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 All withdrawn probes were intact.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.

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.

Frontalis suspension in congenital ptosis using a polyfilament, cable-type suture.

Fifty-five frontalis sling procedures were performed with use of 4--0 polyfilament, cable-type ophthalmic suture (Supramid Extra) as the material for suspension. Recurrences numbered 16, recorded through a follow-up period of one to ten years. Trauma accounted for five recurrences. A pattern of gradual droop of the lid operated on became most obvious between six and ten years postoperatively and accounted for six of seven recurrences during this period. These findings suggest that this suture material is a poor alternative to fascia lata for permanent frontalis suspension in patients with congenital ptosis and should be used only in unusual circumstances, or when a temporary procedure is indicated.

Lattice corneal dystrophy. Report of an unusual case.

The clinical, histochemical, light and electron microscopic evaluation of a case of lattice corneal dystrophy, appears clinically as an atypical granular dystrophy. There is structural and histochemical differentiation of the two dystrophies. Electron microscopy is often an invaluable aid in establishing a definitive diagnosis. The possible sources of the filamentous material found in lattice corneal dystrophy are discussed. It seems that not only keratocytes, but also corneal epithelial cells, occasionally may have the ability to elaborate the abnormal material that is considered to be amyloid in nature.

Ocular and adnexal complications of unilateral orbital advancement for plagiocephaly.

Strabismus, ptosis, lateral canthal dystopia, nasolacrimal obstruction, and cranial nerve palsy were noted preoperatively in 32%, 21%, 14%, 12%, and 9% of 34 patients, respectively, undergoing ophthalmologic evaluation prior to unilateral orbital advancement for plagiocephaly. Thirty-two percent of the patients had normal preoperative ocular and adnexal examination results. Ptosis, strabismus, and amblyopia were frequently acquired postoperative abnormalities in 29%, 18%, and 18% of the patients, respectively. Forty-four percent of the patients had no new abnormalities following craniofacial surgery.

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.

Ocular alignment after craniofacial reconstruction.

We undertook a retrospective study of 160 patients to ascertain if craniofacial reconstruction affected ocular alignment. In most patientspre-existing alignment and binocularity were unaffected by craniofacial surgery; thus, correction of any strabismus can be performed on a child without concern for disruption of muscle tissue by later craniofacial surgery.

Variations in extraocular muscle number and structure in craniofacial dysostosis.

Five of 12 patients with craniofacial dysostosis who needed strabismus surgery had anomalies of extraocular muscle structure and number. Two cul-de-sac incisions per eye are sufficient to investigate all muscles for anomalies and add little time or risk to the planned strabismus procedure. The origin of these anomalies and their frequency in craniofacial stenosis are unknown.

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.

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.

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.

Ophthalmic considerations in cranio-orbital surgery.

Interdisciplinary management of cranio-orbital problems has allowed improved surgical results in the diagnosis and management of motility and adnexal deformities. Careful evaluation of visual function preoperatively, combined with proper timing of repair, can greatly assist the development and maintenance of optimum visual function.

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.

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.

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.

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.