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Journal of Indian Association of Pediatric Surgeons
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Year : 2017  |  Volume : 22  |  Issue : 3  |  Page : 176-178

Surgical repair of congenital abdominal aortic aneurysm in a 1-year-old child with literature review

1 Department of Cardio-thoracic and Vascular Surgery, All Institute of Medical Sciences, New Delhi, India
2 Department of Pediatric Surgery, All Institute of Medical Sciences, New Delhi, India

Date of Web Publication8-Jun-2017

Correspondence Address:
Sandeep Agarwala
Department of Pediatric Surgery, All India Institute of Medical Sciences, New Delhi - 110 029
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Source of Support: None, Conflict of Interest: None

DOI: 10.4103/jiaps.JIAPS_258_16

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Reported here is a case of 1-year-old male child who presented with huge abdominal mass, which on radiological investigation was diagnosed as retroperitoneal pseudoaneurysm of the aorta. On exploration, it was found to be a true aneurysm of infrarenal abdominal aorta with inflow agenesis. Aneurysm was excised, and aorta was reconstructed with 10 mm Dacron graft. Postoperative computed tomography angiography showed patent graft with good distal runoff. Literature review revealed that only 26 cases of congenital abdominal aortic aneurysm had been reported so far. None of them had inflow agenesis which can give false impression of pseudoaneurysm on preoperative evaluation. The case highlights the utility of additional complimentary investigations such as Doppler study in clinching diagnosis and helping plan and execute successful treatment in the difficult diagnostic scenario.

Keywords: Congenital abdominal aortic aneurysm, review, surgical repair

How to cite this article:
Bansal A, Mitra A, Bisoi AK, Agarwala S. Surgical repair of congenital abdominal aortic aneurysm in a 1-year-old child with literature review. J Indian Assoc Pediatr Surg 2017;22:176-8

How to cite this URL:
Bansal A, Mitra A, Bisoi AK, Agarwala S. Surgical repair of congenital abdominal aortic aneurysm in a 1-year-old child with literature review. J Indian Assoc Pediatr Surg [serial online] 2017 [cited 2020 Sep 24];22:176-8. Available from: http://www.jiaps.com/text.asp?2017/22/3/176/207640

   Introduction Top

Congenital abdominal aortic aneurysm (CAAA) is infrequent compared to adult or abdominal aortic aneurysm (AAA). Few previous reports have shown that variable clinical presentation and radiological diagnostic challenge can at times put surgical team in a difficult situation, so it is worth reporting such isolated reports to highlight management strategy adopted and result achieved.

   Case Report Top

A 1-year-old male child of 7 kg weight reported with a history of abdominal distension since day one of life. The child was full term and had passed meconium on day 1 of life. The abdominal distension and gradually and progressively increased in size to its present status. The child was evaluated with ultrasound abdomen and contrast-enhanced computerized tomography scan of the abdomen which showed a 10 cm × 7.8 cm × 9 cm hypodense lesion in retroperitoneum in the infrarenal location. The mass was seen extending from L1 to L5 vertebrae, abutting abdominal wall anteriorly, and vertebral column posteriorly [Figure 1]. On Doppler evaluation, the mass had some color flow suggestive of pseudoaneurysm, but both common iliac and external iliac were patent with normal flow pattern.
Figure 1: Contrast-enhanced computerized tomography showing a large hypodense, retroperitoneal mass

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Abdominal exploration through a midline laparotomy incision revealed a large abdominal aortic aneurysm extending from the infrarenal up to the bifurcation of aorta [Figure 2]a. The left kidney was compressed with the aneurysm and left renal artery and vein were stretched out. Suprarenal aorta was of normal caliber, but part of the abdominal aorta at proximal junction of the aneurysm was of pinhole caliber suggestive of inflow agenesis. Celiac artery, superior mesenteric artery, and bilateral common iliac arteries were looped, and aneurysm wall was opened. The wall of the aneurysm was densely calcified with extensive intramural thrombus. Partial excision of the wall was done [Figure 2]b, and the infrarenal aorta was replaced with 10 mm Dacron graft [Figure 2]c. Postoperatively, the child had an uneventful recovery and a computed tomography (CT) angio performed in the follow-up showed that the graft was patent with good distal run off [Figure 2]d.
Figure 2: Aortic aneurysm extending from the infrarenal up to the bifurcation of aorta (a); partial excision of the wall of the aneurysm (b); infrarenal aorta was replaced with 10 mm Dacron graft (c); postoperative computed tomography angio showing patent graft with good distal run off (d)

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   Discussion Top

The abdomen is the most common site of aortic aneurysm. Aneurysm occurs due to dilatation or bulging of aortic wall. Most of abdominal aortic aneurysms (AAA) are acquired and detected after the age of 60 years. Congenital aortic aneurysms (CAA) are extremely rare, and only 26 cases have been reported so far [1] since the first case was reported by Howorth in 1967.[2] Thirteen of the 26 (50%) of the CAA reported till date has presented at <1 year of age, whereas six (26%) presented after 1 year of age. Seven (27%) were detected in intrauterine period. They are usually asymptomatic, being detected either incidentally or on investigations for nonspecific abdominal complaints such as bloating or dyspepsia.

Congenital AAA is mainly caused by intrauterine infection, connective tissue disorders such as Marfan's syndrome,  Ehlers-Danlos syndrome More Details, or by inflammatory vasculitis such as Takayasu arteritis, Kawasaki syndrome, polyarteritis nodosa, and occasionally by iatrogenic injury following umbilical artery catheterization.[3],[4],[5] Ultrasonography is most common screening investigation and is helpful in diagnosing aneurysm as well as estimating size of aneurysm. For preoperative planning, CT angiography is investigation of choice due to its ability to delineate anatomical details with relation to adjacent organs especially the involvement of renal arteries.[6] Infrarenal involvement of abdominal aorta has been most reported in 18 of the previous 26 cases (70%), whereas thoracoabdominal and juxtarenal involvement being the less commonly observed.[1]

As opposed to acquired AAA in adults, no consensus has so far developed for management strategy of congenital aortic aueurysm. Management options can broadly be divided into three categories: conservative, endovascular aneurysm repair (EVAR), and surgical repair. Conservative management in form of control of blood pressure, use of antiplatelets, and serial follow-up with ultrasound is advisable in if the child is very sick and is unlikely to tolerate major vascular surgery or in a relatively stable smaller child in whom a larger graft (>10 mm) can be placed if operated at a later date. EVAR although has been tried with reasonable amount of success in adults, but in pediatric AAA, it has not been used so far mainly due nonavailability of small size catheters.[1] Surgical repair options include aneurysmorrhaphy and synthetic graft placement (Dacron or Gortex). For smaller aneurysms, some have also used cryopreserved allografts and performed repair with native vessels.[7] Although there has not been any study comparing individual surgical approaches in CAAA, aneurysmorrhaphy has been found to have higher recurrence rate.[8] Among the 26 cases reported so far, surgical correction could not be done in 11 (42%) patients due to late presentation, extensive involvement, renal dysfunction, or rupture of aneurysm during exploration. Of these 11, six either died in theater or in early follow-up after surgery (<1 month). One patient developed aneurysm thrombosis and renovascular hypertension, in two the outcome was not reported while only two patients were healthy in follow-up.[1] Out of 15 patients who underwent surgical correction so far, 11 were healthy in follow-up. One patient had died due to sepsis, and in the remaining three, the outcome was not reported. Therefore, surgical attempt for correction of this difficult subgroup of patients leads to better survival in comparison to conservative approach.

Diagnosis of AAA, though suspected but could not be made in preoperative, evaluation due to inflow agenesis and patient was explored with a working diagnosis of pseudoaneurysm. As proximal diameter was narrow, so an end to side anastomosis was done with 10 mm Dacron graft. At 2 months follow-up, bilateral distal pulses were present, and CT angio showed a normal distal flow. A proper diagnosis or a high degree of suspicion of an odd diagnosis helps to plan a procedure in a place with adequate infrastructure and expertise-like in a cardiac occupational therapy, with available vascular grafts and the assistance of cardiovascular surgeons in the present case.

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Conflicts of interest

There are no conflicts of interest.

   References Top

Wang Y, Tao Y. Diagnosis and treatment of congenital abdominal aortic aneurysm: A systematic review of reported cases. Orphanet J Rare Dis 2015;10:4.  Back to cited text no. 1
Howorth MB Jr. Aneurysm of abdominal aorta in the newborn infant. Report of case. N Engl J Med 1967;276:1133-4.  Back to cited text no. 2
Sarkar R, Cilley RE, Coran AG. Abdominal aneurysms in childhood: Report of a case and review of the literature. Surgery 1991;109:143-8.  Back to cited text no. 3
Sterpetti AV, Hunter WJ, Schultz RD. Congenital abdominal aortic aneurysms in the young. Case report and review of the literature. J Vasc Surg 1988;7:763-9.  Back to cited text no. 4
Darden WA, Fulenwider JT, Smith RB 3rd, Sewell CW. Congenital abdominal aortic aneurysms: Report of a case and review of the literature. Surgery 1984;96:567-73.  Back to cited text no. 5
Buddingh KT, Zeebregts CJ, Tilanus ME, Roofthooft MT, Broens PM. Large neonatal thoracoabdominal aneurysm: Case report and review of the literature. J Pediatr Surg 2008;43:1361-4.  Back to cited text no. 6
Cho YP, Kim SC, Kim SA, Jun H, Kwon TW. An idiopathic congenital abdominal aortic aneurysm with impending rupture in a 23-month-old boy. J Vasc Surg 2013;57:508-10.  Back to cited text no. 7
Malikov S, Delarue A, Fais PO, Keshelava G. Anatomical repair of a congenital aneurysm of the distal abdominal aorta in a newborn. J Vasc Surg 2009;50:1181-4.  Back to cited text no. 8


  [Figure 1], [Figure 2]


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