BritishJ7ournal ofOphthalmology 1993; 77:289-292 289 Distichiasis: management with cryotherapy to the posterior lamella Br J Ophthalmol: first published as 10.1136/bjo.77.5.289 on 1 May 1993. Downloaded from

B A O'Donnell, J Richard 0 Colhn

Abstract Moorfields Eye Hospital and the private practice Distichiasis is the congenital condition. of of one of the authors over the past 13 years were partial orcomplete accessory rows ofeyelashes retrospectively reviewed. Patient names were which exit from the posterior lid margin at or taken from a log kept of all patients with this near the meibomian gland orifices. A series of diagnosis. Data were then gathered from hospital 24 patients with distichiasis is presented with and consultant files. Patient referral was from clinical features and results of treatment. The ophthalmologists and general practitioners. The treatment modes of epilation, lid margin cryo- clinical features, presenting symptoms, family therapy, or eyelid splitting cryotherapy to the history, treatment, and clinical course were posterior lamella are evaluated. The latter was reviewed. Patients not recently reviewed were found to effectively relieve symptoms without recalled for long term follow up. retreatment in 87% patients. Keratinisation of Treatment, if required was in the form of the lid margin was found at long term follow up epilation, electrolysis, lid margin cryotherapy, in a few cases and retinoic acid has been used or lid margin splitting with cryotherapy to the successfully in the treatment of this. Two posterior lamella. The decision to treat was made previously unreported associations of distichi- if symptoms were present, or if significant asis are Pierre Robin syndrome and idiopathic corneal staining or ulceration was observed. The eyelid oedema. A family history of distichiasis type of treatment was chosen according to the was found in 50% patients, and of distichiasis extent of lid margin involved and history of with lymphoedema in 30%. previous failed treatment. Thus if relatively few (BrJ3 Ophthalmol 1993; 77: 289-292) distichiatic lashes were present, electrolysis only was performed. If less than a quarter lid margin extent was involved or previous electrolysis had Method failed, cryotherapy was applied. Cryotherapy All patients with distichiasis who have attended was avoided in dark skinned patients and those http://bjo.bmj.com/ on September 29, 2021 by guest. Protected copyright.

Figure I Eyelid with distichiasis:doublerow oflashes in left upper and lower lids.

Figure 3 The anterior andposterior lamellae are separated.

Moorfields Eye Hospital, City Road, London B A O'Donnell J R 0 Coffin Correspondence to: J R 0 Collin, Department of ...,.~~~~~~~~~~~~~~~~~~~~~.....;i, ...... : . Oculoplastics, Moorfields Eye ...... Hospital, City Road, London . .:.;;...... ECIV 2PD...... :::S.::.. :!.. Figure 4 The cryoprobe applied to the posterior lamella. A Accepted for publication Figure 2 The lid isfixated while performing lid split at the thermocouple isplaced between the lid and a plastic spoon 26 January 1993 grey line. which protects the cornea. 290 O'Donnell, Collin

FigureS The posterior the tarsal plate. A double freeze-thaw technique lamella is advanced in relation to the anterior is applied to the posterior lamella. The tempera- lamella at completion. ture is monitored with a thermocouple placed under the tarsus with a lamellar division to avoid the risk of necrosis of the tarsus. Freezing is Br J Ophthalmol: first published as 10.1136/bjo.77.5.289 on 1 May 1993. Downloaded from applied for the time taken for the thermocouple to register -20°C. This rapid freezing is followed by slow thawing before re-freezing.3 The Collin cryoprobe (Keeler Ophthalmic Instruments), which has been specifically designed for eyelid margin cryotherapy, is used as it allows a greater length oflid margin to be evenly frozen than with an ordinary probe. Following lid split and posterior lamellar advancement, the anterior and posterior lamellae are sutured together with a running 6/0 vicryl suture. Follow up treatment was at 1 week, 4 weeks, and 2 months, but was frequentlyvaried onanindividual basis according with dark lashes to avoid uncosmetic post- to patient travelling distance and postoperative operative depigmentation. If more than half of comfort. the lid margin was involved (Fig 1), then lid splitting with posterior lamellar cryotherapy was performed. Treatment success was recorded if Results there was complete or near complete resolution Twenty four patients have attended Moorfields ofsymptoms, complete or near complete removal Eye Hospital with distichiasis over the past 13 ofthe distichiasis lashes, or disappearance of pre- year period. Nine patients were referred from treatment fluorescein staining. other ophthalmologists and 13 patients were Surgery was performed with general anaes- referred from general practitioners. Two patients thesia in children and with either general or local attended as a result of family history or anaesthesia in adults depending on patient distichiasis. The average age of patients was 9 7 preference and general health. Local anaesthesia years with a range of 1-8 to 50 years. There were was performed with a standard mixture of equal 11 male and 13 female patients. All except one parts of 2% lignocaine with adrenaline and 0 5% patient were symptomatic. Symptoms reported marcaine. were photophobia, lid swelling, conjunctival injection, lacrimation, discharge, and irritation. All patients had four lid involvement with two Surgical technique exceptions: one with only right upper lid The surgical technique used is similar to that involvement and one with bilateral lower lid previously described by Anderson and Harvey' distichiasis sparing them upper lids. Recurrent in which the lid margin is split at the grey line corneal ulceration was present on initial http://bjo.bmj.com/ (Figs 2, 3) to enable a cryoprobe to freeze the examination in two patients with scarring and posterior lamella without freezing the anterior neovascularisation in one ofthese. Family history lamella (Fig 4). However, in this series an was recorded in all patients and 50% had at least anterior lamella recession is performed by one previous generation affected. Two sisters releasing the lid retractors to enable the posterior with distichiasis had a family history affecting lamella to be advanced 4 mm (Fig 5).2 This four generations, and one patient had a family results in more reliable anteriorlamellar recession history with three generations affected. In both on September 29, 2021 by guest. Protected copyright. than does simple recession of the skin and these families, autosomal dominant inheritance orbicularis muscle onto the anterior surface of was evident. Two patients had co-existent lymphoedema (Fig 6): in one of whom the lymphoedema followed pregnancy. Two patients, mother and daughter, gave a history since early teenage, of eyelid swelling. Blepharo- plasty was performed successfully in the mother. Other associated findings were Pierre Robin syndrome (one patient), telecanthus and epicanthus (one patient), and ptosis (two patients). The latter required bilateral brow suspension and anterior levator resection respec- tively, following treatment of the distichiasis. One patient had postoperative eyelid oedema following two anterior approach ptosis corrections, which was not present pre- operatively. Eight patients were treated initially at Moorfields with only electrolysis and/or cryo- therapy. Two required subsequent lid split and cryotherapy. The remaining patients had Figure 6 Lower limb complete removal of lashes although multiple lymphoedema. treatments were required sometimes. One Distichiasis: management with cryotherapy to theposteriorlamella 291

patient refused treatment, and two cases were Individual lash root excision is performed via a treated elsewhere with lid splitting and cryo- razor incision over the distichiatic lash root. therapy and are not included in these results. However, it is time consuming and risks Fifteen patients underwent lid splitting with damaging the posterior lid margin. Excision of Br J Ophthalmol: first published as 10.1136/bjo.77.5.289 on 1 May 1993. Downloaded from posterior lamellar cryotherapy: three patients distichiasis lash roots through a trapdoor incision with all four lids, four with bilateral lower.lids, in the tarsoconjunctival surface just posterior to and eight with bilateral upper lids. Five of these the eyelid margin has been reported to cause less had had previous electrolysis and/or cryotherapy trichiasis, entropion, and eyelid margin distor- (including patients treated initially elsewhere). tion but nevertheless scars the important Of the group with lid splitting, all patients had posterior conjunctival surface of the eyelid. significant improvement in their symptoms Resection of the distal tarsal plate has been tried although theeyelid margins were onlycompletely with simple advancement ofthe cut edge to form cleared of lashes in three cases. Residual lashes a new posterior lid margin.8 Replacement of the were not treated per se but only if significant resected distal posterior lid margin has been tried discomfort or signs of irritation were present. with nasal chondromucosa,9 and buccal mucous Eleven patients in this group had follow up of membrane.'011 more than 2 years: average 4-5 years (range 2 to 9 Cryotherapy is an excellent means of ablating years), four patients had follow up less than 1 lash roots. However, more than half of the year owing to recent surgery or were unable to be patients treated with simple cryotherapy/ reviewed. Since only two patients required electrolysis required retreatment of some kind. retreatment oflashes (byelectrolysis and excision Eyelids with distichiasis tend to have the lashes of lash roots respectively), we report an 87% evenly distributed along the length of the lid success rate for lid splitting with posterior margin necessitating extensive treatment. Cryo- lamellar cryotherapy for congenital distichiasis. therapy is better performed and the compli- Following lid split with cryotherapy, eyelid cations of lash and skin depigmentation can be oedema settled in most cases within 4 to 8 weeks. avoided with the technique of lid splitting at the One patient remained uncomfortable for 6 grey line with cryotherapy to the posterior months. One patient developed pseudoepithelial lamella. This treatment is effective, does not hyperplasia ofthe eyelidmargin which is thought depigment the skin or lashes of the anterior to have occurred because the two lid lamellae lamella, prevents loss of normal anterior lashes, were not sutured but left to granulate following and does not disturb the posterior lid margin. lid splitting. Although uncosmetic, the patient Theoretically cryotherapy of the posterior was comfortable, no further treatment was lamella may cause entropion with trichiasis. This required and the lid margin eventually returned is prevented by the anterior lamellar recession to normal. Following this case, the anterior and advancement of the posterior lamella. No lamella has always been sutured to the advanced complications of entropion or lid margin posterior lamella with an absorbable suture and distortion were found to occur in this series.

this problem has not recurred. Over- Although eyelid erythema and swelling persisted http://bjo.bmj.com/ advancement ofthe posterior lamella occurred in for several months, all except one case fully onepatient. Thiscosmetic problemwas corrected resolved with the latter improving at last follow by shaving down the advanced posterior lamella up visit. The technique of lid splitting and to restore a normal lid margin appearance. On cryotherapy is relatively straight forward but long term review (3 to 11 years), keratin was familiarity with upper lid retractor anatomy is found to develop along the posterior lid margin necessary. We suggest that this is the procedure

in three patients. A course ofretinoic acid 0-01% ofchoice fordistichiasis with extensive lid margin on September 29, 2021 by guest. Protected copyright. was used successfully to remove thekeratin in one involvement. patient without any adverse reactions. Two Complete removal of lashes occurred after lid patients will undergo similar retinoic acid treat- split and cryotherapy in only three patients, yet ment. In cases that underwent lid splitting and retreatment after this procedure was only cryotherapy, the long term postoperative required in two patients. In young children in appearance of the tarsus at the lid margin was whom history was unreliable or examination found to be approximaely half to two thirds difficult, no further treatment was carried out if normal thickness. symptoms or signs were minimal. There was often such a dramatic improvement in the child's postoperative comfort that this may have made Discussion any postoperative minor irritation observed by There have been a considerable number of the parents seem insignificant. In older patients, different treatments tried for permanent cure of it was found that a few remaining lashes were this condition. Electrolysis and cryotherapy most often asymptomatic or caused so little in the remain the simplest forms of treatment for way of irritation, that the patient declined any distichiasis with only minimal lid margin further treatment. The incomplete destruction involvement. For more extensive distichiasis, of the lash roots most probably occurs secondary cryotherapy with nitrous oxide applied to the to inadequate temperature or duration of the conjunctival side of the lid margin effectively freeze. Inaccurate placement ofthe thermocouple removes the unwanted posterior lashes4 but was or calibration may account for the former. found to depigment the normal anterior lashes.5 The associated findings with distichiasis in Surgical excision of the lash roots has been this series were epicanthus, telecanthus, performed individually,6 via a trapdoor in the blepharophimosis, ptosis, colour blindness, conjunctival surface of the tarsal plate,7 and via lymphoedema, eyelid oedema, and the Pierre resection of the distal part of the tarsal plate."-" Robin syndrome. To the authors' knowledge, 292 O'Donnell, Collin

the associations of idiopathic eyelid oedema and 2 Colin JRO. A manual of systemic eyelid surgery. 2nd Ed. Edinburgh: Churchill Livingstone, 1989; 25-6. Pierre Robin syndrome have not previously been 3 Johnson RLC, Collin JRO. Treatment of trichiasis with a lid reported. Distichiasis has been previously cryoprobe. Brj Ophhalmol 1985; 69; 267-70.

4 Matthaus W, Matthes R. Technik und Langzeitergebnisse der Br J Ophthalmol: first published as 10.1136/bjo.77.5.289 on 1 May 1993. Downloaded from reported with hereditary spinal arachnoid kryoepilation bei tichiasis end distichiasis. Klin Monatsbl cysts,'2 lower lid ectropion," reduced serum Augenheilkd 1990; 196: 59-61. 5 Freuh B. Treatment ofdistichiasis with cryotherapy. Ophthal- cholinesterase,'3 cleft palate,'4 trisomy,5 18 mic Surg 1981; 12: 100-3. epicanthus,'6 blepharophimosis, microphthal- 6 Wolfley D. Excision ofindividual follicles for the management of congenital distichiasis and localised trichiasis. Jf Paed mos, dystrophic retinal pigmentation, and optic Ophthalmol Strabismus 1987; 24: 22-6. disc pallorin the oculo-cerebro-renal syndrome,'7 7 Dortzbach RK, Butera RT. Excision of distichiasis eyelashes through a tarso-conjunctival trapdoor. Arch Ophthalmol ectrodactyly,'5 corneal hypoaesthesia,'9 uvula 1978; 96: 111-2. bifida, and submucous cleft of the palate.'0 8 Fox SA. Distichiasis. AmJ Ophthalmol 1962; 53: 14-7. 9 Rodallec A, Krastinova D. Son interet dans la correction de Distichiasis has been reported with both late l'entropion par cicatrice tarso-conjunctivale, du trichiasis et onset Meige type lymphoedema""' and early du distichiasis. La grette chondro-muqueuse. J Fr Ophtal- mol 1983; 6:87-93. onset Milroy type lymphoedema."1 The 10 Metaioreau JP. Traitment du distichiasis par la marginoplasty lymphoedema associated with distichiasis has palpebrale. BullSoc Ophtalmol Fr 1985; 85: 1247-8. 11 White JH. Correction of distichiasis by tarsal resection and been found to be bilateral hyperplasia by mucous membrane grafting. Am J Ophthalmol 1975; 80: lymphography.'3 The association of eyelid 507-8. 12 Schwartz JF, O'Brien MS, Hoffman JC Jr. Hereditary spinal oedema has not previously been reported. arachnoid cysts, distichiasis, and lymphoedema. Ann Neurol The 1980; 7:340-3. inheritance pattern of distichiasis has 13 Shammas HF, Tabbarra KF, Der Kaloustan VM. Atypical been previously reported as autosomal serum cholesterase in a family with congenital distichiasis. JMed ienet 1976; 13: 514-5. dominant.'425 Fifty percent of patients in this 14 Bartley GB, Jackson IT. Distichiasis and cleft palate. Plastic study gave a family history ofdistichiasis. Three ReconstmctSurg 1989; 84: 129-32. 15 Mehta L, Shannon RS, Duckett DP, Young ID. Trisomy 18 in patients, with more than one previous generation a 13 year old girl.J Med Genet 1986; 23: 256-78. affected, displayed an autosomal dominant 16 Deutsche AR. Distichiasis and epicanthus. A study of one family. TennesseeMedAssoc 1969; 62: 1023-5. pattern. Thirty percent ofpatients gave a history 17 Brancato R, Matteini M, Cotrozzi G. Sindrome Oculo- of distichiasis and lymphoedema. The average cerebro-renale in soggott portatore di blefarophimosi e di distichiasisi. Ann Ottal 1965; 91: 35-57. age in this series was less than 10 years and since 18 Collier MM. Ecrodactylie droite et distichiasis inferieur the onset of lymphoedema tends to occur in bilateral. BullSoc D'Ophtalmol 1964; 64: 49-52. 19 Kremer I, Weinberger D, Cohen S, Ben Sira I. Corneal adulthood, this would explain why few patients hypoaesthesia in asymptomatic familial distichiasis. were seen with coexistent lymphoedema in this Brj Ophthalmol 1986; 70: 132-4. series. 20 Jester HG. Lymphoedema distichiasis. A rare hereditary In families with distichiasis and syndrome. Hum Genet 1977; 39: 113-6. lymphoedema affecting more than one previous 21 Campbell KN. Milroy's disease. Chronic hereditary oedema. the Univ BullAnn Arbor 1945; 11: 69-70. generation, distichiasis tended to occur in 22 Kolin T, Johns KJ, WadlingtonWB, Butler MG, Sunlap MA, association with lymophoedema in family Wright KW. Hereditary lymphoedema and distichiasis. Arch Ophthalmol 1991; 109: 980-1. members affected rather than the distichiasis 23 Dale RF. Primary lymphoedema when found with distichiasis occurring alone. is of the type defined as bilateral hyperplasia by lympho- No royalties are received by the author, J R 0 Collin, from Keeler graphy.JMed Genetics 1987; 24: 170-1. Ophthalmic Instruments from the sales ofthe Colfin cryoprobe. 24 Mustonen E. Congenital distichiasis. A report of eleven cases

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