CORRESPONDENCE

The need for preoperative a-adrenergic blockade

for ganglioneuroma excision
SIRGanglioneuromas (GNs) are rare, benign, fully differentiated tumors that arise most commonly from the
sympathetic ganglia in the posterior mediastinum in
adolescents and young adults. The treatment involves
complete surgical excision and has an excellent prognosis. Compared with malignant neuroblastomas and
ganglioneuroblastomas, which frequently produce catecholamines, GNs secrete catecholamines only occasionally. The release of catecholamines during surgery,
which is one of the most feared complications, can be
catastrophic and difficult to manage. For this reason,
the British Paediatric Society of Endocrinology and Diabetes (BPSED) (1) and the panel of experts at the First
International Symposium on Pheochromocytoma (2)
have recommended that all patients with a biochemically positive pheochromocytoma should receive mandatory a-adrenergic blockade before any surgical
intervention. GNs are usually excised without such precaution, and there are no recommendations to suggest
the need for preoperative a-adrenergic blockade in these
cases. We report a unique case of ganglioneuroma excision that was unexpectedly complicated by the episodic
hypertensive spells upon tumor manipulation.
A 4-year-old boy presented with two-day history of
progressive ataxia to pediatric accident and emergency
three weeks after having chickenpox. At presentation,
he had broad-based gait with tendency to fall on right
side. His reflexes were slightly brisk in lower limbs. The
rest of neurological examination and blood pressure was
normal. His blood glucose, serum electrolytes, and full
blood picture were normal. The serum and urine toxicology screen was negative. Neurology opinion was sought,
and the ataxic gait was thought to be consistent with
cerebellitis. Chest X-ray showed a large lobulated posterior mediastinal mass projecting over right hemithorax
with pressure erosion of the right third rib. Computed
tomography (CT) of chest and abdomen showed a posterior mediastinal mass with numerous stippled features
in keeping with neurogenic tumor. Urinary catecholamines were requested, and thoracoscopic biopsy was performed to identify the tumor, which confirmed the
diagnosis of ganglioneuroma. There were no spikes in
blood pressure intraoperatively.
A week later, due to worsening ataxia and inability to
bear weight, magnetic resonance imaging (MRI) of chest
and spine was performed and revealed epidural extension of the tumor through wide intervertebral foramina
from the level of 1st thoracic/2nd thoracic intervertebral
2013 John Wiley & Sons Ltd

disc cranially to the level of 5th thoracic/6th thoracic

caudally with spinal cord compression. He then had
spinal laminectomy to relieve the cord compression.
During this surgery, he started having cyclical spikes of
blood pressure with systolic blood pressures ranging
from 50 to 200 mmHg. This was attempted to be controlled with glyceryl trinitrite infusion and phenylepherine boluses. Postoperatively, plasma normetanephrine
was noted to be increased at 3454 pmoll 1 (normal
range 1201180). Urine catecholamine/creatinine ratios
were increased for norepinephrine at 478 nmolmmol 1
creat (normal range 17194) and for dopamine at
1204 nmolmmol 1 creat (normal range 59901) with
urinary creatinine being 3.5 mM (normal range 7
18 mM). Testing for vanilylmandelic acid (VMA) and
homovanillic acid (HVA) is not available in this institution. A follow-up CT spine after 8 weeks showed small
residual intraspinal tumor with minimally increased thoracic component of the tumor that continued to displace
the mediastinal structures. MRI spine performed
6 months later showed no residual intraspinal soft tissue
component with minimally increased right paramediastinal mass. He then had further surgical excision, and this
time, it was performed with preoperative a-adrenergic
blockade using phenoxybenzamine as per BPSED pheochromocytoma guidelines. He stayed very stable during
the surgery with no hypertensive crises.
The literature regarding childhood GN is scarce, especially on such a relevant issue of surgical approach. A
retrospective study by the Italian Co-operative Neuroblastoma Group concerning a large number of patients
registered as GN over a 27-year period also did not
report any case of surgery complicated by hypertensive
crisis (3). Also, a literature search on the perioperative
management of neuroblastomas was also carried out
with inconclusive results and recommendations. A large
review of 20 years (4) experience showed <3% incidence
of intraoperative hypertension during neuroblastoma
resection and recommended only a-blocking those
patients with significant preoperative signs and symptoms of catecholamine excess. Another large review of
11 years (5) experience confirmed the same and did not
recommend any specific optimal anesthetic regimen. The
downside to routine recommendation of a-adrenergic
blockade for all neurogenic tumors is the subsequent
delay in surgery in newly diagnosed neuroblastomas and
the potential detrimental effect this can have on prognosis of the child.
1

Correspondence

In conclusion, this case highlights the need for the recognition of the fact that, like pheochromocytomas, safe
surgical excision of ganglioneuromas may require
appropriate a-adrenergic blockade and guidelines need
to devised for perioperative management in such
patients. Further studies are needed to compare preoperative urinary catecholamines quantitatively to surgical
complications as a result of catecholamine release which
might provide criteria for a-adrenergic blockade in specific patients. In the meantime, surgeons and anesthetists
need to be aware of this possible complication and be
prepared for hypertensive crises that can occur.

Conflict of interest
No conflict of interests declared.
Chandan Gupta, Noina Abid, Keith Bailie, Mark Terris,
Alistair Dick & Anthony McCarthy
Royal Belfast Hospital for Sick Children, Belfast, UK
Email: chandan_gupta60@yahoo.com
doi:10.1111/pan.12335

References
1 BSPED | British Society for
Paediatric Endocrinology and
Diabetes | Clinical [Internet]. Available
at: http://www.bsped.org.uk/clinical/
clinical_endorsedguidelines.html. Accessed
13, February 2013.
2 Pacak K, Eisenhofer G, Ahlman H et al.
Pheochromocytoma: recommendations for

clinical practice from the First International

Symposium. Nat Clin Pract Endocrinol Metab
2007; 3: 92102.
3 De Bernardi B, Gambini C, Haupt R et al.
Retrospective study of childhood ganglioneuroma. J Clin Oncol 2008; 26: 17101716.
4 Haberkern CM, Coles PG, Morray
JP et al. Intraoperative hypertension

during surgical excision of neuroblastoma.

Case report and review of 20 years
experience. Anesth Analg 1992; 75:
854858.
5 Kain ZN, Shamberger RS, Holzman RS.
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2013 John Wiley & Sons Ltd