Abstract
Symmetrical peripheral gangrene is a severe condition marked by symmetric acral
necrosis without obstruction of the major blood vessels. This case report
examines the critical decisions involved in choosing between early and delayed
amputation, as well as determining the extent of the necessary amputation. We
present three cases: one involving antiphospholipid syndrome, another with
disseminated intravascular coagulation, and a third associated with diabetes
mellitus. All three cases ultimately required amputation due to symmetrical
peripheral gangrene. In the first two cases, amputation was delayed, which is
typically advantageous as it allows for the clear demarcation of necrotic
tissue. However, in the third case, where infection was evident, immediate
amputation was necessary despite the patient's overall poor health.
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Keywords: Amputation; Antiphospholipid syndrome; Disseminated intravascular coagulation; Diabetes mellitus; Gangrene
Introduction
Symmetrical peripheral gangrene (SPG) is a devastating condition characterized by
symmetric acral necrosis without large vessel obstruction, often associated with
septicemic conditions [
1] and disseminated
intravascular coagulation (DIC) [
2]. In this
case report, we describe three cases: one of antiphospholipid syndrome, one of DIC,
and one of diabetes mellitus, all of which resulted in amputation due to SPG. Our
primary protocol is to delay amputation until the gangrene is well established.
However, should signs of infection appear, early amputation is recommended.
Case presentation
Ethics statement
This case report was approved by the institutional ethics committee (IRB NO: KNUH
2021-10-026). Consent for publication was obtained from the patients or their
family members.
Case 1
Patient information and clinical findings
A 76-year-old woman presented to the emergency department with pain in her
bilateral fingers and toes, which began 1 day prior to admission.
Diagnostic assessment and final diagnosis
Photoplethysmographic examination revealed no flow in both the toes and
fingers, with the exception of the right thumb. The patient's
coagulation profile indicated a d-dimer level of 24.63 mg/dL and
antithrombin III at 64.6%. A lupus anticoagulant value of 1.21 was also
detected. Based on these findings, the patient was diagnosed with
antiphospholipid syndrome.
Therapeutic intervention
Given the patient's high risk of developing sepsis, a central line was
placed. The patient was treated with alprostadil and underwent
heparinization. A dark discoloration appeared and progressed symmetrically
up to the middle phalanges (
Fig. 1).
Within 3 days after admission, the patient stabilized, and a clear
demarcation line was established, leading to a series of amputations.
Fig. 1.A 76-year-old woman with sharp aggravating pain and cyanotic
changes on the tips of fingers and toes. She was diagnosed with
antiphospholipid syndrome and later experienced dark discoloration
with symmetric progression up to middle phalanges. The patient then
underwent a series of amputations involving the fingers and toes on
both sides.
Follow-up and outcomes
The patient tolerated the procedures well and did not experience any
recurrence.
Case 2
Patient information and clinical findings
A 49-year-old man diagnosed with rectal carcinoma (stage T3N2M0) underwent
laparoscopic anterior resection of the colon. Three days post-surgery, the
patient experienced anastomotic leakage. As his clinical condition worsened,
he underwent an emergency segmental resection of the colon, coloanal
anastomosis, and loop ileostomy. Following these procedures, he was
transferred to the intensive care unit and placed on a mechanical
ventilator. Blood cultures revealed the presence of Escherichia coli, and
his hemoglobin level dropped to 5.9 g/dL. Treatment included a dobutamine
drip and a norepinephrine drip, stabilizing his blood pressure at 100/60
mmHg.
Diagnostic assessment
Laboratory analysis indicated signs of DIC, with fibrinogen levels at 216
mg/dL, antithrombin III at 31.9%, D-dimer at 1,216 mg/dL, PT at 35.2%, PTT
at 57.6 seconds, INR at 2.15, and a platelet count of 70,000/cc.
Therapeutic intervention
Sixty units of platelets, two pints of packed blood cells, and 10 units of
fresh frozen plasma were transfused. Treatment with vasopressors was
continued, as both hands and feet became cold, and blue discoloration
appeared. Within 2 days, dry gangrene appeared on the digits of all four
limbs and symmetrically progressed up to the bilateral wrists and distal
calves (
Fig. 2). Once the
patient's overall condition and the gangrene stabilized, bilateral
open below-knee and below-elbow amputations were performed.
Fig. 2.A 49-year-old man who later showed a septic manifestation and
consumptive coagulopathy. On the second day of inotropic use, dry
gangrene of the digits developed and progressed symmetrically up to
(A) the bilateral wrists and (B) distal calf, including the ankles
and feet.
Follow-up and outcomes
The patient tolerated the procedures well and did not experience any
recurrence.
Case 3
Patient information
A 53-year-old woman with end-stage kidney disease, who was undergoing
dialysis, and had a long-standing history of diabetes mellitus, was admitted
to the hospital. She had been receiving hemodialysis three times a week and
had been on insulin injections for two years prior to her admission.
Clinical findings
One year before admission, the patient underwent percutaneous transluminal
angioplasty in the right forearm, which was followed by the development of
distal gangrene. Treatment included acetylsalicylic acid, clopidogrel, and
warfarin. Six months before hospital admission, gangrene with infection
appeared at the fourth fingertip of the right hand and on both feet (
Fig. 3). Cultures from wound pus
identified methicillin-resistant
Staphylococcus aureus
(MRSA), leading to the administration of oxacillin and vancomycin. A phase
bone scan indicated a soft tissue infection in the left foot.
Fig. 3.A 53-year-old woman with end-stage kidney disease on dialysis and
a long history of diabetes mellitus had gangrene with infection at
the fourth fingertip of her right hand and both feet. Due to the
presence of an infection, the patient underwent prompt
amputation.
The patient's condition continued to deteriorate, characterized by
persistent fever and poor oral intake. Concurrently, the CRP levels
consistently rose, reaching values above 30.79 mg/dL.
Therapeutic intervention
Amputation was initially performed on the right hand and fourth finger,
followed by the amputation of the first toe on the right foot, and
subsequently, the fourth finger of the left foot. A stronger antibiotic,
meropenem, was administered, along with regular incision and drainage
procedures. Despite these measures, wound healing remained poor.
Follow-up and outcomes
The patient's general condition kept worsening until her death 3
months after admission.
Discussion
In the first case, antiphospholipid syndrome was identified as the underlying cause
of gangrene. Lupus anticoagulants are associated with an odds ratio for thrombosis
that is 5 to 16 times higher than that of controls. This increased risk applies
regardless of the thrombosis's location and type, and whether systemic lupus
erythematosus is present [
3]. The
"two-hit" model of thrombosis associated with antiphospholipid
syndrome proposes an initial "first hit" that disrupts the
endothelium, followed by a "second hit" that promotes thrombus
formation [
4]. The presence of sepsis in this
patient exacerbated the reduction in blood supply to the most distal parts of the
body. Heparinization was implemented to prevent further clot formation, although it
could not reverse the existing damage.
The second patient had a neoplastic condition with
E. coli isolated
from the blood culture.
E. coli contains lipopolysaccharide in its
outer layer, which can trigger endotoxic shock. This, in turn, activates the
coagulation system, leading to consumptive coagulopathy. Fibrin thrombi were
observed in skin biopsy specimens or those from various organs with a history of SPG
during the postmortem examination, suggesting DIC [
2].
Despite recognizing the inotropic agent as the cause of gangrene progression in the
second case, the cost of discontinuing the treatment was deemed too high. A prompt
surgical approach was only considered once the patient's general condition
had stabilized. It is also advisable to wait until a clear demarcation line of
gangrene is established, as viable tissues may exist beneath the necrotic skin
[
5]. The extent of the early symptoms will
likely decrease after revascularization around the gangrene. Therefore, early
amputation could potentially extend the gangrene or lead surgeons to overestimate
the necessary level of amputation.
In the third patient, the cause of gangrene was undetermined. However, the patient
had diabetes mellitus. Microangiopathy in patients with diabetes mellitus impairs
blood flow, especially to the most distal parts, and initially creates dry gangrene
[
6]. Additionally, diabetes mellitus
increases susceptibility to infections [
7].
In the third case, early amputation was warranted because the increasing CRP
indicated infection. For patients with diabetes, many authors advocate early surgery
since infections may further disturb local microcirculation and antibiotics cannot
penetrate the site of infection [
8,
9]. Wound culture also revealed infection by
MRSA, which is associated with worse outcomes in patients with diabetic foot
infections.
The patients in the first two cases tolerated the procedures well and experienced no
recurrence. With the aid of suitable prostheses, they were able to carry out their
daily activities. Therefore, these cases highlight the importance of tailored
assessments that consider the underlying condition, the progression of necrosis, and
the status of infection to enhance outcomes and minimize morbidity in the management
of SPG.
In conclusion, SPG is a severe complication that necessitates careful consideration
of both the timing and level of amputation. The cases discussed here demonstrate
that a delayed approach to amputation is generally advantageous in instances of
antiphospholipid syndrome and DIC, as it allows for a well-demarcated border of
necrosis to form. However, early amputation is imperative in the presence of
infection, such as in diabetes mellitus with MRSA infection, to prevent further
systemic deterioration. The decision on the appropriate level of amputation hinges
on achieving clear demarcation of necrotic tissue to avoid unnecessary removal of
viable tissue, while also taking into account the patient's overall condition
and response to revascularization efforts. In situations where infection impairs
local blood flow or where antibiotics are ineffective in penetrating the affected
area, early intervention at a higher level of amputation may be necessary to control
the infection and improve the potential for healing.
Authors' contributions
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Project administration: Lee HJ, Jeon IH, Kochhar H, Kim HJ, Kim PT
Conceptualization: Deslivia MF, Lee HJ, Kochhar H
Methodology & data curation: Deslivia MF, Kochhar H
Funding acquisition: Lee HJ
Writing – original draft: Deslivia MF, Jeon IH, Kim HJ, Kim PT
Writing – review & editing: Deslivia MF, Lee HJ, Jeon IH, Kochhar
H, Kim HJ, Kim PT
Conflict of interest
-
No potential conflict of interest relevant to this article was reported.
Funding
-
This research was supported by the Korea Health Technology R&D Project
grant through the Korea Health Industry Development Institute (KHIDI), funded by
the Ministry of Health & Welfare, Republic of Korea (grant number:
HR22C1832).
Data availability
-
Not applicable.
Acknowledgments
Not applicable.
Supplementary materials
-
Not applicable.
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Figure & Data
Citations
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