British Journal of Ophthalmology, 1988, 72, 428-431

A fatal case of necrotising fasciitis of the eyelidR WALTERS

From Southampton Eye Hospital, Wilton A venue, Southampton S09 4XW

SUMMARY A fatal case of necrotising fasciitis in a 35-year-old man is described and the differentialdiagnosis and management discussed.

Necrotising fasciitis is a potentially fatal skininfection which is being increasingly recognised as anunderdiagnosed condition. It requires prompt diag-nosis, investigation, and treatment. Early surgicaldebridement is, in combination with suitable intra-venous antibiotics, the mainstay of treatment.

Case report

In December 1985 a previously fit 35-year-old factorymanager was referred by his general practitioner tothe Casualty Department of the Southampton EyeHospital with a 12-hour history of increasing rednessand swelling of his right upper lid. He said that twodays previously he had been poked in the same eye byhis daughter (who had been playing with her guinea-pig) but that he had been symptomless until the dayof presentation.On examination he was febrile (380C) and clearly

unwell. His right eyelid was markedly erythematous,swollen, and tender, and there was a copiouspurulent discharge from the eye. The oedema anderythema extended to his temple as far as the hairlineand down the right side of his face to the mouth.Because of the considerable swelling it was difficult toexamine his eyes, but there was no proptosis and theocular movements were full. The visual acuity of theright eye was reduced (6/24) and that of the left wasgood (6/4). The conjunctiva was very oedematousbut there was no obvious injury, and the anteriorsegment was otherwise unremarkable. The pupilreactions were normal and fundal examinationrevealed no abnormality. The left eye was normal.A provisional diagnosis was made of preseptalcellulitis with septicaemia, and he was admitted tohospital. Swabs from the infected eye were sent forurgent microscopy and culture and blood cultures

Correspondence to Mr R Walters, FRCS.

were taken. The Gram stain revealed Gram-positivecocci. He was then treated with intravenouscefotaxime and gentamicin and topical chlor-amphenicol and gentamicin drops. Because of thepoor visual acuity of the right eye it was thought thatan orbital cellulitis could not be excluded despite thenormal eye movements and absence of proptosis. Hewas therefore transferred to the General Hospitalunder the care of an ear, nose, and throat consultantin order to exclude underlying sinus disease and anassociated abscess.

Skull x-rays (including sinus views) revealed noabnormality and he was therefore continued on hismedical treatment (with the addition of intravenousmetronidazole), the presumed diagnosis beingpreseptal cellulitis. Over the ensuing 12 hours hisgeneral condition improved. His temperature felland his appetite returned. However, the swelling anderythema of the lid and face remained unchanged.Twenty-one hours after his admission he suffered a

Fig. 1 Close up view ofthe upper lid, showing its violet hueand black areas offocal necrosis.

428

on April 10, 2020 by guest. P

rotected by copyright.http://bjo.bm

j.com/

Br J O

phthalmol: first published as 10.1136/bjo.72.6.428 on 1 June 1988. D

ownloaded from

429A fatal case ofnecrotisingfasciitis ofthe eyelid

Fig. 2 Close up view ofdebrided area around the eye. Thelid suture through the upperlid margin enabled the lid to beopenedfor the application ofdrops.

cardiorespiratory arrest and clearly incurred hypoxicbrain damage. He was resuscitated and transferred tothe Intensive Care Unit. The cause of his arrest atthat time remained a mystery in spite of his beingintensively investigated. Chest x-rays, CT brain scan,and lumbar puncture were normal. Specifically, therewas no evidence of intracerebral or intraorbitaldisease. His temperature rose again, and his whitecell count was 22x 109/l (with a predominantneutrophilia).By this stage the lid pathology was beginning to

reveal its true nature. The lids took on a violet hueand bullae developed. Later, dark areas of focalnecrosis appeared (Fig. 1). These features suggestednecrotising fasciitis as the true diagnosis as opposedto the initial diagnosis of cellulitis.Although the original swab grew Streptococcus

viridans and Staphylococcus albus, the original bloodculture and a subsequent lid swab yielded ,3-haemolytic streptococcus (Streptococcus pyogenes).All the organisms were sensitive to cefotaxime (thispresumably accounted for the resolution of the initialbacteraemia). Benzylpenicillin was addedspecifically to counter the streptococci. Anaerobicorganisms were not isolated. Serological testsrevealed a positive anti-DNAse B titre but a negativeanti-streptolysin A titre.As soon as the diagnosis of necrotising fasciitis

was made the patient was taken to theatre andextensive areas of his face, lids, and scalp weresurgically debrided (Fig. 2). Thereafter the patient'sskin condition improved, but the hypoxic braindamage he had incurred during cardiac arrest wassufficient to prevent a recovery of consciousness, andhe died 11 days after admission.The debrided lid and scalp tissue was sent for

Fig. 3 Histological section through the excised upper lidtissue, showing the ulcerated skin and acute inflammationextending through the dermis. Haematoxylin and eosin.

histological examination and showed extensive acuteinflammatory changes. In the upper lid the epidermiswas ulcerated and acute inflammatory changesextended throughout the epidermis and dermis, withthrombus formation in some blood vessels. (Fig. 3).Organisms were not demonstrated despite the use ofspecial stains.

Discussion

Necrotising fasciitis has been recognised as adangerous and often fatal condition since theAmerican Civil War.' However, it was firstaccurately described as a distinct clinical entity byMeleney in 1924.23 Among its synonyms are strepto-coccal gangrene, hospital gangrene, gangrenouserysipelas, and necrotising erysipelas. Fournier'sgangrene is probably also the same condition.4Although originally reported as a relatively rarecondition, it is being increasingly recognised that ithas probably been underdiagnosed56 in the past and

v 4*VR.V.:. .0.1 .1

Ir

on April 10, 2020 by guest. P

rotected by copyright.http://bjo.bm

j.com/

Br J O

phthalmol: first published as 10.1136/bjo.72.6.428 on 1 June 1988. D

ownloaded from

R Walters

is more common than the numbers of reported caseswould at first suggest.

Necrotising fasciitis occurs at all ages, with no

statistical difference in race or sex.' It is an infectionof the skin and subdermal tissue which is mostfrequently caused by Streptococcus pyogenes" "'

(group A P-haemolytic streptococcus (BHS)),though Staphylococcus aureus57X and variousGram-negative rods" 1"-" have also been isolated.The appearance of necrotising fasciitis may be

difficult to diagnose in the early stages. There isoften, but not always, a history of injury, usuallytrivial in nature. In its early stages it is characterisedby the acute onset of a painful erythematous rashwith associated oedema, which is indistinguishablefrom cellulitis or early erysipelas. The patientis usually profoundly unwell, with a fever andtachycardia. Within 24-48 hours the tell-tale patho-gnomonic features develop.239 There is a deepeningred/violaceous discolouration of the skin with blister-ing (these bullae can again be confused witherysipelas). Subsequently, black patches of necrosisappear. The affected area may be numb or anaes-

thetic. The legs are the commonest site for theinfection,9" though it can appear anywhere on thebody. The area around the eye is not an infrequentsite.'""6. After four to five days frank cutaneousgangrene develops, which if untreated tends toseparate by suppuration by the eighth to tenthday. Lymphangitis and lymphadenopathy are

unusual.9 "'The diagnosis is made principally on the clinical

features. However, some investigations are helpful.The white cell count is usually markedly raised (witha neutrophilia). Blood cultures and swabs from theaffected site should be taken before antibiotictherapy is instigated. Evidence of streptococcalinfection is provided by a raised anti-DNAse B titre

Fig. 4 Histopathology ofnecrotisingfasciitis demonstratingthrombosis in the blood vessels, in the acutely inflameddermis. Hand E.

in most cases and the anti-hyaluronidase titre mayalso be raised.5 " 1 The ASO titre, however, is notusually raised in skin infections'8 and therefore isnot helpful in making the diagnosis of necrotisingfasciitis.The essential histological feature of the disease is

necrosis of the deep fascia and spread of the infectionalong the fascial planes, with secondary gangrene ofthe overying skin due to thrombosis of the bloodvessels in the subepidermal and subcutaneoustissues'5" (Fig. 4). A massive infiltrate of polymor-phonuclear leucocytes is present. Antibiotics areunable to reach the necrotic areas, and the bacteriaare able to multiply freely. The bacterial toxins canthus have a widespread effect even after the initialsepticaemia has been treated. The tissue damageis probably caused by bacterial necrotoxins andthus can also be responsible for some of thepathology remote from the site, which may includeglomerulonephritis (resulting in renal failure) andendocarditis.'5

Initial treatment consists of prompt and intensiveintravenous antibiotics. Although the most likelycausative pathogen is Str. pyogenes, it is wise to coverother possible organisms such as Staph. aureus andcoliforms. A cephalosporin such as cefuroxime orcefotaxime in high doses is suitable. Penicillin Gshould be added if streptococci are isolated. Despiteadequate antibiotic treatment, patients may stilldie.5 Therefore as soon as the diagnosis has beenestablished clinically the affected area should besurgically excised.23 10 14 28.23 This is necessary in spiteof the fact that the patient may symptomicallyimprove after treatment with antibiotics.There is a considerable mortality from necrotising

fasciitis (8-50% ).2 8 11 24 2 Although associated ill-nesses, particularly diabetes mellitis, contribute tothis poor prognosis, the principal factors are the timetaken to make the diagnosis and delay in surgicalintervention.' "28 The decision to debride theinfected area is especially difficult when the face isinvolved because the cosmetic implications are con-siderable. However, it must be done, and quickly, ifincreased morbidity and even death are to beavoided.

In summary, necrotising fasciitis is a potentiallylethal condition in which speedy investigation, diag-nosis, intensive antibiotic treatment, and earlysurgical debridement is the correct course ofmanagement.

I would like to thank Mr Andrew Elkington for his help and adviceand for his kindness in allowing me to present this patient, who wasunder his care. I am grateful also to Dr Barbara Leppard for heradvice, to Professor Weller for his help with the histopathologyslides, and to Miss Sara Hipwell for preparing this paper forsubmission.

430

on April 10, 2020 by guest. P

rotected by copyright.http://bjo.bm

j.com/

Br J O

phthalmol: first published as 10.1136/bjo.72.6.428 on 1 June 1988. D

ownloaded from

A fatal case ofnecrotisingfasciitis ofthe eyelid

References

I Investigations upon the Nature, Causes and Treatment ofHospital Gangrene as it Prevailed in the Confederate Armies1861-1865, New York, US Sanitary Commission, SurgicalMemoirs of the War of Rebellion, 1871. Quoted in Meleney FL.Treatise on surgical infections. New York: Oxford UniversityPress, 1948: 15.

2 Meleney FL. Hemolytic streptococcus gangrene. Arch Surg1924; 9: 317-63.

3 Meleney FL. Differential diagnosis between certain types ofinfectious gangrene of skin. Surg Gynecol Obstet 1933; 56:847-67.

4 Cooper DA, Joske RA. Acute streptococcal gangrene of theskin. Aust NZ J Surg 1954; 23: 268-72.

5 Leppard BJ, Seal DV. The value of bacteriology and serology inthe diagnosis of necrotising fasciitis. Br J Dermatol 1983; 109:37-44.

6 Collins RN, Nadel MS. Gangrene due to the haemolyticstreptococcus-a rare but treatable disease. N EnglJ Med 1965;272: 578-8().

7 Rea WJ, Wyrich WJ. Necrotising fasciitis. Ann Surg 1970; 172:957-64.

8 Wilson B. Necrotising fasciitis. Am Surg 1952; 18: 416-31.9 White WL. Haemolytic streptococcus gangrene. A report of

seven cases. Plast Reconstr Surg 1953; 11: 1- 14.10 Strasburg SM, Silver MS. Haemolytic streptococcus gangrene.

An uncommon but frequently fatal infection in the antibiotic era.

Am J Surg 1968; 115: 763-8.11 Crosthwait RW Jr, Crosthwait RW, Jordan GL. Necrotising

fasciitis. J Trauma 1964; 4: 148-57.12 Ledingham IMcA, Tehrani MA. Diagnosis, clinical course and

treatment of acute dermal gangrene. Br J Surg 1975; 62: 364-72.13 Fallahzadeh H, Altenbernd E, Mays E. Necrotizing fasciitis.Am Surg 1972; 40: 352-4.

14 Buchanan CS, Hascrick JR. Nccrotising fasciitis due to group Abeta-haemolytic streptococci. Arc/i Dermnatol 1970; 101: 664-8.

15 Scal D, Leppard B, Widdowson J, McGill J, Tormey P.Necrotising fascitis due to Streptococcus pyogenes. Br Med Jt980; 282: 1419-20.

16 Schott EG. Gangrene of the eyelids. Ind Med Surg 1966; 29:27-9.

17 Hammar H, Wang ERL. Erysipclas and necrotising fasciitis. BrJDermatol 1977; 96: 409-19.

18 Wannamaker LW. Differences between streptococcal infectionsof the throat and skin. N EnglJ Med 1970; 272: 78-85.

19 Kaplan EL, Wannamaker LW. Suppression of the anti-streptolysin 0 (ASO) response by lipids extracted from the skin:an explanation or the feeble ASO response following strepto-coccal pyoderma. Cl/in Res 1973; 21: 881-6.

2t) McCafferty EL Jr, Lyons C. Suppurative fasciitis ias the essentialfeature of haemolytic streptococcus gangrene. With notes on

fasciotomy and early wound closure as the treatment of choice.Surgery 1948; 24: 438-42.

21 Meleney FL. Haemolytic streptococcus gangrene. Importance ofearly diagnosis and early operation. JAMA 1929; 92: 2(X)9-12.

22 Beathard GA, Guckiar JC. Necrotising fasciitis due to group Abeta-hemolytic streptococci. Arch Intern Med 1967; 120: 63-7.

23 Le Froch JL, Molavi A. Necrotising skin and sub-cutaneousinfections. J Antimicrob Chemnother 1982; 9 (suppl A): 183-92.

24 DeforeWW Jr, Mattok KL, Dang MH, Crawford R, Jordan GL.Necrotising fasciitis: a persistent surgical problem. J Am CollEmergency Physicians 1977; 6: 62-5.

25 Galosi AF, Herman WP. Fasciitis necroticans. Dtsch MedWochenschr 1979; 104: 1t)95-9.

26 Rea WJ, Wyrich WJ. Necrotising fasciitis. Ann Surg 1970; 172:957-64.

Accepted for publication 23 April 1987.

431

on April 10, 2020 by guest. P

rotected by copyright.http://bjo.bm

j.com/

Br J O

phthalmol: first published as 10.1136/bjo.72.6.428 on 1 June 1988. D

ownloaded from