IgG and IgM antibodies are available, but may have poor specificity and sensitivity in endemic areas, and may be more useful in visitors from non-endemic countries. Other investigations such as Polymerase Chain Reaction (PCR) and antigen detection systems are not yet in widespread use.
The key to successful management is early diagnosis and prompt treatment. Early appropriate antibiotic and supportive treatment reduces the mortality risk[9]. Delay in treatment is associated with a very high mortality rate exceeding 80%[10]. B. pseudomallei have a characteristic antibiogram[11]. Treatment requires simultaneous multiple antibiotics because single-agent therapy is often ineffective and resistance to antibiotics may occur during the course of treatment[12]. Antibiotic treatment can be divided into the acute phase of treatment (usually about two-three weeks) and the maintenance phase, to complete 20 weeks of total treatment. Ceftazidime, Imipenem or Meropenem are currently the treatments of choice during the acute phase. Medications for the maintenance phase include appropriate doses of amoxicillin, clavulinic acid, Trimethoptim-sulphamethoxazole, Doxycicyline and, Chloramphenicol. Supportive treatment, including drainage of abscesses, is important. Relapse is common especially in immunocompromised hosts.
CONCLUSION
Melioidosis is under-diagnosed and under-reported, and doctors practicing in endemic areas should include it in the differential diagnoses of infections affecting uncontrolled diabetic patients. This case report is an example of the infection in such a clinical situation.
REFERENCES
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