Rapid Communication| Volume 48, ISSUE 2, e5-e8, February 2013

Congenital extensive central venous thrombosis with chylous ascites and chylothoraces


      We describe a case of congenital extensive central venous thrombosis presenting as polyhydramnios and massive ascites, requiring amnioreduction prenatally and refractory chylous ascites and chylothoraces postnatally. Echocardiography, computed tomography angiogram (CTA), and magnetic resonance venogram (MRV) were helpful in defining the nature and extent of the lesion. The patient underwent staged procedures of repeated abdominal paracentesis, chest drain insertion, and right internal jugular vein exploration initially. Subsequently, open thromboembolectomy from the upper venous system veins and pericardial patch angioplasty of the right internal jugular and right innominate veins were required and managed by catheter-directed thrombolysis for the residual thrombosis with successful recovery.

      Key words

      To read this article in full you will need to make a payment

      Purchase one-time access:

      Academic & Personal: 24 hour online accessCorporate R&D Professionals: 24 hour online access
      One-time access price info
      • For academic or personal research use, select 'Academic and Personal'
      • For corporate R&D use, select 'Corporate R&D Professionals'


      Subscribe to Journal of Pediatric Surgery
      Already a print subscriber? Claim online access
      Already an online subscriber? Sign in
      Institutional Access: Sign in to ScienceDirect


        • Zarroug A.E.
        • Srinivasan S.K.
        • Wulkan M.L.
        Incidental chylous fluid during hernia repair may be a harbinger of malrotation.
        J Pediatr Surg. 2010; 45: e17-e18
        • Lloyd D.A.
        Gastroschisis, malrotation, and chylous ascites.
        J Pediatr Surg. 1991; 26: 106-107
        • Casaccia G.
        • Crescenzi F.
        • Palamides S.
        • et al.
        Pleural effusion requiring drainage in congenital diaphragmatic hernia: incidence, aetiology and treatment.
        Pediatr Surg Int. 2006; 22: 585-588
        • Levine C.
        Primary disorders of the lymphatic vessels—a unified concept.
        J Pediatr Surg. 1989; 24: 233-240
        • Mulvihill S.J.
        • Fonkalsrud E.W.
        Complications of superior versus inferior vena cava occlusion in infants receiving central total parenteral nutrition.
        J Pediatr Surg. 1984; 19: 752-757
        • Waterfield T.
        • Lakhoo K.
        Challenges in the management of refractory bilateral idiopathic congenital chylothoraces in a newborn.
        Eur J Pediatr Surg. 2010; 20: 198-201
        • Jones N.M.
        • Kiely E.M.
        Retroperitoneal teratomas—potential for surgical misadventure.
        J Pediatr Surg. 2008; 43: 184-187
        • McCulloch M.A.
        • Conaway M.R.
        • Haizlip J.A.
        • et al.
        Postoperative chylothorax development is associated with increased incidence and risk profile for central venous thromboses.
        Pediatr Cardiol. 2008; 29: 556-561
        • Crowther M.A.
        • Kelton J.G.
        Congenital thrombophilic states associated with venous thrombosis: a qualitative overview and proposed classification system.
        Ann Int Med. 2003; 138: 128-134
        • Phol M.
        • Zimmerhackl L.B.
        • Heinen F.
        • et al.
        Bilateral renal vein thrombosis and venous sinus thrombosis in a neonate with factor V mutation (FV Leiden).
        J Pediatr. 1998; 132: 159-161