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Congenital Abdominal Aortic Aneurysm in a Four Year Old Girl

Open AccessPublished:May 20, 2020DOI:https://doi.org/10.1016/j.ejvsvf.2020.05.004

      Highlights

      • Idiopathic congenital abdominal aortic aneurysm (CAAA) is an extremely rare condition.
      • A CAAA with multiple aneurysms was detected.
      • The CAAA was successfully repaired with a Dacron graft.
      • Aneurysms of the left renal and middle meningeal arteries increased post-operatively.
      • Frequent multimodality follow up imaging for multiple aneurysms is necessary.

      Introduction

      Abdominal aortic aneurysm (AAA) in neonates, infants, and children is uncommon, usually occurring as a result of infections, connective tissue disorders, vasculitis, or iatrogenic trauma. A case of idiopathic congenital AAA, an extremely rare disease of unknown origin, is described.

      Report

      In March 2018, a 40 mm hypoechoic mass adjacent to the left kidney was detected incidentally by abdominal ultrasound for investigation of hypercalciuria in a four year old girl. Contrast enhanced computed tomography (CT) revealed an infrarenal fusiform AAA measuring 39 mm in maximum diameter, a 15 mm left renal artery aneurysm, a 14 mm right hypogastric artery aneurysm, and a 12 mm left hypogastric artery aneurysm. Cerebral magnetic resonance imaging revealed multiple intracranial aneurysms between 8 and 15 mm in diameter. Considering the size of the AAA and risk of rupture, surgical repair was planned. In May 2018, the congenital AAA was successfully repaired with a 10 mm Dacron aorto-aortic tube graft. Increases in the size of the left renal artery aneurysm and a left middle meningeal artery aneurysm were detected 12 and 14 months post-operatively, respectively. Coil embolisations were performed. An intracranial dural arteriovenous fistula (AVF) was discovered incidentally by cerebral angiography for treatment of the left middle meningeal artery aneurysm. Transarterial embolisation for AVF was also performed. At the 21 month post-operative follow up, the patient is doing well, and the untreated aneurysms have not grown.

      Conclusion

      Long term outcomes after surgical repair for congenital AAA are unclear. Moreover, growth of residual aneurysms was detected post-operatively, so follow up with frequent multimodality imaging for multiple aneurysms is necessary.

      Keywords

      Introduction

      Abdominal aortic aneurysm (AAA) in neonates, infants, and children is uncommon and usually caused by bacterial, tuberculous or fungal infection; congenital connective tissue disorders (Marfan syndrome, Ehlers–Danlos syndrome, or Loeys–Dietz syndrome), vasculitis (Takayasu arteritis, Kawasaki syndrome, or polyarteritis nodosa); or trauma from umbilical artery catheterisation.
      • Wang M.
      • Tao Y.
      Diagnosis and treatment of congenital abdominal aortic aneurysm: a systematic review of reported cases.
      Idiopathic congenital AAA (CAAA) is an extremely rare disease of unknown origin. A patient diagnosed with an idiopathic CAAA with multiple aneurysms is described.

      Case report

      In March 2018, a 40 mm hypoechoic mass adjacent to the left kidney was detected incidentally by abdominal ultrasound in a four year old girl with a two year history of cataract, hypercalciuria, and proteinuria. It was initially suspected to be a cystic lesion. Two months later, contrast enhanced computed tomography (CT) revealed an infrarenal fusiform AAA measuring 39 mm in maximum diameter, a 15 mm left renal artery aneurysm, a 14 mm right hypogastric artery aneurysm, and a 12 mm left hypogastric artery aneurysm (Fig. 1A). She had no family history of aneurysmal disease or connective tissue disorders. Her blood pressure was 112/63 mmHg. Clinical examination was unremarkable except for a palpable pulsatile abdominal mass. Cerebral magnetic resonance imaging (MRI) revealed multiple intracranial aneurysms between 8 and 15 mm in the intracavernous portion of the right internal carotid artery and bilateral middle meningeal arteries (Fig. 1B). The patient was referred for further management of the multiple aneurysms. Neurosurgeons considered that surgery or coil embolisation for the multiple cerebral aneurysms carried a high risk of intra-operative rupture. The maximum diameter of the AAA was more than four times the diameter of the normal abdominal aorta. Considering the size and risk of AAA rupture, surgical repair was planned. Regarding the other aneurysms, it was considered that revascularisation was extremely difficult because the normal diameter of the vessels was too small. Therefore, conservative management was planned and angiotensin Ⅱ receptor blocker and beta blocker administration was initiated to prevent hypertension.
      Figure thumbnail gr1
      Figure 1Preoperative images of the abdominal aortic aneurysm and multiple aneurysms. A. Three-dimensional reconstruction of preoperative computed tomography angiography image showing an infrarenal abdominal aortic aneurysm with a maximum aortic diameter of 39 mm, a left renal artery aneurysm, and bilateral internal iliac artery aneurysms. B. Three-dimensional reconstruction cerebral magnetic resonance image showing multiple intracranial aneurysms.
      During the operation (May 2018), the AAA was exposed through a midline incision. The diameter of the infrarenal AAA was 41 mm, and it extended to the aortic bifurcation (Fig. 2A). After heparin infusion at 80 U/kg, the aorta and both common iliac arteries were cross clamped. The aortic aneurysm was opened, and a 10 mm Dacron aorto-aortic tube graft was anastomosed proximally to the infrarenal abdominal aorta and distally to the aortic bifurcation with a running 4-0 Prolene suture. Reconstruction of the inferior mesenteric artery was performed with a running 7-0 Prolene suture considering the risk of intestinal ischaemia following possible subsequent treatment of bilateral hypogastric artery aneurysms (Fig. 2B). A histological examination of the aneurysmal wall revealed thinning of the media with irregularity and fragmentation of the elastic lamina. There was no evidence of cystic degeneration of the media, active aortitis, or infection. These changes in the media were considered the cause of aneurysmal formation. Additionally, diffuse fibro-elastic thickening of the intima, chronic media dissection, slight chronic inflammatory infiltrate, adventitial fibrosis, and dilatation of the vasa vasorum, which were considered secondary changes, were observed (Fig. 3). Whole exome sequencing showed no genetic mutation of the known congenital connective tissue disorders associated with aneurysmal diseases. Contrast enhanced CT on post-operative day seven confirmed complete exclusion of the AAA with patency of the graft and reconstructed inferior mesenteric artery (Fig. 4). Aspirin administration was initiated for post-operative graft thromboprophylaxis. At the 12 month post-operative follow up, abdominal MRI revealed an increase of the left renal artery aneurysm from 15mm to 25 mm (Fig. 5A). Coil embolisation was therefore performed successfully for this aneurysm. Moreover, cerebral MRI revealed an increase of a left middle meningeal artery aneurysm from 15 mm to 19 mm (Fig. 5B) at the 14 month post-operative follow up, for which coil embolisation was also performed successfully. Simultaneously, a superior sagittal sinus dural arteriovenous fistula (AVF) was discovered incidentally on cerebral angiography (Fig. 5C), and transarterial embolisation with N-butyl-2-cyanoacrylate for AVF was also performed. Post-procedure MRI showed disappearance of blood flow into the enlarged aneurysms and decrease of abnormal blood flow into the AVF. At the 21 month post-operative follow up, the patient is doing well, and the untreated aneurysms have not increased in size, as seen on follow up imaging studies.
      Figure thumbnail gr2
      Figure 2Operative findings. A. Operative photograph showing an infrarenal abdominal aortic aneurysm with a maximum aortic diameter of 41 mm. B. Operative photograph of implanted 10-mm Dacron graft in the infrarenal aortic position.
      Figure thumbnail gr3
      Figure 3Pathological findings in the surgically resected abdominal aorta. A. The image shows irregularity and focal loss of the elastic lamina of the media, fibroelastic intimal thickening, and dilatation of the vasa vasorum (arrows). The upper portion of the media shows chronic dissection (asterisk) surrounded by thick elastic tissue (elastica van Gieson stain). B. The high-magnification image of the media (square region of A) shows fragmentation of elastic fibers (elastica van Gieson stain).
      Figure thumbnail gr4
      Figure 4Contrast-enhanced computed tomography on postoperative day 7. Three-dimensional reconstruction of postoperative computed tomography angiography showing patency of the graft and reconstructed internal mesenteric artery.
      Figure thumbnail gr5
      Figure 5Postoperative follow-up imaging. A: Magnetic resonance image at the 12-month follow-up showing an increase in size of the left renal artery aneurysm from 15 mm to 25 mm. B: Magnetic resonance image at the 14-month follow-up showing an increase in size of the left middle meningeal artery aneurysm from 15 mm to 19 mm. C: Cerebral angiography showing superior sagittal sinus dural arteriovenous fistula.

      Consent

      Written consent for publication of this article was obtained from the parents of the patient.

      Discussion

      Idiopathic congenital AAA is an extremely rare disease of unknown origin; only 29 cases have been reported, including the present one (Table 1).
      • Wang M.
      • Tao Y.
      Diagnosis and treatment of congenital abdominal aortic aneurysm: a systematic review of reported cases.
      • Bansal A.
      • Mitra A.
      • Bisoi A.K.
      • Agarwala S.
      Surgical repair of congenital abdominal aortic aneurysm in a 1-year-old child with literature review.
      • Kuboi T.
      • Miyagi M.
      • Kondo S.
      • Arioka M.
      • Yamato S.
      • Sadamura T.
      • et al.
      Congenital abdominal aortic aneurysm discovered incidental to a lower back mass.
      • Sirisabya A.
      • Trinavarat P.
      • Namchaisiri J.
      • Punnahitanonda S.
      • Thaithumyanon P.
      Congenital abdominal aortic aneurysm in a term neonate: a case report.
      The sex ratio among the published reports is 17 (males):nine (females), excluding those reports that fail to mention the sex of patients.
      • Wang M.
      • Tao Y.
      Diagnosis and treatment of congenital abdominal aortic aneurysm: a systematic review of reported cases.
      • Bansal A.
      • Mitra A.
      • Bisoi A.K.
      • Agarwala S.
      Surgical repair of congenital abdominal aortic aneurysm in a 1-year-old child with literature review.
      • Kuboi T.
      • Miyagi M.
      • Kondo S.
      • Arioka M.
      • Yamato S.
      • Sadamura T.
      • et al.
      Congenital abdominal aortic aneurysm discovered incidental to a lower back mass.
      • Sirisabya A.
      • Trinavarat P.
      • Namchaisiri J.
      • Punnahitanonda S.
      • Thaithumyanon P.
      Congenital abdominal aortic aneurysm in a term neonate: a case report.
      The typical presentation of patients at hospital includes abdominal distention, vomiting, or abdominal pulsatile mass, and the diagnosis is confirmed by an abdominal ultrasound or CT. Seven cases (23%) were diagnosed prenatally by foetal ultrasound or MRI.
      • Wang M.
      • Tao Y.
      Diagnosis and treatment of congenital abdominal aortic aneurysm: a systematic review of reported cases.
      Table 1Previously reported cases of congenital abdominal aortic aneurysms.
      Modified from Wang M, Tao Y. Diagnosis and treatment of congenital abdominal aortic aneurysm: a systematic review of reported cases. Orphanet J Rare Dis 2015;10:4
      AuthorGenderAge at discoveryLocationOther aneurysmsSymptoms related to aneurysmsSurgical treatmentOutcome
      1 Howorth Jr MBFemale1 dayInfrarenalNoneLarge abdominal mass, vomiting, anorexiaExploratory laparotomyRuputure and death during Operation
      2 Darden WAMale2.5 yearsInfrarenalNoneNoneDacron aortic graftDied of Pneumonitis at 5 months after surgery
      3 Sterpetti AVMale19 yearsInfrarenalNoneMiddle epigastric pain, abdominal fullness, dysuria, abdominal pulsatile massDacron aortic graft 18 mmHealthy at 9 years after surgery
      4 Odagiri SMale1 yearInfrarenalMultiple left renal artery aneurysrre, bilateral corrmon iliac artery aneurysmsNoneDacron aortic graft 12 mmHealthy at 10 months after surgery
      5 Latter DMale1 monthInfrarenalNonePulsatile abdominal massPolytetrafluoroethylene tube graft 8 mmHealthy at 10 months after surgery
      6 Saad SAMale6 weeksInfrarenalLeft common iliac artery aneurysm massPulsatile abdominal massAneurysmorrhaphyHealthy at 3 months after surgery
      7 Myrmel TMale30 yearsInfrarenalNonePulsatile abdominal mass, acute abdominal painAlbumin coated USCI graft sized 16×8 mmHealthy at 1 year after surgery
      8 Malee MPFemale32 weeks' gestationJuxtarenalAneurysmal dilation of the bilateral iliac artery (details unknown)Palpable abdominal mass, ileus compression from an aneursysmNoneDied of acute pulmonary hypertension and cardiac dysfuncticn at age 9 days
      9 Kim ESFemale9 daysJuxtarenalNoneNoneNoneDied of heart failure secondary to renovascular hypertension at age 20 days
      10 Mehall JRMale6 weeksJuxtarenalRight common iliac artery aneurysmNoneBifurcated Gore-Tex graft 7-4 mmHealthy at 1 month after surgery
      11 Laing AJMale12 monthsInfrarenalNonePale, shocked, in an urresponsive State, vomiting, abdominal distentionExploratory laparotomyRupture and death during operation
      12 Dittrick KMale12 yearsInfrarenalNoneNoneCollagen impregnated Dacron aortic graft 14 mmHealthy at 2 years after surgery
      13 Bell PFemale1 dayInfrarenalNoneBillous vomiting, large abdominal massCryopreserved allograft 5 mmHealthy at 14 months after surgery
      14 Cheung SCWMale6 monthsJuxtarenalBilateral common and external iliac artery aneurysms, right internal iliac artery aneurysmNoneNoneProgression of thrombosis of the aneurysm and renal dysfunction at age 3 years
      15 Buddingh KTMale1 dayJuxtarenalDescending thoracic aortic aneurysm, left common iliac artery aneurysmBilous vomiting, anorexia, pulsatile abdominal massNoneAlive at 7 months, aneurysm has grown to a maximum diameter of 93 mm
      16 Kim JINone reported21 weeks' gestationInfrarenalBilateral common iliac artery aneurysms, left internal iliac artery aneurysmNoneDacron aortic graft 12 mmUneventful postoperative recovery
      17 Malikov SMale28 weeks' gestationInfrarnalNonePulsatile abdominal massRepair with native iliac vesselsHealthy at 39 months after surgery
      18 Cantinotti MNone reported22 weeks' gestationUnspecifiedNone reportedNone reportedNone reportedNone reported
      19 Tsunematsu RMale25 weeks' gestationUnspecifiedNonePulsatile abdominal mass,NoneStable after 6 months follow up
      20 McAteer JFemale32 weeks' gestationThoracoabdominalNoneNoneNoneDied of rupture at age 4 weeks
      21 Cho YPMale23 monthsInfrarenalNoneIrritability, vomiting, poor oral intake, diffuse tenderness, palpable pulsatile abdominal massCryopreserved cadaveric artery 7 mmHealthy at 10 months after surgery
      22 Meyers RLNone reportedNeonateInfrarenalNoneNoneDecellularised, antigen reduced cryopreserved allograftHealthy at 29 months after surgery
      23 Ko YMale2 monthsSupraceliacTwo descending thoracic aortic aneurysmsNone reportedDacron aortic graft 10 mmUneventful postoperative recovery
      24 Fettah NDFemale1 dayInfrarenalNoneVomiting, abdominal distention, palpable pulsatile abdominal massRepair with polytetrafluorethylene patchDied of sepsis and cardiopulmonary insufficiency at 4 weeks after surgery
      25 Bivins HSMale19 weeks' gestationInfrarenalIliac artery aneurysms (details unknown)Large abdominal massNoneDied of renal failure at age 12 days
      26 Bansal AMale1 yearInfrarenalNoneAbdominal distensionDacron aortic graft 10 mmUneventful postoperative recovery
      27 Kuboi TFemaleNeonateInfrarenalNoneLower back mass (subcutaneous vascular malformation)None reportedNone reported
      28 Sirisabya AFemale1 dayInfrarenalLeft common iliac artery aneurysm, two small right renal artery aneurysmsMarked abdominal distension with a large pulsatile massGore-Tex vascular graftThrombosis of the aortic graft and bilateral common iliac, internal iliac, and external iliac arteries at 13 months after surgery. Living a fairly normal life at 26 months after surgery
      29 Higuchi KFemale4 yearsInfrarenaMultiple intracranial aneurysms, bilateral hypogastric artery aneurysms, left renal artery aneurysmPalpable pulsatile abdominal massDacron aortic graft 10 mmAneurysms of the left renal and left middle meningeal arteries were found to have increased and an intracranial arteriovenous fistula was detected. The aneurysms were embolised at 12 to 14 months after surgery. Healthy at 21 months after surgery
      In the current patient, AAA was probably part of a systematic disease rather than a local abnormality as multiple aneurysms were found. Whole exome sequencing showed no genetic mutation specific to the congenital connective tissue disorders associated with aneurysmal diseases. However, some genetic mutations may be involved in multiple aneurysms considering the patient's age.
      No universal management approach for congenital AAA exists;
      • Wang M.
      • Tao Y.
      Diagnosis and treatment of congenital abdominal aortic aneurysm: a systematic review of reported cases.
      conservative management and surgical repair have been reported. Of these reported cases, 18 patients underwent revascularisation with vascular grafts, and Dacron graft or polytetrafluoroethylene graft were most frequently selected.
      • Wang M.
      • Tao Y.
      Diagnosis and treatment of congenital abdominal aortic aneurysm: a systematic review of reported cases.
      ,
      • Bansal A.
      • Mitra A.
      • Bisoi A.K.
      • Agarwala S.
      Surgical repair of congenital abdominal aortic aneurysm in a 1-year-old child with literature review.
      ,
      • Sirisabya A.
      • Trinavarat P.
      • Namchaisiri J.
      • Punnahitanonda S.
      • Thaithumyanon P.
      Congenital abdominal aortic aneurysm in a term neonate: a case report.
      Considering the risk of post-operative vascular graft infection and patient growth, revascularisation with cryopreserved allograft was performed in three patients.
      • Bell P.
      • Mantor C.
      • Jacocks M.A.
      Congenital abdominal aortic aneurysm: a case report.
      • Cho Y.P.
      • Kim S.C.
      • Kim S.A.
      • Jun H.
      • Kwon T.W.
      An idiopathic congenital abdominal aortic aneurysm with impending rupture in a 23-month-old boy.
      • Meyers R.L.
      • Lowichik A.
      • Kraiss L.W.
      • Hawkins J.A.
      Aortoiliac reconstruction in infants and toddlers: replacement with decellularized branched pulmonary artery allograft.
      Revascularisation with native iliac vessels
      • Malikov S.
      • Delarue A.
      • Fais P.O.
      • Keshelava G.
      Anatomical repair of a congenital aneurysm of the distal abdominal aorta in a newborn.
      and aneurysmorrhaphy
      • Wang M.
      • Tao Y.
      Diagnosis and treatment of congenital abdominal aortic aneurysm: a systematic review of reported cases.
      have also been reported. Conservative management was selected for patients in whom surgical repair carried a high mortality risk and was unlikely to improve life expectancy. Conservative management was undertaken in seven patients, four of whom died of heart or renal failure or aneurysm rupture. As conservative management for congenital AAA has a poor prognosis, surgical repair should be considered if the patient's general condition is good and revascularisation is possible anatomically.
      Long term follow up after surgery for congenital AAA in neonates, infants, and children has not been reported. Hence, the results of long term graft patency or need for re-operation are unclear.
      • Wang M.
      • Tao Y.
      Diagnosis and treatment of congenital abdominal aortic aneurysm: a systematic review of reported cases.
      • Bansal A.
      • Mitra A.
      • Bisoi A.K.
      • Agarwala S.
      Surgical repair of congenital abdominal aortic aneurysm in a 1-year-old child with literature review.
      • Kuboi T.
      • Miyagi M.
      • Kondo S.
      • Arioka M.
      • Yamato S.
      • Sadamura T.
      • et al.
      Congenital abdominal aortic aneurysm discovered incidental to a lower back mass.
      • Sirisabya A.
      • Trinavarat P.
      • Namchaisiri J.
      • Punnahitanonda S.
      • Thaithumyanon P.
      Congenital abdominal aortic aneurysm in a term neonate: a case report.
      However, as an implanted Dacron graft does not grow, ischaemic symptoms of the lower limbs may arise as the patient grows. Although collateral circulation may prevent these symptoms, if they do arise, endovascular treatment such as balloon angioplasty or endografting, or surgical repair such as re-implantation with vascular graft or extra-anatomical bypass should be considered.
      • Beckmann E.
      • Jassar A.S.
      Coarctation repair-redo challenges in the adults: what to do?.
      Furthermore, as untreated aneurysms were seen to increase post-operatively in the present patient, frequent follow up with multimodality imaging for multiple aneurysms is necessary.

      Conflicts of interest

      None.

      Funding

      None.

      Acknowledgements

      The authors thank Editage (www.editage.jp) for English language editing. Table 1 was modified from a table in Wang and Tao.
      • Wang M.
      • Tao Y.
      Diagnosis and treatment of congenital abdominal aortic aneurysm: a systematic review of reported cases.
      That article is licensed under the Creative Commons Attribution 4.0 International License (http://creativecommons.org/licenses/by/4.0/).

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