British Journal of Neurosurgery, February 2015; 29(1): 35–36 © 2014 The Neurosurgical Foundation ISSN: 0268-8697 print / ISSN 1360-046X online DOI: 10.3109/02688697.2014.950945


Intracranial abscess in Ectopia Cordis Joseph Merola, Geoffrey Adrian Tipper, Zakier Hussain, Venkataraman Balakrishnan & Peter Gan

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Department of Neurosurgery, Waikato Hospital, Hamilton, New Zealand

depends on classification (thoracic, thoraco-abdominal and cervical as described by Shamberger and Welch 1990)1 and associated congenital defects particularly intracardiac.2,3 The precise aetiology of this condition remains unknown although it has been described in association with chromosomal abnormalities.3 This unusual condition in our patient is made even more rare by the fact that most patients do not survive beyond infancy. There is a well-documented increased risk of intracranial abscess formation in patients with cyanotic congenital heart defects. As in this case, the Tetralogy of Fallot is a major contributor. The underlying process is thought related to the effects of right-to-left shunting – namely repeated polycythaemia-induced cerebral infarcts and poor immunological status from bypass of pulmonary phagocytosis.4 Associated coagulopathy, cardiac dysfunction or rhythm disturbance, electrolyte imbalance and risk of cyanotic spells triggered by increased sympathetic activity4 require that any surgical candidate is managed in a high dependency environment in the perioperative period.

Abstract We present a case of intracranial abscess in a young female with Ectopia Cordis, an exceptionally rare cardiac condition. The neurosurgical implication is the predisposition to intracranial abscess formation. A heightened awareness of this association will aid diagnosis in similar clinical scenarios. Keywords: congenital heart defect; Ectopia Cordis; intracranial abscess

Case report A 34-year-old female presented with rapid onset of occipital headache, vomiting, fever and neck stiffness. She had a background of congenital Ectopia Cordis associated with Tetralogy of Fallot. Of note, she had previously undergone aspiration of an occipital lobe abscess secondary to Aggregatibacter aphrophilus and Actinomyces meyeri. The current examination elicited meningism, fever and lethargy. There were no focal neurological signs and systems examination was normal with the exception of the Ectopia Cordis (Fig. 1) and its associated murmurs. Magnetic Resonance Imaging (MRI) of her brain revealed a solitary intra-axial abscess within the left caudate nucleus (Fig. 1). Following image-guided aspiration she received a prolonged course of intravenous antibiotics with good recovery and satisfactory MRI appearances at three-month evaluation (Fig. 1).

Declaration of interest: The authors report no declarations of interest. The authors alone are responsible for the content and writing of the paper.

References 1. Shamberger RC, Welch KJ. Pediatric sternal defects. Pediatr Surg Int 1990;5:156–64. 2. Amitabh P, Sharan R, Talapatra A . Ectopia cordis. J Clin Neonatol 2012;1:166–7. 3. Pepper MA , Fishbein GA , Teitell MA . Thoracoabdominal wall defect with complete ectopia cordis and gastroschisis: A Case report and review of the literature. Pediatr Dev Pathol 2013;16:348–52. 4. Raha A , Ganjoo P, Singh A , Tandon MS, Singh D. Surgery for brain abscess in children with cyanotic heart disease: An anaesthetic challenge. J Pediatr Neurosci 2012;7:23–6.

Discussion Ectopia cordis is a rare congenital disorder resulting in partial or complete displacement of the heart outside of the thoracic cavity, occurring 8 per million births. The prognosis

Correspondence: Dr. Joseph Merola, Department of Neurosurgery, Waikato Hospital, Corner Selwyn and Pembroke Street, Hamilton, 3240, New Zealand. E-mail: [email protected] Received for publication 16 July 2014; accepted 29 July 2014


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Fig. 1. (A) Axial MRI T1 sequence post-contrast demonstrates a rim enhancing lesion at the head of the left caudate nucleus. (B) Axial MRI T1 sequence post-contrast 3 months post aspiration; no residual abscess cavity seen. Incidental note is made of a carotid body tumour (known to be associated with chronic hypoxia). (C) Oblique view: photo of the sternal wall defect and ectopia cordis (D) AP view.

Intracranial abscess in Ectopia Cordis.

We present a case of intracranial abscess in a young female with Ectopia Cordis, an exceptionally rare cardiac condition. The neurosurgical implicatio...
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