From the Department of Ophthalmology, Ministry of Health, Ankara Education and Research Hospital, Ankara, Turkey.
Address correspondence to Dilek Yuksel, MD, Ministry of Health, Ankara Education and Research Hospital, Ankara, Turkey, Department of Ophthalmology, Hosdere Cad, Çankaya Evleri E Blok No 26 Çankaya, Ankara, 06550, Turkey. E-mail: email@example.com
Congenital tarsal kink syndrome is a rare and severe form of upper eyelid entropion characterized by a horizontal kink within the upper tarsal plate. The cause of the tarsal kink is unknown, but the proposed causes include overaction of the marginal fibers of the orbicularis muscle, primary tarsal defect, and direct mechanical pressure on the tarsus in utero.1,2 Recently, disinsertion of the levator aponeurosis has been reported as a possible cause of congenital entropion of the upper eyelid.3 It is characterized by blepharospasm and absence of an upper eyelid crease. The folded edge of the upper tarsus or the inturned eyelashes may traumatize the cornea and cause corneal abrasion, ulceration, keratitis, and stromal opacification. Surgical treatment is usually required to prevent damage to the cornea. Various surgical procedures have been described for the correction of congenital horizontal tarsal kink syndrome.
We present a case of unilateral congenital horizontal tarsal kink and define our simple and effective surgical procedure for its correction.
A 3-month-old female infant was referred to us for examination of blepharospasm and epiphora that had been noted at birth. The child was born at term after an uncomplicated pregnancy and delivery, and there was no ocular family anamnesis. No systemic abnormalities were present. On external examination, the left eyelid margin was completely rolled in along its entire length, bringing the eyelashes in direct contact with the globe. There was no skin crease (Fig. 1). When we opened the eyelids of the left eye we saw a corneal ulcer (Fig. 2). Corneal cultures were negative. No other ocular abnormality was detected and the examination of the right eye and the eyelid was normal.
Figure 1. (A) A 3-month-old infant with left congenital horizontal tarsal kink. Note the absence of an eyelid crease. (B) Note the inturning of the left upper eyelid margin.
Figure 2. A corneal ulcer was visible on opening of eyelids.
The tarsal kink was corrected as follows. Under general anesthesia, an eyelid incision was made at a level symmetrical to the opposite eyelid crease and dissection in the postorbicular fascial plane was performed to visualize the entire tarsal plate. The kink in the superior tarsus was visible. A horizontal tarsal incision was performed half as thick as the tarsus within the area of the tarsal kink along its entire horizontal extent. Then, dissection was continued superiorly through the orbital septum and levator aponeurosis was found. Terminal levator aponeurosis fibers were reattached to the tarsal incision site to create sufficient everting force for the correction of the entropion. To ensure a deep eyelid fold, the skin was closed with the same sutures (Fig. 3). Two additional sutures were inserted laterally and medially to the eyelid incision in the same manner. Polyglycolic acid sutures (6-0) were used for the procedure. The left upper eyelid was completely rotated externally and an eyelid crease was created (Fig. 4).
Figure 3. Cross-section of the upper eyelid demonstrating the tarsal kink and the placement of the sutures and horizontal tarsotomy.
Figure 4. Appearance of the same child 6 months after the surgery. The tarsal kink disappeared and the eyelid crease was created.
The corneal ulcer healed within 7 days. The tarsal kink disappeared and the eyelid position remained stable during a follow-up period of 2 years. No complications were observed.
Congenital upper eyelid entropion is not commonly seen and is usually caused by disinserted levator aponeurosis or congenital horizontal tarsal kink.3,4 Because the visual axis is involved in most cases and permanent corneal scarring in the newborn may lead to amblyopia with permanent visual loss, early recognition and appropriate management of congenital horizontal tarsal kink by the pediatrician or primary ophthalmologist are important. The ophthalmologist should perform an eversion of the upper eyelid in the diagnosis of tarsal kink to detect the characteristic horizontal kink within the tarsal plate. Both the pediatrician and the ophthalmologist can easily overlook the diagnosis of congenital entropion, particularly because the eyelids are tightly closed in the crying infant and examination of the upper eyelid through eversion is more difficult than that of the lower eyelid. Corneal opacity at birth, absence of upper eyelid crease, and lack of visibility of the upper eyelid margin could be important diagnostic clues for the pediatrician or ophthalmologist. In our case, we observed that the upper eyelid margin was invisible without eyelid eversion and there was no upper eyelid crease. A sterile corneal ulcer was also present. Most of the reported cases had corneal ulcers that were typically sterile.5 Corneal infiltrate was noted in 50% of cases in the collective analysis of prior reports.4
Congenital tarsal kink observed in the neonatal period is more severe than late presentation. In Naik et al.’s study,6 two cases with late presentation manifested with mild kink, an obtuse tarsal bent, and a visible eyelid margin. It can be proposed that the tarsal kink is likely to have a predisposition to stretch and flatten in time. Alternatively, it can be possible for patients with mild congenital horizontal tarsal kink to present symptoms late.
Associated systemic abnormalities affecting the cardiovascular, musculoskeletal, and central nervous system have been reported, as in other types of primary upper eyelid entropion. Hiles and Wilder7 emphasize the association of congenital upper eyelid entropion with other systemic abnormalities, influencing the cardiovascular and skeletal systems and the mental development of 6 of their 14 patients. Zak8 observed multiple cardiovascular, musculoskeletal, and central nervous system abnormalities in a child with congenital primary upper eyelid entropion. Although cases with congenital upper eyelid entropion associated with systemic anomalies were reported, no associations were demonstrated in cases with congenital horizontal tarsal kink.6,9–11 In our case, no cardiovascular or systemic malformations were detected. It should nevertheless be kept in mind that the skeletal, cardiovascular, and nervous systems must be carefully examined by a pediatrician because there are reported cases associated with developmental abnormalities in children with congenital primary upper eyelid entropion.
Surgery is necessary in most reported cases of tarsal kink. Various surgical procedures for the correction of congenital tarsal kink include excision of the kink; lamellar tarsoplasty; eyelid everting sutures; anterior lamellar repositioning, rotation, and excision of the kink with tarsal reapposition; and transconjunctival horizontal tarsotomy.3–6,9–12 Dailey et al. reported a case of congenital upper eyelid entropion that had levator aponeurosis disinsertion treated by resection of the attenuated aponeurosis with reinsertion into the upper tarsus combined with pretarsal crease fixation.3 All of these procedures were successful in correcting the entropion. The surgical goal involves weakening the kink with margin rotation and eyelid crease formation. We believe that our technique of reattaching the upper eyelid retractor to the tarsal plate and skin edges dynamically increases the eversion of the upper eyelid. Recently, overaction of the orbicularis muscle and disinsertion of the levator aponeurosis have been reported as possible causes of congenital tarsal kink and congenital entropion of the upper eyelid.3 We think that orbicular muscle spasm in infants causes levator muscle disfunction resulting in tarsal kink. This technique breaks the vicious cycle caused by spastic orbicular fibers. In addition, absence of the eyelid crease supports insufficiency of the terminal levator aponeurosis fibers.
Congenital horizontal tarsal kink syndrome is a rare and severe condition that does not spontaneously improve and is an important cause of corneal ulceration in infants. Early diagnosis and treatment of the resulting entropion reduce the risk of corneal scarring and amblyopia. We suggest that strengthening the levator muscle by reattaching it to the tarsal plate and skin edges may be an alternative treatment for tarsal kink. The method we employed was safe and effective in this case.
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