Preloaded Monoka (Lacrijet) and congenital nasolacrimal duct obstruction: Initial results.

STUDY OBJECTIVE: To study the performance of a pre-loaded Monoka stent in the management of congenital nasolacrimal duct obstruction (CNLDO). STUDY DESIGN: Non-randomized study of consecutive cases. MATERIALS AND METHODS: A preloaded classic Monoka silicone stent contained entirely inside its introducer (Lacrijet) was used to treat a consecutive series of subjects with CNLDO over an 11-month period (May 2019-March 2020). Only subjects with chronic symptomatic CNLDO were included. Subjects with intermittent tearing, canalicular pathology, trisomy 21, facial cleft, or history of lacrimal surgery were excluded. Intraoperative findings were recorded, including the degree and location of the nasolacrimal obstruction, successful metal to metal contact with the probe, any difficulties encountered by the Lacrijet device itself, procedure duration, tolerability of the fixation punctal plug, and finally, inspection of the stent after withdrawal of the inserter. Functional success was defined as disappearance of all symptoms of epiphora. RESULTS: A total of 45 preloaded Monoka Lacrijet stents (Lcj) were placed consecutively in 38 children. The mean age was 27.9 months (12-78 months). The mean procedural duration was 2.8minutes (range: 1-10min). The overall success with disappearance of all symptoms of epiphora was 88.8% (40/45). Surgery in cases of simple mucosal stenosis was successful in 92.2% (35/38) of cases, with a mean follow-up time of 7.9 months (range: 1 to 12 months). The duration of stent intubation was for this group was 32 days (range: 1-103). The surgical outcomes for the other 7 cases with more complex intraoperative findings are summarized in the publication. All withdrawn probes were intact. CONCLUSIONS: The Lacrijet stent system is a simple and reliable pushed intubation device for CNLDO in appropriately selected cases where bony stenosis of the canal is minimal.

Intralacrimal migration of Masterka® stents.

BACKGROUND: Tearing and conjunctivitis in children are commonly due to lacrimal drainage system obstruction. Congenital nasolacrimal obstruction is a common pathology treated by probing with or without silicone stent insertion, depending upon the age of the child. The silicone stent is self-retaining and placed for at least one month. Masterka® is a recent version of Monoka®, which may lead to the same surgical complications, such as intralacrimal migration. SUBJECTS AND METHODS: The medical records of two patients surgically treated with the Masterka® probe for nasolacrimal duct obstruction, who developed intralacrimal migration of the stent, were retrospectively reviewed and analyzed. A 41-month-old child and an 18-month-old child presented with disappearance of the silicone tube after 7 days and 2 years respectively. In the first case, the tube migrated completely within the lacrimal system and became externalized through the nose at 2 years, while in the second case, the Masterka® was retrieved through a canalicular approach. In both cases, infants had no further tearing. DISCUSSION: The frequency self-retaining stent disappearance is estimated at 15%. Among these cases, intralacrimal migration is only reported in 0.5% of cases. To prevent intralacrimal migration, the surgical technique must follow a certain number of rules. Management, based on residual epiphora, is discussed. CONCLUSION: Prevention of intralacrimal migration of self-retaining stents involves a rigorous analysis of the relationship between the meatus and the fixation head at the time of placement. After lacrimal intubation, scheduled monitoring is necessary to screen for stent disappearance. Management is based on clinical findings, anterior rhinoscopy and even exploratory canaliculotomy.

Phenotype of the fibroblast growth factor receptor 2 Ser351Cys mutation: Pfeiffer syndrome type III.

We present a patient with pansynostosis, hydrocephalus, seizures, extreme proptosis with luxation of the eyes out of the lids, apnea and airway obstruction, intestinal non-rotation, and severe developmental delay. His skeletal abnormalities include bilateral elbow ankylosis, radial head dislocation, and unilateral broad and deviated first toe. The phenotype of this patient is consistent with that previously reported in Pfeiffer syndrome type III, but is unusual for the lack of broad thumbs. Our patient most closely resembles the case described by Kerr et al. [1996: Am J Med Genet 66:138-143] as Pfeiffer syndrome type III with normal thumbs. Mutations in the genes for fibroblast growth factor receptors (FGFR) 1 and 2 have previously been seen in patients with Pfeiffer syndrome type I. The mutation identified in our patient, Ser351Cys in FGFR2, represents the first reported cause of Pfeiffer syndrome type III. An identical mutation was described once previously by Pulleyn et al., in a patient whose brief clinical description included cloverleaf skull, significant developmental delay, and normal hands and feet [Eur. J. Hum. Genet. 4: 283-291, 1996]. In our patient, previously performed single-strand conformation polymorphism analysis failed to detect a band shift; the mutation was identified only after independent sequence analysis.

Unilateral dermis-fat graft implantation in the pediatric orbit.

The purpose of this study was to evaluate the role of the dermis-fat graft (DFG) as an orbital implant in the pediatric age group. A retrospective study was made of a series of 16 patients who had undergone unilateral orbital implantation of a DFG. The ages of the patients at the time of surgery ranged from 2 months to 17 years, with followup ranging from 2 to 15 years. Growth of the graft was clinically apparent in the younger children. Increasing proptosis required surgical debulking of the graft in six of eight children who were 4 years old or younger at the time of DFG implantation. None of the eight children who were 9 years or older at the time of DFG implantation required surgical debulking. Indeed, five of the older patients demonstrated some degree of graft atrophy. Dermis-fat grafts placed in the orbits of young children appear to grow after implantation. This growth of the implant may help stimulate orbital growth, potentially leading to more symmetry between the involved and uninvolved sides.

Oral mucous membrane grafts for corneal protection to permit prosthetic shell wear.

Conjunctival flaps are commonly used to cover the cornea in patients who otherwise can not tolerate a scleral shell. An alternative method of protecting the cornea, oral mucous membrane grafting, is described herein. Ten patients had oral mucous membrane grafting to the cornea. Patients ranged from 5 months to 47 years of age. Diagnoses included partial cryptophthalmia, microphthalmia, congenital orbital fibrosis syndrome, juvenile active ossifying fibroma, and trauma. All patients had poor visual function in the affected eye. Patients were either intolerant of scleral shell wear or had other contraindications to the use of a shell over an unprotected cornea. Following mucous membrane grafting (follow-up, 1-3 years), all patients were successfully fitted with prostheses and obtained good cosmetic results.

Prominent proptosis in childhood thyroid eye disease.

BACKGROUND: Orbital signs and symptoms occur in approximately one half of children with Graves disease, but the symptoms are usually minor and limited to the eyelids. Prominent proptosis is uncommon in children with this disorder. METHODS: Review of eight children with prominent proptosis associated with thyroid eye disease. Four patients were treated at the Children's Hospital of Philadelphia, the other four at the Columbia Presbyterian Medical Center. RESULTS: At initial presentation, children ranged in age from 3 to 16 years. There were five girls and three boys. Seven of eight children had hyperthyroidism at ophthalmic presentation. Four patients had restrictive myopathy, and all of the seven patients who underwent neuroimaging had extraocular muscle enlargement. Five patients were treated with lubrication. Two underwent orbital fat decompression. One patient had thyroid eye disease and myasthenia gravis. CONCLUSIONS: Proptosis in childhood thyroid eye disease usually is associated with a hyperthyroid state. The proptosis may be dramatic, but corneal exposure and restrictive myopathy are seen in only some of the patients. Neuroimaging shows enlarged extraocular muscles. Most children with this complication can be treated conservatively with topical lubrication, but orbital fat decompression may be considered in patients with more advanced conditions.

Results of dacryoscintigraphy in massage of the congenitally blocked nasolacrimal duct.

Between November 1990 and November 1993, 580 children with lacrimal outflow obstruction were examined at the Children's Hospital of Philadelphia. After excluding patients previously treated for nasolacrimal duct obstruction, we obtained a prospectively selected series of 20 children for this study. These patients underwent dacryoscintigraphy before and immediately after lacrimal sac massage to investigate the effect of external compression on fluid movement within the lacrimal outflow system. In 12 patients, tracer did not enter the lacrimal outflow system on the side(s) of obstruction. The absence of radiopharmaceutical correlated with clinical obstruction. In eight patients, tracer was noted to enter the lacrimal sac. After massage of the lacrimal sac, we observed progression of the tracer in five of the eight subjects. In these eight subjects, the pre- and postmassage tear column measurements showed a relative increase of 34.3%. Massage of eight clinically normal ducts showed a relative increase of 2.4% (p = 0.06). We conclude that progression of the tear column after lacrimal massage can be demonstrated on dacryoscintigraphy.

Tonic pupil and orbital glial-neural hamartoma in infancy.

PURPOSE/METHODS: Tonic pupils in early childhood are rare. We studied an otherwise healthy 31/2-month-old girl who had a right pupil that was poorly reactive to light, without other signs of oculomotor nerve palsy. RESULTS/CONCLUSIONS: Constriction of the right pupil after instillation of 0.125% pilocarpine eyedrops confirmed denervation hypersensitivity, consistent with a tonic pupil. There was no strabismus or proptosis. A magnetic resonance imaging scan demonstrated a right orbital mass, interposed between the lateral and inferior recti muscles. Biopsy was consistent with a benign, glial-neural hamartoma. Thus, in this young patient, a tonic pupil was associated with a benign orbital mass.

Congenital and acquired anophthalmia.

Anophthalmia is an absence of ocular tissue in the orbit. Important aspects in the diagnosis, evaluation, and treatment of both congenital and acquired anophthalmic patients are reviewed. Congenital and acquired anophthalmia differ in etiology, but many concepts of management can apply to either category. Specific steps in the evaluation and treatment including the proper timing for medical and surgical intervention will be discussed. The major goals for the ophthalmologist are to optimize motility and symmetry of the eyelids and orbit and coordinate efforts with other medical specialties.

Ophthalmic features and visual prognosis in the Treacher-Collins syndrome.

The ocular findings and visual prognosis were reviewed in 24 patients with the Treacher-Collins syndrome who were evaluated in the craniofacial clinic in the Division of Pediatric Ophthalmology at Children's Hospital of Philadelphia between 1980 and 1991. All patients had some abnormality. Vision loss was present in 37% of patients. Amblyopia was present in 33%, significant refractive errors were present in 58%, and anisometropia was documented in 17%. Strabismus was present in 37% and significant lid and adnexal abnormalities were seen in 96%. The prognosis for normal vision in at least one eye is good but vision loss secondary to amblyopia is more resistant to treatment owing to other medical problems and social concerns.

Congenital unilateral fibrosis, blepharoptosis, and enophthalmos syndrome.

The authors report four cases of the rarest form of the congenital fibrosis syndrome. This disorder is exhibited in infancy as unilateral blepharoptosis, strabismus, limited ductions, globe displacement (enophthalmos and blepharoptosis), and decreased vision, usually due to amblyopia. Forced ductions are positive and surgical exploration confirms anomalous muscle structure. Computed tomography and magnetic resonance imaging studies in these four patients were diagnostically beneficial, showing extraocular muscle and tendinous insertion involvement, and poorly defined intraconal and extraconal masses that had the appearance of scar or inflammatory tissue. All patients had globe displacement. The opposite eye and intracranial contents were normal in all of our patients. Results of histopathologic examination obtained at surgery in three of these patients show replacement of affected structures by fibrous tissue and included the extraocular muscles, orbital fat, Tenon's capsule, and conjunctiva.

Evaluation of a collagen/hydroxylapatite implant for orbital reconstructive surgery.

A variety of autogenous and alloplastic materials have been used to correct enophthalmos. Hydroxylapatite (HA), is a calcium-phosphate-based compound that has been extensively studied as a bone replacement material. We studied the properties of a new dense particulate form of HA in a collagen matrix (PFC/HA) implanted in the subperiosteal space of ten rabbit orbits for a period of 6 months. All animals were studied with pre- and postoperative computed tomography (CT) scans, and measurements of induced proptosis and implant volume were made. The proptosis induced by the implant averaged 2.2 mm and was stable over a 6-month period. Implant volume was constant throughout the study. Three-dimensional computer-generated images of the soft tissue, skeletal, and implant surfaces confirmed the implant stability. All animals were studied histologically with fluorochrome bone markers, which revealed minimal foreign body reaction to the implant, no evidence of infection, and marked fibrovascular ingrowth. We found the PFC/HA to possess properties that make it an ideal implant material: ease of availability, ease of handling, no resorption, minimal immunogenicity, infection resistance, no observed migration, biointegration, and no risk of disease transmission. PFC/HA may make an excellent implant material to manage orbital volume.

Ocular and adnexal findings in patients with facial microsomias.

The authors reviewed the ocular findings in 49 patients with facial microsomias not considered part of a known syndrome. Five patients had bifacial microsomia, and 44 patients had hemifacial microsomia. These patients were evaluated in the Craniofacial Clinic in the Division of Pediatric Ophthalmology at Children's Hospital of Philadelphia between the years 1979 and 1989. Ocular or adnexal abnormalities were present in 67% of patients. Visual loss was present in 8% of eyes. Amblyopia was present in 16% of patients. Significant refractive errors were present in 27% of patients, and anisometropia was documented in 8%. Strabismus was present in 22% of patients, and significant lid and adnexal abnormalities were seen in 41%. Because ocular or adnexal findings present in a significant percentage of patients with more mild forms of facial microsomias, regular examinations to uncover and treat these abnormalities are recommended.

Visual loss in patients with craniofacial synostosis.

We reviewed the ocular findings in patients with cranial stenosis, who were seen in the Craniofacial Clinic at The Children's Hospital of Philadelphia, to determine the prevalence, pattern, and etiology of visual loss. There were 15 patients with Apert syndrome, 25 patients with Crouzon syndrome, and 18 patients with other forms of craniofacial synostoses. Visual loss occurred in 17 eyes of 11 patients with Apert syndrome, 14 eyes of 11 patients with Crouzon syndrome, and 12 eyes of 8 patients with other craniofacial synostosis syndromes. Refractive errors and strabismus were present in the majority of these patients. Amblyopia due to strabismus, ametropia, or ptosis was the major reason for visual loss in all three groups of patients. Structural abnormalities contributed to loss of vision in six eyes.

Congenital craniofacial anomalies of ophthalmic importance.

Congenital craniofacial abnormalities frequently require ophthalmic evaluation and surgical management. Called upon to perform as part of the craniofacial team managing the often severely deformed craniofacial patient, the ophthalmologist must bring a basic knowledge of craniofacial syndromes and developmental anatomy, as well as clinical acumen to help preserve or improve ocular and adnexal function. As an introduction to this area of ophthalmology, the clinical features, classification, appropriate facial embryology, assessment and surgical considerations of the various congenital craniofacial abnormalities are reviewed. The expanding availability of craniofacial surgeons and surgical teams along with improved surgical results will ultimately require an increasing involvement by many more ophthalmologists in the evaluation and management of these congenital abnormalities.

The ophthalmic manifestations and treatment of the amniotic band syndrome.

The amniotic band syndrome is an unusual cause of craniofacial deformities. The syndrome, which is initiated by rupture of the amnion, results in an unpredictable combination of compression deformities of the fetus, construction or amputation defects of the extremities, and craniofacial clefting deformities. The type and extent of ophthalmic abnormalities are dependent on the band location and timing. They include a combination of bony orbital clefts or hypertelorism; lid anomalies such as colobomas, ptosis, and ectropion; lacrimal outflow obstruction; and globe involvement. We review the clinical course of 14 patients diagnosed with, or suspected as having, this syndrome and describe the methods of treatment. In most instances, multiple surgical procedures were required to restore function. Satisfactory cosmesis proved more difficult to obtain.