Katrinka L. Heher

Silent sinus syndrome : A case presentation and comprehensive review of all 84 reported cases

Objectives: The term silent sinus syndrome has been used to describe the constellation of progressive enophthalmos and hypoglobus due to gradual collapse of the orbital floor with opacification of the maxillary sinus, in the presence of subclinical chronic maxillary sinusitis. Currently, it is believed to occur as a result of the sequence of events following maxillary sinus hypoventilation due to the obstruction of the ostiomeatal complex. Methods: In this study, we present a case of true silent sinus syndrome. In addition, we highlight the previously published cases of silent sinus syndrome, as well as provide a review of the etiology, pathophysiology, radiologic diagnosis, surgical treatment, and pitfalls to avoid in the management of patients with silent sinus syndrome. Results: Eighty-three previously published cases of silent sinus syndrome were reported in the literature and are summarized in this review. Conclusions: A well-defined set of criteria is needed to classify a patient under the diagnosis of silent sinus syndrome, which include enophthalmos and/or hypoglobus in the absence of clinically evident sinonasal inflammatory disease.

The natural history of Leber's congenital amaurosis: Age-related findings in 35 patients

The authors studied 35 patients with Lebers congenital amaurosis and assessed visual acuity, fundus appearance, and systemic findings. The patients were arbitrarily divided into five age groups. Visual acuities were comparable at all ages. Of 22 patients seen for follow-up examinations (mean length of follow-up, 5 years), vision worsened slightly in only 4 patients (3 with macular coloboma-like lesions and 1 with keratoconus). Fifty percent of retinal examinations in patients younger than 1 year of age were normal. With increasing age, retinal pigmentary changes became evident. All but four patients seen on more than one occasion developed progressive retinal/retinal pigment epithelium changes. Cataracts (5 patients) and keratoconus (3 patients) were present only in older patients (9 to 33 years of age). In Lebers congenital amaurosis, which probably comprises a number of genetically heterogenous conditions, visual acuity remains stable despite progressive retinal pigmentary changes. The subgroup of patients with macular colobomas, however, may develop progressive decrease in vision. Cataracts and keratoconus are additional factors contributing to visual impairment in older patients.

Unilateral dermis-fat graft implantation in the pediatric orbit.

The purpose of this study was to evaluate the role of the dermisfat 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.

Crush Injuries to the Head in Children

Although the majority of head injuries in children and adults involve dynamic loading conditions, some patients suffer static loading. Static loading occurs when forces are applied slowly to the head, and it produces a much different pattern of injuries. Crush injuries are usually described in the context of industrial accidents, but in our experience, these injuries are not rare in children. We report a series of seven crush injuries in young children admitted during a period of 29 months and describe our experience in the evaluation and treatment of this complex entity. Patient ages ranged from 15 months to 6 years. In four cases, the childs head was run over by a motor vehicle backing up in a driveway or parking lot. In the three other patients, the static loading occurred when the child climbed or pulled on a heavy object, which then fell over with the child and landed on the childs head. One child with cervicomedullary disruption died shortly after his arrival at the hospital. The others showed varying degrees of soft tissue injury to the face and scalp, with Glasgow Coma Scale scores ranging from 7 to 15. Computed tomograms and magnetic resonance images showed multiple and often extensive comminuted calvarial fractures, as well as subarachnoid and parenchymal hemorrhages. All patients had basilar cranial fractures. There was one cervical spine injury but no major vascular injuries. One child had pituitary transection, four had cranial nerve palsies, and another developed a delayed cerebrospinal fluid rhinorrhea 18 months after injury. All children made good cognitive recoveries, with some having relatively mild fixed focal deficits.(ABSTRACT TRUNCATED AT 250 WORDS)

Management of the anophthalmic socket in pediatric patients.

Purpose of review The management of the pediatric anophthalmic socket is distinguished from adult anophthalmia, because normal socket and facial development is dependent on orbital growth. Recent literature on managing anophthalmic sockets is discussed with a focus on the pediatric patient. Recent findings There are many studies discussing the management of the anophthalmic socket, but few are randomized clinical studies. Although the management of anophthalmia in the child is fraught with challenges and nuances, the principal aim is to encourage socket growth. Summary Both congenital and acquired pediatric anophthalmia are discussed. Surgical decisions, choice of orbital implants, various methods of socket expansion, and socket reconstructions are examined.

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 Childrens 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.

Ectopic brain tissue in the orbit

Purpose The authors report findings in a 9-month-old male infant with heterotopic brain tissue in the orbit, and compare and contrast the characteristics in this patient with the few other descriptions of such lesions in the literature.Methods Excisional biopsy of the growth was undertaken by means of an anterior orbitotomy.Results A 9-month-old male infant had a history of congenital left ‘anophthalmia’ and a slowly growing mass in the left orbit. An MRI scan revealed an orbital mass with solid and cystic components. Histological study of the excised tissue was performed and revealed a choristomatous arrangement of dysplastic brain tissue with intermixed primitive retina including pigmented epithelium. There was no connection between the orbit and cranial cavity.Conclusions The mass must be considered a rare example of heterotopic brain tissue in the orbit and is the only instance we could find in the literature in which a formed eye was absent but in which a scattered primitive ocular structure could be identified.

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.

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

Summary 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. SUMMARY Ten patients had mucous membrane grafting to protect the cornea in order to permit essentially constant wear of a prosthetic scleral shell. The results of this technique are gratifying. Only one patient required additional surgery following significant dehiscence of the graft due to self-induced trauma. The oral mucosal donor site healed quickly and well, and no significant scar formation has been observed (9). Unlike previous reports of similar procedures, neither contracture of the grafts nor production of an abnormal discharge has been observed (10). All patients in this series achieved a superior cosmetic result following prosthetic shell fitting. Most importantly, similar to a properly fitted enucleation prosthesis, the shells could be tolerated with only infrequent removal required.

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.