Congenital Eyelid Research Articles

Extensive isolated bilateral Ankyloblepharon Filiforme Adnatum: a case report

We report a case of a rare congenital eyelid abnormality, ankyloblepharon filiforme adnatum, in an otherwise healthy male neonate, bom to healthy parents out of non-consanguineous marriage. This newborn presented with extensive band of tissue connecting upper and lower eyelid of both eyes, resulting in extremely limited palpebral aperture opening. Antenatal history was unremarkable. The parents came to us at the age of one month. The baby was operated on that day at admission under local anesthesia. The eyelid band of both eyes were excised at the level of each lid margin resulting in normal eye opening. Dilated fundoscopic examination was done along wit,h anterior segment examination. No underlying ocular abnormalities were noted. A detail systemic examination by pediatrician failed to reveal any other congenital anomaly. Echocardiography and cerebral echography (both normal) were performed to exclude congenital heart disease and cerebral malformations. The baby was then discharged without complications. Ankyloblepharon filiforme adnatum may be isolated or may present with other congenital anomalies. Therefore, the presence of eyelid bands should alert for complete systemic examination. Prompt treatment is required for proper opening of the eye and to decrease the risk of amblyopia. CME J 2024; 3(2); 47-49

Congenital eyelid imbrication syndrome: a rare occurrence in Pakistan

Congenital eyelid imbrication syndrome (CEIS) is a rare condition presenting at birth and is characterised by overriding of the upper lid on the lower lid. It is due to longer upper lid, than the lower lid. Overriding leads to spontaneous eversion of the upper lids. In our patient, examination revealed canthal tendon laxity and hyperaemia of the tarsal conjunctiva. All the rest of the structures in the eyeball and adnexa were normal. Spontaneous eversion occurred in two weeks as the upper lid grew with time. No treatment was required. Keywords: Congenital imbrication syndrome, Overriding upper lid, Hyperaemic conjunctiva, Floppy eyelids.

TREATMENT OF CONGENITAL EYELID PTOSIS USING FASCIA LATA TENSOR: A CASE REPORT

TREATMENT OF CONGENITAL EYELID PTOSIS USING FASCIA LATA TENSOR: A CASE REPORT

Congenital lower eyelid entropion with corneal ulcer – A case report

Congenital entropion is a rare condition affecting the lower eyelid more commonly than the upper eyelid. We describe a case of congenital lower eyelid entropion in a 3-week-old with corneal ulceration, likely present in utero. The child had successful repair of entropion with Quickert everting sutures and reformation of the lower eyelid skin crease. At 3 months post-surgery, the lid position and corneal ulcer have resolved. The use of non-incisional rotational eyelid surgery for the repair of congenital entropion is quick and simple with good surgical outcomes.

Bilateral Congenital Upper Eyelid Eversion (Ectropion): A Rare Presentation Responded Well to Conservative Treatment at Temeke Regional Referral Hospital-Tanzania

Bilateral Congenital Upper Eyelid Eversion (Ectropion): A Rare Presentation Responded Well to Conservative Treatment at Temeke Regional Referral Hospital-Tanzania

Complete Release of High-riding Septum and Constricted Fibers with Fat Graft for Congenital Depressed Upper Eyelid with Multiple Creases

Complete Release of High-riding Septum and Constricted Fibers with Fat Graft for Congenital Depressed Upper Eyelid with Multiple Creases

Bilateral Congenital Eyelid Eversion: Management of a Case Using the Conservative Approach at the CADESSO of the University Hospital Center, Donka, Conakry, Guinea

Bilateral Congenital Eyelid Eversion: Management of a Case Using the Conservative Approach at the CADESSO of the University Hospital Center, Donka, Conakry, Guinea

Ultrasound-based histological differences of pretarsal fat fascia before and after double eyelid blepharoplasty with the orbital septum method.

To observe the changes of pretarsal fat fascia histological structure after double eyelid blepharoplasty with the orbital septum method. From December 2019 to February 2022, the patients with congenital single eyelid who underwent double eyelid blepharoplasty with the orbital septum method at the Plastic and cosmetic surgery department of Hunan provincial people's Hospital and the same number of congenital double eyelid volunteers. Then divided into three groups: preoperative eyelid ultrasound images in Group A, postoperative eyelid ultrasound images in Group B, and congenital double eyelid volunteers in Group C. To study the histological differences of pretarsal fat fascia before and after double eyelid blepharoplasty with the orbital septum method and congenital double eyelid by ultrasound. Ninety-eight patients who underwent double eyelid blepharoplasty with the orbital septum method met the inclusion criteria. All 98 patients aged 22.88 ± 3.00 years, including 7 males and 91 females, followed up for 6-12 months. Included 98 volunteers with congenital double eyelid, aged 23.58 ± 2.59 years, including 49 males and 49 females. Ultrasound showed that the pretarsal fat fascia was divided into two layers. The dense layer adhered closely to the surface of the tarsus with no statistically significant differences. There was a statistically significant difference in the thickness of the pretarsal loose fat fascia layer between Group A and B. The points a b c in Group A were significantly thicker than those in Group B and C, and there was no significant difference between Group B and C. Postoperative evaluation: 83 cases were satisfied, 11 cases were basically satisfied and 4 cases were unsatisfied. After double eyelid blepharoplasty using orbital septum, the histological structure of pretarsal fat fascia is similar to that of congenital double eyelid, suggesting that double eyelid blepharoplasty with the orbital septum method can change the structure of pretarsal fat fascia and make it similar to congenital double eyelid, which is one of the anatomical bases for its good clinical effect.

A clinical case of fitting scleral contact lenses for congenital bilateral eyelid coloboma complicated by exposure keratopathy

A clinical case of fitting scleral contact lenses for congenital bilateral eyelid coloboma complicated by exposure keratopathy

Orbicularis muscle fixation downward for the correction of congenital epiblepharon in Chinese patients

Congenital epiblepharon (CE) is a congenital eyelid deformity unique to young individuals of Asian descendants, and it might cause a sequence of other complications such as corneal damage and ocular foreign body sensation. Surgery is the most effective approach to treat this condition. Here, this study evaluated the effectiveness and safety of the orbicularis muscle fixation downward (OMFD) procedure for correcting CE. This study enrolled 29 patients who were diagnosed with CE. These patients experienced significant ocular irritation leading to epiphora and corneal injury and, therefore, they underwent the OMFD procedure that was performed by the same surgeon. The cases were divided into 3 grades according to postoperative outcomes. The OMFD procedure was performed on 55 lower lids of the 29 patients. The average age of the patients was 9.6 years. The mean follow-up duration was 17 months. No perioperative complications were observed, and successful surgical outcomes were achieved in all 29 patients. No postoperative necrosis, infection, or corneal epithelial injury occurred. None of the patients exhibited significant scarring. Three patients showed residual 1-3 eyelashes touching the nasal corneal or bulbar conjunctiva at the inner canthus during the last postoperative examination. These patients did not undergo further surgery as there were no obvious irritation symptoms or corneal damage. The fixation of the pretarsal orbicularis muscle is a simple and effective modification of the Hotz procedure to correct CE. The key aspect of this procedure is the suturing of the pretarsal orbicularis muscle and the lower eyelid retractors.

Etiology of congenital ptosis - literature review

Karolina Ridikaitė1, Dovilė Bučaitė1, Dalia Jarušaitienė1, 2 1Lithuanian University of Health Sciences, Academy of Medicine, Faculty of Medicine, Kaunas, Lithuania 2Lithuanian University of Health Sciences Kauno klinikos, Department of Ophthalmology, Kaunas, Lithuania Abstract Background. Ptosis is abnormal drooping of the upper eyelid (> 2 mm). It is divided into two categories: congenital ptosis, which is present at birth, and acquired ptosis. The pathogenesis of congenital ptosis is thought to be due to a disorder of muscle development and damage to the innervation of the muscles. In contrast, there is currently no precise description of the pathogenesis of congenital eyelid ptosis. Aim. To present the main clinical manifestations of congenital ptosis, based on the available scientific literature. Material and methods. A literature review was conducted in the “PubMed” database using the terms “congenital ptosis” and “myogenic congenital ptosis” and “neurogenic congenital ptosis” from 2010 to 2023. Inclusion criteria was represented by articles relevant to these searches. Results. 1) Myogenic ptosis occurs when the development of the muscle responsible for elevating the upper eyelid is affected and replaced with other tissues. The condition is commonly seen as isolated congenital ptosis or blepharophimosis-ptosis-inverse epicanthal syndrome. 2) Neurogenic ptosis is characterized by an interruption in the nerve signals that control the muscles responsible for elevating the upper eyelid. The condition commonly presents as congenital fibrosis of the extraocular muscles, Marcus Gunn syndrome, Duane syndrome, Moebius syndrome, hereditary congenital facial paresis, Horner’s syndrome and congenital III cranial nerve palsy. Conclusions. Myogenic and neurogenic ptosis are most commonly described in the literature, with a higher prevalence in childhood. In contrast, the underlying mechanism of etiopathogenesis remains unclear. Keywords: congenital ptosis, myogenic congenital ptosis, neurogenic congenital ptosis Full article https://doi.org/10.53453/ms.2023.5.12

Subciliary Rotating Sutures Combined With Modified Hotz Procedure for Correction of Congenital Lower Eyelid Entropion.

Standard techniques for the treatment of congenital lower eyelid entropion may not yield suitable outcomes or may result in overcorrection if disinsertion of the lower eyelid retractors is not the primary cause. Herein, we propose and evaluate a technique using subciliary rotating sutures combined with a modified Hotz procedure for repair of lower eyelid congenital entropion that addresses these concerns. A retrospective chart review was conducted of all patients who underwent lower eyelid congenital entropion repair by a single surgeon using subciliary rotating sutures combined with a modified Hotz procedure between 2016 and 2020. Study variables included patient demographics, follow-up period, postoperative complications, operative success, and recurrence. Twelve patients (19 eyelids) met the study inclusion criteria. The mean patient age was 7.1 ± 6.1 years (range, 0.2-22 years). Nine of the patients were female (75%) and 3 were male (25%). The distribution of eyelids was 8 right (42%) and 11 left (58%). The mean follow-up time was 19.5 ± 15 (range 2.5-45) months. There were two eyelids (11%) that had entropion recurrence after initial repair in patients with concomitant compound disease processes. Repeated repair resulted in success with no recurrence at last follow-up. Overall, the described entropion repair technique was successful and without recurrence in 17 eyelids (89%). There were no cases of ectropion, lid retraction, or other complications. Subciliary rotating sutures combined with a modified Hotz procedure are effective for correction of congenital lower eyelid entropion. As the technique does not manipulate the posterior layer of the lower eyelid retractors, it may be useful for when retractor reinsertion does not yield adequate improvement and may also reduce the risk of eyelid retraction and overcorrection in particular cases.

Объем и разновидности офтальмохирургических вмешательств, выполняемых в детском офтальмологическом отделении, их эффективность

Purpose. To evaluate volume and nosological forms of ophthalmic surgery cases in the pediatric department of the ophthalmology surgery and analyze their effectiveness. Material and methods. A continuous retrospective number of all ophthalmic surgery in the pediatric ophthalmology department of the Khabarovsk branch of the S. Fyodorov Eye Microsurgery Federal State Institution for three years (from January 2019 to December 2021). Results. 5025 children aged from 1 month to 18 years old were operated. Surgical treatment of pathology of oculomotor apparatus was performed in 2304 (45.9%) patients, scleral remodeling in progressive myopia – 1088 (21.7%) people, probing for congenital lacrimal ducts obstruction – 619 (12.3%) children. In 463 (9.2%) children, surgical removal of the chalazion was performed. 166 (3.3%) children were operated for eyelid, conjunctival and orbital tumors. 120 patients (2.4%) underwent surgical revascularization for optic nerve atrophy of various origins. 96 children underwent surgical correction of various congenital eyelid anomalies. During this period, 169 premature babies (3.4%) were under observation in the department. Of these, at threshold stages of retinopathy of prematurity (ROP), 34 patients underwent laser coagulation of avascular areas of retina, 22 patients received intravitreal ranibizumab injection, 14 children with stage 4 or 5 ROP underwent endovitreal intervention of traction retinal detachment. Conclusion. The volume of ophthalmic surgery performed in the pediatric department is quite high, the largest proportions are various methods for eliminating the pathology of oculomotor apparatus and scleral remodeling in progressive myopia. In most cases of strabismus and ptosis surgery, it was possible to achieve complete or partial elimination of pathology. Stabilization of the progressive course of myopia was achieved in 67.9% of cases, threshold stages of ROP – in 91.7%. The high demand of population for surgical treatment of pediatric ophthalmopathology indicates the need for further development and improvement of this area. Key words: pediatric ophthalmology, structure of surgical pathology, main trends of ophthalmic surgery

Euryblepharon; A Case Report From Pakistan

Euryblepharon is a rare congenital eyelid anomaly characterized by horizontal enlargement of the palpebral fissure. The eyelid is less in size in the vertical dimension as compared with the horizontal dimension. It may be isolated or associated with other ocular anomalies (e.g. strabismus or telecanthus) or systemic anomalies (blepharo-cheilo-odontic syndrome)There can be associated lateral canthal misalignment and as a consequence lateral ectropion. The condition was first described by Desmarres in 1854. The average length of lid fissures is different in different ages as portrayed by Duke-Elder and Cook (1963) and Waardenburg and others (1961) ranging from 18.35mm at birth to 29.68 at 24 years of age. 50% of this increase occurs during first few (3-4) years of life.

Comparison of the cutaneous orbicularis oculi excision treatment with the inferior eyelid margin fixation treatment for congenital lower eyelid entropion.

To modify the traditional surgical approach to treat patients diagnosed with congenital lower eyelid entropion using inferior eyelid margin fixation of the orbicularis eyelid muscle. Ninety-six participants (180 eyes) with congenital lower eyelid entropion diagnosed between January 2019 and April 2021 were included in this study. The patients were divided into Group A (cutaneous orbicularis oculi excision treatment) and Group B (inferior eyelid margin fixation treatment). The efficiency and recurrence rate of treatments were used to compare the two treatments. There was no significant difference in age, sex, and eyes distribution in both groups. And higher efficiency rate was found in Group B (P < 0.05). And Group A had a higher recurrence rate in the follow-up after surgical treatment (P < 0.05). This modified inferior eyelid margin fixation of the orbicularis eyelid muscle treatment is an ideal procedure with a high degree of efficacy and low recurrence rate in patients with congenital lower eyelid entropion.

A rare case of congenital ectopic eyelid tissue

Ectopic eyelid, an extremely rare entity, is a congenital disturbance in embryogenesis in which lid tissue is present in an abnormal position near the eyelid. We herein report a case of a six-year-old child with a soft mass near the lateral canthus of the left eye having cilia overlying it, with a similar family history in her father. A surgical excision biopsy was performed under general anesthesia. A histopathology report revealed stratified squamous epithelium with numerous dermal pilous units consisting of a central hair shaft and clusters of Moll's glands, suggesting eyelid tissue. Therefore, an ectopic eyelid tissue can be considered a differential diagnosis for any congenital, non-progressive, soft tissue mass near the eyelid with overlying cilia. However, histopathology is required to confirm the diagnosis.

Two Airway Management Techniques in a Pediatric Patient with Temporomandibular Joint Ankylosis: Two-stage Fiberoptic Scope Intubation Technique and Video Laryngoscope-guided Conversion from Nasotracheal to Orotracheal Intubation — A Case Report

Ankyloblepharon-ectodermal dysplasia-clefting (AEC) syndrome is an ectodermal dysplasia presenting with cleft lip or palate and congenital filiform eyelid fusion. This is a case report of a 1-year and 6-month-old girl with AEC syndrome presenting with temporomandibular joint ankylosis who underwent release of temporomandibular joint ankylosis, coronoidectomy, bilateral cheiloplasty, Tajima rhinoplasty, and repair of lower lip pits under general anesthesia. Fiberoptic nasotracheal intubation was done successfully using a two-stage technique originally described by Stiles. As necessitated by the surgical plan, video laryngoscope guidance was subsequently used to facilitate the conversion from nasotracheal to orotracheal intubation.

Clinical value of phenylephrine testing in the upper and lower eyelids of patients with aponeurotic and congenital eyelid ptosis.

To characterize the phenylephrine test in aponeurotic and congenital eyelid ptosis, to determine the appropriate timing of the phenylephrine test, and to assess the responses of the upper and lower eyelids. This was a retrospective analysis of 140 eyes of 87 patients (mean age 52.29±16.45y; 22 males, 65 females) with upper eyelid ptosis. Totally 88.6% had aponeurotic and 11.4% had congenital ptosis. For the evaluation of the responses of the upper and lower eyelids to topical 2.5% phenylephrine, the scleral show height, the marginal reflex distance (MRD) between the inferior margin of the upper eyelid and pupillary light reflex (MRD1), and between the central portion of the lower eyelid and pupillary light reflex (MRD2) were measured at the 2nd, 5th, and 15th minutes. The changes of MRD1 and MRD2 with time (ΔMRD1 and ΔMRD2) were evaluated. The mean MRD1, MRD2, and scleral show heights increased within 5min after testing, remaining largely stable between the 5th-15th minutes. The percentage of eyes with a greater response in MRD1 increased with increased severity of ptosis (P<0.05). Eyes with aponeurotic ptosis were more responsive to phenylephrine testing than congenital ptosis. The mild ptosis group had lower scleral show measurements and higher ΔMRD2 values. The ΔMRD1 and ΔMRD2 values were poorly correlated in all measurement times. Performing the phenylephrine test 5min after instilling the reagent is adequate to assess the maximum response of the upper and lower eyelids. The upper and lower eyelid responses in phenylephrine testing are poorly correlated. However, the ΔMRD2 is related with baseline scleral show degree that may be a postoperative predictive factor. Further studies are necessary to determine the relationship between the responses of the lower eyelids to phenylephrine testing.

Aniridi ile İlişkili Konjenital Glokomlu Bir Olguda Alt Kapak Epiblefaron Cerrahisi: Olgu Sunumu

Aniridi, kromozom 11p13 üzerinde bulunan PAX6 genindeki bozukluğa bağlı gelişen kısmı veya tam iris defektidir. Aniridiye konjenital katarakt, konjenital glokom, keratopati veya foveal aplazi gibi oküler anomaliler eşlik edebilir. Epiblefaronun etyopatojenezi tam olarak bilinmemektedir. Bununla beraber epiblefaron etyopatogenezinde konjenital cilt katlanma anomalisi ve alt kapak retraktörlerinin yanlış yapılanması olduğu düşünülmektedir. Karotiko-kavernöz fistül, tiroid oftalmopati, megalo-oftalmi ve konjenital glokom gibi oftalmolojik hastalıklarla birlikteliği bildirilmiştir. Bu çalışmada aniridiye bağlı konjenital glokom ile takipli Doğu-Asyalı dört yaş erkek olguda gelişen epiblefaronun tanı ve tedavisinin sunulması amaçlandı. Özgeçmişinde atrial septal defekt bulunan hastanın her iki gözünde alt kapakta epiblefaron, aniridi, mikrosferofaki ve katarakt mevcuttu. Bu olguya epiblefaron nedeniyle alt kapak retraktörleri ile orbiküler kası hedef alan cerrahi teknik uygulandı.

Lids in kids

This workshop will discuss surgical and non-surgical management of pediatric eyelid challenges including lid margin disease, eyelid trauma, congenital eyelid malpositions, and ptosis repair. Emphasis will be on treatment of these conditions including pre-operative evaluation and planning, and a post-operative care and complications.