Integration of hospital and community care for paediatric ophthalmology: A mixed-methods study.

BACKGROUND: To explore collaborative care models for paediatric eye care that integrate hospital and community-based care to address access blocks. METHODS: Sequential referrals to a tertiary paediatric ophthalmology clinic between April and October 2019 and subsequent encounters up until July 2020 at a major metropolitan public children's hospital in Sydney, Australia, were reviewed to identify those cases suitable for community care. Semi-structured phone interviews were conducted with eye health service providers, including ophthalmologists, orthoptists and optometrists, as well as service users to explore their perspectives on potential changes to service delivery. Qualitative data were analysed deductively using the Levesque model for access to healthcare and Consolidated Framework for Implementation Research (CFIR) to inform implementation strategies for future models of care. RESULTS: One-third of the 439 audited referrals (30.5%; 134/439) were identified as suitable for community management. Interviews revealed five themes relating to potential models of care, which would support and promote access: integrated health systems, standardised quality of care, interprofessional trust, multidisciplinary governance and patient-centred care. Key recommendations for future implementation included: (i) identifying and preparing clinical champions, (ii) conducting educational meetings, (iii) conducting local needs assessments and (iv) informing local opinion leaders. CONCLUSIONS: This audit highlights access blocks and poor targeting of referrals to tertiary paediatric ophthalmology services in a metropolitan hospital. Integration with community practitioners was identified as an acceptable way to streamline services, and strategies that may support successful implementation in this setting were identified.

Role of Optic Nerve Sheath Fenestration in Inflammatory Pachymeningitis Causing Visual Impairment.

Eye disease due to pachymeningitis caused by immunoglobulin G4-related disease (IgG4-RD) is a rare occurrence. Here, the authors report a unique case of a patient presenting with visual loss from raised intracerebral pressure from pachymeningitis most likely related to IgG4-RD. The patient was treated with acetazolamide and steroids, and an optic nerve sheath fenestration was performed to successfully save the patients vision.

A Case of Acute Myeloid Leukaemia Presenting as Subacute Intracranial Hypertension in a Young Adult.

We report a case of an 18-year-old woman presenting with headache, papilloedema, and cerebrospinal fluid (CSF) pleocytosis. She was subsequently diagnosed with acute myeloid leukaemia, which is to date the only reported case manifesting as central nervous system-localised disease in an adult. The intracranial hypertension was treated successfully with chemotherapy, acetazolamide, and CSF drainage, with no permanent visual impairment. The mechanism by which haematological malignancy causes intracranial hypertension is not fully elucidated, but we hypothesise that in our case, blast infiltration interfered with CSF reabsorption at the arachnoid granulations.

A Case Report of Appendicitis Causing Cecal Volvulus: A Rare Occurrence.

This case report presents a fascinating scenario involving a 60-year-old female who was diagnosed with cecal volvulus secondary to appendicitis. The patient's initial presentation included a three-day history of periumbilical pain accompanied by reduced oral intake and an inability to pass stool. Through a systematic approach involving detailed history-taking, comprehensive physical examinations, and pertinent imaging studies, a precise diagnosis of cecal volvulus induced by appendicitis was established. Subsequently, the patient underwent a timely operation, leading to a successful resolution of her condition and a remarkably swift recovery post-surgery. This unique case prompts a deeper exploration into the incidence and management of this rare phenomenon, where the seemingly unrelated condition of appendicitis precipitated a cecal volvulus. Given the unusual nature of this presentation, it underscores the importance of considering atypical etiologies in patients presenting with signs and symptoms of bowel obstruction. This discussion aims to shed light on the diagnostic challenges, treatment strategies, and outcomes associated with this intriguing interplay of pathologies, offering valuable insights for clinicians encountering similar cases in their practice.

Pancreatic heterotopia of the gallbladder - possible cause of right upper quadrant pain, a case report.

Pancreatic heterotopia is characterized by ectopic pancreatic tissue found outside the pancreas without any anatomical or vascular connection to the pancreas. Pancreatic heterotopia of the gallbladder is a rare histological finding; there have been only a handful of cases described in the literature. Pancreatic heterotopia of the gallbladder is usually diagnosed incidentally at histological examination following cholecystectomy or autopsy. Clinical presentation of pancreatic heterotopia of the gallbladder can vary from biliary colic, biliary obstruction or it can be completely asymptomatic. It has been suggested that gallbladder pancreatic heterotopia may lead to pancreatitis of this ectopic tissue, which may present differently to typical biliary colic. Here, we present the case of a 43-year-old male that presented with 2 years of significant postprandial nausea and right upper quadrant pain. Histopathology following cholecystectomy revealed chronic cholecystitis with cholelithiasis, in addition to a focus of pancreatic heterotopia in the gallbladder wall.

Perforated Meckel's diverticulitis secondary to a corn kernel obstruction of lumen in an elderly patient.

Meckel's diverticulum (MD) is the most common congenital malformation of the gastrointestinal tract. The most frequent complications are bleeding, intestinal obstruction, intussusception and neoplasm. Perforation because of diverticulitis is very rare and usually associated to foreign bodies. We report a case of an elderly man who presented with right iliac fossa pain, raised inflammatory markers and a computer tomography (CT) scan suggestive of Meckel's diverticulitis. He underwent a laparoscopic converted to open small bowel resection and primary anastomosis for perforated MD, which revealed a corn kernel at its base in the absence of ectopic gastric or pancreatic tissue. Symptomatic and perforated MD secondary to a phytobezoar is extremely rare in elderly patients, hence the importance of clinical suspicion in patients with abdominal pain, independent from their age, to avoid delay in diagnosis, which can lead to increased morbidity and mortality in this demographic.

Diagnostic Difficulties and Complexities in the Management of Dermatofibrosarcoma Protuberans in the Breast: A Case Report and Review of the Literature.

Dermatofibrosarcoma protuberans (DFSP) is an extremely rare sarcoma with an incidence between 0.8-5.0 cases per one million persons per year. DFSP accounts for less than 0.1% of all malignancies and approximately 1-6% of all soft tissue sarcomas. Only a few cases of DFSP have been found within the breast tissue. We report a case of DFSP in a 30-year-old female within the left breast. The sarcoma presented as a painless, rubbery, mobile lump that gradually increased in size. It was initially identified on an ultrasound scan and subsequently confirmed with MRI and core biopsy. DFSP is a rare condition and treatment guidelines are not well established. The current recommendation is for surgical excision with 2-cm margins. Mastectomy may be considered in some circumstances. In our case, the aim was for surgical resection with 2-cm margins at both breast tissue and skin, but insufficient margins were taken. The recommendation for re-excision of the inadequate margins was declined. It is uncertain what the implications of this are given the lack of research on DFSP in the breast. Therefore, close surgical surveillance will be imperative. We present this case to highlight the difficulties associated with the diagnosis, treatment, and management of DFSP due to the lack of literature on this disease.

Cutaneous metastases as a presenting sign of metastatic NSCLC.

Cutaneous metastases are rare and often late signs of an internal malignancy; however, this can be the presenting sign in very rare cases. These typically have a characteristic appearance and distribution based on the underlying malignancy but can be difficult to distinguish from other malignant or benign dermatoses. We report a case of a 69-year-old female who presented for elective excision of two cutaneous, fast-growing lesions on the chest wall. The patient's past medical history is significant for a 45-pack-year smoking history. Pre-operative chest radiograph revealed a right upper lobe mass, with the lesions ultimately representing cutaneous metastases of a non-small-cell lung cancer. Although cutaneous metastases have been reported, difficulty remains in distinguishing other causes of cutaneous lesions from cutaneous metastases in the predominantly asymptomatic patient.

Intra-abdominal tuberculosis masquerading as ovarian carcinoma.

Intra-abdominal tuberculosis (TB) is rare in Australia, but it can be found in those who emigrate from endemic areas. We report a rare case of a 28-year-old lady from the Philippines who presented with abdominal pain, ascites and an elevated cancer antigen (CA) 125 with an initial concern of ovarian malignancy. She underwent a diagnostic laparoscopy which revealed typical features of intra-abdominal TB and histological features of granulomatous inflammation. The symptoms and signs of intra-abdominal TB are non-specific and can mimic many other conditions. The gold-standard mode of diagnosis in intra-abdominal TB is laparoscopy with tissue biopsy. Ovarian malignancy is relatively rare in pre-menopausal women; hence an elevated CA 125 warrants a broader differential diagnosis. It is important to have intra-abdominal TB as a differential even in the non-endemic settings to avoid delay in diagnosis and appropriate management.

Visual manifestations of craniofrontonasal dysplasia.

In this sample of craniofrontonasal dysplasia, a 44.4% prevalence of visual impairment was observed, with more than half being due to potentially correctable causes of visual loss, including amblyopia and anisometropia. High prevalences of strabismus (88.9%) and V-pattern (55.5%) in craniofrontonasal dysplasia were also demonstrated. All three patients who underwent strabismus surgery showed improvement in ocular alignment postoperatively. This group needs regular eye examinations to assess for visual impairment and provide timely intervention for modifiable causes of visual loss.

Surgical Technique for Pulled in Two Syndrome: Three Cases With Chronic Progressive External Ophthalmoplegia.

The authors describe three examples of "pulled in two syndrome" (PITS) from a series of 13 patients undergoing strabismus surgery with underlying chronic progressive external ophthalmoplegia (CPEO) and illustrate techniques for recovery of the "pulled in two" extraocular muscle should the complication arise. In all cases, a rectus muscle snapped under minimal tension while held on a strabismus hook during strabismus surgery. Two patients suffered from CPEO as a result of genetic mitochondrial disease, whereas one resulted from presumed mitochondrial toxicity induced by HAART. In cases 1 and 3, the proximal medial rectus segment was retrieved and reattached. In case 2, the fragmented superior rectus muscle was too friable to be reattached. All three patients were satisfied with the outcome, having reduced their angles of misalignment postoperatively. All three had improved cosmesis, and the two who had complained of diplopia preoperatively found their diplopia to be eliminated or improved. With anticipation of muscle friability in patients with previous extraocular surgery or degenerative muscle changes such as CPEO, the likelihood of the complication arising may be reduced. Should it occur, the loss of a snapped rectus muscle may be avoided through careful manipulation of the globe. [J Pediatr Ophthalmol Strabismus. 2017;54:e83-e87.].

Ocular overelevation in adduction in craniosynostosis: is it the result of excyclorotation of the extraocular muscles?

PURPOSE: We sought to determine whether radiological evidence supports excyclorotation of the extraocular muscle cone as a cause of overelevation in adduction in children with craniosynostosis. METHODS: This was a retrospective case series of 40 patients with craniosynostosis. Ophthalmic findings were assessed for incomitant vertical strabismus in particular excessive elevation in adduction. Computed tomography and magnetic resonance imaging scans were reviewed. Those scans with adequate coronal imaging of their orbits to assess the position and angulation of the horizontal extraocular muscles were identified, and the degree of rotation of the muscles formally measured along with aged matched controls. These groups were analyzed for the association between presence of overelevation in adduction and degree of excyclorotation of the extraocular muscle cone. RESULTS: The identified updrift on adduction that mimics inferior oblique muscle overaction was present in 63% (25/40) of patients. Imaging that permitted accurate measurement of the muscles positions was available in 10 of the 40 patients. Of these 10, 8 had the updrift, and 7 of these 8 (88%) demonstrated more excyclorotation than their aged matched controls. Comparison of scan measurements of patients revealed a significant difference in degree of excyclorotation (mean difference = 16.2, 95% confidence interval 6.2-31.5; P = 0.006) between patients with and without excess elevation in adduction. CONCLUSION: Overelevation in adduction is significantly associated with excyclorotation of the extraocular muscle cone in children with craniosynostosis. We demonstrate a simple method to assess for the excyclorotation. Our findings support the importance of imaging of the orbits in these children before strabismus surgery.

Terson syndrome with ipsilateral severe hemorrhagic retinopathy in a 7-month-old child.

In infants with intracranial hemorrhage, the most common cause of intraocular hemorrhages is abusive head trauma. Terson syndrome is rare in infants, and the retinal findings, although not well reported in the literature, are generally limited to the posterior pole. We report a case of a 7-month-old boy who developed ipsilateral, extensive preretinal and intraretinal hemorrhage after subarachnoid hemorrhage from a ruptured intracranial aneurysm.

Prevalence and causes of visual impairment in craniosynostotic syndromes.

BACKGROUND: To assess the prevalence and causes of visual impairment in patients with craniosynostotic syndromes of Apert, Crouzon, Pfeiffer, Saethre-Chotzen and craniofrontonasal dysplasia. METHODS: The medical records of patients who attended the Craniofacial Clinic at two large paediatric hospitals in Sydney, Australia between 1983 and 2004 were retrospectively reviewed. Presenting visual acuity (VA) was assessed using tests appropriate to age and cognition: 'fix and follow' in infants (<18 months old), Teller card acuity in preverbal children (18 months to less than 3 years old), Kay picture test or Sheridan-Gardiner test in children aged between 3 and less than 6 years and Snellen chart in those aged 6 years or older. Visual impairment was defined as the inability to fix and follow or presenting VA < 6/12 in the better eye. Amblyopia was defined as a two-line difference in VA between both eyes in the absence of an organic eye disease. RESULTS: Sixty-three patients with craniosynostotic syndromes were identified, of whom 55 had VA assessed at the first visit. Of these 55, 19 (35.5%) had bilateral visual impairment and 5 (9.1%) had unilateral visual impairment. Causes of visual impairment include amblyopia (16.7%), ametropia (25%), optic atrophy (16.7%) and exposure keratopathy (4.2%). Risk factors for amblyopia include strabismus (43.3%), astigmatism (> or =1.5 dioptres) (39.5%), hypermetropia (18.4%) and anisometropia (> or =1.5 dioptre difference between both eyes) (15.8%). Six of the 63 patients (9.5%) had papilloedema; those who were followed up showed gradual resolution of papilloedema following timely decompressive surgery. CONCLUSIONS: A high prevalence of visual impairment in patients with craniosynostotic syndromes was found, almost half of them due to potentially correctable causes, including amblyopia and ametropia. Optic atrophy remains an important cause of visual impairment. Further studies are needed to assess the timing and efficacy of intervention for modifiable causes of visual loss in craniosynostotic syndromes.

Orbital apex syndrome secondary to Pseudallescheria boydii fungal sinusitis in an immunocompetent patient.

Orbital apex syndrome secondary to mucormycosis in immuno-compromised patients is well described; however, few reports exist of a paranasal sinus mycetoma resulting in this presentation in the immuno-competent patient. The case is reported of a 92-year-old man who developed orbital apex syndrome secondary to a sphenoidal sinus mycetoma of Pseudallescheria boydii.