Postgrad Med J: first published as 10.1136/pgmj.69.807.6 on 1 January 1993. Downloaded from Postgrad Med J (1993) 69, 6- 11 © The Fellowship of Postgraduate Medicine, 1993

Review Article Atypical manifestations ofruptured abdominal aortic A. Banerjee

Accident and Emergency Department, East Birmingham Hospital, Bordesley Green East, Birmingham B95ST, UK

Introduction The rupture of an abdominal aortic is a The majority of published series of ruptured catastrophic event with a uniformly fatal outcome abdominal aortic aneurysms deal with the outcome if untreated. The triad of abdominal and/or back of those submitted to surgery. Untreated cases, pain, a pulsatile abdominal mass, and , misdiagnoses and delayed diagnoses are generally is said to be diagnostic. However, this triad may not not discussed. Hence the precise extent of these be present in its entirety, or when present may not problems is unclear. be recognized, as one of the components However, data from various sources suggest that predominates. Clinical diagnosis is thus 'not infre- the diagnosis of ruptured abdominal aortic quently missed in the emergency rooms ofeven the aneurysms is difficult and often missed. In a study most prestigious medical centers." of 9.894 autopsies in two Glasgow hospitals,6 41 To add to this, difficulties in diagnosis may arise patients were noted to have died in hospital with owing either to an impalpable aneurysm or to unoperated ruptured abdominal aortic aneurysms. copyright. atypical presentations.2'3 The clinical diagnosis is The diagnostic triad was present in nine. The often difficult and not infrequently missed. Conse- correct diagnosis had been made in 24. In a series of quently, appropriate treatment in the form of 100 ruptured aneurysms admitted to hospital,7 in expeditious laparotomy, rapid control of the prox- 58 cases surgery was delayed. In 42 instances this imal aorta and graft replacement ofthe aneurysmal was due to an incorrect diagnosis. Emergency aorta is not achieved leading to possibly avoidable operation was performed for an incorrect diagnosis mortality. in five cases. The purpose of this report is to discuss less The implications of this are thatjunior staff may http://pmj.bmj.com/ common presentations of ruptured aneurysms of not be prepared to deal with the ruptured aneurysm the abdominal aorta, concentrating on the clinical when encountered and cross-matched may aspects. not be available. Of 44 admissions to a hospital with ruptured aneurysms of the abdominal aorta,8 in 15 patients Extent ofthe problem the diagnosis was missed altogether. In another

study of 47 patients with ruptured abdominal on September 28, 2021 by guest. Protected The incidence of abdominal aortic aneurysms who died in hospital over a 4 year appears to be rising, independent of any increased period, the diagnostic triad was noted in only 19 detection from improved diagnostic techniques.4 In patients.9 Twenty-four patients underwent surgery, the absence of any effective community screening only 16 having had the correct preoperative diag- programme and also because symptomatic aneur- nosis. ysms are treated surgically at an early stage, In a series of 119 patients with ruptured ruptured previously undiagnosed aneurysms con- abdominal aortic aneurysms who reached a hos- tinue to arrive in hospital. Methods to detect pital alive,'0 20 died in the Accident and Emergency aneurysms based on community screening by Department (only five being diagnosed correctly), clinical examination are, in any case, likely to be and 24 died on the ward without surgery (nine inaccurate.5 This implies that rupture is often the being undiagnosed). first clue to the presence of an aneurysm. Of 120 patients with ruptured abdominal aortic aneurysms in one health district," 12 died in hospital from undiagnosed rupture. Of 56 deaths Correspondence: A. Banerjee, F. R.C.S. outside hospital, 27 had been seen before death by a Accepted: 18 June 1992 doctor but the correct diagnosis was not suspected. Postgrad Med J: first published as 10.1136/pgmj.69.807.6 on 1 January 1993. Downloaded from RUPTURED ABDOMINAL AORTIC ANEURYSMS 7

In the above series, failure to consider the correct presentation of abdominal aortic aneurysmal rup- diagnosis and to recognize atypical presentations ture, in association with fistula formation with contributed directly to most of the deaths. It is either the inferior vena cava or the left renal unfortunately not possible to comment on the .'8-20 Haematuria in the presence of a symp- relative proportions. Inappropriate orthopaedic, tomatic aneurysm must be considered as being due urological, neurological and medical referrals to the aneurysm until proven otherwise. Systemic resulted in many instances. Commonly recorded circulatory effects ofthe fistula must be sought. The misdiagnoses are enumerated in Table I. haematuria has been attributed to venous conges- tion of the kidney and bladder, and characteris- tically clears promptly with resection of the Urological presentations aneurysm. Urological presentations are not infrequent. Ten per cent of a series of 400 patients with abdominal Neurological presentations aortic aneurysms (intact and ruptured) who under- went surgery for the aneurysm presented with The precise incidence of neurological complica- features suggesting genitourinary disease.12 tions associated with ruptured abdominal aortic Unilateral flank pain, either right- or left-sided, aneurysm is not known. However, they appear to with distal radiation to the genitalia, hip and thigh, be relatively uncommon. A ruptured abdominal is often a presenting feature ofaneurysmal rupture. aortic aneurysm can present as an acute unilateral This may be misdiagnosed as ureteric colic.'3 4 The painful neuropathy affecting the femoral and/or cause of this pain is believed to be irritation of the obturator nerves.2'125 A similar constellation of ureteric pain fibres in the sympathetic plexus by the symptoms and signs has been previously en- retroperitoneal haematoma. This appears to be a countered with patients on anticoagulant therapy relatively common diagnostic problem. In one and in haemophiliacs. The mechanism is that of series of 44 cases of aneurysmal rupture, five were pressure on these nerves by the extraperitoneal noted to have ureteric colic-like presentation.8 In haematoma present in each instance. copyright. another series of 105 patients, 18 presented with The presentation is with severe hip and thigh unilateral loin pain.'5 pain, paraesthesiae and sensory loss of femoral In many cases, the misdiagnosis of ureteric colic nerve distribution (anteromedial thigh), weakness leads to inappropriate intravenous urography, of hip flexion (iliopsoas) and of knee extension with deterioration during, or immediately after, the (quadriceps femoris) and loss of the knee jerk. In procedure (Figure 1). One must be wary ofthe first many reported instances, inappropriate neuro- episode of ureteric colic in the middle aged and logical and orthopaedic investigation has been elderly, and leaking abdominal aortic aneurysm associated with delayed diagnosis. The neuropathy http://pmj.bmj.com/ must be part of the differential diagnosis. In the is reversible with early definitive treatment of the haemodynamically stable patient, ultrasound aneurysm but, if this is delayed, residual assessment is recommended.'6 This can demon- neurological deficit with prominent quadriceps strate both aneurysmal leakage as well as urinary wasting ensues. Intractable sciatica owing to sciatic tract obstruction. Three cases of isolated severe nerve compression by haematoma has also been unilateral have been reported as the described. The presentation has been with severe sole presentation of aneurysmal rupture."' The gluteal pain, restricted straight leg raising and postulated mechanism is haematoma causing pres- normal neurological examination.26 Clearly in the on September 28, 2021 by guest. Protected sure on visceral afferent pain fibres from the testes. situation with a normal lumbar spine X-ray and Massive haematuria has been described as a aortic calcification, one must be wary of ruptured abdominal aortic aneurysm. Other reported presentations include lateral Table I Common misdiagnoses in patients with popliteal nerve palsy with foot drop (attributed to ruptured abdominal aortic aneurysms occlusion of the to the sciatic nerve)27 and various root compression syndromes.28 Acute Ureteric colic paraplegia has also been reported as a presentation Prolapsed lumbar intervertebral disc of aneurysmal rupture,27'29 although this is more Sciatica likely to occur as a result ofaortic or as Acute myocardial infarction a postoperative complication. Acute sympathetic Perforated peptic ulcer paralysis causing a unilateral warm dry foot can Acute pancreatitis Acute cholecystitis occur as a complication of aneurysmal rupture. Mesenteric vascular occlusion Haematoma causing damage to the sympathetic Acute diverticulitis ganglia has been implicated as the cause.30 The message is that acute painful neuropathies in Postgrad Med J: first published as 10.1136/pgmj.69.807.6 on 1 January 1993. Downloaded from 8 A. BANERJEE the lower limbs and acute nerve root compression .41- This may lead to inguinal syndromes in the middle aged or elderly can be herniorrhaphy as an emergency procedure, when a caused by aortic aneurysmal rupture, which must haemorrhagic hernial sac and/or retroperitoneal be considered in the differential diagnosis. tissues may be noted. These, in conjunction with hypotension and anaemia, should suggest the cor- rect diagnosis. Orthopaedic presentations Intractable backache of acute or subacute onset Gastrointestinal presentations may be the sole presentation of ruptured abdominal aortic aneurysms. This, owing to its Abdominal aortic aneurysms may rupture into the frequency cannot be considered to be an atypical gastrointestinal tract, usually into the duo- presentation. Often, in this instance, diagnostic denum45 51 or less commonly into the colon.52-53 failure appears to be due to lack of awareness and Aortoenteric fistulas are notoriously difficult to failure to examine the abdomen. diagnose. The triad of abdominal or flank pain, However, a more chronic form of backache has intermittent gastrointestinal bleeding and a pul- been described in conjunction with chronic con- satile abdominal mass is diagnostic. tained ruptures of aortic aneurysms.3-34 The Upper gastrointestinal bleeding is characteris- special features ofthis entity are: (a) the presence of tically intermittent, with minor 'herald' or 'sentinel' abdominal aortic aneurysm; (b) previous pain that bleeds preceding an episode of major exsan- may have resolved; (c) a stable patient with normal guinating haemorrhage. Gastrointestinal contrast haematocrit; (d) computed tomography (CT) scan studies and endoscopy are usually non- showing retroperitoneal haematoma (soft tissue contributory. Diagnosis may be confirmed by mass silhouetting psoas muscle, associated with either flush aortography or CT scanning. Selective renal displacement); and (e) pathological con- arteriography may fail to demonstrate the lesion. firmation of organized haematoma. Any gastrointestinal bleeding in the presence of a A rare with severe chronic presentation symptomatic aneurysm of the abdominal aorta copyright. backache and systemic symptoms mimicking sepsis must be considered as being due to the aneurysm in association with vertebral body erosion and disc until proven otherwise. space narrowing has also been described.35 One Recurrent lower gastrointestinal bleeding with must beware of chronic intractable backache discharge offresh blood is the presenting feature of especially where spinal X-rays do not show a aortocolic fistulas. Abdominal pain and systemic contributory lesion. toxicity may be associated. The diagnosis is usually missed and the condition is almost uniformly fatal. Rarely, ruptured aneurysms can present with pain- Presentations with manifestations of ful obstructive jaundice'M or a cholecystitis-like http://pmj.bmj.com/ retroperitoneal bleeding picture owing to bleeding around the gallbladder." The latter presentation was said to herald the Ruptured abdominal aortic aneurysms can present demise of Albert Einstein. with secondary manifestations of extensive retro- peritoneal bleeding. They can divert attention from the primary pathological process, although these Cardiovascular complications

are seldom isolated presentations. on September 28, 2021 by guest. Protected Rapid onset of a bulging diamond-shaped Rupture of the aneurysmal abdominal aorta into perianal haematoma has been reported.36-38 This is either the inferior vena cava or the left renal vein due to extraperitoneal rupture ofthe aneurysm into are well described. A total of 159 cases of aorto- the sigmoid mesocolon. The haematoma therefore caval fistula from the world literature were extends into Waldeyer's fascia and transverses the reviewed in a recent article56 and this complication levator ani muscle sling into the ischiorectal fossa. was noted to occur with 3-4% of ruptured Localization in the ischiorectal fossa accounts for abdominal aortic aneurysms. The syndrome of the characteristic shape. Subsequently tracking aortocaval fistula is well delineated.57'58 It is typical across into the subcutaneous perianal space occurs. that, in spite of a multitude of physical signs, the Retroperitoneal bleeding has been known to pro- diagnosis is missed owing to lack of awareness of duce periumbilical bruising, known as Cullen's the condition. sign.39 Presentation with Cullen's sign has accord- The features of aortocaval fistula include: ingly been noted with aneurysmal rupture.' a. Severe abdominal or back pain Another effect of retroperitoneal bleeding is to b. Systemic venous overload with acute onset high cause acute incarceration of a previously reducible output congestive cardiac failure owing to the , owing to increased pressure in the large arteriovenous shunt. This causes a Postgrad Med J: first published as 10.1136/pgmj.69.807.6 on 1 January 1993. Downloaded from RUPTURED ABDOMINAL AORTIC ANEURYSMS 9

Table II Suspect ruptured abdominal aortic aneurysm Unexplained severe abdominal or back pain in middle aged or elderly Ureteric colic in the elderly Acute painful femoral neuropathy Unexplained high output cardiac failure Unexplained compression of inferior vena cava Acute incarceration of inguinal hernia Unexplained gastrointestinal bleeding, especially if recurrent Unexplained massive haematuria

Figure 1 Intravenous urogram showing lateral displace- ment of left kidney and ureter by ruptured aortic The clinical diagnosis can usually be confirmed aneurysm. The patient died a few minutes later. (Kindly angiographically or by CT scanning. supplied by Dr D. Hakhamaneshi, M.D., F.R.C.R.) Aorto-left renal vein fistula is much rarer6'1-66 but can be suspected in the presence of left flank (ureteric colic-like) pain, a pulsatile abdominal widened pulse pressure, with reduced diastolic mass, haematuria, proteinuria and azotemia. The , resulting in bounding peri- large majority of reported cases occur in the pheral pulses, and venous with a presence of an anomalous left retro-aortic renal raised jugular venous pressure. The shunt fur- vein which occurs in 2% ofthe general population.

ther produces a widely transmitted bruit accom- Left renal vein hypertension may cause rapid onset copyright. panied by a palpable thrill. The murmur is of a left scrotal varicocele.67 Features of high usually continuous but may be only systolic or output circulatory state are usually lacking owing absent in the presence of intraluminal throm- to the much smaller shunt. Intravenous urography bosis or venous outflow tract obstruction by the shows the left kidney to be enlarged and non- aneurysm. The heart failure is characteristically functioning. Aortography is diagnostic. refractory to the usual anti-failure regimens. c. Systemic arterial insufficiency causing myocar- dial ischaemia (angina),59 cerebral ischaemic Conclusions http://pmj.bmj.com/ (syncope) and renal insufficiency (oliguria or anuria). The diagnosis of a ruptured abdominal aortic d. Regional venous overload with massive lower aneurysm can be difficult. This is partly due to the limb oedema extending to the lower trunk, fact that this condition mimics a variety of other haematuria, rectal bleeding and priapism. This abdominal and extra-abdominal conditions (Table constellation can occur in the absence of heart II). Greater awareness ofthe varied clinical presen- failure by a mechanism of inferior vena caval tations is necessary as well as of the value ofurgent obstruction.6 This causes confusion with ret- ultrasonic assessment in establishing the diagnosis on September 28, 2021 by guest. Protected roperitoneal tumours. in haemodynamically stable patients. e. Paradoxic via fistula into the pul- monary circulation. f. Branham Nicoladoni sign ofreflex bradycardia Acknowledgement on aortic compression, usually noted intraoperatively, but rarely demonstrable pre- I wish to thank Mrs A. Danks for kindly typing the operatively. manuscript.

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