Eyelid Necrosis and Periorbital Necrotizing Fasciitis Report of a Case and Review of the Literature JAN
w.
KRONISH, MD, WILLIAM M. McLEISH, MD
Abstract: Necrotizing fasciitis is an uncommon and severe soft tissue infection characterized by cutaneous gangrene, suppurative fasciitis, and vascular thrombosis. The disease is usually preceded by trauma in patients that have systemic problems, most commonly diabetes and alcoholism. Streptococcus pyogenes and Staphylococcus aureus are the most frequent bacterial etiologies; however, combinations of numerous facultative and anaerobic organisms have also been isolated. Involvement of the face and periocular region is rare. A case is presented here, as well as a review of the clinical features of 15 other patients previously described, in whom eyelid necrosis due to periorbital necrotizing fasciitis developed. Early surgical debridement and drainage of necrotic tissues and appropriate parenteral antibiotics are the mainstay of therapy. The mortality rate in patients with periorbital spread was 12.5%, with the prognosis known to be adversely affected by delay in diagnosis and treatment and/or extension of infection from the face to the neck. Reconstruction of the eyelids with skin grafts was necessary in most cases to avoid such complications as cicatricial lid retraction, lid malpositions, and lagophthalmos. Ophthalmology 1991; 98:92-98
Necrotizing fasciitis is a severe and potentially fatal soft tissue infection that rarely involves the head and neck. 1-3 Extensive necrosis of the superficial fascia with widespread undermining of surrounding tissues and secondary gangrene of the overlying skin characterize this disease. 2,4,5 Before the discovery that numerous facultative and anaerobic bacteria could lead to similar clinical features, infections with subcutaneous tissue necrosis had been referred to as streptococcal gangrene. This disease most commonly involves the extremities and trunk. The term necrotizing fasciitis is preferred as it provides an anatomic description of the planes of infection regardless of the bacterial etiology.5-7 Early recognition and prompt ag-
gressive medical and surgical intervention are critical for proper management of this serious condition. Most of the previously reported cases of eyelid necrosis caused by periorbital necrotizing fasciitis are presented in the general medicalliterature. 4 ,8-18 We present a patient in whom bilateral eyelid necrosis due to necrotizing fasciitis of the scalp and face developed that was successfully managed with proper wound care, antibiotics, reconstructive surgery, and adjunct hyperbaric oxygen therapy, This case and a review of the literature are provided so that ophthalmologists can identify this underdiagnosed condition and institute immediate therapy to minimize morbidity and mortality.
Originally received: June 18, 1990. Manuscript accepted: August 13, 1990.
CASE REPORT
From the Department of Ophthalmology, Bascom Palmer Eye Institute, University of Miami School of Medicine, Miami. The authors have no proprietary interest in any drug mentioned. Reprint requests to Jan W. Kronish, MD, Bascom Palmer Eye Institute, PO Box 016880, Miami, FL 33101.
92
A 26-year-old woman presented to the Jackson Memorial Hospital emergency room several hours after being struck over the left temple region with a metal pipe. She experienced no loss of consyiousness or visual disturbance. Results of an examination revealed a 4-cm full-thickness temporal scalp laceration, fluc-
KRONISH AND McLEISH
Fig I. Computed tomographic scan shows marked soft tissue swelling of the eyelids (arrows), temporal and frontal scalp regions.
tuant swelling of the forehead, tense edema and ecchymosis of the upper and lower eyelids, and generalized facial swelling. Skull radiographs failed to reveal any bony fractures or sinus opacification . A head computed tomography scan revealed marked bilateral soft tissue swelling of the temporal scalp, forehead, and periocular regions with no intracranial abnormalities (Fig I). Hematoma formation within the anterior scalp extending into the preseptal eyelid tissues was suspected. The scalp wound was cleansed and sutured closed. The patient was released and given a 7-day course of prophylactic oral oxacillin 500 mg every 6 hours. On the following day, she was referred to the Bascom Palmer Eye Institute for evaluation. The eyelids remained markedly swollen, while the forehead and temporal scalp appeared less distended than on the previous day. The superficial layers of epidermis of the pretarsal and preseptal areas of both upper lids and preseptal portion of the medial right lower lid had sloughed, leaving a gray-tan discoloration of the excoriated skin (Fig 2). Except for the inability to open her eyes due to the swelling, the patient's ocular examination was normal, including a visual acuity of 20/20 in both eyes, unrestricted motility, and quiet anterior segments. Application of polysporin ointment to the eyelids was initiated four times a day. Four days after the injury, the scalp, facial, and eyelid edema had markedly diminished. The previously eroded eyelid skin had begun to reepithelialize, and she could open her eyes spontaneously.
•
NECROTIZING FASCIITIS
Over the next few days, the patient noted the onset of progressive swelling and redness of both upper lids and gradually increasing purulent discharge. She did not seek further medical attention until 12 days after the initial injury when she was unable to open her eyelids. She denied having such constitutional symptoms as sweats, chills, or rigors, and was found to have a low-grade fever of 38°C. Results of physical examination showed the entire forehead and both brows were elevated and fluctuant, and purulent discharge could be expressed from the temporal scalp wound dehiscence. Necrosis of the preseptal and pretarsal skin and superficial layers of orbicularis muscle of the upper eyelids was noted, along with eschar formation and marked underlying purulent discharge (Fig 3). The lid margins were spared and the well-defined borders of the necrotic wounds were indurated. Her vision remained stable with no signs of orbital inflammation. Initial management included hospital admission for extensive surgical debridement of all the necrotic tissues of the eyelids and scalp wound (Fig 4). Inspection of the scalp wound revealed necrosis to the depth of the temporalis fascia, and a blunt hemostat could be passed with minimal resistance across the forehead in this plane. Aerobic and anaerobic cultures from these areas, as well as samples of blood and urine, were obtained. Gram's stain of the eyelid discharge revealed a few gram-positive cocci and many gram-negative rods. Administration of intravenous penicillin, methicillin, and gentamicin was begun immediately for suspected necrotizing fasciitis. Wound care consisted of alternating wet-to-dry dressing changes with dilute povidone-iodine every 2 hours and cleansing of the eyelids with hydrogen peroxide and irrigation into the scalp wound with gentamicin and penicillin solution. Results of laboratory examination were remarkable for a leukocyte count of 5400/cu mm, a hemoglobin count of8.6 g/dl, aWestegren sedimentation rate of 134 mm/hr, an RPR titer of 1:32, and a negative HIV-1 ELISA and Western blot. Repeat skull radiographs and orbital computed tomography scan failed to disclose any sinus disease or orbital abnormalities. Over the next 2 days, the patient's clinical condition gradually improved with reduction in purulent drainage and diminished edema from the upper lids, as well as slow resolution of the fluctuance in the forehead. Eyelid and scalp wound cultures were positive for Citrobacter diversus and Enterobacter aerogenes, both of which were sensitive to gentamicin. Results of all remaining cultures were negative. After a week of intravenous antibiotics and topical wound care, all signs of active infection had subsided. The necrotic areas in both upper lids had begun to spontaneously granulate with the early development of lid retraction and lagophthalmos. On the tenth hospital day, the patient underwent reconstruction of both upper lids, which involved the removal of granulation tissue and placement of partial-thickness skin grafts over the exposed pretarsal and preseptal orbicularis muscle. Pressure bandages were applied bilaterally after traction sutures were secured to maintain the lids on stretch. The left temporal wound edges, which had previously separated, were reapproximated. Hyperbaric oxygen therapy using 100% oxygen at two atmospheres absolute pressure for 2 hours daily was begun immediately after surgery and continued for 7 days. Parenteral gentamicin was given for 3 more days, at which time the patient began taking oral ciprofloxacin and received 2.4 million units of intramuscular benzathine penicillin for suspected primary syphilis. The pressure patches and traction sutures were removed 7 days after surgery, and the final result was a 100% take of the grafts. Moderate limitation of levator function and ptosis were present initially, but there was no lagophthalmos in either eye. At the time of discharge from the hospital, the patient was instructed to apply antibiotic ointment to her upper lids and
93
OPHTHALMOLOGY
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JANUARY 1991
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VOLUME 98
•
NUMBER 1
Fig 2. Top left. appearance of patient 36 hours after head trauma with diffuse swelling of the forehead and massive edema of both upper lids. Epidermal sloughing of the preseptal and pretarsai areas of the lids exposes the pale, avascular dermis with slightly erythematous borders. Fig 3. Second row left. Extensive gangrenous ulceration of both upper eyelids with underlying suppuration 12 days after injury. Fig 4. Third row left. debridement of the wounds consists of removal of eschar, purulent discharge, and residual necrotic tissues. Notice the well-vascularized underlying orbicularis muscle (arrow). Fig 5. Bottom left. appearance of patient with an acceptable cosmetic and functional result 3 months after eyelid reconstruction with partial-thickness skin grafts. Notice the moderate ptosis of both upper lids. Fig 6. Top right. complete eyelid closure is achieved with no associated lid margin malposition or cicatricial lid reo traction. Fig 7. Center right. diffuse edema involving th~ dermis and subcutaneoU! tissue with a predominantl) neutrophilic infiltrate (he· matoxylin-eosin; origina magnification, X60). Fig 8 Bottom right. focally enlarge< collection of neutrophils an( neutrophilic debris associate< with a secondary vasculiti: (hematoxylin-eosin; origina magnification, X400).
massage them regularly to soften the skin grafts. Three months later, she recovered most of the levator function with mild residual ptosis of both upper lids and maintained complete eyelid closure bilaterally (Figs 5 and 6).
DISCUSSION Necrotizing fasciitis was recognized as early as the American Civil War and referred to by Jones l9 as hospital
94
gangrene in which "the skin of an affected part melt~ away." Melenelo accurately described this infection ill 1924 based on 20 patients in which hemolytic strepto· coccus was cultured, leading him to coin the term "he· molytic streptococcus gangrene." Other names became synonymous with this condition including "gangrenom erysipelas," "necrotizing erysipelas," "acute infective gangrene," and "suppurative fasciitis." In 1952, Wilson~ introduced the currently preferred term of "necrotizin! fasciitis" to emphasize the constant feature of necrotic
KRONISH AND McLEISH
fascia with spread of the infection along fascial planes and the non specificity of the bacterial etiology. More recent bacteriologic studies have confirmed Wilson's findings that most cases can be attributed to streptococcal and staphylococcal organisms, although a mixture offacultative and anaerobic bacteria also can lead to this type of infection. 5,21 Necrotizing fasciitis can develop in patients of all ages and has no sex or race predilection. 5 Minor trauma, such as abrasions, lacerations, insect bites, and hypodermic needle injections, is the most common initiating factor. The onset of infection may also follow surgery or blunt injuries, and, occasionally, develop without apparent cause. I,5,6,IO Predisposing factors have been reported to include diabetes mellitus,I,2,6,ls,22 arteriosclerosis and peripheral vascular disease,I,l s alcoholism,I,2,ls malignancy,6.IS polymyositis,13,ls intravenous drug abuse, and the postpartum state. 6 Most reported cases involve the lower extremities, whereas the upper extremities, trunk, and genitalia also are frequently affected. 4,7 Necrotizing fasciitis of the head and neck is relatively uncommon and may have a worse prognosis.2,6 This fulminating infection usually develops within 2 to 4 days after the traumatic event and progresses with alarming speed. The skin is initially pale red, tensely swollen, and accompanied by pain as extensive necrosis spreads in the subcutaneous tissue planes. Within 1 to 2 days the pathognomonic features become apparent as the skin turns a dusky, gray-blue color with irregular, erythematous borders. Blisters containing yellow or reddishblack fluid may also be found . Frank cutaneous gangrene develops 4 to 5 days later, which sloughs due to underlying suppuration by the eighth to tenth day. The involved area becomes anesthetic due to the destruction ofthe cutaneous nerves passing through the superficial fascia. Patients are usually toxic on presentation with a low- to medium-grade fever. Lymphadenopathy and lymphangiitis are not usually found. 4. IO,ls Group A and non-group A streptococcus and staphylococcus species are the most common etiologic organisms, and are usually found in synergistic combination with other facultative bacteria, such as enterobacteriaceae, or anaerobic bacteria. Rarely, patients have been infected with enteric gram-negative organisms alone. 5,ls,21 Anaerobic bacteria were found in 34% of patients, although in none exclusively, by Fisher and co-workers. 23 Some of the isolated organisms may represent superinfection in areas of widespread necrosis, which provides a favorable medium for bacterial colonization. 11,22 Bacteremia has been found in up to 46% of patients with streptococcal infections, and is associated with metastatic foci to distant subcutaneous sites causing thrombosis and gangrene. 1.6.10,22,23 Although the diagnosis is made on results ofthe clinical findings, laboratory studies are useful. Bacteriologic studies should be obtained before antibiotic therapy, including Gram's stains of the discharge from affected areas and ' aerobic and anaerobic cultures of the wounds and blood. The Gram's stain is useful for identifying the predominant organism and has been found to correlate closely with culture results.2l,23 Cultures are ideally obtained from the edges of the wound, tissue debrided from the center of the lesions, or fluid from hemorrhagic bullae.
•
NECROTIZING FASCIITIS
Marked leukocytosis (> 30,OOO/cu mm) is common, although leukopenia due to convergence of granulocytes in the involved areas, spleen, and lymphatics may occur in more severe cases.IA.IO,IS Elevated anti-DNAse B titers and anti-hyaluronidase titers may provide serologic evidence of streptococcal infection. Anti-streptolysin 0 titers and the sedimentation rate may be raised but are not of diagnostic significance. 4,24 A profound anemia may develop from bacterial hemolysins or inhibition of the bone marrow by toxemia. Sequestration of calcium in areas of fat necrosis may lead to hypocalcemia.I,IO,ls Radiographs and computed tomography scans often show subcutaneous gas in the affected sites. 6,ls,23 Results of microscopic examination of the gangrenous tissues show focal necrosis, microabscesses, fibrosis, and hemorrhage in the fascia and subcutaneous tissues, with an intense polymorphonuclear cell infiltrate (Figs 7 and 8). Thrombosis and transmural thickening of the small arterioles and venules that traverse from the subcutaneous fat to the dermal papillae may be found. 4,6,11,21 Frozen section biopsy has been advocated by Stamenkovic and Lew 25 to confirm the diagnosis and expedite the provision of therapy. The pathogenesis of necrotizing fasciitis has been studied by numerous investigators, but the exact mechanism of this rapidly spreading gangrenous infection has not been established. Meleney26 proposed a hypersensitivity reaction of the Arthus or localized Shwartzman type after producing focal necrosis in rabbits by injecting viable streptococci subcutaneously. McCafferty and Lyons 27 postulated that streptokinase or staphylokinase activates proteolytic enzyme activity, resulting in collagen necrosis. The mucopeptide fraction of the cell wall of group A Streptococcus was later shown by Schwab2s ,29 to combine with dermal collagen; Cromartie and co-workers30 injected these cell walls intradermally and were able to produce lesions characterized by inflammation and necrosis of dermal connective tissue. Hyaluronidase released by the organism may enhance the spread and toxicity of these factors. 22 An extensive review of the literature since 1947 yielded 15 cases of necrotizing fasciitis of the scalp and face with associated eyelid necrosis (Table 1).4,S-18 The clinical features and results of diagnostic studies in our patient and previously reported cases are similar to those described for infections involving the extremities and trunk. Blunt or penetrating trauma, most often to the periocular region or scalp, was the etiologic factor in 13 patients. Five patients had a history of alcoholism, and two had diabetes. Bilateral eyelid necrosis developed in 10 cases, whereas both the upper and lower lids were involved in the remaining six unilateral cases. Extension of infection to adjoining sites, including the brow, cheek, and scalp, occurred in all but two patients; neck and mandibular involvement was seen in only one patient each. {3-hemolytic streptococcus was the only organism identified in half of the cases. Staphylococcus species were cultured in combination with streptococci from seven patients. Our patient was the only one in whom streptococcus was not isolated from the wound; however, gram-positive cocci were seen on stained smears of the discharge and 7 days 95
\0 0"1
271M
351M
Walters, 19884
Current study
15
16
26/F
Laceration
Trauma
Blunt trauma
Laceration
Laceration
None
Abrasion
Wasp sting
Blunt trauma
None
Blunt trauma
Unknown
Hairspray
Blunt trauma
Blunt trauma
Laceration
Type of Injury
=
Associated Diseases
RUL, RLL, right cheek, right forehead BUL, RLL, forehead
BUL, BLL, forehead LUL, LLL, left cheek
RUL, RLL, neck, right cheek
LUL, LLL, left cheek, neck BUL, BLL, left scalp, both cheeks LUL, LLL, left cheek, left mandible LUL, BLL, left cheek BUL, LLL
BUL, BLL
=
None
None
=
left upper lid; BLL
Citrobacter diversus, Enterobacter aerogenes
Group A tl-hemloytic Streptococcus, Staphyococcus epidermidis, Bacteroides melaninogenicus Streptococcus viridans, S. albust
Group A tl-hemolytic Streptococcus
Group A ~-hemolytic Streptococcus, S. aureus
tl-hemolytic Streptococcus, Staphylococcus albus tl-hemolytic Streptococcus
tl-hemolytic Streptococcus
Group A tl-hemolytic Streptococcus, Staphylococcus aureus Group A ~-hemolytic Streptococcus, S. aureus Group A tl-hemolytic Streptococcus, S. aureus "Aerobic Streptococcus"
=
=
=
Survived
Died
Survived
Survived
Survived
Survived
Survived
Survived
Survived
Survived
Survived
Died
Survived
Survived
Survived
Survived
Outcome
not provided;
PTSG BUL
PTSG and FTSG LUL, LLL, and cheek
NP
FTSG LUL and LLL FTSG LLL, PTSG BUL PTSG and FTSG RUL
FTSG BUL and BLL, PTSG forehead PTSG and FTSG LUL and LLL
None
PTSG BUL
None
PTSG
FTSG BUL, PTSG scalp FTSG LUL, LTSG LLL
Type of Reconstruction
not known; NP
PCN, gentamicin, methicillin
PCN, cefotaxime, gentamicin
Oxacillin, cefotaxime, metromidazole
NP
Cefuroxime, metronidazole
Ampicillin, gentamicin Gentamicin'
PCN, gentamicin
PCN, oxacillin, chloramphenicol
Cephaloridine
Lincomycin
PCN, methicillin
PCN, tetracycline
both lower lids; NK
12
2
2
9
3
3
2
28
NP
NK
NK
2
3
Group A ~-hemolytic Streptococcus Group A tl-hemolytic Streptococcus
Erythromycin
7
tl-hemolytic Streptococcus
PCN, cephalothin
PCN, sulfathiazole
6
Group A ~-hemolytic Streptococcus
Parenteral Antibiotics
Days Before Therapy
Wound Culture Results
partial-thickness skin graft; LUL
Alcoholism
Alcoholism, discoid lupus Alcoholism
Alcoholism
None
None
None
Polymyositis
Alcoholism
BUL, LLL, scalp, Diabetes right cheek LUL, LLL, left Arteriosclerosis cheek, left temporal scalp BUL, BLL, Diabetes forehead, both cheeks BUL BLL, right None cheek BUL, BLL Hepatomegaly
Area of Infection
full-thickness skin graft; PTSG
Left temporal scalp
Right eye
Face
Occipital scalp
Right upper lid
Left brow
Left mandible
Face
Both eyes
Both eyes
Right brow
Left occipitoparietal scalp Left lateral orbital rim
Site of Injury
BUL = both upper lids; LLL = left lower lid; PCN = penicillin; FTSG RUL = right upper lid; RLL = right lower lid. , Erythromycin administered orally. ttl-hemolytic Streptococcus was cultured from blood .
14
47 IF
Rosenthal et aI., 198717 Balcerak et aI., 198816
13
38/F
Lloyd, 198716
12
62/F
11
59/M
59/F
Einarsson and Pers, 1986 15
10
9
55/F
46/F
7
8
43/F
78/M
6
Carruthers et aI., 197513 Klabacha et aI., 198214
Buchanan and Haserick, 197011 Ross & Kohlhepp, 197312
4
5
67/F
Beathard and Guckian, 196710
3
73/F
93/F
Schott,
2
65/F
Age (yrs)1 Sex
Moore, 19476
Reference
19669
Case No.
Table 1. Clinical Summary of Sixteen Cases of Eyelid Necrosis and Periorbital Necrotizing Fasciitis
m
s:
:0
III
c
z
•
ex>
w.
KRONISH, MD, WILLIAM M. McLEISH, MD
Abstract: Necrotizing fasciitis is an uncommon and severe soft tissue infection characterized by cutaneous gangrene, suppurative fasciitis, and vascular thrombosis. The disease is usually preceded by trauma in patients that have systemic problems, most commonly diabetes and alcoholism. Streptococcus pyogenes and Staphylococcus aureus are the most frequent bacterial etiologies; however, combinations of numerous facultative and anaerobic organisms have also been isolated. Involvement of the face and periocular region is rare. A case is presented here, as well as a review of the clinical features of 15 other patients previously described, in whom eyelid necrosis due to periorbital necrotizing fasciitis developed. Early surgical debridement and drainage of necrotic tissues and appropriate parenteral antibiotics are the mainstay of therapy. The mortality rate in patients with periorbital spread was 12.5%, with the prognosis known to be adversely affected by delay in diagnosis and treatment and/or extension of infection from the face to the neck. Reconstruction of the eyelids with skin grafts was necessary in most cases to avoid such complications as cicatricial lid retraction, lid malpositions, and lagophthalmos. Ophthalmology 1991; 98:92-98
Necrotizing fasciitis is a severe and potentially fatal soft tissue infection that rarely involves the head and neck. 1-3 Extensive necrosis of the superficial fascia with widespread undermining of surrounding tissues and secondary gangrene of the overlying skin characterize this disease. 2,4,5 Before the discovery that numerous facultative and anaerobic bacteria could lead to similar clinical features, infections with subcutaneous tissue necrosis had been referred to as streptococcal gangrene. This disease most commonly involves the extremities and trunk. The term necrotizing fasciitis is preferred as it provides an anatomic description of the planes of infection regardless of the bacterial etiology.5-7 Early recognition and prompt ag-
gressive medical and surgical intervention are critical for proper management of this serious condition. Most of the previously reported cases of eyelid necrosis caused by periorbital necrotizing fasciitis are presented in the general medicalliterature. 4 ,8-18 We present a patient in whom bilateral eyelid necrosis due to necrotizing fasciitis of the scalp and face developed that was successfully managed with proper wound care, antibiotics, reconstructive surgery, and adjunct hyperbaric oxygen therapy, This case and a review of the literature are provided so that ophthalmologists can identify this underdiagnosed condition and institute immediate therapy to minimize morbidity and mortality.
Originally received: June 18, 1990. Manuscript accepted: August 13, 1990.
CASE REPORT
From the Department of Ophthalmology, Bascom Palmer Eye Institute, University of Miami School of Medicine, Miami. The authors have no proprietary interest in any drug mentioned. Reprint requests to Jan W. Kronish, MD, Bascom Palmer Eye Institute, PO Box 016880, Miami, FL 33101.
92
A 26-year-old woman presented to the Jackson Memorial Hospital emergency room several hours after being struck over the left temple region with a metal pipe. She experienced no loss of consyiousness or visual disturbance. Results of an examination revealed a 4-cm full-thickness temporal scalp laceration, fluc-
KRONISH AND McLEISH
Fig I. Computed tomographic scan shows marked soft tissue swelling of the eyelids (arrows), temporal and frontal scalp regions.
tuant swelling of the forehead, tense edema and ecchymosis of the upper and lower eyelids, and generalized facial swelling. Skull radiographs failed to reveal any bony fractures or sinus opacification . A head computed tomography scan revealed marked bilateral soft tissue swelling of the temporal scalp, forehead, and periocular regions with no intracranial abnormalities (Fig I). Hematoma formation within the anterior scalp extending into the preseptal eyelid tissues was suspected. The scalp wound was cleansed and sutured closed. The patient was released and given a 7-day course of prophylactic oral oxacillin 500 mg every 6 hours. On the following day, she was referred to the Bascom Palmer Eye Institute for evaluation. The eyelids remained markedly swollen, while the forehead and temporal scalp appeared less distended than on the previous day. The superficial layers of epidermis of the pretarsal and preseptal areas of both upper lids and preseptal portion of the medial right lower lid had sloughed, leaving a gray-tan discoloration of the excoriated skin (Fig 2). Except for the inability to open her eyes due to the swelling, the patient's ocular examination was normal, including a visual acuity of 20/20 in both eyes, unrestricted motility, and quiet anterior segments. Application of polysporin ointment to the eyelids was initiated four times a day. Four days after the injury, the scalp, facial, and eyelid edema had markedly diminished. The previously eroded eyelid skin had begun to reepithelialize, and she could open her eyes spontaneously.
•
NECROTIZING FASCIITIS
Over the next few days, the patient noted the onset of progressive swelling and redness of both upper lids and gradually increasing purulent discharge. She did not seek further medical attention until 12 days after the initial injury when she was unable to open her eyelids. She denied having such constitutional symptoms as sweats, chills, or rigors, and was found to have a low-grade fever of 38°C. Results of physical examination showed the entire forehead and both brows were elevated and fluctuant, and purulent discharge could be expressed from the temporal scalp wound dehiscence. Necrosis of the preseptal and pretarsal skin and superficial layers of orbicularis muscle of the upper eyelids was noted, along with eschar formation and marked underlying purulent discharge (Fig 3). The lid margins were spared and the well-defined borders of the necrotic wounds were indurated. Her vision remained stable with no signs of orbital inflammation. Initial management included hospital admission for extensive surgical debridement of all the necrotic tissues of the eyelids and scalp wound (Fig 4). Inspection of the scalp wound revealed necrosis to the depth of the temporalis fascia, and a blunt hemostat could be passed with minimal resistance across the forehead in this plane. Aerobic and anaerobic cultures from these areas, as well as samples of blood and urine, were obtained. Gram's stain of the eyelid discharge revealed a few gram-positive cocci and many gram-negative rods. Administration of intravenous penicillin, methicillin, and gentamicin was begun immediately for suspected necrotizing fasciitis. Wound care consisted of alternating wet-to-dry dressing changes with dilute povidone-iodine every 2 hours and cleansing of the eyelids with hydrogen peroxide and irrigation into the scalp wound with gentamicin and penicillin solution. Results of laboratory examination were remarkable for a leukocyte count of 5400/cu mm, a hemoglobin count of8.6 g/dl, aWestegren sedimentation rate of 134 mm/hr, an RPR titer of 1:32, and a negative HIV-1 ELISA and Western blot. Repeat skull radiographs and orbital computed tomography scan failed to disclose any sinus disease or orbital abnormalities. Over the next 2 days, the patient's clinical condition gradually improved with reduction in purulent drainage and diminished edema from the upper lids, as well as slow resolution of the fluctuance in the forehead. Eyelid and scalp wound cultures were positive for Citrobacter diversus and Enterobacter aerogenes, both of which were sensitive to gentamicin. Results of all remaining cultures were negative. After a week of intravenous antibiotics and topical wound care, all signs of active infection had subsided. The necrotic areas in both upper lids had begun to spontaneously granulate with the early development of lid retraction and lagophthalmos. On the tenth hospital day, the patient underwent reconstruction of both upper lids, which involved the removal of granulation tissue and placement of partial-thickness skin grafts over the exposed pretarsal and preseptal orbicularis muscle. Pressure bandages were applied bilaterally after traction sutures were secured to maintain the lids on stretch. The left temporal wound edges, which had previously separated, were reapproximated. Hyperbaric oxygen therapy using 100% oxygen at two atmospheres absolute pressure for 2 hours daily was begun immediately after surgery and continued for 7 days. Parenteral gentamicin was given for 3 more days, at which time the patient began taking oral ciprofloxacin and received 2.4 million units of intramuscular benzathine penicillin for suspected primary syphilis. The pressure patches and traction sutures were removed 7 days after surgery, and the final result was a 100% take of the grafts. Moderate limitation of levator function and ptosis were present initially, but there was no lagophthalmos in either eye. At the time of discharge from the hospital, the patient was instructed to apply antibiotic ointment to her upper lids and
93
OPHTHALMOLOGY
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JANUARY 1991
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VOLUME 98
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NUMBER 1
Fig 2. Top left. appearance of patient 36 hours after head trauma with diffuse swelling of the forehead and massive edema of both upper lids. Epidermal sloughing of the preseptal and pretarsai areas of the lids exposes the pale, avascular dermis with slightly erythematous borders. Fig 3. Second row left. Extensive gangrenous ulceration of both upper eyelids with underlying suppuration 12 days after injury. Fig 4. Third row left. debridement of the wounds consists of removal of eschar, purulent discharge, and residual necrotic tissues. Notice the well-vascularized underlying orbicularis muscle (arrow). Fig 5. Bottom left. appearance of patient with an acceptable cosmetic and functional result 3 months after eyelid reconstruction with partial-thickness skin grafts. Notice the moderate ptosis of both upper lids. Fig 6. Top right. complete eyelid closure is achieved with no associated lid margin malposition or cicatricial lid reo traction. Fig 7. Center right. diffuse edema involving th~ dermis and subcutaneoU! tissue with a predominantl) neutrophilic infiltrate (he· matoxylin-eosin; origina magnification, X60). Fig 8 Bottom right. focally enlarge< collection of neutrophils an( neutrophilic debris associate< with a secondary vasculiti: (hematoxylin-eosin; origina magnification, X400).
massage them regularly to soften the skin grafts. Three months later, she recovered most of the levator function with mild residual ptosis of both upper lids and maintained complete eyelid closure bilaterally (Figs 5 and 6).
DISCUSSION Necrotizing fasciitis was recognized as early as the American Civil War and referred to by Jones l9 as hospital
94
gangrene in which "the skin of an affected part melt~ away." Melenelo accurately described this infection ill 1924 based on 20 patients in which hemolytic strepto· coccus was cultured, leading him to coin the term "he· molytic streptococcus gangrene." Other names became synonymous with this condition including "gangrenom erysipelas," "necrotizing erysipelas," "acute infective gangrene," and "suppurative fasciitis." In 1952, Wilson~ introduced the currently preferred term of "necrotizin! fasciitis" to emphasize the constant feature of necrotic
KRONISH AND McLEISH
fascia with spread of the infection along fascial planes and the non specificity of the bacterial etiology. More recent bacteriologic studies have confirmed Wilson's findings that most cases can be attributed to streptococcal and staphylococcal organisms, although a mixture offacultative and anaerobic bacteria also can lead to this type of infection. 5,21 Necrotizing fasciitis can develop in patients of all ages and has no sex or race predilection. 5 Minor trauma, such as abrasions, lacerations, insect bites, and hypodermic needle injections, is the most common initiating factor. The onset of infection may also follow surgery or blunt injuries, and, occasionally, develop without apparent cause. I,5,6,IO Predisposing factors have been reported to include diabetes mellitus,I,2,6,ls,22 arteriosclerosis and peripheral vascular disease,I,l s alcoholism,I,2,ls malignancy,6.IS polymyositis,13,ls intravenous drug abuse, and the postpartum state. 6 Most reported cases involve the lower extremities, whereas the upper extremities, trunk, and genitalia also are frequently affected. 4,7 Necrotizing fasciitis of the head and neck is relatively uncommon and may have a worse prognosis.2,6 This fulminating infection usually develops within 2 to 4 days after the traumatic event and progresses with alarming speed. The skin is initially pale red, tensely swollen, and accompanied by pain as extensive necrosis spreads in the subcutaneous tissue planes. Within 1 to 2 days the pathognomonic features become apparent as the skin turns a dusky, gray-blue color with irregular, erythematous borders. Blisters containing yellow or reddishblack fluid may also be found . Frank cutaneous gangrene develops 4 to 5 days later, which sloughs due to underlying suppuration by the eighth to tenth day. The involved area becomes anesthetic due to the destruction ofthe cutaneous nerves passing through the superficial fascia. Patients are usually toxic on presentation with a low- to medium-grade fever. Lymphadenopathy and lymphangiitis are not usually found. 4. IO,ls Group A and non-group A streptococcus and staphylococcus species are the most common etiologic organisms, and are usually found in synergistic combination with other facultative bacteria, such as enterobacteriaceae, or anaerobic bacteria. Rarely, patients have been infected with enteric gram-negative organisms alone. 5,ls,21 Anaerobic bacteria were found in 34% of patients, although in none exclusively, by Fisher and co-workers. 23 Some of the isolated organisms may represent superinfection in areas of widespread necrosis, which provides a favorable medium for bacterial colonization. 11,22 Bacteremia has been found in up to 46% of patients with streptococcal infections, and is associated with metastatic foci to distant subcutaneous sites causing thrombosis and gangrene. 1.6.10,22,23 Although the diagnosis is made on results ofthe clinical findings, laboratory studies are useful. Bacteriologic studies should be obtained before antibiotic therapy, including Gram's stains of the discharge from affected areas and ' aerobic and anaerobic cultures of the wounds and blood. The Gram's stain is useful for identifying the predominant organism and has been found to correlate closely with culture results.2l,23 Cultures are ideally obtained from the edges of the wound, tissue debrided from the center of the lesions, or fluid from hemorrhagic bullae.
•
NECROTIZING FASCIITIS
Marked leukocytosis (> 30,OOO/cu mm) is common, although leukopenia due to convergence of granulocytes in the involved areas, spleen, and lymphatics may occur in more severe cases.IA.IO,IS Elevated anti-DNAse B titers and anti-hyaluronidase titers may provide serologic evidence of streptococcal infection. Anti-streptolysin 0 titers and the sedimentation rate may be raised but are not of diagnostic significance. 4,24 A profound anemia may develop from bacterial hemolysins or inhibition of the bone marrow by toxemia. Sequestration of calcium in areas of fat necrosis may lead to hypocalcemia.I,IO,ls Radiographs and computed tomography scans often show subcutaneous gas in the affected sites. 6,ls,23 Results of microscopic examination of the gangrenous tissues show focal necrosis, microabscesses, fibrosis, and hemorrhage in the fascia and subcutaneous tissues, with an intense polymorphonuclear cell infiltrate (Figs 7 and 8). Thrombosis and transmural thickening of the small arterioles and venules that traverse from the subcutaneous fat to the dermal papillae may be found. 4,6,11,21 Frozen section biopsy has been advocated by Stamenkovic and Lew 25 to confirm the diagnosis and expedite the provision of therapy. The pathogenesis of necrotizing fasciitis has been studied by numerous investigators, but the exact mechanism of this rapidly spreading gangrenous infection has not been established. Meleney26 proposed a hypersensitivity reaction of the Arthus or localized Shwartzman type after producing focal necrosis in rabbits by injecting viable streptococci subcutaneously. McCafferty and Lyons 27 postulated that streptokinase or staphylokinase activates proteolytic enzyme activity, resulting in collagen necrosis. The mucopeptide fraction of the cell wall of group A Streptococcus was later shown by Schwab2s ,29 to combine with dermal collagen; Cromartie and co-workers30 injected these cell walls intradermally and were able to produce lesions characterized by inflammation and necrosis of dermal connective tissue. Hyaluronidase released by the organism may enhance the spread and toxicity of these factors. 22 An extensive review of the literature since 1947 yielded 15 cases of necrotizing fasciitis of the scalp and face with associated eyelid necrosis (Table 1).4,S-18 The clinical features and results of diagnostic studies in our patient and previously reported cases are similar to those described for infections involving the extremities and trunk. Blunt or penetrating trauma, most often to the periocular region or scalp, was the etiologic factor in 13 patients. Five patients had a history of alcoholism, and two had diabetes. Bilateral eyelid necrosis developed in 10 cases, whereas both the upper and lower lids were involved in the remaining six unilateral cases. Extension of infection to adjoining sites, including the brow, cheek, and scalp, occurred in all but two patients; neck and mandibular involvement was seen in only one patient each. {3-hemolytic streptococcus was the only organism identified in half of the cases. Staphylococcus species were cultured in combination with streptococci from seven patients. Our patient was the only one in whom streptococcus was not isolated from the wound; however, gram-positive cocci were seen on stained smears of the discharge and 7 days 95
\0 0"1
271M
351M
Walters, 19884
Current study
15
16
26/F
Laceration
Trauma
Blunt trauma
Laceration
Laceration
None
Abrasion
Wasp sting
Blunt trauma
None
Blunt trauma
Unknown
Hairspray
Blunt trauma
Blunt trauma
Laceration
Type of Injury
=
Associated Diseases
RUL, RLL, right cheek, right forehead BUL, RLL, forehead
BUL, BLL, forehead LUL, LLL, left cheek
RUL, RLL, neck, right cheek
LUL, LLL, left cheek, neck BUL, BLL, left scalp, both cheeks LUL, LLL, left cheek, left mandible LUL, BLL, left cheek BUL, LLL
BUL, BLL
=
None
None
=
left upper lid; BLL
Citrobacter diversus, Enterobacter aerogenes
Group A tl-hemloytic Streptococcus, Staphyococcus epidermidis, Bacteroides melaninogenicus Streptococcus viridans, S. albust
Group A tl-hemolytic Streptococcus
Group A ~-hemolytic Streptococcus, S. aureus
tl-hemolytic Streptococcus, Staphylococcus albus tl-hemolytic Streptococcus
tl-hemolytic Streptococcus
Group A tl-hemolytic Streptococcus, Staphylococcus aureus Group A ~-hemolytic Streptococcus, S. aureus Group A tl-hemolytic Streptococcus, S. aureus "Aerobic Streptococcus"
=
=
=
Survived
Died
Survived
Survived
Survived
Survived
Survived
Survived
Survived
Survived
Survived
Died
Survived
Survived
Survived
Survived
Outcome
not provided;
PTSG BUL
PTSG and FTSG LUL, LLL, and cheek
NP
FTSG LUL and LLL FTSG LLL, PTSG BUL PTSG and FTSG RUL
FTSG BUL and BLL, PTSG forehead PTSG and FTSG LUL and LLL
None
PTSG BUL
None
PTSG
FTSG BUL, PTSG scalp FTSG LUL, LTSG LLL
Type of Reconstruction
not known; NP
PCN, gentamicin, methicillin
PCN, cefotaxime, gentamicin
Oxacillin, cefotaxime, metromidazole
NP
Cefuroxime, metronidazole
Ampicillin, gentamicin Gentamicin'
PCN, gentamicin
PCN, oxacillin, chloramphenicol
Cephaloridine
Lincomycin
PCN, methicillin
PCN, tetracycline
both lower lids; NK
12
2
2
9
3
3
2
28
NP
NK
NK
2
3
Group A ~-hemolytic Streptococcus Group A tl-hemolytic Streptococcus
Erythromycin
7
tl-hemolytic Streptococcus
PCN, cephalothin
PCN, sulfathiazole
6
Group A ~-hemolytic Streptococcus
Parenteral Antibiotics
Days Before Therapy
Wound Culture Results
partial-thickness skin graft; LUL
Alcoholism
Alcoholism, discoid lupus Alcoholism
Alcoholism
None
None
None
Polymyositis
Alcoholism
BUL, LLL, scalp, Diabetes right cheek LUL, LLL, left Arteriosclerosis cheek, left temporal scalp BUL, BLL, Diabetes forehead, both cheeks BUL BLL, right None cheek BUL, BLL Hepatomegaly
Area of Infection
full-thickness skin graft; PTSG
Left temporal scalp
Right eye
Face
Occipital scalp
Right upper lid
Left brow
Left mandible
Face
Both eyes
Both eyes
Right brow
Left occipitoparietal scalp Left lateral orbital rim
Site of Injury
BUL = both upper lids; LLL = left lower lid; PCN = penicillin; FTSG RUL = right upper lid; RLL = right lower lid. , Erythromycin administered orally. ttl-hemolytic Streptococcus was cultured from blood .
14
47 IF
Rosenthal et aI., 198717 Balcerak et aI., 198816
13
38/F
Lloyd, 198716
12
62/F
11
59/M
59/F
Einarsson and Pers, 1986 15
10
9
55/F
46/F
7
8
43/F
78/M
6
Carruthers et aI., 197513 Klabacha et aI., 198214
Buchanan and Haserick, 197011 Ross & Kohlhepp, 197312
4
5
67/F
Beathard and Guckian, 196710
3
73/F
93/F
Schott,
2
65/F
Age (yrs)1 Sex
Moore, 19476
Reference
19669
Case No.
Table 1. Clinical Summary of Sixteen Cases of Eyelid Necrosis and Periorbital Necrotizing Fasciitis
m
s:
:0
III
c
z
•
ex>
