MyMedR

Skeletal expansion via craniofacial distraction osteogenesis technique in syndromic craniosynostosis: impact on ophthalmic parameters

Kai LC, Khaliddin N, Hassan MK, Hariri F

Int Ophthalmol, 2024 Mar 19;44(1):147.

PMID: 38499845 DOI: 10.1007/s10792-024-03084-y

BACKGROUND: This study aims to compare the changes in ophthalmic parameters among syndromic craniosynostosis patients who underwent craniofacial skeletal expansion procedures via distraction osteogenesis (DO).

METHOD: A retrospective study was conducted involving syndromic craniosynostosis patients who underwent surgical expansion via the DO technique from the year 2012 to March 2022. Changes in six parameters which consist of visual acuity, refractive error, optic disc health, intraocular pressure, degree of proptosis and orbital volume were measured objectively pre and post-surgery. For categorical parameters, the Chi-square cross-tab test was done. Paired sample T-test was used for normally distributed variables. Wilcoxon signed-rank test was used for non-normally distributed data.

RESULTS: Visual impairment was present in 21.4% of eyes before surgery and increased to 28.5% post-surgery. Three patients had changes of refractive error post-surgery with one developed hypermetropia, another developed anisometropia and the last had improvement to no refractive error. Two patients had optic disc swelling which was resolved post-surgery. Intraocular pressure changes were inconsistent post-surgery. All patients achieved a significant reduction in the degree of proptosis post-surgery. Orbital volume calculation using computed tomography (CT) scans shows a significant increase in volume post-surgery for all patients.

CONCLUSION: Our study shows a significant increase in orbital volume post-surgery with a reduction in the degree of proptosis. Optic disc and nerve health improved after the surgery. Changes in terms of visual acuity, refractive error and IOP were inconsistent after the surgical intervention.

Matched MeSH terms: Exophthalmos*

Fulltext Adult embryonal rhabdomyosarcoma of the ethmoid: a rare entity

Rohaizam J, Doris EJ, Tang PI, Lee SC, Uchang J

Med J Malaysia, 2010 Jun;65(2):160-1.

PMID: 23756807 MyJurnal

Embryonal rhabdomyosarcoma is an exceedingly rare tumor in adult. We report an embryonal rhabdomyosarcoma of the ethmoid in a 59-year-old Iban lady who presented with proptosis and complete ptosis of her left eye for two months. Imaging investigations showed left ethmoidal and left orbital soft tissue mass with extradura and dura involvement. The patient was planned for chemotherapy. Unfortunately, in such an advanced disease, she succumbed before treatment.

Matched MeSH terms: Exophthalmos*

Spontaneous orbital haematoma in a scurvy child: A forgotten diagnosis

Cheah SC, Tang IP, Matthew TJH, Ooi MH, Husain S

Int J Pediatr Otorhinolaryngol, 2020 Oct;137:110224.

PMID: 32896344 DOI: 10.1016/j.ijporl.2020.110224

Spontaneous unilateral orbital haematoma in children is not common and very rarely caused by scurvy. Scurvy is a clinical syndrome with a spectrum of clinical manifestations due to severe prolonged vitamin C deficiency leading to impairment of collagen synthesis over skin, bone, teeth and blood vessels. This paper presents a unique case of a 7- year-old girl with learning difficulty who presented with spontaneous right proptosis due to scurvy. Imaging studies suggestive of intra- orbital extraconal haemorrhage. The child was treated with a higher than recommended dose of vitamin C initially in emergency situation. She responded well and discharged without complication. Spontaneous orbital haematoma due to scurvy is very rare with less than 10 cases published in literature. The present case should raise the awareness regarding this forgotten disease and importance of balance nutrition amongst children.

Matched MeSH terms: Exophthalmos/diagnosis; Exophthalmos/etiology*

A Simple Bone Cyst Requiring Exenteration: A Case Report With Literature Review

Yi Wen L, Singh S, Mohamad NFB, Toh YF, Khalil AAK

Ophthalmic Plast Reconstr Surg, 2020 5 20;37(1):e23-e25.

PMID: 32427737 DOI: 10.1097/IOP.0000000000001713

Simple bone cyst is a benign osteolytic lesion, found primarily in the mandible, occasionally in the maxilla, and rarely in the zygoma. A 17-year-old male patient with 6-month history of left facial mass presented with worsening proptosis and displacement of left eyeball associated with reduced OS vision. The lesion was painless to start with, but with time, he reported OS pain. There was no history of any trauma. CT scan and MRI of orbit performed revealed cystic bony lesion involving greater wing of sphenoid. He subsequently had excision of the left facial mass with exenteration of OS due to poor visual prognosis, followed by reconstruction with osteomyocutaneous flap from left scapular region. The histopathologic features were consistent with a diagnosis of simple bone cyst of zygomatic arch.

Matched MeSH terms: Exophthalmos*

Exophthalmos in ritual sculptures in the Sunda Isles

Ber A

Int Surg, 1970 Apr;53(4):279-85.

PMID: 4907253

Matched MeSH terms: Exophthalmos/history*

Fulltext Post-traumatic Carotico-cavernous fistula: A report of two cases

Primuharsa Putra. S.H.A., Nurliza, I., Roszalina, R., Saim, L., Zahiah, M.

International Medical Journal Malaysia, 2007;6(2):1-8.

MyJurnal

We present two cases of direct traumatic carotico-cavernous fistula following motor vehicle accident. Their main symptoms include loss of vision, progressive proptosis and recurrent epistaxis. Angiogram confirmed presence of carotico-cavernous fistula in both cases. One was successfully treated by open surgery and the other by embolization with Guglielmi detachable coils (GDC). In both cases, proptosis and epistaxis resolved completely.

Matched MeSH terms: Exophthalmos

Meningioma Mimicking Fibrous Dysplasia

Kamaluddin NA, Samsudin AHZ, Wan Hitam WH, Ibrahim M

Cureus, 2019 Sep 27;11(9):e5782.

PMID: 31723541 DOI: 10.7759/cureus.5782

Despite being among the common primary intracranial tumors, intraosseous craniofacial meningioma is the least common subtype of meningioma accounting for only 1-2% of intracranial meningiomas. Interestingly, it can display clinical and radiologic features that can be confused for fibrous dysplasia. Scan imaging and biopsy are crucial for the diagnosis as well as for further proper treatment. We report a case of unilateral eye proptosis and optic neuropathy which was initially thought for fibrous dysplasia. Later the histopathology revealed meningioma grade 1. As the clinical presentations are almost undifferentiated, diagnosis and further prompt treatment are challenging.

Matched MeSH terms: Exophthalmos

Distraction osteogenesis in the surgical management of syndromic craniosynostosis: a comprehensive review of published papers

Al-Namnam NMN, Hariri F, Rahman ZAA

Br J Oral Maxillofac Surg, 2018 06;56(5):353-366.

PMID: 29661509 DOI: 10.1016/j.bjoms.2018.03.002

Our aim was to summarise current published evidence about the prognosis of various techniques of craniofacial distraction osteogenesis, particularly its indications, protocols, and complications. Published papers were acquired from online sources using the keywords "distraction osteogenesis", "Le Fort III", "monobloc", and "syndromic craniosynostosis" in combination with other keywords, such as "craniofacial deformity" and "midface". The search was confined to publications in English, and we followed the guidelines of the PRISMA statement. We found that deformity of the skull resulted mainly from Crouzon syndrome. Recently craniofacial distraction has been achieved by monobloc distraction osteogenesis using an external distraction device during childhood, while Le Fort III distraction osteogenesis was used in maturity. Craniofacial distraction was indicated primarily to correct increased intracranial pressure, exorbitism, and obstructive sleep apnoea in childhood, while midface hypoplasia was the main indication in maturity. Overall the most commonly reported complications were minor inflammatory reactions around the pins, and anticlockwise rotation when using external distraction systems. The mean amount of bony advancement was 12.3mm for an external device, 18.6mm for an internal device and 18.7mm when both external and internal devices were used. Treatment by craniofacial distraction must be validated by long-term studies as there adequate data are lacking, particularly about structural relapse and the assessment of function.

Matched MeSH terms: Exophthalmos/etiology; Exophthalmos/surgery

Fulltext A rare case of recurrent orbital solitary fibrous tumour with intracranial extension

Ainal Adlin N, Umi Kalthum MN, Amizatul Aini S, Reena Rahayu MZ

Journal of Surgical Academia, 2016;6(1):59-61.

MyJurnal

A 47-year-old lady, presented with progressive proptosis of left eye with deterioration of vision. She had a history of left solitary fibrous tumour and had undergone left frontal craniotomy and orbitotomy in 2004. Surveillance Magnetic resonance imaging (MRI) six years later showed tumour recurrence with intracranial extension. However, she did not follow-up and only presented again 3 years, later. Tumour resection and left exenteration was performed. Histology showed ‘patternless’ pattern of neoplastic cells, and CD34 staining was diffusely positive. Diagnosis of recurrent solitary fibrous tumour with intracranial extension was made.

Matched MeSH terms: Exophthalmos

Fulltext Managing Subacute Subperiosteal Hematoma with High Intraocular Pressure: A case report

Hashim SE, Fatisha A, Nazri MN

International Medical Journal Malaysia, 2017;16(1):135-138.

MyJurnal

Subperiosteal haematoma of the orbit is an uncommon complication of maxillofacial trauma, hence easily missed. It usually presents subacutely with proptosis and diplopia. In our case, the subperiosteal haematoma is complicated with high intraocular pressure, necessisating measures to reduce the intraocular pressure. Unresponsive to only medical treatment, surgical evacuation was carried out in this patient. Removal of the clot finally relieved the intraocular pressure and simultaneously improved the proptosis and the cumbersome diplopia.

Matched MeSH terms: Exophthalmos

Fulltext Proptosis presenting as a delayed sign of frontal extradural haematoma

Saiful MN, Azmi A, Saffari MH

Med J Malaysia, 2007 Jun;62(2):156-7.

PMID: 18705451 MyJurnal

We report a case of a young man who presented with proptosis as a delayed manifestation of a frontal extradural haematoma (EDH) following a minor head injury. A computed tomography (CT) of the brain done 72 hours after trauma revealed a large extradural haematoma in the right anterior cranial fossa with orbital roof fracture and subperiosteal clot extension into the orbital cavity. Right frontal craniotomy with evacuation of haematoma was done and the proptosis completely resolved after surgery. The clinical course, possible mechanism and management of the patient are discussed.

Matched MeSH terms: Exophthalmos/etiology*

Proptosis--Correlation and Agreement between Hertel Exophthalmometry and Computed Tomography

Ramli N, Kala S, Samsudin A, Rahmat K, Abidin ZZ

Orbit, 2015;34(5):257-62.

PMID: 26186249 DOI: 10.3109/01676830.2015.1057291

To determine the correlation and agreement between Hertel exophthalmometry and computed tomography (CT) of the orbits in measuring proptosis.

Matched MeSH terms: Exophthalmos/diagnosis*

Metastatic osteosarcoma of the ethmoid: an unusual cause of recurrent epistaxis and proptosis

Sharma HS, Reddy SC, Mohamad A, Kamal MZ, Halder D

J Laryngol Otol, 1996 Jul;110(7):676-8.

PMID: 8759546

Osteosarcoma is the most common primary malignant tumour of bone and it usually metastasises to the lung. In the nasal cavity metastatic disease is extremely rare. We describe a case of osteosarcoma presenting with recurrent epistaxis, and proptosis due to secondaries in the nasal cavity. To our knowledge such a case has not been reported previously in the available English literature.

Matched MeSH terms: Exophthalmos/etiology

Fulltext Proptosis: a rare presentation of metastatic renal cell carcinoma

Ho CC, Krishna KK, Praveen S, Goh EH, Lee BC, Zulkifli MZ

Med J Malaysia, 2010 Sep;65(3):229-30.

PMID: 21939176

We present a case of a middle-aged man who was incidentally found to have right renal solid mass while investigating for his left eye proptosis. Computerised tomography (CT) scan confirmed the diagnosis of renal cell carcinoma and the tumour was successfully excised via open surgery. The histopathology examination revealed the 10x7x8 cm mass to be a clear cell type renal cell carcinoma. The rare presentation of this metastatic renal cell carcinoma, its diagnosis and management will be discussed.

Matched MeSH terms: Exophthalmos/diagnosis*; Exophthalmos/etiology

A prospective study of ocular manifestations in childhood acute leukaemia

Reddy SC, Menon BS

Acta Ophthalmol Scand, 1998 Dec;76(6):700-3.

PMID: 9881556

PURPOSE: To determine the prevalence of ocular manifestations in childhood acute leukaemia at the time of presentation.

METHODS: Eighty-two children with acute leukaemia were examined for ocular lesions within two days of diagnosis before starting chemotherapy. The detailed ocular examination of both eyes was carried out by the ophthalmologist irrespective of the presence or absence of eye symptoms in all cases.

RESULTS: Only 3 out of 82 children presented with eye symptoms (3.6%). However, ocular changes were found in 14 children (17%); ten with lymphoblastic and four with myeloid leukaemia. The ocular lesions observed were proptosis, intraretinal haemorrhages, white centered haemorrhages, cotton wool spots, macular haemorrhage, subhyaloid haemorrhage, vitreous haemorrhage, papilloedema, cortical blindness, sixth nerve palsy, and exudative retinal detachment with choroidal infiltration.

CONCLUSION: In view of the high prevalence of asymptomatic ocular lesions in childhood acute leukaemia, routine ophthalmic examination should be included as a part of evaluation at the time of diagnosis.

Matched MeSH terms: Exophthalmos/diagnosis; Exophthalmos/etiology

Fulltext Diagnostic dilemma of dural carotid cavernous fistula

Noorlaila B., Zunaina E., Raja Norliza R.O., Nor Fadzillah A.J., Alice, G.K.C.

Journal of Biomedical and Clinical Sciences, 2017;2(1):38-41.

MyJurnal

We report a case of dural carotid cavernous fistula (CCF) pose a diagnostic dilemma with initial symptoms of the arteriovenous shunt. A 56 year-old man presented with right eye diplopia, thensubsequently developed ptosis, congestion of conjunctiva, dilated episcleral vessels, and gradual rise in intraocular pressure. Initial diagnosis of pseudotumour was made based on negative finding of CCF by computed tomography angiography (CTA). In view of persistent clinical manifestations in spite of steroid therapy, and with the presence of new ocular signs; cock-screw conjunctival vessels, dilated retinal veins, and proptosis, digital subtraction angiography (DSA) was performed and confirmed the diagnosis of dural CCF.The ocular symptoms resolved completely post embolization of the fistula.

Matched MeSH terms: Exophthalmos

Fulltext Importance of clinical and morphological correlations in diagnosing Langerhans cell histiocytosis

Seb, Omayma, Fauzana, K., Aisyah, M.R., Noraidah, M., Noor Hamidah, H.

Medicine & Health, 2018;13(1):220-226.

MyJurnal

Langerhans cell histiocytosis (LCH) is a clonal histiocytic disorder. The variable clinical manifestations from isolated bone lesion to multisystem disease can cause difficulties and delay in diagnosis. We report a 2 years and 8 months-old girl who presented with a 2 weeks history of persistent fever and weight loss associated with progressive abdominal distension. Physical examination revealed pallor, bilateral proptosis, seaborrheic dermatitis over the scalp and hepatosplenomegaly. Skull X-ray demonstrated multiple lytic lesions at the base and the skull vault. Bone marrow morphology showed numerous abnormal Langerhans cells (LCs) and foamy macrophages. The trephine immunohistochemistry (IHC) stains for CD1a, S-100 and CD68 were inconclusive. The diagnosis of multisystem Langerhans cell histiocytosis (MS-LCH) in this patient was based on the clinical presentation, radiological and morphological analysis. She subsequently received chemotherapy and currently she is on maintenance therapy with a good clinical response. LCH is a rare disease and although the IHC was inconclusive, the correlation of clinical, radiological and morphological data are essential for the diagnosis.

Matched MeSH terms: Exophthalmos

Fulltext Orbital rhabdomyosarcoma: a case series

Chen, C.Y., Nor Hidayah, A.B., Adil, H.

Malaysian Journal of Medicine and Health Sciences, 2013;9(2):69-72.

MyJurnal

In this paper, we report three cases of orbital rhabdomyosarcoma in different age groups with different histopathologic types. Case 1 is a 10-year old Malay boy who presented with painless proptosis of the left eye. Magnetic resonance imaging (MRI) showed soft tissue mass arising from the lateral rectus muscle. Tissue biopsy revealed rhabdomyosarcoma of embryonal type. Case 2 is a 32-year old Malay man who presented with a progressive left eye proptosis associated with pain and redness. Computed Tomography (CT) scan showed a retrobulbar mass extending medially in the left orbit. Meanwhile, biopsy showed rhabdomyosarcoma of pleomorphic type. Case 3 is a 67-year old Malay woman who presented with proptosis and fungating growth of left orbital region, epistaxis and nasal blockage. CT scan revealed an ill-defined mass filling up the left orbital cavity. Biopsy showed rhabdomyosarcoma of alveolar type. Rhabdomyosarcoma is the most common in childhood but it should be considered as a differential diagnosis of orbital tumours irrespective of age.

Matched MeSH terms: Exophthalmos

Spontaneous retrobulbar haemorrhage secondary to orbital varices of inferior ophthalmic vein-a case report

Haizul, I.M., Umi Kalthum, M.N., Mae-Lynn, C.B., Faridah, H.A.

Journal of Surgical Academia, 2012;2(2):49-51.

MyJurnal

A 50-year-old Chinese man presented with sudden onset of painful right eye, diplopia, and redness associated with headache and deteriorating vision. Examination revealed obvious proptosis with elevated intraocular pressure. Computed tomography (CT) scan showed presence of retrobulbar haemmorhage. Emergency lateral canthotomy and cantholysis was performed followed by medical orbital decompression, resulting in improvements in visual acuity, and other ocular symptoms. The diagnosis of thrombosed orbital varices involving inferior ophthalmic vein was confirmed on radiological- angiographic study. To date, he is symptoms-free with good visual acuity. Immediate surgical decompression with lateral cantholysis for retrobulbar haemorrhage was effective in the treatment of retrobulbar haemorrhage.

Matched MeSH terms: Exophthalmos

Crouzon Syndrome: A Case Series of Craniomaxillofacial Distraction Osteogenesis for Functional Rehabilitation

Hariri F, Abdul Rahman ZA, Bahuri NFA, Azmi MN, Abdullah NA, Ganesan D

J Oral Maxillofac Surg, 2018 03;76(3):646.e1-646.e12.

PMID: 29268076 DOI: 10.1016/j.joms.2017.11.029

Crouzon syndrome (CS) is the most common craniosynostosis syndrome and requires a comprehensive management strategy for the optimization of care and functional rehabilitation. This report presents a case series of 6 pediatric patients diagnosed with CS who were treated with distraction osteogenesis (DO) to treat serious functional issues involving severe orbital proptosis, an obstructed nasopharyngeal airway, and increased intracranial pressure (ICP). Three boy and 3 girls were 8 months to 6 years old at the time of the operation. The mean skeletal advancement was 16.1 mm (range, 10 to 27 mm) with a mean follow-up of 31.7 months (range, 13 to 48 months). Reasonable and successful outcomes were achieved in most patients as evidenced by adequate eye protection, absence of signs and symptoms of increased ICP, and tracheostomy tube decannulation except in 1 patient. Complications were difficult fixation of external stabilizing pins in the distraction device (n = 1) and related to surgery (n = 4). Although DO can be considered very technical and can have potentially serious complications, the technique produces favorable functional and clinical outcomes in treating severe CS.

Matched MeSH terms: Exophthalmos

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