Congenital unilateral orbital fibrosis with suspected prenatal orbital penetration

Congenital fibrosis of the extraocular muscles includes various abnormalities ranging from isolated fibrosis of a single muscle to bilateral involvement of all extraocular muscles. Congenital unilateral orbital fibrosis is a specific fibrosis syndrome with only five cases previously reported. Although these reports postulated that a prenatal orbital inflammatory process caused the fibrosis, no etiology was proposed, nor was a mechanism suggested for entrance of the inflammation into the orbit. We discuss three additional cases in which evidence suggested prenatal orbital penetration. In one, a dense scar was found at birth from the skin of the upper lid to beyond the trochlea. The second had a dense fibrous tract extending from the upper lid skin to the trochlea and beyond into the orbit without a skin scar. These lesions could have been inflicted by the fetuses' own fingers or toes or, in the second case, by the twin fetus. The third had a benign mesenchymoma of the nasopharynx and skull that had eroded the medial orbital wall, which was seen on computed tomography scan but not on routine x-rays. The nature of the inflammatory substance is unknown. There was no other evidence of inflammation or infection in the eight reported cases. Possibly amniotic fluid itself, entering the orbit "late" in gestation, might cause this inflammation. We recommend that all children with congenital unilateral orbital fibrosis be examined specifically for defects in the orbit, and that computed tomography be employed rather than plain x-rays.     

Management of periorbital and orbital cellulitis

Swelling of the tissues surrounding the eye is a relatively common presentation in paediatric practice. Many of the mild, pre-septal cases of periorbital inflammation and infection are caused by insect bites, trauma and conjunctivitis. Frequently infection spreads from the ethmoid sinuses and invades orbital tissues. The distinction between pre-septal and orbital involvement can be difficult based on clinical examination only, and the research base supporting management of periorbital and orbital cellulitis is limited. This review addresses the role of investigations and the multi-disciplinary approach needed in order to establish accurate diagnosis, appropriate treatment and prevention of serious complications including blindness and venous sinus thrombosis.     

Computerized tomography in the diagnosis and treatment of orbital cellulitis

Acute sinusitis remaines the major cause of orbital cellulitis in children, despite the extensive use of antibiotics. Staging of orbital cellulitis on the basis of clinical signs is difficult but is easily achieved by computerized tomography. The CT findings in a series of 9 children with orbital cellulitis in different stages are reported. The information obtained by CT contributes to the selection of an adequate surgical approach, if a therapeutic trial with antibiotics fails.     

Sclerosing idiopathic orbital inflammation

A 5-year-old girl referred for orbital cellulitis was found to have a right orbital mass. Computed tomography revealed a mass occupying the inferotemporal orbit, extending into the maxillary sinus. Biopsy yielded a diagnosis of sclerosing idiopathic orbital inflammation. She was successfully treated with prednisone.     

The role of computed tomography in the diagnosis of subperiosteal abscess of the orbit.

Periorbital cellulitis is a frequent complication of sinusitis in children. Subperiosteal abscess (SPA), however, is an uncommon sequela and may lead to serious complications if not promptly and adequately treated. A series of 13 consecutive patients who underwent orbital computed tomographic (CT) scans from January 1, 1986, to June 30, 1989, to investigate the possibility of SPA were reviewed. As demonstrated in this series, the diagnosis of subperiosteal abscess remains a clinical one that may be supported, but not determined, by an orbital CT scan. Cautious interpretation of these studies is advocated. Aggressive surgical therapy in appropriate clinical situations will lead to more rapid resolution of the disease process and fewer complications.     

Acute orbital pseudotumor with iatrogenic intraorbital air: a diagnostic dilemma

A 13-year-old black female was referred with unilateral granulomatous uveitis and orbital inflammation of sudden onset. Hematologic, serologic, bacteriologic, and ultrasonographic studies along with high resolution CT scans of the orbits were employed to determine the diagnosis and appropriate treatment. Subconjunctival corticosteroid injection containing air prior to referral obfuscated the ultimate diagnosis of pseudotumor. The Pediatric Infectious Disease service delayed definitive treatment with systemic steroids. Differential diagnosis of granulomatous uveitis with orbital inflammation are discussed. CT scan has significantly advanced the diagnosis and management of orbital pseudotumor.     

Evidence supporting extraocular muscle pulleys: refuting the platygean view of extraocular muscle mechanics

BACKGROUND: Late in the 20th century, it was recognized that connective tissue structures in the orbit influence the paths of the extraocular muscles and constitute their functional origins. Targeted investigations of these connective tissue "pulleys" led to the formulation of the active pulley hypothesis, which proposes that pulling directions of the rectus extraocular muscles are actively controlled via connective tissues. PURPOSE: This review rebuts a series of criticisms of the active pulley hypothesis published by Jampel, and Jampel and Shi, in which these authors have disputed the existence and function of the pulleys. METHODS: This article reviews published evidence for the existence of orbital pulleys, the active pulley hypothesis, and physiological tests of the active pulley hypothesis. Magnetic resonance imaging in a living subject and histological examination of a human cadaver directly illustrate the relationship of pulleys to extraocular muscles. RESULTS: Strong scientific evidence is cited that supports the existence of orbital pulleys and their role in ocular motility. The criticisms of the hypothesis have ignored mathematical truisms and strong scientific evidence. CONCLUSIONS: Actively control led orbital pulleys play a fundamental role in ocular motility. Pulleys profoundly influence the neural commands required to control eye movements and binocular alignment. Familiarity with the anatomy and physiology of the pulleys is requisite for a rational approach to diagnosing and treating strabismus using emerging methods. Conversely, approaches that deny or ignore the pulleys risk the sorts of errors that arise in geography and navigation from incorrect assumptions such as those of a flat ("platygean") earth.     

Orbital hydatid cyst manifested as expanding exophthalmos following blunt orbital trauma

A 14-year-old boy suffered right blunt orbital trauma. X-ray revealed a compressed fracture of the right ethmoidal sinuses. Three months after the trauma, progressive, painless right proptosis developed. Ocular examination revealed a severe proptosis and pronounced down displacement of the right globe without signs of orbital inflammation. A well-demarcated, extraconal right orbital cystic mass was seen on computerized tomography scan. The cyst contents were aspirated and found to contain echinococcal scolices and "hydatid sand." Hypertonic saline was injected and the cyst was excised. Progressive proptosis after blunt orbital trauma in patients from endemic areas should be suspected of being an orbital hydatid cyst.     

Anterior segment ischemia after two-muscle surgery in a patient with radiation-treated nasopharyngeal carcinoma

Anterior segment ischemia after strabismus surgery is a rare occurrence. Risk factors include single-stage surgery of three or more muscles in one eye, dysthyroid eye disease, and advanced age. The authors report a case of anterior segment ischemia after single-stage surgery of two horizontal muscles without previous reported risk factors. However, the patient had received radiotherapy for nasopharyngeal carcinoma more than 20 years ago. The case was successfully treated with systemic steroids but with reduced best-corrected visual acuity. Previous radiotherapy in the related orbital field may be a new risk factor for this condition.     

Influence of antineoplastic therapy on function of the masticatory system,tooth development,and cariogenic status: A case report

Antineoplastic therapy causes developmental disturbances in the dental enamel and root if children are treated during tooth development. Increased caries activity has also been reported. The effect of anticancer therapy on the function of the masticatory system (i.e., jaws, dentition, masticatory muscles) is not well known. A case report of a 9-year-old girl with right auricular rhabdomyosarcoma is presented. She received irradiation of 50 Gy to the right auricular area and chemotherapy. A year and a half after cessation of cancer therapy, she was disease free and the clinical stomatognathic examination combined with electromyogram (EMG) registration of the masseter and temporal muscles and magnetic resonance imaging (MRI) examination of the temporomandibular joints (TMJ) revealed a strongly restricted mouth opening capacity, painful right TMJ, and flattened head of the right mandibular condyle. Muscle atrophy in the right masseter muscle was clearly visible but EMG activities of the masseter and temporal muscles, however, were higher on the right than on the left. More severe developmental defects, and worse gingival and cariological health were observed on the right side than on the left side. She developed 12 carious lesions and all the lesions were on the right maxilla or mandible or on anterior teeth. The left side was not affected. Intensive prophylactic dental care after cancer treatment is important in order to prevent caries and gingival inflammation. Stomatognathic treatment (i.e., management of occlusal and dysfunctional problems) may improve the mouth opening capacity and relieve pain. © 1996 Wiley-Liss, Inc.     

Familial aplasia of the inferior rectus muscles

PURPOSE: We describe inferior rectus aplasia in three members of a family. No family member showed signs of craniofacial dysostosis syndromes or neurofibromatosis. PATIENTS AND FINDINGS: A woman with abnormal head posture, right exotropia, and right hypotropia presented to our clinic. She mentioned that her two children had similar problems. A provisional diagnosis of ocular fibrosis or atypical Duane syndrome was made and she underwent surgery. During surgery, the inferior rectus muscle could not be identified in either eye. Subsequently, her children were examined and orbital magnetic resonance imaging (MRI) was performed for all family members. MRI confirmed bilateral absence of the inferior rectus muscles in the mother and unilateral absence with atrophic and fibrotic contralateral inferior rectus muscles in the children. CONCLUSION: This is the first report in the literature of a family with aplastic inferior rectus muscles with no signs of craniosynostosis or neurofibromatosis.     

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??Idiopathic inflammatory sympathies are a group of rare autoimmune disorders??characterized by inflammation in striated muscle. Dermatomyositis is the most common subtype in children.Though a number of diagnostic criteria have been proposed??the diagnosis of dermatomyositis is based on a combination of clinical??laboratory and electromyography findings.Typical features are subacute-onset?? proximal??symmetric muscle weakness??pathognomonic skin rashes.Muscle biopsy demonstrated inflammatory infiltrates confined to perimysium??often around blood vessels??and perifascicular muscle fiber atrophy. The uses of magnetic resonance imaging of skeletal muscles??detection of myositis-specific autoantibodies and nailfold capillaroscopy have become useful additions to our diagnostic test.     

Coincidence of congenital infiltrative facial lipoma and lingual myxoma in a newborn Holstein calf

A one-day-old male Holstein calf was presented with a palpable subcutaneous mass, extending from the parotid to the orbital region, involving the entire right side of the face and a large flabby mass without any evidence of inflammation or edema on the tongue. Macroscopically, the cut surface of the lingual mass appeared slightly lobulated, pink, with a mucoid appearance and gelatinous consistency. Histopathological examination confirmed the infiltrative subcutaneous lipoma and lingual myxoma evidenced by low cellularity and abundant basophilic, mucinous stroma. In this report, clinical and detailed histhopathological findings of congenital infiltrative myxoma and its coincidence with infiltrative facial lipoma is reported in a newborn calf.Key Words: Calf, Histopathology, Infiltrative lipoma, Infiltrative myxoma     

The clever fetus: responding to inflammation to minimize lung injury

Clinical and experimental information indicate that fetal exposure to inflammation can induce lung maturation. This inflammation may be chronic and indolent. We present clinical and experimental information that challenge assumptions about chorioamnionitis associated infection/inflammatory exposures to the fetus. We question the assumption that the fetal compartment is sterile, and that delivery is inevitable if chronic infection/inflammation is present. We demonstrate that the preterm fetus can develop a brisk and adequate inflammatory response, and the fetus also can quickly modulate and downregulate inflammation to prevent injury. The fetus at risk of early preterm delivery may be exposed to both chorioamnionitis and glucocorticoids. Both exposures can either increase or decrease fetal inflammatory responses depending on the timing of the exposures. The immunomodulatory ability of the fetus to fetal exposures remains an unexplored research field.     

Graves' ophthalmopathy evolution studied by MRI during childhood and adolescence

Pediatric patients with Graves' disease (n=26) were studied longitudinally by magnetic resonance imaging of the orbits, allowing an assessment of the enlargement of the extraocular muscles and orbital volume variations. The positive outcome of Graves' ophthalmopathy correlated with low TRAb (autoantibodies to thyroid-stimulating hormone receptor) titers at diagnosis and during follow-up and with prepubertal condition at diagnosis.     

Posterior scleritis in a 7-month-old infant

This article describes a 7-month-old infant with posterior scleritis, diagnosed on the basis of B-scan ultrasonography and computed tomography. The patient was initially diagnosed with preseptal cellulitis and endophthalmitis. Posterior scleritis should be considered in the differential diagnosis of acute orbital inflammation in children younger than 1 year.     

Preseptal and orbital cellulitis: 15-year experience with sulbactam ampicillin treatment

The infection of the orbita and ocular tissues can result in severe local and systemic complications. We aimed to determine the predisposing factors for preseptal and orbital cellulitis, the clinical and routine laboratory differences between orbital and preseptal cellulitis, and the change in the spectrum of the pathogens and the antibiotics used in the last 10 years. One hundred thirty-nine patients, hospitalized in Hacettepe University Faculty of Medicine Children's Hospital between 1 January 1990 and 31 December 2003 with diagnosis ofperiorbital or orbital cellulitis, were reviewed retrospectively. Ten of the patients (7%) had orbital and 129 (93%) had preseptal cellulitis. The male/female ratio was 1.7:1. The average age (mean+/-standard deviation) was 5.7+/-4 years. The seasonal distribution was most marked in spring and fall periods. When compared with preseptal cellulitis, the mean blood cell count, erythrocyte sedimentation rate and C-reactive protein levels were significantly higher in patients with orbital cellulitis. Staphylococcus aureus was isolated in 13 (41.9% of total microbiologically confirmed cases), coagulase-negative staphylococcus in 8 (25.8%), and H. influenza type b in 2 patients (6%). Thirty out of 77 clinical sample cultures (39%) were positive. In clinical studies, etiological agents of orbital and preseptal cellulitis could be identified in only 20-30% of cases, so in clinical practice treatment is usually empiric. We observed that sulbactam-ampicillin was a safe and effective choice of treatment in orbital and preseptal cellulitis in our cases.     

Trauma to elbows, knees, and ankles

In summary, the emergency department or office-based physician should distinguish first between inflammation and injury. A clinical diagnosis of fracture should be made before obtaining and reading films. Comparison views help to resolve doubt. A neurologic examination should be documented before undertaking reduction. Finally, if in doubt, a splint for 24 to 48 hours until an orthopedic opinion is available causes no harm.     

Tension pneumo-orbitus and pneumocephalus induced by a nasal oxygen cannula: report on two paediatric cases

The present paper highlights the potential dangers of misplaced nasopharyngeal oxygen cannulae causing secondary pneumo-orbitus and pneumocephalus in two paediatric patients. While this complication is uncommon, early recognition allows prompt and appropriate intervention, with cessation of nasal oxygen, cannula removal, early investigation with computed tomography (CT) head/orbit scan and orbital or cranial decompression, if required. Early CT imaging identifies medial orbital or paranasal sinus fractures, the presence of sinusitis, associated intracranial air and assessment of the degree of orbital or intracranial tension. Antibiotics are not usually required for this type of clean injury unless pre-existing sinusitis is identified. In both cases, direct orbital decompression was performed with excellent results after identification of marked unilateral tense exophthalmos, delayed pupillary reactions to light and ophthalmopegia.     

Growing skull fracture of the orbital roof. Case report

Growing skull fractures are rare complications of head trauma and very rarely arise in the skull base. The clinical and radiological finding and treatment of a growing fracture of the orbital roof in a 5-year-old boy are reported, and the relevant literature is reviewed. The clinical picture was eyelid swelling. Computed tomography (CT) scan was excellent for demonstrating the bony defect in the orbital roof. Frontobasal brain injury seems to play an important role in the pathogenesis of the fracture growth. Growing skull fracture of the orbital roof should be considered in the differential diagnosis in cases of persistent ocular symptoms. Craniotomy with excision of gliotic brain and granulation tissue, dural repair and cranioplasty is the treatment of choice.