Apraxia of lid opening ALO is defined as non-paralytic motor abnormality of the upper eyelid characterized by inability to open the eyes intentionally once closed1. It is not considered to be a true apraxia. A true apraxia is defined as inability to perform a motor action to command despite both an adequate understanding of the action and the elementary ability to carry it out. However ALO is confirmed when there is inability to open the lids at will but lids do open at other times when patients is not trying it willingly2. Exact patho-physiology of ALO is not established, however few mechanisms have been considered in its explanation. There is believed to be an abnormality in the supranuclear control of voluntary eyelid elevation, which requires the activation of the levator palpebrae superioris and the concurrent inhibition of orbicularis oculi activity3.
1. Head of Ophthalmology Department Bakhtawar Amin Medical and Dental College, Multan 2. Assistant Professor, Isra postgraduate Institute of ophthalmology, Al Ibrahim Eye Hospital, Karachi 3 Associate Professor Bakhtawar Amin Medical and Dental College, Multan 4. Consultant Ophthalmologist, Bakhtawar Amin Medical and Dental College, Multan 5. Senior Registrar, Ophthalmology Department, Bakhtawar Amin Medical and Dental College, Multan 6. Assistant Professor Neurosurgery Department Bakhtawar Amin Medical and Dental College, Multan
Correspondence. Dr. Syed Hassan Raza Jafri, Professor of
Ophthalmology, Department of Ophthalmology, Bakhtawar Amin
Medical and Dental College, Northern Bypass Road, Multan.
Cell: 0333-2114785, E-mail: firstname.lastname@example.org
Received. May2019 Accepted: June 2019
Frontalis sling is minimally invasive and effective procedure for treatment of apraxia of lid opening. It shows promising results with fewer complications.
Electromyographic studies have demonstrated that involuntary levator palpebrae inhibition (ILPI) 4, intermittent 5or prolonged6, persistent pretarsal Orbicularis contraction 7and ILPI (either intermittent or prolonged) with persistent pretarsal Orbicularis contraction8can cause ALO. Which is sometimes associated with essential blepharospasm. Such cases have to be differentiated clinically as their management is different from simple ALO9. ALO can be caused by a variety of CNS lesions in non-dominant hemisphere 10 medial frontal lobe, 11,12basal ganglia 13 or rostral brainstem14,15 Progressive supra nuclear palsy is one of the most common causes of ALO 16. Other causes include Parkinson disease 17, Idiopathic dystonia 5, Hydrocephalus 18, Motor neuron disease 19, Dystonia due to kernicterus 4, Choreoathetosis 4, Huntington chorea, Shy-Drager syndrome 8, Post-encephalitic parkinsonism 8,and Neuro-acanthocytosis 2. Drugs like Lithium and sulpiride are also associated with ALO.,ALO can be treated medically or surgically. Medical treatment include Injection of a botulinum toxin A (BOTOX) 21,Levodopa, sodium valproate22and anticholinergic agent trihexyphenidyl, however the efficacy is dubious and indications are limited. Frontalis sling gives a promising surgical correction in majority of patients. It is minimally invasive. With lesser invasive approaches such as modified fox pentagon though Suprabrow single stab incision23 (SBSS), the cosmetic outcome is even better. Various sling materials can be used; however we used silicone tube because it provides elasticity to allow the movement of upper eyelid on downward gaze, effectively reducing lid lag and lagophthalmos
MATERIAL AND METHODS
Patients were selected on non-probability purposive basis from Ophthalmology department, Bakhtawar Amin medical and dental college/hospital, Multan from January, 2016 to December, 2017. Permission was taken from ethical review committee. All the patients were diagnosed as having Apraxia of lid opening were included. Patients having associated blepharospasm with continuing botulinum toxin therapy or having active neurological disease were excluded, they were followed up for 6 months. Preoperative and postoperative photographs of the patients were taken on every visit after informed consent. Neurological assessment for exclusion of active neurological disease was carried out by a competent neurologist. Routine preoperative investigations including viral markers were done for all patients. Cardiac clearance was taken from cardiologist and anticoagulants settled in case of patients having any cardiac issue. SBSS frontalis sling was done in all patients, which included marking of the pentagon angles, incisions over the superior tarsus. Single stab incision was given on the frontalis muscle. A silicone tube was used as the sling material. Sling was introduced through the tarsal incisions and then brought out at the Suprabrow incision following the marks at the eyebrow, deflecting beneath the skin and orbicularis muscle without piercing them and passing through the superior orbital septum. The two ends of the sling were fixed as per desired correction and Suprabrow incision was closed with a single 5/0 polypropylene suture with the knot buried by making a shelving facial pocket. The upper lid was supported with a frost suture from the lower lid in cases of larger corrections. Post-operatively patients were given oral antibiotics and NSAIDs. Bandage was removed on the following day. Topical ointment containing antibiotics were prescribed to be applied over the wound. Frequentlubrication in form of artificial tears and gel was prescribed to counter lagophthalmos. Follow up was done on 1stpost-operative day then at 1st week, 3rd week and finally at 6th month. At all visits palpabrel fissure height (PFH), and marginal reflex distance (MRD) were measured, photographs were taken and any complication was looked for. Functional outcome was documented on the basis of patient’s ability to open the eye intentionally. Cosmeticoutcome was graded according to the post-operative MRD in comparison with the normal (normal 4-4.5mm) as follows:
Good : within 1mm of normal
Fair : within 2mm of normal
Under corrected : less than 2 mm from normal
Over corrected : more than 2mm from normal 24,25
The various complications that have been reported in literature are under correction, overcorrection, exposure keratitis, lid crease abnormalities, eyelid margin contour abnormalities, lagophthalmos, ectropion and entropion26
21 eyes of 19 patients were included in this study. All patients underwent Frontalis sling through Suprabrow single stab incision (SBSS) with silicone tube. Age of the patients range from 49years to 71 years, (mean of 59.98 years). 13(68.42%) patients were female while 06(31.57%)were male. 17 (89.47%) patients had bilateral disease while 02(10.52%) had unilateral disease.14 (66.67%) eyes had good outcome, 04(19.04%) had fair outcome, while 03 (14.28%) had under correction (table 1).
As far as complications are concerned 02 (9.52%) eyes had sling failure, while 01 (4.76%) had knot exposure with wound infection (table 2).
Inability to open the eyes willingly can make a patient practically blind especially when he needs them for a desired visual task. ALO is a debilitating condition in which the patient is unable to see despite having good vision. Classical apraxia is characterized by loss of controlled motor function all the times however, in ALO patients do respond to unintentional phenomena. The levator palpabrae superioris LPS is innervated bilaterally from the central caudal subdivision of the oculomotor nucleus, while the Orbicularis Oculi is innervated unilaterally from the facial nucleus. The cortex, extrapyramidal motor systems, and rostral midbrain structures may control LPS motor neuron activity13 EMG studies have proposed two possible pathophysiological phenomena for ALO. Either there is involuntary inhibition of LPS (ILPI)4 or Persistent pretarsal Orbicularis Oculi contraction5. In ALO alone ILPI is dominant while in cases associated with essential blepharospasm the element of persistent orbicularis contraction is also contributing to the disease. Therefore it is imperative to clinically differentiate both entities as their management differs at a certain stage. Essential blepharospasm with ALO is treated with Botulinum toxin injections first and then surgical options are added to supplement its effect. However in pure ALO botulinum toxinhas limited effect and surgical options have primary role in its management. Botulinum toxin provides a very handy treatment for blepharospasm. ALO associated with blepharospasm has also been treated with botulinum first and then augmented with surgery. However as Botulinum is a toxin, it can have some adverse effects as well. These effects include hypersensitivity, respiratory problems, dysphagia, seizures, flu like syndrome, facial and other muscle weakness, ptosis, skin and injection site reactions27. Tolerance is another issue and the response becomes refractory as the number of injections increases. Different surgical options have been tried successfully by various surgeons for the correction of ALO with or without blepharospasm. These include frontalis sling28, brow lift29, myectomy of orbicularis30, corrugator and protractor muscles, partial or limited myectomy31, levator aponeurosis repair and frontalis advancement32. However, frontalis sling alone provides with a minimally invasive approach and is relatively easier to revert in case of a spontaneous recovery. We did frontalis sling in all our patients without any botulinum injection. The technique used was Suprabrow single stab incision SBSS with modified fox pentagon which is relatively less invasive and has better cosmetic results then other techniques23. We used silicone tubes as sling material. Surgeons have used different materials in this regard including PTFE28, Polypropylene29 and Nylon etc. we preferably used silicon due to its elasticity, inertness and easy availability. Due to its elasticity we didn’t experience any significant lid lag, lagophthalmos or exposure keratopathy. Due to its inert nature and softness, knot extrusion and suture granulomas were also well controlled. Unilateral ALO is very rare33 but we experienced two cases. Both had isolated ALO and no systemic or neurological deficit was found. Unilateral cases also responded to frontalis sling very well with good cosmetic and functional outcome. Frontalis sling in unilateral cases has always been a dilemma for discussion however we have previously managed unilateral cases of ptosis treated with frontalis sling with commendable outcomes34. Patients are to be guided about moving the head instead of moving the eyes to mask any difference causing obvious lid lag. Our surgical outcome is comparable to other researchers. We achieved a good result in 66.67% and a fair result in 19.04%, this adds upto 85.71% of cosmetically satisfied patients. This is comparable to Karapantzo C28 74.6% and 76.92% by De Groot V35. As far as complications are concerned we had 02 (9.52%) sling failures due to breaking of the tube. These cases had repeat procedure and finally had a good outcome. We experience 02 (9.52%) under corrections, but as the patients were cosmetically and functionally satisfied, revision was not necessitated. 01 (4.76%) patient had knot exposure with wound infection in one eye. After controlling the infection the knot was buried deeply in the frontalis and wound closed in layers to avoid recurrence; the outcome remained unaltered.
Frontalis sling is minimally invasive and effective procedure for treatment of Apraxia of lid opening. It shows promising results with fewer complications.