Abdominal Aortic Aneurysm in a Neonate By Saad A. Saad and Allan May Riyadh, Saudi Arabia 0 This is the second reported case of abdominal aotiic aneurysm in neonates to be presented in the English language literature. The first case was reported by Howorth in 1967. In addition to the presence of abdominal aortic aneurysm, this neonate has nesidioblastosis and sequestration of the lungs. The combination of these three rare anomalies in one infant is fascinating and has not been reported. Copyright o 1991 by W.B. Saunders Company INDEX WORDS: sis; sequestration

Aneurysm, of lung,

abdominal neonate.

aorta;

nesidioblasto-

ESIDIOBLASTOSIS is not uncommon in the neonate with severe hypoglycemia. The association of other congenital anomalies such as sequestration of the lungs and cardiovascular anomalies is quite unique. The presence of abdominal aortic aneurysm in this neonate makes the present case report very special.

N

CASE REPORT A 6-week-old boy newborn presented with severe hypoglycemia and pulsatile huge abdominal mass. The hypoglycemia, after thorough workup, was related to nesidioblastosis. The pulsatile abdominal mass was investigated. Abdominal ultrasound and computed tomography scan showed a solid mass in the retroperitoneal area along the aorta (Fig 1). Abdominal aortic aneurysm was not suspected at this time, but was confirmed after performing an aortogram (Fig 2). The aneurysm was infrarenal, extending to the bifurcation of the aorta. Another small aneurysm, originating from the left common iliac artery, was noted. The neonate underwent laparotomy and 90% pancreatectomy to correct his hypoglycemia. Histopathological diagnosis of the resected pancreas proved to be nesidioblastosis. Abdominal aortic aneurysmorrhaphy was performed after completing the pancreatectomy. The aneurysm was repaired in the following fashion. The mesentery of the small bowel along the abdominal aorta was dissected. Multiple small blood vessels connecting the mesentery of the small bowel to the wall of the aneurysm were resected after evaluating the vascularity of the small bowel. The abdominal aorta measuring 0.5 cm in diameter was dissected proximal to the aneurysm and umbilical tape was passed around it. The four iliac arteries were dissected and umbilical tapes were passed around them. The abdominal aortic aneurysm was saccular, measuring 4.5 cm in diameter, extended to the left common iliac artery, and was isolated. The lumbar blood vessels arising from the posterior surface of the aorta were circumferentially dissected and 00 silk tie was passed around them. The neonate received 1 mg/kg of heparin and aortic crossclamping was carried out proximally and distally. The abdominal aortic aneurysm wall, which was very soft and threatening to rupture, was opened (Fig 3). The excess wall of the aneurysm was resected and aneurysmorrhaphy was performed to restore the normal diameter of the aorta (Fig 4). Aortic cross-clamping time was 28 minutes. The left common iliac artery aneurysm was not corrected at this time because it was relatively small and because

JOUrna/OfPediafricSurgery,

Vol26,

No 12 (December),

1991: pp 1423-1424

Fig 1. centrally

Abdominal computed located in the posterior

tomography abdominal

scan shows area.

a solid mass

the infant had already undergone long surgical procedures to correct his hypoglycemia and the abdominal aortic aneurysm. Follow-up and possible surgery are planned in the future for this aneurysm. A postoperative angiogram showed normal diameter of the aorta and of the iliac arteries. The angiogram also showed abnormal infradiaphragmatic vascularity of the right lower lobe of the lung, and sequestration of the right lung was diagnosed. Right lower lobectomy was carried out without any complications. The infant was discharged 3 months after admission weighing 6.2 kg (the weight on admission was 3.8 kg). DISCUSSION

Few reports have been presented in the literature regarding abdominal aortic aneurysms in infants and children. The majority of abdominal aortic aneurysm (16 cases) present in association with Marfan’s syndrome’ and tuberous sclerosis.’ Congenital abdominal aortic aneurysm has been reported in four children varying in age from 13 years to 14 years. Traumatic abdominal aortic aneurysm has been re-

From the Section of Pediahic Surgery, Department of Surgery, King Faisal Specialist Hospital and Research Centre, Riyadh, Saudi Arabia. Address reprint requests to Saad A. Saad, MD, 20 White Rd, Shrewsbury, NJ 07702. Copyright o 1991 by W B. Saunders Company 0022-346819112612-0024$03.00/O

1423

1424

SAAD

Fig 4. rhaphy.

Fig 2. Abdominal aortogram and left internal iliac aneurysm

shows aortic (small one).

aneurysm

(large

one)

ported by Gibson3 and by Takayanagi and Bader.4 The majority of children with abdominal aortic aneurysm have one aneurysm; however, two cases reported in the literature also had multiple peripheral arterial aneurysm.5 Most aortic aneurysms are infrarenal, but Gauderer et al6 reported one child with suprarenal abdominal aortic aneurysm. Congenital abdominal aortic aneurysm in neonates was reported

Fig 3. The abdominal eortic aneurysm opened and trimmed pair of scissors. The suction catheter is inside the aneurysm.

with

The

abdominal

aortic

aneurysm

repaired

AND

MAY

by aneurysmor-

in 1967 by Howorth. The 6-week-old neonate in this report had similar-type aneurysm and is considered the second case of abdominal aortic aneurysm in a newborn in the literature. The etiology of this aneurysm is not known and, therefore, is considered congenital. The association of this aneurysm with nesidioblastosis and sequestration of the right lung in the present case is fascinating and has not been reported previously. This report shows the importance of preoperative diagnosis of rare anomalies such as abdominal aortic aneurysm, in addition to nesidioblastosis, to plan safe elective surgery for both anomalies. Also, it shows that one laparotomy can be done safely to correct these two anomalies, without serious complications. REFERENCES 1. Darden WA, Fulenwider JT, Smith RB, et al: Congenital abdominal aortic aneurysm. Report of the case and review of literature. Surgery 96:567-573,1984 2. Hagood C, Gatvin DD, Lachina FM, et al: Abdominal aortic aneurysm and renal hamartoma in an infant with tubeous sclerosis. Surgery 79:713-7151916 3. Gibson TA: Aneurysm of lower abdominal aorta with rupture in a 16-month old infant. Am J Dis Child 71:654-658,1956 4. Takayanagi K, Bader M: Rupture of abdominal aortic aneurysm in a seven-year old girl. J Pediatr Surg 18:193-195,1983 5. Shiller M, Gordon R, Shifrine E, et al: Multiple arterial aneurysm. J Pediatr Surg 18:27-29, 1983 6. Gauderer MW, Wolfkoff JS, Izant R: Aortic aneurysm of the suprarenal abdominal aorta: Surgical reconstruction in a seven-yearold patient. J Pediatr Surg 17:940-943,1982 7. Howorth MB: Aneurysm of the abdominal aorta in the newborn infant. N Engl J Med 276:1133-1134,1967