Periorbital Necrotizing Fasciitis in a Young Woman with Anorexia Nervosa-the Management and Considerations of Cosmetic and Functional Outcomes of Fulminant Disease in a Critically Unwell Patient-Juniper publishers
JUNIPER PUBLISHERS-OPEN ACCESS JOURNAL OF HEAD NECK & SPINE SURGERY
Introduction
Necrotizing Fasciitis (NF) is a devastating rapidly
progressive bacterial infection involving the superficial fascia leading
to
necrosis of overlying skin and systemic toxicity [1,2]. We present a
case of periorbital NF in a chronically malnourished and unwell patient
following facial trauma.
Case Report
A 36-year-old female presented to the emergency
department following a mechanical fall with extensive left facial
bruising extending to the neck and chest wall. The patient featured a
complex medical history including severe anorexia nervosa. Pathology
revealed significant electrolyte derangement, hypothermia, osteoporosis,
bone marrow suppression and synthetic hepatic impairment. A clinical
diagnosis of NF was made 6 days into her ICU admission following the
development of anaesthetic, necrotic-appearing skin associated with
purulent
exudates from the palpebral fissure and a corresponding rise
in the patient’s White Cell Count (WCC) and C-Reactive Protein (CRP).
Examination of the eye was unremarkable, with the exception of a
subconjunctival hemorrhage and nasally oriented corneal dellen. Initial
emergency debridement was undertaken as a joint operation between
ophthalmology and ENT teams, stripping back necrotic periorbital tissues
down to the lower cheek and performing a lateral tarsorrhaphy to
prevent exposure keratopathy. ENT undertook further exploration and
debridement down to the neck and submental region, leaving a VAC
dressing in-situ and harvesting multiple tissue samples for
histopathological evaluation. Histology revealed marked suppurative
inflammatory changes in the reticular dermis, skeletal muscle and
subcutaneous fat in association with venous congestive changes and large
regions of muscular and dermal necrosis (Figure 1).
0018
Pan-sensitive Streptococcus pyogenes and penicillinresistant
Staphylococcus aureus (MSSA) were isolated from the
excised tissues, while Pseudomonas species were isolated from
the superficial left facial and eye swabs. Pending sensitivities,
targeted antimicrobial therapy was instituted including the use
of intravenous flucloxacillin, vancomycin, piperacillin-tazobactam
and meropenem. Subsequent operative debridement and
exploration was undertaken two days later, excising further nonviable
periorbital tissues down to the buccal fat pad. Inspection
of oral tissues was performed under direct laryngoscopy along
with extension of tissue debridement from the neck area, noting
that progression did not appear to track along fascial planes.
Two months later, initial reconstruction surgery was performed.
After washout of the left cheek and periorbital wounds, a Split
Skin Graft (SSG) was applied to the left cheek (from a left thigh
donor site) and Full-Thickness Skin Graft (FTSG) was applied
to the superior and inferior left eyelids (abdominal donor site).
The left tarsorrhaphy was also revised. Six months following her
initial presentation, horizontal lateral canthotomy and division of
tarsorrhaphy was performed to allow for a wider palpebral fissure.
Finally, nine months after the initial diagnosis of NF, debulking of
the upper lid was performed in conjunction with suturing of trace
remaining elevator fibers to the tarsal plate. Reconstruction of the
lower eyelid with FTSG (donor site medial upper arm) was also
performed to relax the cicatricial nature of the earlier procedures
(Figure 2).
Post-operative best corrected visual acuity was 6/9+2 in
the affected eye with 5-6mm of lagophthalmos and complete
pupillary exposure, allowing for good binocular vision without
altered head posture. The patient remains able to use frontalis and
orbicularis action to consciously moisten her cornea. The patient
continued to use regular preservative-free lubricants to prevent
exposure keratopathy. Periorbital NF is a rare rapidly progressing
ophthalmic emergency that requires early recognition and
operative debridement to prevent a fulminant course [1,3-5]. First
described by Hippocrates in the fifth century B.C. as a complication
of erysipelas [5], NF was then first properly described in modern
literature by Confederate army surgeon Joseph Jones in 1871.
Inconsistent nomenclature and inadequate linkage to sufficient
bacteriological data led to confusion in the literature and a
delay in the understanding of NF as a single disease entity [6].
Infection of the head and neck is uncommon in NF with only asmall number of cases being described in literature since 1960.
In the post-antibiotic era, the overall mortality of head and neck
NF has been estimated at between 9 and 31%, but its relatively
frequent association with multi-system shock and permanent
disfigurement render NF a particularly devastating and terrifying
illness [3,7] (Figure 3).
The most common trigger for periorbital NF is an antecedent
injury leading to the breakdown of the skin barrier and concomitant
inoculation of the causative organism(s) [6-8]. Swelling, pain,
erythema, fever, haemorrhagic bullae, skin necrosis and crepitus
are the most common clinical signs relevant to diagnosis [2,4].
Diabetes mellitus, immunosuppression, chronic renal impairment,
pulmonary disease, recent surgery and traumatic injury have been
identified as predisposing factors for NF; however, it is worthwhile
noting that a large number of published cases remain idiopathic
in nature [1,9-10]. Immunosuppression, age greater than 50
years, cirrhosis, congestive cardiac failure, gout and development
of toxic shock syndrome (a late sign of infection) have been
identified as independent risk factors most associated with
increased mortality from NF [1,2,11]. Relative risk indicators have
been identified, utilizing biochemical indicators such as white cell
count, haemoglobin, CRP, serum creatinine, sodium and blood
glucose to stratify NF severity in the acute setting [12].
Periorbital NF is divided into 4 classifications on the basis
of microbiological culture. Type 1 (polymicrobial) infections
are related to both anaerobic and aerobic (including facultative
anaerobes such as Enterobacteriaciae and non-typable
Streptococci) organisms most commonly manifesting on the
abdomen, trunk and perineum. Type 1 NF is the most common
type to develop in patients with diabetes. Type 2 (monomicrobial)
NF involves the extremities, cutaneous and muscular tissues;
and features no clear underlying risk factors. Group-A betahaemolytic
Stretococcus (S. pyogenes) and/or S. aureus are
the most commonly implicated organisms in Type 2 disease,
characterizing its strong association with toxic-shock syndrome.
Type 3 infections are caused by Clostridium, Vibrio spp. and other
gram-negative organisms, typically involving the abdominal wall
and/or perineum. They are often rapidly progressive infectionsfollowing salt-water injuries, seafood ingestion and penetrating
traumas. Type 4 NF describes fungal infections involving the
extremities, most frequently occurring in immunosuppressed
individuals [1,9,10,13].
Our patient was a young, immunosuppressed, malnourished
female. On hospital presentation she had severe electrolyte
derangement (hypomagnesaemia, hypokalaemia and
hyponatreamia), hypothermia, impaired hepatic synthetic
function and coagulopathy (INR 2.0 with hypovitaminosis-K) and
ischaemic digits. She developed severe facial Type 2 NF (S. aureus
and S. pyogenes) following a break in the periorbital skin overlying
a large haematoma, initially caused by blunt mechanical trauma.
No underlying fractures were identified on computed tomography
imaging. Anaesthetic necrosis of the thin eyelid skin allowed the
early clinical recognition and diagnosis of necrotizing fasciitis,
nevertheless the infection was rapidly progressive in nature,
resulting in severe permanent disfigurement despite prompt
operative debridement and broad-spectrum antimicrobial cover.
This case affords some insights into the pathophysiology of
NF and also presented challenging aspects in the consideration of
reconstructive techniques, attempting to maximize both long-term
functional stability of the eye in limiting exposure keratopathy
whilst optimizing the patient’s cosmesis
Operative exploration and debridement in this patient
demonstrated that this infection did not respect fascial planes,
considered anomalous in the typical subcutaneous spread pattern
of NF [14]. It is possible that traumatic disruption of subcutaneous
compartments involving all skin and muscular layers, in
conjunction with a large haematoma facilitated alternative
pathways for the dispersion of infection in this patient. This is
of particular importance to keep in mind in such cases wherethe musculoaponeurotic structures of the face and neck may
be breached, allowing the spread of infection into the superior
mediastinum [11].
Discussion
Periorbital NF is the most commonly involved facial site
and behaves differently from NF elsewhere in that the highly
vascularized orbicularis oculi acts as an effective barrier to prevent
the spread of infection from the skin to the underlying periorbita,
thereby retarding the spread of infection into the orbit. As in this
case, necrosis of the thin eyelid skin occurs rapidly, facilitating
early detection of the condition due to the obvious cosmetic
abnormality on examination [1]. Given the fragile general health
and constitution of the patient, decisions regarding the donor sites
for reconstructive skin grafting were based purely on pragmatism,
with cosmesis playing a clearly secondary consideration to what
were emergent and life-saving surgeries. Gross malnutrition
and immunosuppression meant that donor tissue was chosen
to maximize graft viability, to facilitate adequate coverage of the
relatively large areas left exposed following extensive surgical
debridement and to maximize the chances of adequate postoperative
healing in this setting. Subsequent procedures were
required in this case to balance the cosmesis and function of the
patients eyelids, her vision (and the initial requirement for a chinup
posture to peer through her tight palpebral fissure,) and the
necessity to prevent an exposure keratopathy in a patient with
significant lagophthalmos secondary to an almost obliterated
orbicularis.
Conclusion
The rapidly destructive nature of NF necessitates early
recognition and diagnosis to minimize the risk of a fulminant
course with high morbidity and mortality. Physicians must
remain vigilant and mount a thorough clinical suspicion for NF
in suitably vulnerable patients with clinical features suggestive
of the disease such as severe pain, swelling, erythema, bullae
and tactile anaesthesia. Urgent resuscitation, emergency surgical
debridement, broad-spectrum IV antibiotics and other novel
therapies such as hyperbaric oxygen must be implemented
without delay to prevent the frequently debilitating, disfiguring
and life-threatening sequelae of NF [1,8,11,15]. This case report
demonstrated some of the complexities in managing periorbitalNF in the context of pre-existing medical comorbidities and
highlighted the challenges in optimizing the functional and
cosmetic outcomes of reconstructive surgery following extensive
emergency debridements of neck and facial tissues.
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