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Child's cardiac tumour unmasked during tonsillectomy

Leontidou, A.; Papadopoulou, K.; Vlachtsis, K.; Douvantzi, A.; Zambouri, A.; Petropoulou, P.

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European Journal of Anaesthesiology: March 2004 - Volume 21 - Issue 3 - p 246-249


In the present day practice of anaesthesia, unnecessary laboratory tests in healthy individuals during preoperative preparation are usually avoided. Especially in children, the prevalent view is not to perform preoperative laboratory tests before minor procedures provided that both personal and family history, and clinical examination are normal.

The case of an apparently healthy five-and-a-half year old male, body weight 19 kg, who exhibited intraoperative electrocardiographic (ECG) disorders that finally led to the diagnosis of a heart tumour is presented. The patient had no abnormal perinatal history and was the third child of apparently healthy parents. He was scheduled for tonsillectomy and ade-noidectomy. The child's medical history included an episode of bronchiolitis at 18 months of age and an episode of laryngospasm at 30 months, both of which responded to treatment. Preoperative clinical examination showed nothing abnormal apart from a mild spinal scoliosis and mild nasal congestion. The child had no symptoms or signs to indicate a heart disorder and therefore a preoperative ECG was not performed. A chest radiograph and a complete blood count were performed with normal findings.

Thirty minutes before induction of anaesthesia, midazolam 8 mg was given orally for premedication. Anaesthesia was provided with propofol 50 mg, fentanyl 0.05 mg, and maintained with N2O/O2 (60/40) and sevoflurane 1.0-1.5%. Atracurium 10 mg was given for muscular relaxation and a plain 5.5 mm endotracheal tube inserted. Acetaminophen (paracetamol) 400 mg was given rectally for postoperative analgesia. Intraoperative monitoring included continuous ECG (leads II and V5), pulse oximetry (SPO2), non-invasive blood pressure (NIBP) and capnography (ETCO2). The ECG showed a normal sinus rhythm of 120 beats min−1. During surgery, three episodes of wide complex tachycardia of 140-170 beats min−1 occurred, each lasting less 1 min. Heart rate decreased to 120 beats min−1 automatically followed by negative T-waves for several seconds and then reverted to sinus rhythm. These episodes were unrelated to surgical or anaesthetic manipulations such as intubation or mouth gag placement. Neither blood pressure nor SPO2 were affected during these episodes. The operation was successfully completed and the child was fully awake and extubated after atropine 0.2 mg and neostigmine 1 mg had been given for antagonism of the residual effects of neuromuscular blockade. On departure from the operating room, the patient's vital signs were: heart rate, 110 beats min−1; blood pressure, 90/60 mmHg; and SPO2, 100%. The child was transported to the postanaesthesia care unit (PACU) with a heart rate of 110 beats min−1. Twenty minutes later, he suddenly developed supraventricular tachycardia of 180 beats min−1 with wide QRS, which was primarily treated with lidocaine 20 mg, and fentanyl 0.05 mg was given intravenously (i.v.). Because the dysrhythmia persisted, a bolus of amiodarone 50 mg was given i.v. followed by an infusion at 10 mL h−1 (50 mg in NaCl 0.9% 250 mL). The heart rate finally reduced to 110-120 beats min−1. Although the postanaesthetic condition of the child was extremely good and despite the fact that the amiodarone infusion was continued, there were many episodes of non-persistent supraventricular tachycardia (140 beats min−1) followed by a few ventricular extrasystoles during the child's stay in the PACU (Figs 1-3).

Figure 1
Figure 1:
Patient's ECG during an episode of tachycardia (144 beats min−1).
Figure 2
Figure 2:
ECG 7 min later.
Figure 3
Figure 3:
ECG 12 min later.

The next day, the child was transported to the Cardiology Department at Hippokratio Children's General Hospital. The amiodarone infusion was discontinued 18 h later because of bradycardia. Amiodarone was replaced by propranolol 25 mg four times daily. At that time, the ECG showed negative T-waves in lead II, aVF, V4, V5, biphasic T-waves in V6 and positive T-waves in aVR. The two-dimensional echocardiogram revealed a large echogenic mass involving the apex of the left ventricle and the apical portion of the intraventricular septum. No left ventricular outflow tract obstruction or pericardial effusion was observed and the left ventricular systolic function was satisfactory. Magnetic resonance imaging (MRI) also showed a large mass (5.6 × 3.7 × 3.4 cm) involving the inferoseptal and apical wall of the left ventricle, probably a fibroma (Fig. 4). Seven months later, the child was successfully operated on in the Children's Hospital, Boston, MA, USA, and the tumour was completely removed. The operating findings confirmed a huge fibroma (5.4 × 4.5 × 2.7 cm), which was extended to the endocardium on both right and left ventricles and the posterior apical septum. The child is now in a very good physical condition. Although he is under oral medication consisting of amiodarone 100 mg daily and atenolol 12.5 mg daily, his ECG presents permanent disorders such as negative T-waves in lead II, aVF and in V3-V6.

Figure 4
Figure 4:
Transoesophageal echocardiography image showing a large tumour involving the inferoseptal and apical wall of the left ventricle. Reproduced with the kind permission of the Children's Hospital, Boston, MA, USA.

Primary heart tumours are uncommon in all age groups, with an incidence rate of 0.0017% in a large autopsy series reviewed by Straus and Merliss [1]. Primary heart tumours are extremely rare in infants and children under 16 yr of age [2]. Nadas and Ellison [3] reviewed the large experience of the Boston Children's Hospital and cited a frequency of 0.027% among 11 000 paediatric autopsies. These studies were all carried out before the routine application of cross-sectional electrocardiography. According to data from 1980 to 1995 from the Hospital for Sick Children at Toronto, Canada [4], 27 640 subjects (from fetuses to patients of 18 yr of age) were referred for cardiac disease, and 56 of these had a primary cardiac tumour. The most common primary heart tumours that occur in children are rhabdomyomas and cardiac fibromas [2,5]. Cardiac fibromas are histologically benign but potentially lethal. Clinical signs and symptoms depend largely on the location and size of the tumour. Symptoms might include heart failure, arrhythmias (supraventricular or ventricular tachycardia is a known complication of these tumours and may cause sudden death) [6], murmurs, sudden death, cyanosis and chest pain. Fibromas can present as intramural or intracavitary masses. The left ventricular free wall and the septum are most often involved [7]. Right ventricular fibromas are rare. Occasionally, a cardiac fibroma might grow to an enormous size while the patient is asymptomatic. In a retrospective review of the files of the Armed Forces Institute of Pathology between 1967 and 1993, 23 cases of cardiac fibromas were identified. Two were incidental findings [8].

The present case made us reconsider the need for further preanaesthetic investigation in children in order to detect similar cases before a scheduled operation. The current data suggest that there is no need for preoperative ECG if there are no abnormal findings from clinical examination. Additionally, the possibility of a preanaesthetic diagnosis of such a disorder by a single ECG is very small. Further, establishing echocardiography as a routine preanaesthetic evaluation is extremely expensive. This fact, in addition to the rarity of such disorders, led us to the decision of maintaining the same practice of avoiding unnecessary examinations. Although there is no doubt that such rare cases may cause major problems during anaesthesia, it was considered that it would be rather unusual for the anaesthesiologist to face medico-legal consequences because the vast majority would not perform such investigations in children for routine surgery merely on the off-chance of detecting an extremely rare condition.

A. Leontidou

K. Papadopoulou

K. Vlachtsis

A. Douvantzi

A. Zambouri

P. Petropoulou

Departments of Anaesthesiology, Paediatrics and Laryngology; Papageorgiou General Hospital; Thessaloniki, Greece


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© 2004 European Society of Anaesthesiology