Volume 2, Issue 3 , Pages 141-143, September 2007
Prevention over an 8-year period of laryngeal stenosis in LOGIC syndrome using a silastic keel
Article Outline
Summary
Laryngeal stenosis is a key feature of LOGIC syndrome and maintaining patency is important to prevent loss of airway and voice. Loss of airway can be prevented via a tracheostomy. This will not preserve voice in a larynx which in this syndrome closes due to granulations and fusion of the cords. Previous work has reported maintaining a voice using mitomycin C over a 1-year period. We report a patient where we have been able to maintain a patent larynx and voice over an 8-year period using a silastic keel secured in the glottis to prevent restenosis.
Keywords: LOGIC syndrome, Laryngo-onycho-cutaneous syndrome, Laryngeal stenosis, Silastic keel
1. Introduction
LOGIC syndrome (LOGIC syndrome “Laryngeal and Ocular Granulation tissue in children from the Indian subcontinent”, also known as laryngo-onycho-cutaneous syndrome, LOC or Shabbir syndrome) is a very rare condition which to date is restricted to families of Punjabi origin [1], [2]. It is characterised by chronic granulations and was first described in 1986 Pakistan [3] in a series of 22 patients with a collection of previously undescribed clinical features. The condition was first described in European journals in 1991 [2]. The children are generally of normal appearance at birth but begin to develop the features soon after [1], [2]. Initially granulation tissue develops in mucosal regions, nail beds and larynx followed by development of chronic skin ulcers and conjunctival lesions. Dental enamel fails to develop and dental malformations are common [1], [2], [3], [4], [5] (Fig. 1).
Fig. 1.
Image of the patient's mouth showing gross granulations of the gums and malformed teeth with poor enamel formation.
In the original series [3] 18 of the 22 patients were children born from consanguineous marriages of clinically normal parents. This and more recent work [1] has the identified the genetic mutation and confirmed the condition to have an autosomal recessive inheritance pattern.
Laryngeal stenosis is a key feature of the disease and maintaining patency is important to prevent loss of airway and loss of voice. The use of mitomycin C has previously been reported for this [6]. We report the use of a silastic keel placed in the larynx of a patient to prevent stenosis of the larynx and permit voicing over an 8-year period.
2. Case report
Our female patient was born from a Punjabi family in June 1986. She originally presented aged 3 years old, to a speciality paediatric hospital via a referral to paediatric ophthalmology. This was with granulomata and epithelial breakdown on her cornea and other mucous membranes. She also had fibromata on her skin and nail beds. The consultant ophthalmologist noted at this point that she was also very hoarse and referred her onto the laryngeal surgeon.
In January 1990 her first laryngeal biopsy showed chronic granulation tissues and she required laser treatment of her larynx later in 1990 for laryngeal stenosis. This was primarily caused by fusion of the cords from the anterior commisure extending posteriorly leading to loss of airway and voice. Further laser treatment was required on four occasions in 1991 for laryngeal stenosis and anterior webbing before a tracheostomy was finally required in September 1991. At this point a diagnosis of LOGIC syndrome was made. Further laser treatment was carried out before in 1992 a silastic keel was placed to maintain her voice. This stent was removed after 4 months and over the next 7 years, until 1999, she underwent numerous more laser treatments to preserve her voice and had 4 further short-term silastic keels placed. None of these was left for longer than 4 months. A final silastic keel was placed in February 1999 and this remains in situ currently (January 2007). She has had a functional voice since then and has not required any further laser treatment.
As noted earlier granulation tissue builds up in the larynx of patients with LOGIC syndrome and whilst an airway can be maintained effectively with a tracheostomy, voice will not be preserved. In our patient a silastic keel was placed between the cords and secured with nylon sutures. This has effectively splinted open the glottic aperture and permitted air transfer via the glottis. It has also preserved her voice. The current keel in situ is shown in Fig. 2.
Fig. 2.
Image of larynx taken using 0° Hopkins rod through Dado laryngoscope. The stent is seen placed in the anterior commisure (anterior is towards the top of the image) with patent airway and vocal cords to either side. The generally distorted nature of the larynx can also be seen.
3. Discussion
As part of the disease progression of LOGIC syndrome granulation tissue accumulates rapidly in the larynx leading to laryngeal stenosis. Tracheostomy is often required to maintain an airway [2], [5] and prevent airway obstruction which is the most commonly reported cause of premature death [2], [5]. Whilst a tracheostomy will maintain the airway, it will not maintain voice and other methods are required to prevent full closure of the larynx. Previous work reports the use of mitomycin C to prevent re-stenosis in a patient presenting with complete laryngeal stenosis and who had his airway re-established surgically [6]. In this work the patency of the larynx and also a functioning voice was reportedly maintained over a 1-year period.
Keels have been used for many years to prevent and during treatment of anterior commissure webbing [7], [8], [9]. Historically the early keels used were of silver [7] but this has been adapted over time and silastic keels are more commonly used. Keels are commonly left in for up to 6 weeks only and in those left for longer periods there has been some suggestion that there can be an increase in granulation tissue [10]. Similarly children who have either had congenital laryngeal stenosis [11] or developed laryngeal stenosis following prolonged intubation [12], [13] have been treated successfully with silastic splints left in place for periods of up to 3 months [10]. In our patient there appears to have been no adverse reaction to the keel and the granulation tissue which developed would be in keeping with that expected from the disease [2], [4].
Our patient has been left with a narrow but functional laryngeal airway and to protect this has not been decannulated but continues to wear a Great Ormond Street style tracheostomy tube (currently a size 6). Throughout the period however the laryngeal stenting has maintained a glottic airway and she has had a functioning voice since insertion of the keel. Management of the tracheostomy has not been an issue for her or her family.
The treatment reported here has been successful over a long period of time and the patient has maintained her voice and airway throughout. Mitomycin C is now being used on the patient to reduce granulations around her tracheostomy site and laryngeal keel. We will continue to review her, both with respect to her voice and airway, and it is not planned to remove her keel nor try to decannulate her.
References
- . An unusual N-terminal deletion of the laminin alpha3a isoform leads to the chronic granulation tissue disorder laryngo-onycho-cutaneous syndrome. Hum. Mol. Genet. 2003;12:2395–2409
- . Laryngeal and ocular granulation tissue formation in two Punjabi children: LOGIC syndrome. Eye. 1991;5:717–722
- . Laryngo-onycho-cutaneous syndrome: a study of 22 cases. Biomedica. 1986;2:15–25
- . Laryngo-onycho-cutaneous syndrome: an inherited epithelial defect. Arch. Dis. Child. 1994;70:319–326
- . Multisystem disorder of Punjabi children exhibiting spontaneous dermal and submucosal granulation tissue formation: LOGIC syndrome. Clin. Dysmorph. 1992;1:3–14
- . Successful treatment of laryngeal stenosis in laryngo-onycho-cutaneous syndrome with topical mitomycin C. Pediatr. Dermatol. 2006;23:75–77
- . Ein Fall von Membranbildung im Larynx. Eine neue Methode zu ihrer Behebung. Monatschr Ohrenheilk. 1924;58:174
- . An endoscopic silastic keel for anterior glottic webs. J. Laryngol. Otol. 1987;101:1055–1061
- . Outpatient transoral laser vaporization of anterior glottic webs and keel placement: risks of airway compromise. J. Voice. 1998;12:536–539
- . Intralaryngeal approach to laryngeal web using lateralization with silastic. Laryngoscope. 2000;110:1780–1782
- . Congenital laryngeal webs: surgical management and clinical embryology. Int. J. Pediatr. Otorhinolaryngol. 2000;52:1–9
- . Tratamiento quirurgico de la estenosis laringotraqueal adquirida en ninos. Cir. Pediatr. 1995;8:135–138(surgical treatment of acquired laryngotracheal stenosis in children, Spanish)
- . Update of the Cincinnati experience in pediatric laryngotracheal reconstruction. Laryngoscope. 1989;99:1111–1116
PII: S1871-4048(07)00027-5
doi:10.1016/j.pedex.2007.04.001
© 2007 Elsevier Ireland Ltd. All rights reserved.
Volume 2, Issue 3 , Pages 141-143, September 2007
