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Journal of Indian Society of Pedodontics and Preventive Dentistry Official publication of Indian Society of Pedodontics and Preventive Dentistry
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Year : 2011  |  Volume : 29  |  Issue : 6  |  Page : 66-69

Dental management of PHACE syndrome under general anesthesia

1 Department of Anesthesiology, T. N. Medical College and B. Y. L. Nair Charitable Hospital, Mumbai, India
2 Department of Pediatric Dentistry, Nair Hospital Dental College, Mumbai, India

Date of Web Publication12-Dec-2011

Correspondence Address:
A Kakade
Department of Pediatric Dentistry, Nair Hospital Dental College, Dr. A. L. Nair Road, Mumbai Central, Mumbai - 400 008
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Source of Support: None, Conflict of Interest: None

DOI: 10.4103/0970-4388.90745

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PHACE syndrome was first described by Dr. Ilonia Frieden and colleagues in 1996. It is an under-recognized rather than a very rare condition among patients with large facial hemangiomas. It is challenging as it has significant neurological, vascular and airway implications. Vascular malformations compromising cerebral blood flow predispose the patient to strokes and seizures. Subglottic hemangiomas, if present, could bleed during intubation. Meticulous neurological monitoring is mandatory in those undergoing repair of the great vessels. We describe the perioperative management of a child with PHACE syndrome subjected to dental treatment under general anesthesia.

Keywords: Dental treatment, general anesthesia, PHACE syndrome

How to cite this article:
Fernandes S, Kakade A, Jetpurwala A M, Patil B, Jain S, Kasar P. Dental management of PHACE syndrome under general anesthesia. J Indian Soc Pedod Prev Dent 2011;29, Suppl S1:66-9

How to cite this URL:
Fernandes S, Kakade A, Jetpurwala A M, Patil B, Jain S, Kasar P. Dental management of PHACE syndrome under general anesthesia. J Indian Soc Pedod Prev Dent [serial online] 2011 [cited 2023 Jan 29];29, Suppl S1:66-9. Available from: http://www.jisppd.com/text.asp?2011/29/6/66/90745

   Introduction Top

The association of cerebrovascular and facial arterial anomalies with hemangiomas was first noticed by Pascual-Castroviejo. [1] Later, Reese et al.[2] described the association of facial hemangiomas with Dandy Walker and other posterior fossa malformations. The acronym PHACE was proposed by Frieden et al. PHACE syndrome is a phakomatosis which is easily confused with  Sturge- Weber syndrome More Details More Details.

In November 2008, at the PHACE syndrome Research Conference in Houston, a panel was formed to discuss the key features of PHACE. They defined major and minor characteristics for diagnosis.

The major criteria are the following:

  1. Cerebrovascular: Anomalies of the major cerebral arteries, posterior fossa anomalies
  2. Cardiovascular: Aortic arch anomaly, aberrant origin of the subclavian artery
  3. Ocular: Posterior segment abnormalities
  4. Ventral or midline: Sternal defects
The minor criteria are the following:

  1. Cerebrovascular: Persistent embryonic artery other than trigeminal artery
  2. Structural brain: Enhancing extra-axial lesion with features consistent with intracranial hemangioma
  3. Cardiovascular: Ventricular septal defect, right aortic arch
  4. Ocular: Anterior segment anomalies
  5. Ventral or midline: Hypopituitarism

The diagnosis of PHACE syndrome requires the presence of a segmental hemangioma or hemangioma >5 cm 2 of the head (face or scalp) plus one major or two minor criteria. [3]

It has been termed as PHACES syndrome when associated with sternal defects and/or supraumbilical raphae. Females are affected eight times as compared to males. The predominance of female gender led to the speculation that PHACE syndrome might represent an X-linked dominant condition with lethality in males.

   Case Report Top

A 3-year-old boy (weight 11 kg, height 93 cm) reported to the Department of Pediatric Dentistry, Nair Hospital Dental College, Mumbai (NHDC). The parents complained of multiple decayed teeth. The child showed delayed developmental and motor milestones. He was having weakness on the right side of both the upper and lower extremities and impaired speech. There were no cranial nerve anomalies. A detailed medical history, clinical evaluation and previous medical reports revealed that the child was a known case of PHACE syndrome. There was a hemangioma near the right upper lip and right eyelid. On presentation, the hemangiomas measured approximately 3 cm × 2 cm on the upper lip [Figure 1]. Parents gave a history that the hemangioma had decreased in size after treatment with propranolol. The right eye was amblyopic due to the ptosis caused by the hemangioma. The child was taking Tab. aspirin 75 mg OD and Tab. propranolol 10 mg BD since 1 year of age. Preliminary oral examination revealed multiple badly carious deciduous teeth; however, detailed examination of the oral cavity was not possible. As the child was uncooperative and extensive dental treatment was required, it was decided to perform the same under general anesthesia (GA). Pre-operative pulse rate was 80/min, regular and equal in all four extremities, blood pressure was 100/60 mm Hg. There were no abnormal findings in the cardiovascular and respiratory systems. Hemogram, biochemical parameters, X-ray chest, electrocardiography (ECG), two-dimensional echocardiography (2D Echo), electroencephalogram (EEG) and ultrasonography (USG) abdomen were within normal limits. Computed tomography (CT) scan showed Dandy Walker variant with bilateral hypoplastic cerebellar hemispheres [Figure 2]. Magnetic resonance imaging (MRI) angiography revealed agenesis of the left internal carotid artery and its reformation by the ascending pharyngeal artery [Figure 3]. The left anterior cerebral artery (ACA) and middle cerebral artery (MCA) were seen to originate from the reformed left internal carotid artery (ICA) [Figure 4]. The right ICA was moderately dilated and tortuous in its entire course. Rest of the major intracranial and neck vessels were normal in size and caliber. MRI neck ruled out any airway hemangiomas. Orthopantomogram (OPG) did not reveal any hemangiomas in the jaw. Propranolol was continued, but aspirin was stopped 5 days prior to the procedure.
Figure 1: Hemangioma of upper lip and eyelid and patient consent

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Figure 2: Dandy Walker variant: Slit-like communication between the fourth ventricle and cisterna magna. Inferior vermis and cerebellar hemispheres are hypoplastic

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Figure 3: Agenesis of left internal carotid artery (ICA). Ectasia of right common carotid artery (CCA) and ICA noted

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Figure 4: Left middle cerebral artery (MCA) is reformed by left posterior communicating artery which is tortuous and ectatic. M1 segments of bilateral MCAs are ectatic. Left anterior cerebral artery (ACA) is reformed by anterior communicating artery

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Intravenous access was secured the night prior to surgery to facilitate hydration. Glycopyrolate 0.04 mg/kg i.v. and midazolam 0.03 mg/kg i.v. were used as premedicants. Anesthesia was induced with sevoflurane and fentanyl (2 mcg/kg). When a bispectral index (BIS) of 40 was reached, orotracheal intubation was performed. Muscle relaxants were not used. Anesthesia was maintained using sevoflurane (1.5-2%) and fentanyl. Monitoring included non-invasive blood pressure (NIBP), ECG, pulse oximetry, capnography and BIS. The procedure included endodontic management of 85, restoration of 55, 65, 75, and stainless steel crowns adjusted and cemented on 55, 65, 75, 85. Extraction of all remaining deciduous teeth was done and resorbable sutures were taken [Figure 5] and [Figure 6]. The procedure lasted for 2 hours. The child was extubated after hemostasis was achieved. The postoperative period was uneventful and the child was discharged on the second day.
Figure 5: Maxillary arch after treatment

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Figure 6: Mandibular arch after treatment

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   Discussion Top

PHACE syndrome was first described in 1996 by Frieden et al. in a review of 43 patients with facial hemangiomas and central nervous system abnormalities. [4] Our patient had all the components of PHACE except aortic coarctation which was ruled out on a 2D Echo. Patients with PHACE syndrome may present for ophthalmic, vascular, neurologic or otolaryngologic surgery.

The neurologic features of PHACES can be either congenital anomalies which include structural malformations of the cerebral vasculature, cerebellum and cerebrum or progressive stenosis and occlusions of the principal cerebral arteries. Steno-occlusive arterial disease leads to arterial ischemic stroke and a moyamoya-like vasculopathy in some affected children. [5] They are usually started on aspirin as they are prone to cerebrovascular thrombosis. The results of a study conducted by Joel et al. suggest that invasive dental treatment can be performed safely in patients who are receiving single or dual anti-platelet therapy without altering or discontinuing the use of their anti-platelet medications. The risk of postoperative bleeding complications in these patients is low to negligible and the use of local hemostatic measures is sufficient. [6] However, the neurologists and dental surgeons at our institution decided to discontinue aspirin 5 days prior to the procedure.

Strokes and seizures are generally at their worst when the hemangioma is in its growth phase - can last anywhere from 6 to 18 months. Hypotension in the perioperative period should be treated immediately as these patients can suffer strokes due to cerebral ischemia. Neuromonitoring can detect changes in cerebral perfusion and oxygenation during surgery. BIS, near-infrared spectroscopy (NIRS) and transcranial Doppler have been reliably used to detect and prevent prolonged cerebral ischemia. BIS reflects EEG activity in pediatric patients under GA and has been shown to be a quantitative, nondisruptive and easy-to-use depth of sedation monitor in children. [7] Decrease in cerebral blood flow may sometimes be associated with a decrease in BIS. We maintained a BIS of around 50-60 during the intraoperative period. Aurelia et al. have reported a case of thoracic aorto-aortic bypass in a child with PHACE syndrome where they successfully used near-infrared spectroscopy (NIRS) to monitor cerebral oxygen saturation. They maintained NIRS values above 50 throughout surgery. [8] Patients with seizures would be on anticonvulsants which have to be continued in the perioperative period. Our patient had a normal EEG and no history of convulsions. We chose to avoid the use of muscle relaxants as the child was hypotonic. Also, the dental procedure did not necessitate profound relaxation.

The most conspicuous vascular malformations are often nearest to the facial hemangioma itself or stand in direct relation to the intracranial feeder vessels. Oslow et al. found that airway hemangiomas occur much more commonly (63%) in children with cutaneous hemangiomas, so these children should be considered to have an airway hemangioma until proved otherwise. Subglottic hemangiomas might bleed with instrumentation or intubation of the airway. Awake flexible fiberoptic endoscopy provides the optimal means of evaluating the nasopharynx, tongue base and supraglottic region. [9] As oral/airway hemangiomas had been ruled out by MRI angiography, we decided to proceed with routine laryngoscopy and orotracheal intubation. Facial hemangiomas may make mask holding difficult.

Propranolol, a nonselective beta blocker, has been used to treat infantile capillary hemangiomas. It has been found to induce vasoconstriction, decreased expression of vascular endothelial growth factor and basic fibroblast growth factor through the downregulation of the RAF-mitogen-activated protein kinase pathway and the triggering of apoptosis of capillary endothelial cells. [10] Propranolol is contraindicated in patients who have bronchospasm, CCF, hypotension, bradycardia and heart blocks. As the muscarinic effect of succinylcholine on the beta blocked heart is a cause for concern, it is best reserved for situations when it is absolutely indicated. Profound bradycardia after neostigmine has been reported in a patient taking propranolol and close observation of heart rate and blood pressure during this period is mandatory. [11] Hypoglycemia is an adverse effect in patients receiving propranolol; hence, we monitored the blood glucose levels during the perioperative period. Bonifazi and colleagues reported severe hypoglycemia and a resultant seizure in an infant who had received 2 mg/kg propranolol for 5 months to treat diffuse hemangiomatosis. [12]

PHACE syndrome has multisystem involvement, and a thorough evaluation prior to surgery is mandatory.

   Acknowledgments Top

We express our gratitude to Dr. Ravi Rananavare (Dean, Professor and Head) and Dr. Bhakti Yergi (Assistant Professor) from Department of Radiology, T. N. Medical College and B. Y. L. Nair Hopsital, Mumbai, for their assistance in interpretation of the MRI reports.

   References Top

1.Pascual-Castroviejo I. Vascular and nonvascular intracranial malformations associated with External capillary hemangiomas. Neuroradiology 1978;16:82-4.  Back to cited text no. 1
2.Reese V, Frieden IJ, Paller AS, Esterly NB, Ferriero D, Levy ML, et al. Association of facial hemangiomas with Dandy Walker and other posterior fossa malformations. J Pediatr 1993;122:379-84.  Back to cited text no. 2
3.Metry D, Heyer G, Hess C, Garzon M, Haggstrom A, Frommelt P, et al. Consensus statement on diagnostic criteria for PHACE syndrome. Pediatrics 2009;124:1447-56.  Back to cited text no. 3
4.Frieden JJ, Reese V, Cohen D. PHACE syndrome. The association of posterior fossa brain malformations, hemangiomas, arterial anomalies, coarctation of the aorta and cardiac defects and eye abnormalities. Arch Dermatol 1996;132:307-11.   Back to cited text no. 4
5.Drolet BA, Dohil M, Golomb MR, Wells R, Murowski L, Tamburro J, et al. Early stroke and cerebral vasculopathy in children with facial hemangiomas and PHACE association. Pediatrics 2006;117:959-64.  Back to cited text no. 5
6.Napen~ as JJ, Hong CH, Brennan MT, Furney SL, Fox PC, Lockhart PB. The frequency of bleeding complications after invasive dental treatment in patients receiving single and dual antiplatelet therapy. J Am Dent Assoc 2009;140:690-5.  Back to cited text no. 6
7.Sadhasivam S, Ganesh A, Robison A, Kaye R, Watcha MF. Validation of the bispectral index monitor for measuring the depth of sedation in children. Anesth Analg 2006;102:383-8.  Back to cited text no. 7
8.Javault A, Metton O, Raisky O, Bompard D, Hachemi M, Gamondes D, et al. Anesthesia management in a child with PHACE syndrome and agenesis of bilateral internal carotid arteries. Pediatr Anesth 2007;17:989-93.  Back to cited text no. 8
9.Bent JP. Airway hemangiomas: Contemporary management. Lymphat Res Biol 2004;2:56-60.  Back to cited text no. 9
10.Sommers Smith SK, Smith DM. Beta blockade induces apoptosis in cultured capillary endothelial cells. In vitro Cell Dev Biol Anim 2002;38:298-304.  Back to cited text no. 10
11.Sprague DH. Severe bradycardia after neostigmine in a patient taking propranolol to control paroxysmal atrial tachycardia. Anaesthesiology 1975;42:208.  Back to cited text no. 11
12.Bonifazi E, Acquafredda A, Milano A, Montagna O, Laforgia N. Severe hypoglycemia during successful treatment of diffuse hemangiomatosis with propranolol. Pediatr Dermatol 2010;27:195-6.  Back to cited text no. 12


  [Figure 1], [Figure 2], [Figure 3], [Figure 4], [Figure 5], [Figure 6]

This article has been cited by
1 Anesthetic considerations for a child with PHACE syndrome: a case report
Wadha Alotaibi, Zainab Alzayer
European Journal of Medical Case Reports. 2018; : 45
[Pubmed] | [DOI]


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