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Congenital Bilateral Internal Jugular Venous Malformation in an Infant with Spinal Osteomyelitis and Discitis - Nature's Recipe for Disaster at Vascular Access

Ramnik Patel¹ , David Horton², Graham Robinson³, Raghuram Lakshminarayan³, Rejoo D Daniel¹

¹Department of Paediatric Surgery, Women’s and Children’s Hospital, University Hospitals of Hull and East Yorkshire NHS Trust, Hull, UK

²Department of Paediatric Radiology, Women’s and Children’s Hospital, University Hospitals of Hull and East Yorkshire NHS Trust, Hull, UK

³Vascular Interventional and Diagnostic Radiology, Women’s and Children’s Hospital, University Hospitals of Hull and East Yorkshire NHS Trust, Hull, UK


Correspondence:

Rejoo D. Daniel

Department of Pediatric Surgery, Children’s Hospital

University Hospitals of Hull and East Yorkshire NHS Trust

Hull Royal Infirmary, Anlaby Road, Hull HU3 2JZ United Kingdom

Telephone: 00441482675650 Fax: 00441482674687

E-mail: This email address is being protected from spambots. You need JavaScript enabled to view it.


Abstract

We report an infant who presented with lumbar spinal osteomyelitis and discitis. Patient had a rare congenital right sided agenesis/atresia and left sided phlebectasia and stenosis of internal jugular veins making central vascular access a challenge. This was diagnosed by imaging and treated with angioplasty successfully allowing conservative management of her acute sepsis. Patient made complete recovery with excellent results.

Keywords: vascular malformation, internal jugular vein, agenesis/atresia, stenosis, phlebectasia, angioplasty, osteomyelitis, discitis, central venous access

 

Introduction

Lumbar spinal osteomyelitis and discitis is very rare in infants. Vascular access in infants is a very common procedure in pediatric surgery for those infants requiring long term intravenous antibiotics with monitoring of inflammatory markers and side effects of antibiotics. We wish to describe and unusual vascular malformation in the cerebrovascular system consisting of hypoplasia of right vertebral artery, atresia or agenesis of right internal jugular vein with well-developed collaterals and phlebectasia of left internal jugular vein in association of a concentric stenosis at its junction with left subclavian vein leading to this unusual metastatic infection in the first place and the challenges at vascular access therapeutically.

Case Report

An 18-month old girl presented with a 3 days history of gradually worsening lower back pain, limp and abnormal gait following upper respiratory tract infection which responded well to amoxicillin. Patient was twin 2 born by Caesarean section and her twin 1 brother was normal. There was family history of Best disease.

On examination, there was asymmetry of gait with lurching to the right hand side somewhat resembling a Trendelenburg gait and there was tenderness over lower lumbar spine. Full blood count revealed haemoglobin 84 g/L, white blood cell count 12.2 x 109/L, neutrophils 4.2 x 109/L, platelets 341 x 109/Land C-reactive protein 12 mg/L. Blood culture did not grow any organisms. Pelvis radiograph and hip ultrasounds were normal. Magnetic resonance imaging (MRI) scan with contrast of the whole spine and pelvis showed unusual focal osteomyelitis of the posterior quadrant of 4th lumbar vertebra involving the L4/L5 disc (Fig. 1).

Figure 1: MRI scans showing osteomyelitis of 4th lumbar vertebra and discitis of L 4/5 with contrast enhancement.

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Multidisciplinary team recommended 6 weeks of intravenous ceftriaxone via central line followed by 6 weeks of oral cephalexin and 12 weeks of oral fusidic acid with liver function monitoring. Patient underwent cuffed tunnelled Hickman line insertion into right internal jugular vein (RIJV) uneventfully (Fig. 2A). Patient improved and began to walk comfortably in one week and was discharged home with ambulatory team administering antibiotics.

On 12th day she was readmitted as community nurses were unable to aspirate blood through the line. Chest radiograph showed that the line previously in the superior vena cava had flipped upwards pointing into the direction of right internal jugular vein (Fig. 2B). A linogram confirmed the findings and showed very small calibre of presumed right internal jugular vein and some collaterals (Fig. 3A). It was decided not to use this line which was removed and replaced with left internal jugular vein. At insertion, left internal jugular vein (LIJV) was extremely dilated but a size 14 gauge cuffed tunnelled Hickman line was easily inserted and the position of line was confirmed fluoroscopically (Fig. 3B).

Figure 2: Chest radiographs at A. insertion and B. follow up

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Figure 3: Right IJV Linogram and position of left IVJ insertion.

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On removal of the drapes from the head; facial plethora with hyperaemic face, swollen cyanotic lips, peri-orbital oedema and prominent veins in the head and neck area was immediately evident. Rest of the body has normal colour. There were reduced saturations of 80-85% in the ear/neck as compared to 100% in the rest of the body below the neck. Within 15 minutes patient’s face blew up with oedema so much so that patient found it difficult to open her eyes. Over the next 2-3 hours, the oedema gradually settled but the face remained dusky and discoloured. Ultrasound scan of the neck veins showed small calibre presumed right internal jugular vein with multiple collaterals (Fig. 4A) and hugely dilated left jugular vein with sluggish/slow moving blood flow without any evident thrombus (Fig. 4B).

Figure 4: Neck ultrasound and colour Doppler showing right internal jugular vein and left internal jugular vein

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MRI head was normal. MRA neck showed asymmetric calibre vertebral arteries with lesser expression on the right (Fig. 5). MRV cranial diffusion weighted scans showed that the calibre of jugular veins was asymmetric; right one atretic and heterogeneously small calibre with recruitment of several collateral veins and on the left one was significantly enlarged calibre with a concentric stenosis at the origin of the left jugular vein (Fig. 6).

Figure 5: MRA-Neck. Note small calibre right vertebral artery.

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Figure 6: MRV- neck showing atretic right internal jugular vein with multiple collaterals and ectatic left internal jugular vein and stenosis at junction.

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Patient underwent left arm and central venogram which confirmed MRI findings of widely patent SVC, brachiocephalic veins and left subclavian veins (Fig. 7). Left internal jugular venogram using right femoral approach demonstrated a large vein with a functional stenosis at the junction with the subclavian vein (Fig. 8A). Left internal jugular angioplasty of the stenosis with a 3mm balloon produced immediate functional improvement in drainage (Fig. 8B). The improvement was dramatic clinically with reversal of all symptoms within 15 minutes of the procedure and she was back to normal. Patient completed course of antibiotics and Hickman line was removed uneventfully. Fundoscopy and regular head circumference measurements were normal. Aspirin 2mg/kg daily with a maximum dose of 75mg has been started. At 12 months follow up, patient is asymptomatic with normal gait and no limp or pain.

Figure 7: Left arm central venogram demonstrating normal patent left sided central veins

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Figure 8: Left internal jugular venogram and balloon angioplasty: note several collaterals in the former and immediate good drainage in the later.

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Discussion

The rare combination of congenital vascular anomalies of central venous agenesis or atresia of RIJV and phlebectasia of LIJV associated with junctional concentric stenosis associated with vertebral artery hypoplasia in an infant associated with unusual complications of metastatic lumbar osteomyelitis and discitis is an extremely rare event. Extensive search through the literature failed to reveal a previously reported combination of such lesions.

Congenital vascular anomalies are classified into two groups as vascular tumours and vascular malformations [1]. Most of the vascular anomalies are present prenatally or diagnosed soon after birth. Vascular malformations are sub-classified into arterial, venous, capillary, lymphatic or mixed based on the nature of the anomalous vessels anatomically and slow-flow or fast-flow(arterial or mixed) functionally [2]. Congenital extensive central venous thrombosis in a neonate causing a challenge to central venous access and diagnostic difficulties during imaging studies created by associated collaterals has previously been highlighted [3]. Hand held ultrasound at percutaneous insertion of line usually is unable to differentiate between normal vein and large collateral developed due to congenital chronic venous insufficiency in these cases. Generally in a previously healthy infant, routine mapping of the neck veins is not carried out in the first placement of central venous catheters. Even post-operative ultrasound and colour Doppler studies on neck veins missed the anomaly in our case due to extensive collateral circulation and although phlebectasia was easy to diagnose on the left side, the stenosis was missed. Our case demonstrates limitations of diagnostic accuracy of the scans and therapeutic confirmation of fluoroscopy at insertion which may give false sense of security leading to a trap.

Although the development of these congenital anomalies happens early in the embryonic life, the effects of chronic venous insufficiency with development of collateral circulation take some time. Prenatal ultrasound anomaly scan will still miss such anomalies. Even in the post natal period both arterial and venous malformations remains silent and unless gets complicated, there detection is exceptional [4-5]. We believe that in the presence of bilateral venous insufficiency, the upper respiratory tract infection was taken via collaterals to paravertebral vein plexus which led to metastatic septic focus in unusual site. Such an infection is rare but very serious associated with significant mortality and morbidity in infants. If the first line on right side would have no complication, these congenital vascular anomalies and the possible mechanism of such an unusual metastatic infection would have been missed easily. Stenotic LIVJ phlebectasia was the only main channel of venous drainage from the head and neck and our catheter converted the partial chronic obstruction into an acute complete obstruction leading to acute venous obstruction syndrome.

Conclusion

Our case is the congenital form of benign chronic cerebrovascular venous insufficiency syndrome recently described in adults in association with multiple sclerosis [6]. Recent advances and availability of advanced paediatric interventional diagnostic and therapeutic procedures made such a successful management in our case. We believe that interventional balloon angioplasty even in acute emergency is feasible and safe in specialist centres of excellence with adequate expertise available even at very young age.

 

Acknowledgements

We are grateful to Miss S Besarovic MD, FRCS (Paeds) Consultant Paediatric Surgeon at Hull Royal Infirmary; Dr Colin Ferrie and Dr Helen McCullagh, Consultant Paediatric Neurologists at Leeds General Infirmary; Dr Simon Magurk and Dr Ian McCavity at Birmingham Children’s Hospital; Mr Giles Peek, and Dr Demetris Taliotis, Consultants at Leicester Glenfield Hospital, Leicester for their expertise and help in the management of this difficult and challenging case.

 

 

References:

1. Patel R, Curry JI: Vascular Malformations. In: Handbook of Pediatric Surgery. Part 4 Chap 13, 1st Ed, CK Sinha & Mark Davenport (Eds), Springer Verlag, London, 2010, 221-228.

2. Legiehn GM, Heran MKS. Venous malformations: classifications, development, diagnosis, and interventional radiological management. Radio Cl North Am 2008; 46:545-597.

3. Patel R, Griselli M, Barrett AM. Congenital extensive central venous thrombosis with chylous ascites and chylothoraces. J Pediatr Surg 2013; 48:e5-8.

4. Lane V, Patel R, Daniel RD. Prolapsed urachal sinus with pyourachus in an infant. J Pediatr Surg 2013; 48:e17-9.

5. Siani A, Flaishman I, Schioppa A. et al. Jugular venous phlebectasia: uncommon in children, anecdotal in adults. Am J Surg 2008; 195:419-20.

6. Zamboni P, Galeotti R, Menegatti E, et al Chronic cerebrospinal venous insufficiency in patients with multiple sclerosis. J Neurol Neurosurg Psychiatry 2009; 80: 392–9.