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Aberrant facial flushing following monobloc fronto-facial distraction
Journal of Cranio-Maxillo-Facial Surgery 43 (2015) 1511e1515
Contents lists available at ScienceDirect
Journal of Cranio-Maxillo-Facial Surgery journal homepage: www.jcmfs.com
Aberrant facial flushing following monobloc fronto-facial distraction Alistair R.M. Cobb a, *, Michael Vourvachis b, Jahangir Ahmed c, Michelle Wyatt d, David Dunaway b, 1, Richard Hayward e a
Department of Oral & Maxillofacial Surgery, United Hospitals Bristol NHS Trust, Bristol, UK Craniofacial Service, Great Ormond Street Hospital for Children, London, UK c Department of Otolaryngology, Royal London Hospital, London, UK d Department of Paediatric Otolaryngology, Great Ormond Street Hospital for Children, London, UK e Department of Paediatric Neurosurgery, Great Ormond Street Hospital for Children, London, UK b
a r t i c l e i n f o
a b s t r a c t
Article history: Paper received 13 April 2015 Accepted 14 July 2015 Available online 20 July 2015
Background: Patients with syndromic forms of craniosynostosis may experience functional problems such as raised intracranial pressure, proptosis, obstructive sleep apnoea and failure to thrive. The monobloc fronto-facial advancement with osteogenic distraction is increasingly used to correct these functional problems in one procedure as well as improve appearance. The authors report the phenomenon of post operative aberrant facial flushing e an unusual and previously unreported complication of the procedure. Methods: The case notes of 80 consecutive patients undergoing fronto-facial advancement by distraction using the rigid external distraction device (RED) were reviewed for features of aberrant facial flushing. Results: Four out of eighty individuals developed facial flushing after monobloc fronto-facial distraction using the rigid external distractor (RED) frame. All were female with Crouzon or Pfeiffer syndromes causing the severe functional problems for which they underwent the surgery. They were aged 6e8 years. Following removal of the frame, they developed intermittent but severe facial flushing. The flushing spontaneously settled in three patients after up to four years but persists in the other child seven years after her surgery. Conclusion: Aberrant facial flushing is a rare but significant complication of monobloc fronto-facial surgery. It occurred in 4 of our 80 (5%) patients. The skull base osteotomies essential for the procedure are made anterior to the pterygopalatine ganglion and it is our contention that damage from these was responsible for a neuropraxia of its efferent nerve branches. A review of the autonomic control of the facial vascular system suggests that the phenomenon is due to an unequal process of recovery that leaves the cutaneous vasodilating parasympathetic or beta-adrenergic innervation relatively unopposed e a situation that persists until with time a normal balance of autonomic input is achieved. © 2015 European Association for Cranio-Maxillo-Facial Surgery. Published by Elsevier Ltd. All rights reserved.
Keywords: Monobloc Fronto-facial Craniosynostosis Facial flushing Autonomic dysfunction
1. Introduction Patients with syndromic forms of craniosynostosis may experience functional problems such as raised intracranial pressure, proptosis, obstructive sleep apnoea and failure to thrive. Cosmetic concerns become increasingly important as the child ages. The monobloc fronto-facial advancement with osteogenic distraction is
* Corresponding author. Southwest UK Cleft Service, Lower Maudlin Street, Bristol BS1 2LY, UK. Tel.: þ44 117 342 1180. E-mail address: [email protected] (A.R.M. Cobb). 1 Craniofacial Service, Great Ormond Street Hospital. Lead: David Dunaway.
increasingly used to correct these functional problems in one procedure as well as improve appearance (Ortiz-Monasterio et al.,1978). In this paper we describe the presentation of post-operative facial flushing e an unusual and previously unreported complication of the procedure. The potential mechanism is discussed. 2. Patients and methods The case notes of 80 consecutive patients undergoing frontofacial advancement by distraction using the rigid external distraction device (RED) were reviewed. The surgical technique was as described by Ortiz-Monasterio (Ortiz-Monasterio et al., 1978;
http://dx.doi.org/10.1016/j.jcms.2015.07.005 1010-5182/© 2015 European Association for Cranio-Maxillo-Facial Surgery. Published by Elsevier Ltd. All rights reserved.
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A.R.M. Cobb et al. / Journal of Cranio-Maxillo-Facial Surgery 43 (2015) 1511e1515
Molina, 2004). The distraction process itself was commenced on the seventh post operative day and continued until the required advance had been achieved. The frame was then left on for a further few weeks to allow bony consolidation to occur e usually 6e8 weeks (Jones et al., 2004). 3. Results Four of eighty individuals (5%) undergoing a monobloc frontofacial advance using external distraction with or (usually) without bipartition developed a phenomenon we have called aberrant facial flushing. The details of the four cases are as follows: 3.1. Case 1 This girl presented to the craniofacial department at Great Ormond Street Hospital with a diagnosis of Pfeiffer Syndrome at age 3 months. Since then she had undergone a number of operations including a cranial vault expansion and a ventriculoperitoneal (VP) shunt for increased intra-cranial pressure and an adeno-tonsillectomy to reduce her upper airway obstruction. At age 7 years, she underwent monobloc fronto-facial distraction according to the protocol described above for recurrence of her increased intra-cranial pressure associated now with severe (cornea threatening) proptosis and continuing upper airway difficulties due to her maxillary hypoplasia. Approximately 3e4 months following the removal of the RED frame, she presented with intermittent attacks of severe bilateral facial flushing and swelling, streaming nasal discharge (non cerebrospinal fluid rhinorrhoea) and excessive epiphora lasting over 2 h each time. This was far worse on the right side. This is triggered by any more marked change in temperature e eg going outside or returning into a house, or emotional triggers e eg crying (not anger). These symptoms, which are a source of considerable discomfort to the patient, persist 7 years following surgery. 3.2. Case 2 This girl with Pfeiffer Syndrome had previously undergone two cranial vault expansions but aged 6 years was considered on the basis of her optic disc appearances and recent deterioration in her visual evoked potentials to have recurrence of increased intracranial pressure. She had a moderate airway obstruction for which CPAP was being considered and her degree of proptosis was starting to threaten her corneas. She was also being teased at school because of her appearance. In view of these functional and cosmetic issues a fronto-facial monobloc distraction procedure was performed. Three months after RED frame removal a dramatic improvement in her airway obstruction was recorded, her proptosis had been eliminated and her visual evoked potentials (Liasis et al., 2006) showed no further deterioration. She was delighted too with the improvement in her appearance. However, following the removal of the RED frame, she developed attacks of severe intermittent facial flushing. These continued for two years and then reduced in their intensity, disappearing altogether by four years after her surgery. 3.3. Case 3 This girl with Crouzon syndrome (Fig. 1) who had previously required an adenotonsillectomy and a nasopharyngeal airway for airway obstruction and had also undergone two vault expansions and an endoscopic third ventriculostomy for raised ICP underwent monobloc fronto-facial advancement with distraction at nearly six
Fig. 1. Case 3 e 3D reconstructed CT pre-operatively showing markedly retruded middle and upper 1/3s of the craniofacial skeleton.
years of age for a combination of recurrent ICP, advancing exorbitism, continuing airway problems requiring increasing CPAP pressures together with psychosocial issues relating to her appearance (Fig. 2). At her three-month review following the removal of the RED frame she reported episodic right sided facial flushing. The phenomenon presented around the orbit and would come on when she cried. This was accompanied by a non-CSF rhinorrhoea. By twenty months post-surgery it had resolved. 3.4. Case 4 This girl with Crouzon syndrome underwent monobloc frontofacial advancement by distraction at eight years of age for obstructive sleep apnoea and advancing exorbitism that threatened the corneas. Her surgery and the distraction period itself passed without complications but following the frame removal she reported facial flushing, with a clearly demarcated pattern particularly around the nose, that came on after crying or exertion. 4. Discussion Four out of 80 children with complex craniosynostosis-associated syndromes undergoing monobloc distraction surgery developed aberrant facial flushing that was first noticed 2e3 months after the procedure and recovered spontaneously in 3 after 2e4 years. It is our contention that the procedure produced an imbalance between the sympathetic and parasympathetic inputs that are normally responsible for facial cutaneous vasomotor control. The autonomic supply to the face is from the pterygopalatine ganglion, which lies in the pterygopalatine fossa close to the sphenopalatine foramen and 2.7 mm from the end of the Vidian
A.R.M. Cobb et al. / Journal of Cranio-Maxillo-Facial Surgery 43 (2015) 1511e1515
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Fig. 2. Case 3 e 3D reconstructed CT post operatively showing corrected forehead and facial form.
canal (Fig. 3), (Alvernia et al., 2007; Roberti et al., 2007) Parasympathetic efferent fibres run from the superior salivary nucleus via the nerve of the pterygoid canal (Vidian's nerve) and greater petrosal nerve from the nervus intermedius of the facial nerve to reach the ganglion. Here they synapse and then the secretomotor parasympathetic supply to the lacrimal gland runs via zygomatic and lacrimal nerves and to the mucous glands of the nose, nasopharynx and palate via maxillary nerve branches. The sympathetic supply arises from the superior cervical ganglion and then runs via the internal carotid nerve, deep petrosal nerve and Vidian's nerve to the pterygopalatine ganglion before continuing through the same routes as the parasympathetic fibres although without synapsing (McMinn, 1994). There is evidence that facial veins may have a specialized vasodilatatory mechanism compared to other cutaneous vessels, with intrinsic basal tone. They are supplied with beta-adrenoceptors in addition to alpha-adrenoceptors. Vasoconstrictor tone in the face is low (van der Meer, 1985) and Alpha-blockade has been demonstrated to have no effect on facial vasodilatation in blushing experiments. Vasodilator effects mediated via beta-adrenoceptors within the facial cutaneous venous plexus (Mellander et al., 1982) are therefore more likely to be responsible for aberrant facial flushing (Drummond, 1999). The association with lacrimation and rhinorrhoea (cases 1 & 3) however suggests an increased parasympathetic effect. This is also seen in cluster headaches (Goadsby, 2013), a severe unilateral and periorbital facial pain syndrome often associated with such autonomic symptoms as ipsilateral lacrimation, nasal congestion, conjunctival injection, meiosis, ptosis, and eyelid oedema (Lad et al., 2007) that can be alleviated (temporarily) by local anaesthetic blockade, or (more permanently) by gamma knife destruction of the pterygopalatine ganglion (Lad et al., 2007; Narouze et al., 2009). The fact that Horner syndrome is characterised (amongst other features such as pupillary changes etc) by impairment of flushing
Fig. 3. The sphenopalatine ganglion (indicated by red arrow) lies immediately behind the external osteotomy cuts (dotted lines) which places it at risk during surgery.
and sweating on the affected side suggests that the cervical sympathetic outflow is the main pathway for thermoregulatory and emotional flushing of the face (Drummond and Lance, 1987). In a study of 21 patients with a facial nerve lesion compromising parasympathetic outflow to the lacrimal gland, and 13 patients undergoing diagnostic blockade of the stellate ganglion, Drummond (1995) demonstrated different neurovascular mechanisms for the forehead and face with a parasympathetic vasodilator reflex in the facial nerve contributing to gustatory flushing in the forehead, but some other unidentified mechanism for vascular responses in the cheeks. In brief, while aberrant facial flushing following monobloc surgery could be due to either exaggerated parasympathetic or betasympathetic activity or to a failure of sympathetic inhibition, the evidence argues against the latter. In theory either the pterygo-palatine ganglion itself or outflow from it could be damaged during monobloc surgery by the osteotomy cut through the vomer and septum or the pterygomaxillary dysjunction (Fig. 4) (necessary for the separation/ down-fracture of the viscerocranium from the neurocranium) or by the down-fracture process itself. It is unlikely that the ganglion itself would be directly damaged as it lies anterior to the opening of pterygoid canal on the skull base
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An alternative scenario suggested by the occurrence of unilateral facial flushing at times of increased emotion or exertion (cases 3 and 4) is that it is the reddening side that is normal while the expected physiological response on the opposite side has been inhibited by a defect of beta-sympathetic input (Lance et al., 1988) In this case it would be the parasympathetic input to the facial vascular system that was taking longer to recover leaving the sympathetic unopposed. 5. Conclusions Aberrant facial flushing is a rare complication of monobloc fronto-facial surgery. It occurred in 4 of our 80 (5%) patients, being first noticed 2e3 months following the operation (around the time of the external distraction frame removal) and resolving over 2e4 years in 3 of them. The skull base osteotomies essential for the procedure are made anterior to the pterygopalatine ganglion and it is our contention that damage from these was responsible for a neuropraxia of its efferent nerve branches. A review of the autonomic control of the facial vascular system suggests that the phenomenon is due to an unequal process of recovery that leaves the cutaneous vasodilating parasympathetic or beta-adrenergic innervation relatively unopposed e a situation that persists until with time a normal balance of autonomic input is achieved. Unfortunately the essential requirement of the monobloc procedure (the disconnection of the viscerocranium from the neurocranium) makes it difficult to avoid a degree of surgical trauma in this region that may rarely be responsible for aberrant facial flushing. Fig. 4. The internal osteotomy cuts during craniofacial dysjunction disrupt the internal nasal anatomy made by the craniofacial dysjunction (arrows) as seen on this postoperative axial view CT of case 3. This may affect the nasal efferent autonomic supply.
Conflict of interest None.
(McMinn, 1994) because the osteotomy cuts and the down-fracture are carried out forward of this point. This would however still leave it vulnerable to trauma causing a gangliopraxia. More plausible is functional damage to its anterior (efferent) branches. The fact that in those in whom it resolved it took 2e4 years to do years implies a lengthy process of either recovery from neuropraxia or of reinnervation e a phenomenon known to occur following Vidian neurectomy performed for severe chronic vasomotor rhinitis resistant to medical treatment (Krajina, 1989; Konno, 2010). We suggest that it is damage to efferents from the pterygopalatine ganglion followed by an inequality in their speed of recovery (either from neurotmesis or regeneration/reinnervation in the case of complete disruption) that is responsible for the phenomenon of aberrant facial flushing we have described. This theory implies that greater vulnerability of the alpha-adrenergic fibres (or a less effective capacity for recovery or regeneration) allows for excessive parasympathetic, or beta-adrenergic, activity e a situation that continues until (in 3 of 4 cases) a state of balanced innervation is eventually restored. Monobloc surgery causes a major disruption to the soft tissues of the face to produce often severe swelling that may persist for several weeks. This could account for the onset of aberrant facial flushing being first noticed by all four patients around the time of the removal of the distraction apparatus (the skull pins and the transcutaneous posts that connect to the Polley plates) e the problem may have present since surgery but “occult” until then. But if at surgery all autonomic input to the facial vascular system was abolished and subsequent (unequal) functional reinnervation was not present until 2e3 months later, this could explain the “late” onset of aberrant facial flushing without having to invoke it having previously been “hidden” by facial swelling and soft tissue bruising.
Funding No sources of support in the form of grants were received. Acknowledgement We would like to thank David Smithson at the ICH/GOS Graphic Design Studio for the illustrations. References Alvernia JE, Spomar DG, Olivero WC: A computed tomography scan and anatomical cadaveric study of the pterygopalatine ganglion for use in Gamma Knife treatment of cluster headache. J Neurosurg 107: 805e808, 2007 Drummond PD, Lance JW: Facial flushing and sweating mediated by the sympathetic nervous system. Brain 110: 793e803, 1987 Drummond PD: Mechanisms of physiological gustatory sweating and flushing in the face. J Auton Nerv Syst 8: 117e124, 1995 Drummond PD: Facial flushing during provocation in women. Psychophysiology 36: 325e332, 1999 Goadsby PJ: Sphenopalatine (pterygopalatine) ganglion stimulation and cluster headache: new hope for ye who enter here. Cephalalgia 33: 813e815, 2013 Jones BM, Dunaway DJ, Hayward RD: Surgery. In: Hayward RD, Jones BM, Dunaway DJ, Evans RD (eds), The clinical management of craniosynostosis, 1st ed. London: Mac Keith Press, 391e395, 2004 Konno A: Historical, pathophysiological, and therapeutic aspects of vidian neurectomy. Curr Allergy Asthma Rep 10: 105e112, 2010 Krajina Z: Critical review of vidian neurectomy. Rhinology 27: 271e276, 1989 Lad SP, Lipani JD, Gibbs IC, Chang SD, Adler Jr JR, Henderson JM: Cyberknife targeting the pterygopalatine ganglion for the treatment of chronic cluster headaches. Neurosurgery 60: E580eE581, 2007 Lance JW, Drummond PD, Gandevia SC, Morris JG: Harlequin syndrome: the sudden onset of unilateral flushing and sweating. J Neurol Neurosurg Psychiatry 51: 635e642, 1988 Liasis A, Nischal KK, Walters B, Thompson D, Hardy S, Towell A, et al: Monitoring visual function in children with syndromic craniosynostosis: a comparison of 3 methods. Arch Ophthalmol 124: 1119e1126, 2006 McMinn RMH: Last's anatomy, 9th ed. Edinburgh: Churchill Livingstone, 34, 1994
A.R.M. Cobb et al. / Journal of Cranio-Maxillo-Facial Surgery 43 (2015) 1511e1515 Mellander S, Andersson PO, Afzelius LE, Hellstrand P: Neural beta-adrenergic dilatation of the facial vein in man. Possible mechanism in emotional blushing. Acta Physiol Scand 114: 393e399, 1982 Molina F: From midface distraction to the “true monoblock”. Clin Plast Surg 31: 463e479, 2004 Narouze S, Kapural L, Casanova J, Mekhail N: Sphenopalatine ganglion radiofrequency ablation for the management of chronic cluster headache. Headache 49: 571e577, 2009
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Ortiz-Monasterio F, del Campo AF, Carrillo A: Advancement of the orbits and the midface in one piece, combined with frontal repositioning, for the correction of Crouzon's deformities. Plast Reconstr Surg 61: 507e516, 1978 Roberti F, Boari N, Mortini P, Caputy AJ: The pterygopalatine fossa: an anatomic report. J Craniofac Surg 18: 586e590, 2007 van der Meer C: Pharmacotherapy of “idiopathic” excessive blushing and hyperhidrosis. Acta Neurochir (Wien) 74: 151e152, 1985
Contents lists available at ScienceDirect
Journal of Cranio-Maxillo-Facial Surgery journal homepage: www.jcmfs.com
Aberrant facial flushing following monobloc fronto-facial distraction Alistair R.M. Cobb a, *, Michael Vourvachis b, Jahangir Ahmed c, Michelle Wyatt d, David Dunaway b, 1, Richard Hayward e a
Department of Oral & Maxillofacial Surgery, United Hospitals Bristol NHS Trust, Bristol, UK Craniofacial Service, Great Ormond Street Hospital for Children, London, UK c Department of Otolaryngology, Royal London Hospital, London, UK d Department of Paediatric Otolaryngology, Great Ormond Street Hospital for Children, London, UK e Department of Paediatric Neurosurgery, Great Ormond Street Hospital for Children, London, UK b
a r t i c l e i n f o
a b s t r a c t
Article history: Paper received 13 April 2015 Accepted 14 July 2015 Available online 20 July 2015
Background: Patients with syndromic forms of craniosynostosis may experience functional problems such as raised intracranial pressure, proptosis, obstructive sleep apnoea and failure to thrive. The monobloc fronto-facial advancement with osteogenic distraction is increasingly used to correct these functional problems in one procedure as well as improve appearance. The authors report the phenomenon of post operative aberrant facial flushing e an unusual and previously unreported complication of the procedure. Methods: The case notes of 80 consecutive patients undergoing fronto-facial advancement by distraction using the rigid external distraction device (RED) were reviewed for features of aberrant facial flushing. Results: Four out of eighty individuals developed facial flushing after monobloc fronto-facial distraction using the rigid external distractor (RED) frame. All were female with Crouzon or Pfeiffer syndromes causing the severe functional problems for which they underwent the surgery. They were aged 6e8 years. Following removal of the frame, they developed intermittent but severe facial flushing. The flushing spontaneously settled in three patients after up to four years but persists in the other child seven years after her surgery. Conclusion: Aberrant facial flushing is a rare but significant complication of monobloc fronto-facial surgery. It occurred in 4 of our 80 (5%) patients. The skull base osteotomies essential for the procedure are made anterior to the pterygopalatine ganglion and it is our contention that damage from these was responsible for a neuropraxia of its efferent nerve branches. A review of the autonomic control of the facial vascular system suggests that the phenomenon is due to an unequal process of recovery that leaves the cutaneous vasodilating parasympathetic or beta-adrenergic innervation relatively unopposed e a situation that persists until with time a normal balance of autonomic input is achieved. © 2015 European Association for Cranio-Maxillo-Facial Surgery. Published by Elsevier Ltd. All rights reserved.
Keywords: Monobloc Fronto-facial Craniosynostosis Facial flushing Autonomic dysfunction
1. Introduction Patients with syndromic forms of craniosynostosis may experience functional problems such as raised intracranial pressure, proptosis, obstructive sleep apnoea and failure to thrive. Cosmetic concerns become increasingly important as the child ages. The monobloc fronto-facial advancement with osteogenic distraction is
* Corresponding author. Southwest UK Cleft Service, Lower Maudlin Street, Bristol BS1 2LY, UK. Tel.: þ44 117 342 1180. E-mail address: [email protected] (A.R.M. Cobb). 1 Craniofacial Service, Great Ormond Street Hospital. Lead: David Dunaway.
increasingly used to correct these functional problems in one procedure as well as improve appearance (Ortiz-Monasterio et al.,1978). In this paper we describe the presentation of post-operative facial flushing e an unusual and previously unreported complication of the procedure. The potential mechanism is discussed. 2. Patients and methods The case notes of 80 consecutive patients undergoing frontofacial advancement by distraction using the rigid external distraction device (RED) were reviewed. The surgical technique was as described by Ortiz-Monasterio (Ortiz-Monasterio et al., 1978;
http://dx.doi.org/10.1016/j.jcms.2015.07.005 1010-5182/© 2015 European Association for Cranio-Maxillo-Facial Surgery. Published by Elsevier Ltd. All rights reserved.
1512
A.R.M. Cobb et al. / Journal of Cranio-Maxillo-Facial Surgery 43 (2015) 1511e1515
Molina, 2004). The distraction process itself was commenced on the seventh post operative day and continued until the required advance had been achieved. The frame was then left on for a further few weeks to allow bony consolidation to occur e usually 6e8 weeks (Jones et al., 2004). 3. Results Four of eighty individuals (5%) undergoing a monobloc frontofacial advance using external distraction with or (usually) without bipartition developed a phenomenon we have called aberrant facial flushing. The details of the four cases are as follows: 3.1. Case 1 This girl presented to the craniofacial department at Great Ormond Street Hospital with a diagnosis of Pfeiffer Syndrome at age 3 months. Since then she had undergone a number of operations including a cranial vault expansion and a ventriculoperitoneal (VP) shunt for increased intra-cranial pressure and an adeno-tonsillectomy to reduce her upper airway obstruction. At age 7 years, she underwent monobloc fronto-facial distraction according to the protocol described above for recurrence of her increased intra-cranial pressure associated now with severe (cornea threatening) proptosis and continuing upper airway difficulties due to her maxillary hypoplasia. Approximately 3e4 months following the removal of the RED frame, she presented with intermittent attacks of severe bilateral facial flushing and swelling, streaming nasal discharge (non cerebrospinal fluid rhinorrhoea) and excessive epiphora lasting over 2 h each time. This was far worse on the right side. This is triggered by any more marked change in temperature e eg going outside or returning into a house, or emotional triggers e eg crying (not anger). These symptoms, which are a source of considerable discomfort to the patient, persist 7 years following surgery. 3.2. Case 2 This girl with Pfeiffer Syndrome had previously undergone two cranial vault expansions but aged 6 years was considered on the basis of her optic disc appearances and recent deterioration in her visual evoked potentials to have recurrence of increased intracranial pressure. She had a moderate airway obstruction for which CPAP was being considered and her degree of proptosis was starting to threaten her corneas. She was also being teased at school because of her appearance. In view of these functional and cosmetic issues a fronto-facial monobloc distraction procedure was performed. Three months after RED frame removal a dramatic improvement in her airway obstruction was recorded, her proptosis had been eliminated and her visual evoked potentials (Liasis et al., 2006) showed no further deterioration. She was delighted too with the improvement in her appearance. However, following the removal of the RED frame, she developed attacks of severe intermittent facial flushing. These continued for two years and then reduced in their intensity, disappearing altogether by four years after her surgery. 3.3. Case 3 This girl with Crouzon syndrome (Fig. 1) who had previously required an adenotonsillectomy and a nasopharyngeal airway for airway obstruction and had also undergone two vault expansions and an endoscopic third ventriculostomy for raised ICP underwent monobloc fronto-facial advancement with distraction at nearly six
Fig. 1. Case 3 e 3D reconstructed CT pre-operatively showing markedly retruded middle and upper 1/3s of the craniofacial skeleton.
years of age for a combination of recurrent ICP, advancing exorbitism, continuing airway problems requiring increasing CPAP pressures together with psychosocial issues relating to her appearance (Fig. 2). At her three-month review following the removal of the RED frame she reported episodic right sided facial flushing. The phenomenon presented around the orbit and would come on when she cried. This was accompanied by a non-CSF rhinorrhoea. By twenty months post-surgery it had resolved. 3.4. Case 4 This girl with Crouzon syndrome underwent monobloc frontofacial advancement by distraction at eight years of age for obstructive sleep apnoea and advancing exorbitism that threatened the corneas. Her surgery and the distraction period itself passed without complications but following the frame removal she reported facial flushing, with a clearly demarcated pattern particularly around the nose, that came on after crying or exertion. 4. Discussion Four out of 80 children with complex craniosynostosis-associated syndromes undergoing monobloc distraction surgery developed aberrant facial flushing that was first noticed 2e3 months after the procedure and recovered spontaneously in 3 after 2e4 years. It is our contention that the procedure produced an imbalance between the sympathetic and parasympathetic inputs that are normally responsible for facial cutaneous vasomotor control. The autonomic supply to the face is from the pterygopalatine ganglion, which lies in the pterygopalatine fossa close to the sphenopalatine foramen and 2.7 mm from the end of the Vidian
A.R.M. Cobb et al. / Journal of Cranio-Maxillo-Facial Surgery 43 (2015) 1511e1515
1513
Fig. 2. Case 3 e 3D reconstructed CT post operatively showing corrected forehead and facial form.
canal (Fig. 3), (Alvernia et al., 2007; Roberti et al., 2007) Parasympathetic efferent fibres run from the superior salivary nucleus via the nerve of the pterygoid canal (Vidian's nerve) and greater petrosal nerve from the nervus intermedius of the facial nerve to reach the ganglion. Here they synapse and then the secretomotor parasympathetic supply to the lacrimal gland runs via zygomatic and lacrimal nerves and to the mucous glands of the nose, nasopharynx and palate via maxillary nerve branches. The sympathetic supply arises from the superior cervical ganglion and then runs via the internal carotid nerve, deep petrosal nerve and Vidian's nerve to the pterygopalatine ganglion before continuing through the same routes as the parasympathetic fibres although without synapsing (McMinn, 1994). There is evidence that facial veins may have a specialized vasodilatatory mechanism compared to other cutaneous vessels, with intrinsic basal tone. They are supplied with beta-adrenoceptors in addition to alpha-adrenoceptors. Vasoconstrictor tone in the face is low (van der Meer, 1985) and Alpha-blockade has been demonstrated to have no effect on facial vasodilatation in blushing experiments. Vasodilator effects mediated via beta-adrenoceptors within the facial cutaneous venous plexus (Mellander et al., 1982) are therefore more likely to be responsible for aberrant facial flushing (Drummond, 1999). The association with lacrimation and rhinorrhoea (cases 1 & 3) however suggests an increased parasympathetic effect. This is also seen in cluster headaches (Goadsby, 2013), a severe unilateral and periorbital facial pain syndrome often associated with such autonomic symptoms as ipsilateral lacrimation, nasal congestion, conjunctival injection, meiosis, ptosis, and eyelid oedema (Lad et al., 2007) that can be alleviated (temporarily) by local anaesthetic blockade, or (more permanently) by gamma knife destruction of the pterygopalatine ganglion (Lad et al., 2007; Narouze et al., 2009). The fact that Horner syndrome is characterised (amongst other features such as pupillary changes etc) by impairment of flushing
Fig. 3. The sphenopalatine ganglion (indicated by red arrow) lies immediately behind the external osteotomy cuts (dotted lines) which places it at risk during surgery.
and sweating on the affected side suggests that the cervical sympathetic outflow is the main pathway for thermoregulatory and emotional flushing of the face (Drummond and Lance, 1987). In a study of 21 patients with a facial nerve lesion compromising parasympathetic outflow to the lacrimal gland, and 13 patients undergoing diagnostic blockade of the stellate ganglion, Drummond (1995) demonstrated different neurovascular mechanisms for the forehead and face with a parasympathetic vasodilator reflex in the facial nerve contributing to gustatory flushing in the forehead, but some other unidentified mechanism for vascular responses in the cheeks. In brief, while aberrant facial flushing following monobloc surgery could be due to either exaggerated parasympathetic or betasympathetic activity or to a failure of sympathetic inhibition, the evidence argues against the latter. In theory either the pterygo-palatine ganglion itself or outflow from it could be damaged during monobloc surgery by the osteotomy cut through the vomer and septum or the pterygomaxillary dysjunction (Fig. 4) (necessary for the separation/ down-fracture of the viscerocranium from the neurocranium) or by the down-fracture process itself. It is unlikely that the ganglion itself would be directly damaged as it lies anterior to the opening of pterygoid canal on the skull base
1514
A.R.M. Cobb et al. / Journal of Cranio-Maxillo-Facial Surgery 43 (2015) 1511e1515
An alternative scenario suggested by the occurrence of unilateral facial flushing at times of increased emotion or exertion (cases 3 and 4) is that it is the reddening side that is normal while the expected physiological response on the opposite side has been inhibited by a defect of beta-sympathetic input (Lance et al., 1988) In this case it would be the parasympathetic input to the facial vascular system that was taking longer to recover leaving the sympathetic unopposed. 5. Conclusions Aberrant facial flushing is a rare complication of monobloc fronto-facial surgery. It occurred in 4 of our 80 (5%) patients, being first noticed 2e3 months following the operation (around the time of the external distraction frame removal) and resolving over 2e4 years in 3 of them. The skull base osteotomies essential for the procedure are made anterior to the pterygopalatine ganglion and it is our contention that damage from these was responsible for a neuropraxia of its efferent nerve branches. A review of the autonomic control of the facial vascular system suggests that the phenomenon is due to an unequal process of recovery that leaves the cutaneous vasodilating parasympathetic or beta-adrenergic innervation relatively unopposed e a situation that persists until with time a normal balance of autonomic input is achieved. Unfortunately the essential requirement of the monobloc procedure (the disconnection of the viscerocranium from the neurocranium) makes it difficult to avoid a degree of surgical trauma in this region that may rarely be responsible for aberrant facial flushing. Fig. 4. The internal osteotomy cuts during craniofacial dysjunction disrupt the internal nasal anatomy made by the craniofacial dysjunction (arrows) as seen on this postoperative axial view CT of case 3. This may affect the nasal efferent autonomic supply.
Conflict of interest None.
(McMinn, 1994) because the osteotomy cuts and the down-fracture are carried out forward of this point. This would however still leave it vulnerable to trauma causing a gangliopraxia. More plausible is functional damage to its anterior (efferent) branches. The fact that in those in whom it resolved it took 2e4 years to do years implies a lengthy process of either recovery from neuropraxia or of reinnervation e a phenomenon known to occur following Vidian neurectomy performed for severe chronic vasomotor rhinitis resistant to medical treatment (Krajina, 1989; Konno, 2010). We suggest that it is damage to efferents from the pterygopalatine ganglion followed by an inequality in their speed of recovery (either from neurotmesis or regeneration/reinnervation in the case of complete disruption) that is responsible for the phenomenon of aberrant facial flushing we have described. This theory implies that greater vulnerability of the alpha-adrenergic fibres (or a less effective capacity for recovery or regeneration) allows for excessive parasympathetic, or beta-adrenergic, activity e a situation that continues until (in 3 of 4 cases) a state of balanced innervation is eventually restored. Monobloc surgery causes a major disruption to the soft tissues of the face to produce often severe swelling that may persist for several weeks. This could account for the onset of aberrant facial flushing being first noticed by all four patients around the time of the removal of the distraction apparatus (the skull pins and the transcutaneous posts that connect to the Polley plates) e the problem may have present since surgery but “occult” until then. But if at surgery all autonomic input to the facial vascular system was abolished and subsequent (unequal) functional reinnervation was not present until 2e3 months later, this could explain the “late” onset of aberrant facial flushing without having to invoke it having previously been “hidden” by facial swelling and soft tissue bruising.
Funding No sources of support in the form of grants were received. Acknowledgement We would like to thank David Smithson at the ICH/GOS Graphic Design Studio for the illustrations. References Alvernia JE, Spomar DG, Olivero WC: A computed tomography scan and anatomical cadaveric study of the pterygopalatine ganglion for use in Gamma Knife treatment of cluster headache. J Neurosurg 107: 805e808, 2007 Drummond PD, Lance JW: Facial flushing and sweating mediated by the sympathetic nervous system. Brain 110: 793e803, 1987 Drummond PD: Mechanisms of physiological gustatory sweating and flushing in the face. J Auton Nerv Syst 8: 117e124, 1995 Drummond PD: Facial flushing during provocation in women. Psychophysiology 36: 325e332, 1999 Goadsby PJ: Sphenopalatine (pterygopalatine) ganglion stimulation and cluster headache: new hope for ye who enter here. Cephalalgia 33: 813e815, 2013 Jones BM, Dunaway DJ, Hayward RD: Surgery. In: Hayward RD, Jones BM, Dunaway DJ, Evans RD (eds), The clinical management of craniosynostosis, 1st ed. London: Mac Keith Press, 391e395, 2004 Konno A: Historical, pathophysiological, and therapeutic aspects of vidian neurectomy. Curr Allergy Asthma Rep 10: 105e112, 2010 Krajina Z: Critical review of vidian neurectomy. Rhinology 27: 271e276, 1989 Lad SP, Lipani JD, Gibbs IC, Chang SD, Adler Jr JR, Henderson JM: Cyberknife targeting the pterygopalatine ganglion for the treatment of chronic cluster headaches. Neurosurgery 60: E580eE581, 2007 Lance JW, Drummond PD, Gandevia SC, Morris JG: Harlequin syndrome: the sudden onset of unilateral flushing and sweating. J Neurol Neurosurg Psychiatry 51: 635e642, 1988 Liasis A, Nischal KK, Walters B, Thompson D, Hardy S, Towell A, et al: Monitoring visual function in children with syndromic craniosynostosis: a comparison of 3 methods. Arch Ophthalmol 124: 1119e1126, 2006 McMinn RMH: Last's anatomy, 9th ed. Edinburgh: Churchill Livingstone, 34, 1994
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