International Journal of Surgery Case Reports 90 (2022) 106713

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International Journal of Surgery Case Reports

journal homepage: www.elsevier.com/locate/ijscr

Case report

Ruptured abdominal aortic aneurysm in a young male patient, a rare

case report
Kush Raj Lohani *, Geraldine Wong Yan Xin, Jiaqian Cui, Vikram Vijayan Sannasi
Division of Vascular Surgery, Department of General Surgery, Ng Teng Fong General Hospital, National University Health System, Singapore

A R T I C L E I N F O A B S T R A C T

Keywords: Introduction and importance: Abdominal aortic aneurysm (AAA) is commonly a disease of the elderly population
Abdominal aortic aneurysm with an atherosclerotic aorta. We present a rare case scenario of a large ruptured AAA in a young patient.
Early onset aortic aneurysm Case presentation: A 32-year-old man presented to the Emergency Department with abdominal pain. On exami­
Connective tissue disorder
nation he had hypotension with a severely tender abdomen. Imaging revealed a ruptured 10 cm abdominal aortic
Case report
aneurysm (AAA). He underwent an emergency open aneurysm repair and was discharged well on post-operative
day 12. Apart from smoking, he had no known significant risk factors contributing to an AAA of such size.
Clinical features and family history suggested a possible underlying connective tissue disorder.
Clinical discussion: A painful abdomen and hypotension in a young patient should prompt investigations to rule
out a rare but life-threatening diagnosis of a ruptured AAA.
Conclusion: A possible underlying connective tissue disorder should be investigated for in any young patient
presenting with an AAA.

1. Introduction Examination revealed a severely tender and tense abdomen. A

bedside ultrasound demonstrated free fluid, with a large 8x9cm AAA. A
An abdominal aortic aneurysm (AAA) is defined as an aorta having a CT aortogram further confirmed tortuous and aneurysmal dilatation of
diameter of at least one and a-half times the normal diameter of the the abdominal aorta measuring up to 10.8 × 10.8 cm in axial dimension
aorta at the level of the renal arteries [1]. Common risk factors for AAA and 24.1 cm in cranio-caudal length extending into the left common iliac
include advancing age, smoking, hypercholesterolemia, hypertension artery, with the proximal aspect involving the origin of the bilateral
and the male gender. AAAs are more common in patients with athero­ renal arteries. A large retroperitoneal hematoma was also seen (Fig. 1a,
sclerosis, but other pathologies have been associated as well including b,c).
connective tissue diseases such as Ehlers-Danlos syndrome and infective The patient underwent an emergency open abdominal aortic aneu­
diseases such as syphilis or HIV [2,3]. rysm repair (Fig. 2). The surgical procedure and overall management of
This case report has been reported in accordance with the SCARE patient was performed under the leadership of VVS, a Senior Consultant
criteria [4]. Vascular Surgeon with decades of experience in open and endovascular
repair of Aortic emergencies. Access was difficult and due to the hae­
2. Presentation of case modynamic instability and the need for an expedited procedure, an
aortic cross clamp was applied beneath the right renal artery but just
A 32-year-old Vietnamese man presented to the emergency depart­ proximal to left renal artery. This gave sufficient room for a bifurcated
ment with sudden onset severe abdominal pain associated with syncope aortic graft to be anastomosed proximally without need for renal artery
and hypotension (systolic blood pressure 50+). He was a smoker of 2- implantation and distally to the bilateral common iliac arteries.
pack year history, an occasional drinker and worked as a technician in An on table angiogram of the left lower limb was also performed as it
a factory. There was no change in bowel habit, no recent loss of appetite appeared pale intraoperatively. This demonstrated an abrupt cut-off at
or loss of weight and he had never experienced abdominal pain like this the level of the left popliteal artery. A transverse arteriotomy was made
before. over the left common femoral artery and a Fogarty balloon catheter was

* Corresponding author.
E-mail addresses: [email protected], [email protected] (K.R. Lohani).

https://doi.org/10.1016/j.ijscr.2021.106713
Received 19 November 2021; Received in revised form 13 December 2021; Accepted 14 December 2021
Available online 22 December 2021
2210-2612/© 2021 Published by Elsevier Ltd on behalf of IJS Publishing Group Ltd. This is an open access article under the CC BY-NC-ND license
(http://creativecommons.org/licenses/by-nc-nd/4.0/).
K.R. Lohani et al. International Journal of Surgery Case Reports 90 (2022) 106713

used to trawl out multiple thrombi. A final angiogram demonstrated in-

line flow to the foot via the anterior tibial artery. Foot pulses were
palpable and strong at the end of the procedure.
The patient was transferred to the intensive care unit (ICU), and was
extubated on post-operative day (POD) 1. He was given intensive chest
physiotherapy and incentive spirometry and started on nasogastric
feeding on POD2, escalating to oral diet by POD 6. On the 4th day of his
ICU stay he developed a fever, likely secondary to basal atelectasis. He
was empirically started on a course of piperacillin-tazobactam, which
was stopped 4 days later in view of a negative septic work up and down-
trending inflammatory markers. His pro-calcitonin level was within
normal limits. The surgical sites were healing well with no hematoma or
signs of infection. During his ICU stay, the patient developed an initial
acute kidney injury which was managed with proper hydration with
normalising of his renal function. A transient liver enzyme rise was also
noted which resolved gradually. His coagulation profile was preserved.
By POD 12, he was clinically stable, independently mobile and fit for
discharge from hospital. The patient was grateful to the surgical and
intensive care unit for saving his life and taking good care of him.
Histopathological examination revealed degenerative changes of the
aortic wall that was consistent with aortic rupture. However, no gran­
ulomas, giant cells, obliterative phlebitis or storiform fibrosis nor ma­
lignancy were identified. The appearance was also not suggestive of
tuberculosis, vasculitic changes or IgG4 sclerosing disease. There was no
evidence of any infective process in the aortic wall on microbiological
investigation.
The etiology of the AAA was of interest due to its large size and his
Fig. 2. Intraoperative picture demonstrating the aneurysmal dilatation of
young age at presentation and he had multiple investigations while in abdominal aortic aorta (yellow arrow). Bowel has been eviscerated into bowel
hospital. However, soon after his discharge, he returned to Vietnam to bag to the patient's right and the retractor is on the left. Surgeon's hand is
be with his family and was lost to follow up before genetic testing could holding the transverse colon cranially. (For interpretation of the references to
be arranged. colour in this figure legend, the reader is referred to the web version of
this article.)
3. Discussion
ectopic pregnancy. A ruptured abdominal aortic aneurysm is a very rare
Common causes of an acute abdomen with hypovolemic shock in a contributing factor. However, anticipation with prompt diagnosis and
young patient usually include a bleeding intestinal ulcer or a ruptured treatment of such a grievous condition cannot be overemphasized.

Fig. 1. CT aortogram (a. Axial section b. Coronal Section c. Parasagital section) demonstrating the large ruptured abdominal aortic aneurysm (10.8 × 10.8 × 24.1
cm) (yellow arrow) and the retroperitoneal haematoma (red arrowhead). (For interpretation of the references to colour in this figure legend, the reader is referred to
the web version of this article.)

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K.R. Lohani et al. International Journal of Surgery Case Reports 90 (2022) 106713

Aortic aneurysms have a strong association with atherosclerosis and classically characterised by arterial tortuosity and aneurysm formation,
arterial remodeling resulting in thinning of the tunica media and hypertelorism and bifid uvula or cleft palate [11]. MFS mainly presents
decrease in elastin and collagen [5]. Risk factors such as hypercholes­ with lens dislocation, myopia and mitral valve prolapse whereas the
terolemia, hypertension and smoking are commonly attributed to vascular type of EDS presents with fragile skin and lax joints. Our patient
atherosclerosis. In addition, several connective tissue disorders have displayed mild cranio-facial features, a tortuous aneurysmal aorta,
been implicated in aneurysms of the young population. Genetic associ­ arachnodactyly and pectus likely fitting into LDS type IV. He also
ation is noted mainly in proximal or thoracic aortic aneurysms of the developed keloid at the midline laparotomy wound which further sup­
young population and can be syndromic or sporadic [6–8]. Syndromic ports a possible TGFB pathway abnormality as noted in LDS [12]. Our
aortopathies are usually autosomal dominant disorders mainly due to patient had a juxta-renal aortic aneurysm supporting the proximal aortic
defect in the extracellular matrix such as fibrillin-1 of elastin in Marfan aneurysms seen in connective tissue disorders. Type IV EDS presents
syndrome (MFS) and type 3 collagen in vascular type (type IV) of Ehlers- with loss of tensile strength, vascular fragility and brittle tissue and is
Danlos syndrome (EDS), cytokine pathway overexpression of trans­ usually associated with poor operative outcome as compared to LDS
forming growth factor beta (TGFB) pathway in Loeys-Dietz syndrome [11]. Our patient also survived the acute contained rupture of his large
(LDS) or ciliopathy like autosomal dominant polycystic kidney disease. juxta-renal aortic aneurysm favouring LDS over EDS.
Whereas, non-syndromic aneurysms can be due to defects in neural crest Our patient presented with very high C-reactive protein (241.6 mg/
migration (bicuspid aortic valves), smooth muscle contraction proteins L) that remained elevated on discharge (71.6 mg/L). His erythrocyte
(familial disorders) or those involving TGF-beta pathway resulting in sedimentation rate was also elevated at 35 mm/h. The elevated CRP
Loeys-Dietz variants. reflects the severe acute systemic inflammatory state relating to the
The probability of a connective tissue disorder with genetic predis­ aortic wall degeneration [ [13,14]] with associated poor prognosis [15].
position is high in our patient. Early onset aortic aneurysms (<51 years) AAAs are commonly associated with atherosclerosis and inflammation
tend to be associated with smoking, other more commonly identifiable while thoracic aortic aneurysms are mainly hereditary without signifi­
causes of aneurysms, be more symptomatic, more commonly proximal cant atheroma [8]. About 20% of thoracic aortic aneurysms or dissec­
aorta (juxta renal, supra renal and thoraco- abdominal) and larger sized tions display an autosomal dominant pattern of inheritance while
aneurysms (>6.9 cm at presentation) [9]. This is similar to the scenario abdominal aortic aneurysms usually do not [16]. However, our patient
of our patient who was 32 years old and a smoker, presenting acutely had no evidence of atheroma on imaging and had normal serum
with abdominal pain and with a 10 cm juxta-renal aortic aneurysm. cholesterol. Also, normal homocysteine level precluded the chances of
The patient and his family originated from Nghe An in Central precocious atherosclerosis and aneurysmal dilatation of aorta.
Vietnam. He was married with a healthy 5-year-old son. However, his Young-onset aortic aneurysms are also noted in rheumatological
mother had significant bilateral hallux valgus. He had 2 younger sib­ conditions such as systemic lupus erythematosus [17]. Our patient
lings, the youngest being a 25-year-old male with a history of pectus lacked the tell-tale clinical features of rheumatological disorders such as
carinatum, which developed in his teenage years. Otherwise, there was dry eyes, xerostomia, abnormal hair loss or rashes. The histopatholog­
no known cardiac, lung or rheumatological disease in the family. The ical examination of the aortic wall was also unremarkable for any
patient was notably taller than his peers and stood at 179 cm tall. His vasculitis.
body mass index was 24.2 kg/m2. He had an average head width of 19 We explained to the patient multiple times about the need for genetic
cm and head length of 22 cm, a normal arched palate and a triangular testing for prognostication and the possible need to screen family
uvula centre. He did have a mild form of pectus excavatum. There was members. We also offered to provide free genetic testing. However, the
no obvious scoliosis or truncal striae. His fingers and toes were unusu­ patient was lost to follow up before this could be arranged. Echocardi­
ally long and slender without any deformities or positive thumb sign. He ography was also not performed that would help to characterize any
also had bilateral pes planus. (Fig. 3). Despite the lack of histological concomitant aortic root or cardiac involvement.
evidence, these clinical features point towards a connective tissue
disorder. 4. Conclusion
There might be phenotypic overlap among patients with MFS,
vascular type (type IV) EDS and LDS. However, a few distinctive features We present a rare case of a ruptured AAA in a young man with likely
might help to differentiate them [10]. Our patient likely fits into LDS. connective tissue disorder. He was successfully treated and discharged
Loeys-Dietz is a relatively new connective tissue disorder and is with good post-operative outcomes. It is imperative to consider the rare

Fig. 3. Photos of the right and left hands and feet of the patient demonstrating arachnodactyly and pes planus.

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K.R. Lohani et al. International Journal of Surgery Case Reports 90 (2022) 106713

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None.