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 All withdrawn probes were intact.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.

Insertion of a preloaded Monoka™ stent for congenital nasolacrimal obstruction: Intraoperative observations. A preliminary study.

STUDY OBJECTIVE: To study the intraoperative deployment of a pre-loaded probe for a "pushed" monocanalicular nasolacrimal intubation. STUDY DESIGN: Non-randomized study of consecutive cases. MATERIALS AND METHODS: Description: A classical Monoka™ silicone stent with the silicone tube attached at right angles to the punctal plug is contained entirely inside an introducer connected to a piston. Insertion: The procedure begins with intubation of the nasolacrimal duct with the metallic introducer. Traction on the piston retracts the metallic introducer inside the handpiece. This relative shortening progressively ejects the stent, starting with its free end at the bottom of the introducer. The operation was observed endoscopically under single-blind conditions. RESULTS: Twenty-eight preloaded Monoka™ stents were placed consecutively, in 28 congenital nasolacrimal duct intubations in 22 patients (28 sides). Endoscopic examination showed that the free part of the stent was progressively ejected from the introducer during retraction of the piston. Insertion of this pushed stent into the nasal cavity was effective in 23/28 cases (82.1%). A total of 28 preloaded stent insertions were attempted and 23 were correctly deployed. DIFFICULTIES ENCOUNTERED: At the end of nasolacrimal duct intubation, contact between the punctal plug and the lacrimal punctum was problematic in four cases (4/28=14.2%). At the beginning of stent placement, premature ejection of the punctal plug within the end of the introducer occurred in five cases (5/28=17.8%). These five stents failed to insert properly into the nasal cavity. At the end of insertion, retention of the punctal plug in the introducer occurred in two cases (2/28=7.1%). COMPLICATIONS: No cases of intraoperative or postoperative epistaxis were observed. CONCLUSIONS: Intraoperative nasal endoscopy validated the concept of the preloaded Monoka stent and its deployment. Reproducibility and improved reliability may require a change in stenting technique and a design modification.

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.

Pushed monocanalicular intubation. Pitfalls, deleterious side effects, and complications.

PURPOSE: To present our experience with pushed monocanalicular nasolacrimal intubation in the management of 90 consecutive cases of nasolacrimal outflow obstruction. MATERIALS AND METHOD: This paper reports a non-randomized study of 90 consecutive cases treated with a pushed Monoka intubation system (Masterka™). A metal guide is placed inside a silicone tube rather than being attached at the distal end of the tube, as done with traditional pulled intubations. Three probe lengths are available: 30, 35, and 40 mm. SURGICAL PROCEDURE: The silicone stent was pushed into a punctum, canaliculus, and nasolacrimal duct by means of the guide. After passing through the valve of Hasner and reaching the nasal floor, the guide was then delicately withdrawn while remaining oriented along the axis of the lacrimal sac and duct. Throughout this phase, the anchoring plug was held in contact with the punctum. Three study groups were set up chronologically: group 1: endo-DCR procedures done with Masterka insertions under endoscopic observation. Group 2: Masterka insertions done with endoscopic guidance. Group 3: blind Masterka insertions without endoscopic guidance. The patients in groups 2 and 3 were selected on the information obtained by lacrimal probing. Only cases with mucosal nasolacrimal stenoses were included. All patients had surgery under general anesthesia with mechanically assisted ventilation (groups 1 and 2) or spontaneous ventilation (group 3). The anchoring plug was inserted into the punctum and vertical canaliculus, either by pulling on the probe (group 1) or using an inserting instrument. RESULTS: A total of 90 pushed Monoka intubations were done. Endoscopic examination (groups 1 and 2) demonstrated visually that the pushed intubation method was effective. In none of the 28 cases did the silicone bunch up when the guide was withdrawn. DEGREE OF DIFFICULTY: This was dependent upon proper selection for pushed Monoka intubation; the length of the probe and confirmation that there no false passage was created. The pushed intubation technique was only slightly more difficult than a simple lacrimal probing. The average operating time, excluding the anesthetic procedures, was respectively 5 min (group 2) and 4 min (group 3). COMPLICATIONS DURING SURGERY: There were no anesthetic or general problems observed in the three groups. Epistaxis was also not noted. POSTOPERATIVE COMPLICATIONS: Fifteen percent (13/90). The 13 complications noted were: two cases of canaliculitis, one intracanalicular migration, eight probes that disappeared, one keratitis, and one case of involuntary removal by the patient. DELETERIOUS SIDE EFFECTS: Tearing with the probe was in place was noted in 21.1% of the cases (19/90). This tearing disappeared as soon as the probe was removed in 50% of these cases (10/19). FUNCTIONAL RESULTS: Overall, the success rate (absence of epiphora, absence of mucous discharge) was 90% (81/90) with an average follow-up period of 19 weeks (Range, 1 day to 60 weeks). Two cases were lost to follow-up at day 1 and day 7. Group 1: 90.9% (20/22 cases; average age: 65 years, with an average follow-up period of 24 weeks). Group 2: 100% (6/6 cases; average age: 3.1 years, with an average follow-up period of 14 weeks). Group 3: 88.3% (53/60 cases excluding the two cases that were lost to follow-up; mean age: 2.3 years, with an average follow-up period of 16 weeks). CONCLUSIONS: From a technical perspective, pushed nasolacrimal intubation is much simpler than the traditional pulled types of nasolacrimal intubation. The anesthetic procedure required is the same as that for a late probing procedure, but the functional results are better. The Masterka is an alternative to simple late probing in the treatment of mucosal nasolacrimal stenoses in patients of over 12 months of age.

"Pushed" monocanalicular intubation in children under general anesthesia with spontaneous ventilation. A preliminary report.

PURPOSE: We studied the possibility of placing a new type of monocanalicular nasal intubation under general anesthesia with spontaneous mask ventilation in congenital nasolacrimal duct obstruction. PATIENTS AND METHOD: This was a non-randomized study of consecutive cases using a monocanalicular stent called the "pushed Monoka". The benefits of anchoring with meatus fixation are similar to the original Monoka device, but the probe guide or introducer is inside the silicone tube. The external diameter of the "pushed Monoka" is 0.96 mm (versus 0.64 mm in the traditional Monoka). There are three lengths: 30, 35, and 40 mm. General anesthesia was administered by inhalation of a halogen gas using a facial mask. The technique was selected by lacrimal exploration to evaluate the extent of the stenosis (simple or complex). The location and freedom of movement of the stent into the inferior nasal meatus was tested using a second lacrimal probe. Only simple stenosis cases with positive metal-to-metal contact were included in the study. INSERTION TECHNIQUE: The introducer pushes the stent into the lacrimal duct. The introducer should be removed from the silicone sleeve very carefully by gently pulling it out, millimeter by millimeter. This action is carried out while paying careful attention to keeping the stent aligned with the major axis of the lacrimal sac. Throughout this phase, the anchoring plug should remain in contact with the lacrimal punctum. Once the introducer is completely removed, the anchoring plug is secured into the vertical canaliculus. A single-use plug inserter was used. RESULTS: Fourteen children (18 sides) with congenital nasolacrimal duct obstruction were consecutively included in the study. The pushed intubations were all performed under general anesthesia with spontaneous ventilation. The average age of the children was 26.2 months (range: 14 to 46 months). The average duration of the procedure, measured between the moment that the facial mask was put into place and the child's awakening (crying, restlessness) was 14 minutes (range: 9 to 27 minutes). The most variable parameter was the use of the venous portal. The introduction of the pushed probe itself required an average 7 minutes (range: 3 to 11 minutes). None of the children showed epistaxis. In general terms, no intraoperative or postoperative complications were noted. The "pushed Monoka" tubes were withdrawn during postoperative appointments with a mean intubation duration of 34 days (range: 1 to 59 days). Postoperative success (absence of epiphora, absence of mucous discharge) was achieved in 88% of cases (16/18 sides). The average follow-up was 8.7 weeks (range: 3 to 26 weeks). Complications and side effects were minimal. One stent was withdrawn on day 1 due to a keratitis with respect to the anchoring plug. Three stents were spontaneously lost (16%) between day 2 and day 30. Anterior rhinoscopy found none of the stents in the inferior nasal meatus. These four cases were all considered successful as there was no postoperative epiphora noted. CONCLUSIONS: Pushed nasolacrimal intubation can be safely utilized under general anesthesia with spontaneous mask ventilation. This technique appears to be a simple and safe alternative to late and very late probing in the treatment of membranous congenital nasolacrimal duct obstruction in children older than 12 months.

Hemifacial microsomia in a newborn with hypoplastic skin lesions, an eyelid skin tag, and microphthalmia: an unusual presentation of Delleman syndrome.

Delleman syndrome (oculocerebrocutaneous syndrome) is characterized by orbital cysts, periorbital skin appendages, and focal cutaneous hypoplasia. We describe a male infant with findings associated with this condition, including an eyelid appendage, discrete hypoplastic skin lesions, unilateral microphthalmia, and hydrocephalus. In addition, he had striking unilateral mandibular hypoplasia and microtia, features often present in the oculoauriculovertebral (OAV) spectrum. However, hypoplastic skin lesions and eyelid appendages are not features of the OAV spectrum. The marked degree of hemifacial microsomia present in this child has not been previously noted in Delleman syndrome. Two patients with Delleman syndrome have been previously described who have features typically present in the OAV spectrum. This case demonstrates that characteristics of both Delleman syndrome and the OAV spectrum may be present in one individual.

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.

The surgical management of orbitofacial dermoids in the pediatric patient.

Orbitofacial dermoids in the pediatric population represent a diverse group of lesions. Because of their variability in both clinical presentation and contiguous structure involvement, a thorough understanding of their surgical-pathologic anatomy is imperative. In this retrospective review of 84 lesions in 84 patients, we noted segregation of the lesions into three distinct subgroups: brow region dermoids, orbital region dermoids, and nasoglabellar dermoids. Further, we identified physical characteristics within each subgroup that appeared to direct diagnostic workup and subsequent surgical intervention. From this experience, a treatment algorithm was developed that gives the practitioner more precision in the management of these lesions.

Orbital wall infarction in sickle cell disease: MR evaluation.

Orbital wall infarction in a patient with sickle cell disease can present with periorbital swelling and subperiosteal collection and thus can mimic infection on CT. However, MR not only provides excellent morphologic information but, by characterizing the nature of the collection as containing blood, and by identifying bone marrow abnormality, can lead to the diagnosis of orbital wall infarction.