25-year-old man with sudden onset paraplegia

Abstract

A 25-year-old man with Crohn’s disease presented to the emergency department with sudden onset bilateral leg pain, paraplegia and loss of anal sphincter tone. CT angiography revealed a massive abdominal aortic thrombosis. Revascularisation surgery was performed promptly, saving most of the abdominal organs, but motor function of the legs was not restored. No coagulation disorder was identified, and no other underlying cause for thrombosis was found. It was speculated that it may be related to the patient’s underlying Crohn’s disease.

Full text

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ManieriM. BMJ Case Rep 2019;12:e228281. doi:10.1136/bcr-2018-228281
Case report
25-year-old man with sudden onsetparaplegia
Marco Manieri
Rare disease
To cite: ManieriM. BMJ
Case Rep 2019;12:e228281.
doi:10.1136/bcr-2018-
228281
Emergency Department, Skanes
universitetssjukhus Malmo,
Malmo, Sweden
Correspondence to
Marco Manieri;
marco. manieri@ skane. se
Accepted 25 September 2019
© BMJ Publishing Group
Limited 2019. Re-use
permitted under CC BY-NC. No
commercial re-use. See rights
and permissions. Published
by BMJ.
Figure 1 CT angiography sagittal image. Red arrows
point to the proximal and distal end of the thrombus in
the aorta. There is passage of contrast medium, but the
distal diameter is less due to decreased flow.
SUMMARY
A 25-year-old man with Crohn’s disease presented to
the emergency department with sudden onset bilateral
leg pain, paraplegia and loss of anal sphincter tone.
CT angiography revealed a massive abdominal aortic
thrombosis. Revascularisation surgery was performed
promptly, saving most of the abdominal organs,
but motor function of the legs was not restored. No
coagulation disorder was identified, and no other
underlying cause for thrombosis was found. It was
speculated that it may be related to the patient’s
underlying Crohn’s disease.
BACKGROUND
Aortic thrombosis is a rare disease, even more so in
a young athlete. There is a suspected connection to
inflammatory bowel disease (IBD) which may be of
interest to future researchers.
CASE PRESENTATION
A 25-year-old man presented to the emergency
department with sudden onset paraplegia and pain in
the lower extremities. His medical history included
Crohn’s disease, managed with sulfasalazine. He
was otherwise healthy, worked as a tradesman, and
was competing in fitness championships. There
was no history of trauma to the back or abdomen.
Earlier in the morning, while lifting a 10 kg object
at work, he experienced sudden onset of bilateral
leg pain and felt the urge to defecate. While in the
bathroom, he lost power in his lower extremities
and was found on the floor by his coworkers. Vital
signs were stable upon ambulance crew arrival. The
patient presented with abdominal and bilateral leg
pain in addition to numbness and paralysis of the
legs. He had faecal incontinence and had soiled his
clothes and the floor. During ambulance transport,
he received intravenous crystalloid fluids, ondanse-
tron and a total of 25 mg of intravenous morphine.
On arrival to the resuscitation room at Skåne
University Hospital in Malmö, the patient was still
complaining of pain, now primarily localised to
the perianal region, and persistent paraplegia. His
vital signs were within normal range, except blood
pressure that was elevated at 180/90 mm Hg. His
legs were pale, cold and numb with flaccid paral-
ysis. Femoral and distal peripheral pulses were
impalpable. Sensory loss extended distally from
the umbilical level, whereas examination above the
umbilical level was normal. Bedside ultrasonog-
raphy revealed no free intra-abdominal fluid and
the aorta appeared normal. On log-rolling, there
was no sign of trauma or midline tenderness on
palpation of the spine. Rectal examination revealed
total loss of sphincter tone and sensation in the
perianal area. Bedside arterial blood gas showed
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2ManieriM. BMJ Case Rep 2019;12:e228281. doi:10.1136/bcr-2018-228281
Rare disease
Learning points
►When clinical findings point strongly in one direction, this
must be pursued, however uncommon the diagnosis. The
combination of ischaemic lower extremities and spinal
ischaemia (paralysis and sensory loss) is strongly suggestive
of an abdominal aortic occlusion.
►Inflammatory bowel disease is an independent risk factor for
thromboembolic disease.
►Abdominal aortic thrombosis is a true vascular emergency
and, even when handled in a timely fashion, patients are at a
high risk of severe morbidity and mortality.
Figure 2 3D reconstruction of CT angiography. White arrows point to
the proximal and distal end of the thrombus.
metabolic acidosis with pH 7.2, base excess −13 mmol/L, lactate
6.2 mmol/L, an elevated creatinine at 155 µmol/L and otherwise
unremarkable. More analgesia was given.
INVESTIGATIONS
The vascular surgery consultant was summoned and the patient
was expedited to the CT scanner for imaging. CT angiography
of the whole aorta revealed aortic thrombosis, extending from
the level of the diaphragm to the aortic bifurcation (figures 1 and
2). No signs of atherosclerosis were visible.
TREATMENT
The patient was transferred directly to the operating theatre
where emergency hybrid surgery was performed. Open explo-
ration revealed extensive visceral ischaemia. The occluded arte-
rial branches were clamped distally in an effort to avoid further
embolisation. Thereafter the following endovascular interven-
tions were performed: thrombectomy and stenting of the right
renal artery, superior mesenteric artery and distal aorta. There
was no macroscopic anomaly of the aorta that the surgeons could
identify. On reperfusion, there was massive swelling of both legs
requiring several fasciotomies. The patient was transferred to
the intensive care unit (ICU) where he remained intubated for 2
days. Second-look surgery was performed 24 hours after the first
intervention and revealed viable abdominal organs except for
the gallbladder, which was removed. During the patient’s 58-day
ICU stay, multiple complications occurred, including local and
systemic infections, renal failure secondary to rhabdomyolysis
and reactive depression with profound anxiety.
OUTCOME AND FOLLOW-UP
After transfer from the ICU, a further month of admission to
the vascular surgery ward was followed by another 3.5 months
of inpatient spinal rehabilitation before discharge. The patient
had regained some sensory function of the lower extremities but,
unfortunately, he remained wheelchair-dependent.
The patient was investigated by the coagulation department, but
no coagulation disorder was identified and the underlying reason
for developing this massive aortic thrombosis remains a mystery.
DISCUSSION
Aortic thrombosis in an otherwise normal aorta is a rare occur-
rence.1 2
A link between IBD and thromboembolism has been estab-
lished, but primarily in the venous system.3 A link between IBD
and aortic thromboembolism has also been suggested.4 One
can only speculate that this case is related to IBD as no actual
evidence to support this explanation is available.
The patient was previously engaged in fitness competitions
(akin to body building), and the issue of anabolic steroid use was
raised on multiple occasions, something that the patient denied
decidedly, and continues to deny to this day. He was also tested
negative for illicit substances in conjunction with a competition
only 6 months prior to the incident.
In previously published cases of aortic thrombosis, the
outcomes range from full recovery to death. Azzarone et al
reported full recovery in a patient with an operative approach
similar to this case,2 while Matsuoka and Hashizume reported
a case where the patient suffered cardiopulmonary arrest while
waiting for the CT to be performed.5 The patient described
here was diagnosed promptly and treated in a timely fashion,
but nonetheless suffered severe complications. This illustrates
and stresses the severity of this rare condition as a true vascular
emergency.
Contributors MM: planned, conducted and reported.
Funding The authors have not declared a specific grant for this research from any
funding agency in the public, commercial or not-for-profit sectors.
Competing interests None declared.
Patient consent for publication Obtained.
Provenance and peer review Not commissioned; externally peer reviewed.
Open access This is an open access article distributed in accordance with the
Creative Commons Attribution Non Commercial (CC BY-NC 4.0) license, which
permits others to distribute, remix, adapt, build upon this work non-commercially,
and license their derivative works on different terms, provided the original work
is properly cited and the use is non-commercial. See: http:// creativecommons. org/
licenses/ by- nc/ 4. 0/.
REFERENCES
1 Fayad ZY, Semaan E, Fahoum B, etal. Aortic mural thrombus in the normal or minimally
atherosclerotic aorta. Ann Vasc Surg 2013;27:282–90.
2 Azzarone M, De Troia A, Iazzolino L, etal. Hybrid treatment of acute abdominal aortic
thrombosis presenting with paraplegia. Ann Vasc Surg 2016;33:228.e5–8.
3 Giannotta M, Tapete G, Emmi G, etal. Thrombosis in inflammatory bowel diseases:
what’s the link? Thromb J 2015;13:14.
4 Novacek G, Haumer M, Schima W, etal. Aortic mural thrombi in patients with
inflammatory bowel disease: report of two cases and review of the literature. Inflamm
Bowel Dis 2004;10:430–5.
5 Matsuoka Y, Hashizume M. Cardiopulmonary arrest caused by acute abdominal aortic
thrombosis: a case report. BMJ Case Rep 2009;2009:bcr09.2008.0947.
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ManieriM. BMJ Case Rep 2019;12:e228281. doi:10.1136/bcr-2018-228281
Rare disease
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