Qualitative ultrasound training: defining the learning curve.

AIM: To assess the developing competence of two trainees learning groin and shoulder ultrasound when compared to an expert practitioner. MATERIALS AND METHODS: Specific pro formas were used to record ultrasound trainee performance in each scan region and their diagnosis was compared to the opinion of the expert. The data derived were reviewed using kappa analysis and training end points were defined as a minimum 80% trainee agreement with the expert. Retrospectively, cumulative sum analysis was applied to the data to assess case-by-case performance. RESULTS: For groin hernias, reporting an average of 70 examinations was required to become competent and inguinal hernias required higher numbers of examinations than femoral hernias. For shoulders, an average of 80 examinations was required and the supraspinatus and infraspinatus tendons proved the most challenging structures. CONCLUSIONS: Kappa analysis demonstrated a differential in the learning curves for individual structures within each examination region. Sequential kappa scores are consistent with a sigmoid learning curve. The numbers required to achieve satisfactory agreement are suggested as required minima for ultrasound training curricula. Cumulative sum analysis provided a sensitive indicator of trainee performance, quickly highlighting individual learning difficulties when they arose. Its prospective use can ensure extra training support is instigated quickly and appropriately.

Changing trends in postoperative cataract care: impact of electronic patient records in optometrist-delivered shared care.

BACKGROUND: This study evaluates a community optometrist-delivered postoperative care scheme in patients discharged from the hospital ophthalmology department following uncomplicated cataract surgery. AIM: The aim of this study is to assess the efficacy of electronic patient records (EPR) in facilitating co-managed cataract care. METHODS: We performed a retrospective analysis of a prospectively maintained Medisoft EPR database of postoperative cataract review data at a single centre, Sligo University Hospital (SUH), which serves a large and predominantly rural catchment area. All patients undergoing cataract surgery at SUH from October 2012 to September 2013 were included in this study. A total of 39 optometric practices, all with access to the Medisoft EPR software, participated in this pilot co-management scheme. RESULTS: One thousand four hundred and twenty-two cataract surgeries were performed in SUH (55% female, 45% male); 1011 patients (71%) were discharged to the community on the day of cataract surgery. Complete postoperative feedback (i.e. data on refraction, visual acuity and intraocular pressure) was available in 97% of these patients compared to 50% of patients reviewed in the hospital. Patients followed up by optometrists were twice as likely to have complete postoperative clinical details (RR = 1.934, 95% CI: 1.759-2.126, p < 0.0001). Overall, 65% of operations were performed on first eyes. Hospital doctors were more likely to document requirement for second eye surgery compared to community optometrists (RR = 1.434, 95% CI: 1.302-1.580, p < 0.0001). CONCLUSIONS: Optometrists provided an excellent postoperative care service with superior postoperative feedback rates compared to hospital doctors. EPRs facilitate a postoperative shared-care pathway that is of high quality and efficiency with major economic advantages.

Benign hidradenoma: magnetic resonance and ultrasound features of two cases.

Tumors of sweat gland origin are rare in the hand but should be considered in the differential diagnosis when certain imaging features are present. We present a case of nodular hidradenoma of the hand, with previously unreported magnetic resonance imaging features, and a further case in the thigh, both with ultrasound and histopathological correlation. The imaging literature of this tumor is reviewed, and its significance is discussed with respect to the current understanding of its malignant potential.

Posterior dislocation of the long head of biceps tendon: case report and review of the literature.

Posterior or lateral dislocation of the long head of biceps is a rare complication of shoulder dislocation that can result in inability to relocate the humerus. The diagnosis should be suspected when certain radiographic features are present at the initial presentation. Other imaging modalities can aid diagnosis when clinical management is unsuccessful or protracted. We present a case of surgically proven posterior dislocation of the biceps tendon with conventional radiographic, computed tomography and magnetic resonance imaging assessment. The literature on this subject is reviewed, and imaging features associated with the diagnosis are described.

Appraisal of compliance with the UICC/AJCC staging system in the staging of gastric cancer. Union Internacional Contra la Cancrum/American Joint Committee on Cancer.

BACKGROUND: In the surgical management of gastric carcinoma, regional lymphatic spread is of prognostic importance. The fifth edition of the Union Internacional Contra la Cancrum classification has been shown to be reproducible, practical and of significant prognostic use. The tumour node metastasis (TNM) system requires at least 15 lymph nodes to be acquired and examined for staging to be accurate. This has raised concern over the consistency with which the requisite numbers of nodes would be acquired. This study was performed to assess how consistently surgically managed cases of gastric cancer in the West Midlands fulfilled this requirement to allow accurate staging. METHODS: Data from the West Midlands Cancer Intelligence Unit on all cases of gastric cancer registered from 1998 to 1999 were obtained and the number of lymph nodes documented for each surgically managed case was assessed. RESULTS: Overall, only 31.0 per cent of surgically resected cases could be assessed accurately according to the TNM system. The proportion staged accurately varied widely across hospitals from 10.9 to 76.0 per cent. CONCLUSION: These results reflect the need for improved N staging across the region to aid the appropriate multimodal treatment of patients.

Mimotopes and proteome analyses using human genomic and cDNA epitope phage display.

In the post-genomic era, validation of candidate gene targets frequently requires proteinbased strategies. Phage display is a powerful tool to define protein-protein interactions by generating peptide binders against target antigens. Epitope phage display libraries have the potential to enrich coding exon sequences from human genomic loci. We evaluated genomic and cDNA phage display strategies to identify genes in the 5q31 Interleukin gene cluster and to enrich cell surface receptor tyrosine kinase genes from a breast cancer cDNA library. A genomic display library containing 2 x 106 clones with exon-sized inserts was selected with antibodies specific for human Interleukin-4 (IL-4) and Interleukin-13. The library was enriched significantly after two selection rounds and DNA sequencing revealed unique clones. One clone matched a cognate IL-4 epitope; however, the majority of clone insert sequences corresponded to E. coli genomic DNA. These bacterial sequences act as 'mimotopes' (mimetic sequences of the true epitope), correspond to open reading frames, generate displayed peptides, and compete for binding during phage selection. The specificity of these mimotopes for IL-4 was confirmed by competition ELISA. Other E. coli mimotopes were generated using additional antibodies. Mimotopes for a receptor tyrosine kinase gene were also selected using a breast cancer SKBR-3 cDNA phage display library, screened against an anti-erbB2 monoclonal antibody. Identification of mimotopes in genomic and cDNA phage libraries is essential for phage display-based protein validation assays and two-hybrid phage approaches that examine protein-protein interactions. The predominance of E. coli mimotopes suggests that the E. coli genome may be useful to generate peptide diversity biased towards protein coding sequences.

Homozygous mutations in ARIX(PHOX2A) result in congenital fibrosis of the extraocular muscles type 2.

Isolated strabismus affects 1-5% of the general population. Most forms of strabismus are multifactorial in origin; although there is probably an inherited component, the genetics of these disorders remain unclear. The congenital fibrosis syndromes (CFS) represent a subset of monogenic isolated strabismic disorders that are characterized by restrictive ophthalmoplegia, and include congenital fibrosis of the extraocular muscles (CFEOM) and Duane syndrome (DURS). Neuropathologic studies indicate that these disorders may result from the maldevelopment of the oculomotor (nIII), trochlear (nIV) and abducens (nVI) cranial nerve nuclei. To date, five CFS loci have been mapped (FEOM1, FEOM2, FEOM3, DURS1 and DURS2), but no genes have been identified. Here, we report three mutations in ARIX (also known as PHOX2A) in four CFEOM2 pedigrees. ARIX encodes a homeodomain transcription factor protein previously shown to be required for nIII/nIV development in mouse and zebrafish. Two of the mutations are predicted to disrupt splicing, whereas the third alters an amino acid within the conserved brachyury-like domain. These findings confirm the hypothesis that CFEOM2 results from the abnormal development of nIII/nIV (ref. 7) and emphasize a critical role for ARIX in the development of these midbrain motor nuclei.

Abnormal development of the lesser wing of the sphenoid with microphthalmos and microcephaly.

We report two patients with abnormal development of the lesser wing of the sphenoid bone, globe, optic nerve and cerebral hemisphere without stigmata of neurofibromatosis type 1. The lesser wing of the sphenoid bone was abnormally formed and was not ossified ipsilateral to the dysmorphic eye and underdeveloped cerebral hemisphere. Maldevelopment of the sphenoid wing may interfere with the normal closure of the optic vesicle and normal growth of encephalic structures, possibly by disturbing developmental tissue interactions. These patients may exhibit a type of restricted primary sphenoid dysplasia, while the sphenoid dysplasia of neurofibromatosis type 1 may be secondary to orbital or ocular neurofibromas and other factors associated with that disease.

Ophthalmic involvement as a presenting feature of nonorbital childhood parameningeal embryonal rhabdomyosarcoma.

OBJECTIVE: To describe presenting ophthalmic signs and symptoms in children with parameningeal rhabdomyosarcoma. DESIGN: Retrospective, noncomparative case series. PARTICIPANTS: Twenty-three children with parameningeal rhabdomyosarcoma treated from 1978 through 1998. MAIN OUTCOME MEASURES: Each patient's presenting symptoms and history, the ophthalmic signs at presentation, the location of the tumor on computed tomographic scanning, the pathologic diagnosis after tumor biopsy, and the child's outcome after chemotherapy and radiotherapy. RESULTS: Eight of 23 patients with parameningeal rhabdomyosarcoma had ophthalmologic signs at presentation. All patients had sixth nerve palsies, and four had additional third nerve palsies. Fifth and seventh nerve involvement was seen in three children each, and two manifested additional fourth nerve palsies. In two patients, invasion of the optic canals and posterior orbit resulted in unilateral blindness in one and marked unilateral visual loss in the other. In no patient was the symptomatic history longer than 12 weeks. Seven of eight patients were treated with a combination of surgery, chemotherapy, and radiotherapy. The mean survival time for five patients who died from parameningeal rhabdomyosarcoma associated with ophthalmic signs was 27.2 months. Three patients are currently alive, with a mean survival time of 33.7 months after diagnosis. The survival time of 12 patients with parameningeal rhabdomyosarcoma and without ophthalmologic signs at presentation was better, in that nine are alive, with a mean survival time of 54 months. CONCLUSIONS: Advanced parameningeal rhabdomyosarcoma with skull base invasion may present with ophthalmic signs. These patients sought treatment late and had advanced tumors because their initial signs and symptoms were often attributed to more common ailments. Usually, the onset of the cranial nerve palsy initiated referral to the tertiary care center. The sixth nerve was the most vulnerable. Further invasion of the skull base or cavernous sinus may cause additional third, fourth, and fifth nerve palsies. The optic nerve was involved when the optic canals and orbit were encroached on by the tumor. The prognosis for survival was poor when these tumors were accompanied by ophthalmic signs.

Recognized globe perforation during strabismus surgery: incidence, risk factors, and sequelae.

BACKGROUND: Inadvertent perforation of the globe is a well-recognized complication of extraocular muscle surgery. We evaluated the incidence, risk factors, and sequelae of this complication at our institution. METHODS: Medical records of patients who underwent extraocular muscle surgery at King Khaled Eye Specialist Hospital, Saudi Arabia, between September 1983 and April 1997, were reviewed for the occurrence of globe perforation. We documented preoperative visual acuity and refraction, surgical procedure, how the perforation occurred, and immediate management, as well as the sequelae of the perforation, its management, and final outcome. RESULTS: Recognized perforations occurred in 15 of 4886 procedures, for an overall incidence rate of 3/1000. Perforations were 3 times more common in myopic eyes (>-6.00 D, P =.05) and 2 times more common in eyes with previous extraocular muscle surgery. Perforations occurred during muscle reattachment (5 cases), placement of traction sutures at the limbus (4 cases with transient hyphema), muscle disinsertion (3 cases), and placement of sutures at the muscle insertion before disinsertion (3 cases). One patient had a large scleral laceration with uveal prolapse, necessitating scleral patch graft at the time of surgery, and later had retinal detachment surgery with loss of 2 lines of visual acuity. Endophthalmitis, cataract, glaucoma, and phthisis bulbi were not encountered in our review. CONCLUSION: The current incidence of globe perforation is low and only rarely associated with serious sequelae.

The natural history and ophthalmic involvement in childhood myasthenia gravis at the hospital for sick children.

OBJECTIVE: To characterize signs, symptoms, and the natural history of myasthenic syndromes in pediatric patients. DESIGN: Retrospective noncomparative case series. PARTICIPANTS: Thirty-four patients with a diagnosis of myasthenia were identified from either the hospital's or treating physician's database. METHODS: Retrospective chart review, clinical examination, and telephone interview. MAIN OUTCOME MEASURES: Information pertaining to the ophthalmologic and neurologic examination, diagnostic interventions, and treatment was noted. Patients with active disease, attending during the study period, were examined at their outpatient visits. Those who no longer attended the hospital were contacted by means of a telephone interview to complete their follow-up. RESULTS: Thirty-four children were found to have myasthenia. Two had transient neonatal myasthenia, which resolved quickly. Seven (20.6%) patients had congenital myasthenic syndromes (CMS) and 25 (73.5%, 19 females) were affected with autoimmune myasthenia gravis (AMG). In those patients with severe CMS, three showed signs of generalized weakness, including failure to thrive, frequent apneas, and aspirations. In four patients with mild CMS, eye signs were relatively more prominent. In all patients with CMS, strabismus, ophthalmoplegia, and ptosis were the main ophthalmologic signs and remained relatively constant. Fourteen (56%) patients with AMG had ocular signs and symptoms, and five of them progressed to systemic involvement in 7.8 months on average (range, 1-23). The remaining nine patients with ocular AMG had either strabismus or ptosis and were treated with pyridostigmine (nine patients) and prednisone (two patients). Patients with ocular AMG were seen at 78 months on average, those with systemic AMG at 85.6 months. Systemic AMG was seen in 16 patients. No thymomas were found in 14 patients who underwent thymectomy. Of the 25 patients with AMG, 8 are still being treated, 8 are in remission for an average of 65.2 months and are asymptomatic, 4 patients are receiving long-term immunosuppressants (1 has likely sustained permanent damage to her extraocular muscles with complete ophthalmoplegia and ptosis), and 4 have been lost to follow-up. Finally, one patient died after aspiration because of bulbar weakness. CONCLUSIONS: Patients with CMS varied in the degree of severity. Apneic attacks, aspiration, and failure to thrive may obscure the diagnosis. Compared with AMG, their ophthalmologic signs and symptoms were usually permanent. Visual signs and symptoms were usually prominent in those patients with active AMG, but those in remission were asymptomatic. More than half of the patients with juvenile AMG had ocular symptoms. Generalization occurred in a minority in an average of 7.8 months. Patients entered remission after approximately 2 years of treatment and were visually asymptomatic. This study suggests that long-term permanent damage to the extraocular muscles as a result of juvenile AMG is rare. Myasthenia gravis is a life-threatening disease as evidenced by the death of one of our patients. Many of these patients are first seen by the ophthalmologist who can aid the diagnosis, screen for amblyopia, and monitor the patient's response to therapy.

Canalis basilaris medianus: MRI.

We report the MRI appearances of an developmental anatomical variant of the basiocciput, with neuroimaging findings (CT and MRI). Such variants are commonly asymptomatic, but may be associated with episodes of meningitis.

Ophthalmological and intracranial anomalies in patients with clinical anophthalmos.

PURPOSE: To better describe the clinical and neuroimaging spectrum of abnormalities in clinical anophthalmos. METHODS: We performed a retrospective review of all 17 patients admitted to the King Khaled Eye Specialist Hospital with clinical anophthalmos over a 15 year period who had a complete ophthalmological examination documented and received computed tomographic (CT) imaging of the orbits and brain. RESULTS: Patients with clinical anophthalmos had a high incidence of developmental abnormalities involving both eyes (15/17 patients, 88%), the brain (12/17 patients, 71%) and the body (7/12, 58%). The incidence of central nervous system anomalies reached 100% in patients with bilateral small optic nerves on CT scan. CONCLUSIONS: Patients with clinical anophthalmos share a similar constellation of neurological, somatic and neuroradiological abnormalities as patients with microphthalmos, septo-optic dysplasia and clinical optic nerve hypoplasia. This fact may provide insight into developmental abnormalities of the afferent visual system and brain.

Pyramidal anterior polar cataracts.

OBJECTIVE: To document clinical features and subsequent management of pyramidal anterior polar cataracts in children. DESIGN: Retrospective, noncomparative case series and clinicopathologic correlation. PARTICIPANTS: Fifteen patients who presented to the pediatric ophthalmology clinic. INTERVENTION: All patients underwent measurement of visual acuity, assessment of ocular motility, examination of the anterior and posterior segments, and cycloplegic refraction. Amblyopia treatment was instituted when appropriate. When visual impairment occurred from cataract progression or amblyopia or both, cataract removal with or without lens implantation was performed. After surgery, correction of refractive error and treatment of amblyopia were instituted. Several pyramidal opacities were retrieved during cataract extraction and examined by light and electron microscopy. MAIN OUTCOME MEASURES: Visual acuity at initial presentation, size of lens opacity before surgery, amblyopia status, most recent visual acuity after cataract extraction, and histologic examination of lens opacity. RESULTS: Nine children had bilateral and six had unilateral pyramidal cataracts (24 eyes). There was no discernible inheritance pattern. Patients were followed for 27 months on average. Twenty of 24 eyes developed cortical opacification that extended significantly beyond the base of the pyramidal lesion. Nineteen eyes required cataract surgery: 10 eyes underwent lensectomy with anterior vitrectomy and 9 had extracapsular cataract extraction, 8 of which had insertion of a posterior chamber intraocular lens. Amblyopia was present or developed in all six patients with unilateral cataract and in eight of nine patients with bilateral cataract. Visual acuity in many eyes remained poor despite amblyopia therapy. The pyramidal opacities consisted of hyperplastic lens epithelium, which exhibited a loss of polarity and was surrounded by a collagenous matrix. CONCLUSIONS: Pyramidal anterior polar cataracts are present at birth and may represent a variant of anterior polar lens opacities. They may be unilateral or, if bilateral, they may be either symmetric or asymmetric. They consist of hyperplastic lens epithelium in a collagenous matrix. Patients with pyramidal cataracts are likely to develop amblyopia. This can result from either unilateral occurrence or asymmetry of bilateral opacities and is often worsened by surrounding cortical opacification. Many patients require cataract surgery. All infants and young children with anterior polar opacities showing this configuration should be followed for cataract progression and amblyopia.

Combined trabeculotomy and trabeculectomy as an initial procedure in uncomplicated congenital glaucoma.

OBJECTIVE: To review the results of combined trabeculotomy and trabeculectomy as a primary procedure in congenital glaucoma. METHODS: A retrospective review of 100 consecutive eyes in 60 children undergoing surgery from December 30, 1991, to April 17, 1996. Features at initial examination, ie, corneal size and clarity, presence or absence of anterior segment structural abnormalities, and intraocular pressure (IOP), were noted. Data pertaining to perioperative use of mitomycin and the occurrence of complications were collected. After surgery, all patients had IOP, corneal integrity, and any postoperative complication recorded under chloral hydrate sedation. RESULTS: Mean preoperative IOP was 31 mm Hg. Average horizontal corneal diameter was 12.60 mm. Ninety-five eyes had corneal opacification. Twenty-nine eyes had additional anterior segment anomalies, with ectropion uveae (n = 11), Peters anomaly (n = 9), and partial aniridia (n = 7) being the most common. Mitomycin (0.2 or 0.4 mg/mL) was used in 87 eyes. Eleven eyes sustained hyphemas during or just after surgery. Total average follow-up was 304 days. Eyes in which no coexistent anterior segment anomalies were present had a 78% (49 eyes) operative success (IOP, <21 mm Hg); however, in eyes with associated anterior segment anomalies, the success rate was much lower (45% [18 eyes]). The difference in success rates between both groups was statistically significant (P = .03, chi2 test). CONCLUSIONS: Primary combined trabeculotomy and trabeculectomy was a useful initial procedure in uncomplicated congenital glaucoma. This was particularly true where corneal opacification, as in nearly all our eyes, precluded goniotomy, however, where other stigmata of anterior segment dysgenesis coexisted, results were significantly poorer.

Glaucoma in Sturge-Weber syndrome.

BACKGROUND: In glaucoma associated with Sturge-Weber syndrome (SWS), medical treatment often fails to control intraocular pressure, thus requiring surgical intervention that may result in serious complications. METHODS: Eighteen consecutive patients with SWS were reviewed retrospectively at the King Khaled Eye Specialist Hospital. An intraocular pressure less than 20 mm Hg, plus stable optic nerve cup-to-disc ratio and corneal diameter (or visual fields where appropriate), were parameters chosen to indicate that the glaucoma was being controlled. RESULTS: Glaucoma was found in 15 of 18 patients (22 eyes). The mean follow-up time was 62 months (range, 12 to 148 months). Medical treatment alone was successful in 5 patients (7 eyes); the remainder required surgical intervention. The initial surgical procedures included cyclocryotherapy, YAG laser goniotomy, surgical goniotomy, and trabeculotomy or trabeculectomy. Eight eyes required subsequent surgery, 5 with Molteno or Ahmed implants. Early postoperative choroidal effusion and hemorrhage occurred in 4 eyes and resolved spontaneously. Hemorrhagic choroidal detachment with total retinal detachment developed in 2 patients 3 to 5 months after surgery. In 1 patient a recurrent serous choroidal detachment after suture lysis was associated with total optic atrophy. CONCLUSIONS: Glaucoma in SWS is common. Control of glaucoma was successfully achieved with medical treatment in 7 of 22 eyes of our 15 SWS-glaucoma patients, and we consider it the initial treatment of choice. Of the 15 eyes that required surgery, late postoperative complications resulted in loss of vision as a result of persistent postoperative hypotony in 3 eyes that underwent surgical procedures.

Orbital color Doppler imaging of optic nerve tumors.

OBJECTIVE: To report changes in retinal arterial and venous blood flow pattern in two patients with tumors involving the entire optic nerve. METHODS: Retrospective review of one patient with clinical and neuroimaging characteristics typical of bilateral optic nerve gliomas and one patient with a probable meningioma of the left optic nerve sheath. RESULTS: The optic nerve glioma patient had reduced peak systolic velocity of central retinal arteries bilaterally, while the patient with an optic nerve sheath meningioma had relatively low central retinal artery flow velocity and intermittent blood flow in the central retinal vein on the affected side. CONCLUSIONS: Reduced retinal arterial flow velocities in the setting of optic nerve gliomas may correlate with the presence of optic nerve disease. Phasic blood flow in the central retinal vein with optic nerve sheath meningioma may be the reason that some patients with this tumor develop retinal choroidal venous anastomoses.