Penile fracture associated with complete urethra and bilateral corpora cavernosa transection.

Penile fracture is a rare urological emergency caused by blunt trauma to the erect penis. It occurs due to the forcible bending of the turgid erect penis against resistance leading to tunica albuginea tear. The rupture of tunica albuginea surrounding the corpora cavernosa leads to hematoma formation and classical 'aubergine' deformity. Timely intervention is essential to improve sexual function. Urethral injury may occur concomitantly in case of severe trauma. Blood at the meatus, inability to void and haematuria are distinctive features. We describe a case of 36-year-old man who presented to the emergency department with penile fracture during sexual intercourse associated with blood at the meatus and voiding difficulty. On surgical exploration, complete bilateral corpora cavernosa tear and penile urethral transection was noted. The patient was successfully managed with timely repair. This case highlights the need for suspicion of an associated urethral injury in patients of penile fracture with blood at the meatus.

Retroperitoneal paraganglioma: a chameleon masquerading as an adrenal pheochromocytoma.

Retroperitoneal paragangliomas are rare tumours arising from neural crest cells located near or in the autonomic nervous system; right from the skull bass to the pelvic floor. Often called the great masqueraders, their diagnosis and treatment is often difficult and fraught with danger, considering the close proximity of these tumours to important retroperitoneal organs. A 29-year-old woman presented to the outpatient department with complaints of paroxysms of headache, sweating and palpitations for one year. She was found to have elevated urinary metadrenalines (metanephrines) and a suprarenal mass on contrast-enhanced computed tomography of the abdomen, which demonstrated increased tracer activity on I-131 iodine-123 meta-iodobenzylguanidine scintigraphy. Adrenal pheochromocytoma was diagnosed and she underwent laparoscopic adrenalectomy after stabilisation of her blood pressure. Laparoscopy revealed a large suprarenal mass with dense adhesions to the inferior vena cava that was carefully separated from the vein and surrounding structures. The cut section revealed a heterogeneous mass encasing a normal adrenal gland and histopathology confirmed the same, confirming the final diagnosis of retroperitoneal paraganglioma.

Chronic maternal hyperglycemia induced during mid-pregnancy in rats increases RAGE expression, augments hippocampal excitability, and alters behavior of the offspring.

Maternal diabetes during pregnancy may increase the risk of neurodevelopmental disorders in the offspring by increasing inflammation. A major source of inflammatory signaling observed in diabetes is activation of the receptor for advanced glycation end-products (RAGE), and increased RAGE expression has been reported in psychiatric disorders. Thus, we sought to examine whether maternal diabetes creates a proinflammatory state, triggered largely by RAGE signaling, that alters normal brain development and behavior of the offspring. We tested this hypothesis in rats using the streptozotocin (STZ; 50mg/kg; i.p.) model of diabetes induced during mid-pregnancy. Following STZ treatment, we observed a significant increase in RAGE protein expression in the forebrain of the offspring (postnatal day 1). Data obtained from whole-cell patch clamping of hippocampal neurons in cultures from the offspring of STZ-treated dams revealed a striking increase in excitability. When tested in a battery of behavioral tasks in early adulthood, the offspring of STZ-treated dams had significantly lower prepulse inhibition, reduced anxiety-like behavior, and altered object-place preference when compared to control offspring. In an operant-based strategy set-shifting task, STZ offspring did not differ from controls on an initial visual discrimination or reversal learning but took significantly longer to shift to a new strategy (i.e., set-shift). Insulin replacement with an implantable pellet in the dams reversed the effects of maternal diabetes on RAGE expression, hippocampal excitability, prepulse inhibition and object-place memory, but not anxiety-like behavior or set-shifting. Taken together, these results suggest that chronic maternal hyperglycemia alters normal hippocampal development and behavior of the offspring, effects that may be mediated by increased RAGE signaling in the fetal brain.

Multifocal electroretinogram contributes to differentiation of various clinical pictures within a family with Bardet-Biedl syndrome.

PURPOSE: To demonstrate the use of the multifocal electroretinogram (mfERG) in addition to the full-field electroretinogram (ERG) in defining varying clinical pictures in children within a family with Bardet-Biedl syndrome (BBS). METHODS: All members from a family generation underwent a detailed history and examination before proceeding to a detailed ERG in accordance with the International Society of Clinical Electrophysiology for Vision protocol and a rapid, low-resolution mfERG. Of the sibling pair, the 13-year-old boy showed reduced vision and atypical maculopathy and the 10-year-old sister showed normal vision and atrophic maculopathy. Parents had normal ocular examination. RESULTS: The male sibling had reduced rod and cone full-field ERG responses with a relatively spared central response from the mfERG suggesting central macular sparing. In contrast, for the female sibling, the ERG was normal for the cone pathway although reduced for rod pathway, with mfERG showing central involvement. The mother had rod responses at the lower end of normal range, a normal cone pathway, and a normal mfERG. The father showed a normal ERG and mfERG. CONCLUSION: The mfERG is a useful adjunct to full-field ERG in the paediatric population and in family studies.

Childhood sight impairment: a 10-year picture.

INTRODUCTION: Registering a child as visually impaired is a potentially traumatic, but necessary, milestone in paediatric ophthalmology. Registration enables the provision of services essential to maximise the child's potential. PURPOSE: This study was carried out to investigate the changes over a 10-year period in the rates of registration of childhood blindness at a tertiary paediatric ophthalmology department. Particular attention was given to diagnosis, whether the disease was preventable, time to registration, age at registration, and the socioeconomic status of the patient's family. METHODS: A retrospective analysis of all children registered blind or partially sighted over a 10-year period until December 2006. RESULTS: A total of 256 children were registered blind or partially sighted over the 10 years. All cases were analysed. Of these, 58.2% were male and the average age at registration was 76 months; 52.0% were registered as severely sight-impaired. The most common primary diagnosis was cerebral visual impairment (CVI) in 27% cases, followed by optic atrophy in 16%, and the commonest anatomical site involved was the retina in 30.9%. An average of 25.6 (SD 8.0) registrations were carried out each year. The number of registrations per year is increasing. Seven cases (2.7%) were deemed avoidable and 61 cases (23.8%) were deemed potentially treatable. The mean index of multiple deprivation (IMD) score for the English cases (45.1) was significantly higher than of the surrounding area (P<0.0001). CONCLUSIONS: The yearly rates of registration increased slowly over the 10 years. The most common underlying cause for registration remains CVI, with the yearly proportion of registrations because of CVI not altering. The average age of registration was 6.3 years. A significant proportion of the cases of visual impairment, are because of potentially modifiable causes.

Magnetic susceptibility artifact in orbital magnetic resonance imaging.

Case report of a 16-year-old boy presenting with total hyphema due to blunt trauma to the left eye. During a subsequent cinematic MRI scan to identify salvageable lateral rectus muscle, the attachment of the left lateral rectus was obscured by a 20-mm diameter signal void. A subsequent CT scan for a suspected metallic foreign body revealed the muscle attachment after all. The theoretical explanations for this finding are discussed.

Clinical features and outcome of surgery in 30 patients with acromegaly.

OBJECTIVE: To assess the surgical outcome of hypophysectomy in acromegalic patients, differences in response to surgery in micro and macroadenoma and the development of associated hormonal deficiency after surgery. METHODS: Retrospective analysis of charts of acromegalic patients who were operated upon at Aga Khan University Hospital, Karachi, was done. Information regarding presentation, laboratory data, radiological assessment, details of surgery and postoperative outcome was recorded. Surgical outcome was classified depending on the degree of disease control after surgery as biochemical cure, remission and treatment failure. RESULTS: Out of 30 patients, eighteen (60%) were males and 12 (40%) females with mean age at the time of diagnosis 35.6+/-10.4 years. The mean duration of symptoms was 3.2+/-2.4 years. Twenty-five patients had macroadenoma and five had microadenoma. Preoperative mean GH level was 42.4 ng/ml with range of 2.5 to 534 ng/ml. Following surgery, 13 had biochemical cure, 4 were in remission and 13 failed to respond. The outcome in microadenoma was better than in macroadenoma i.e. 100% vs. 32%. Preoperatively four patients had panhypopituitarism with additional six patients (20%) developing hypopituitarism postoperatively. CONCLUSION: Our study showed that acromegaly was seen at a much earlier age, outcome of surgery was comparable to other international studies and postoperative panhypopituitarism was seen in significant number of patients. The outcome in microadenoma was better than in macroadenoma but the number of patients seen with microadenoma was small.

Linear assessment of anteroposterior jaw relationship.

Three Linear methods of establishing the sagittal relationship of the maxillary and mandibular apical bases were compared using Lateral skull radiographs of thirty individuals having Class II div. 1 malocclusion. Statistical evaluation was done for wit appraisal, AD' distance, and Wylie analysis. The A-D' distance is recommended for use during sagittal apical base relationship.

Contour integration deficits in anisometropic amblyopia.

PURPOSE: Previous retrospective studies have found that integration of orientation information along contours defined by Gabor patches is abnormal in strabismic, but not in anisometropic, amblyopia. This study was conducted to reexamine the question of whether anisometropic amblyopes have contour integration deficits prospectively in an untreated sample, to isolate the effects of the disease from the effects of prior treatment-factors that may have confounded the results in previous retrospective studies. METHODS: Contour detection thresholds, optotype acuity, and stereoacuity were measured in a group of 19 newly diagnosed anisometropic amblyopes before initiation of occlusion therapy. Contour detection thresholds were measured using a card-based procedure. RESULTS: Significant interocular differences in contour detection thresholds were present in 14 of the 19 patients with anisometropic amblyopia. CONCLUSIONS: Contour integration deficits are a common, but not universal, finding in untreated anisometropic amblyopia. Differences in the prevalence of contour integration deficits between the present study and that of another study may lie in differences in treatment history and/or in the sensitivity of the two different contour integration tasks.

The use of the fluorescein disappearance test in the management of childhood epiphora.

PURPOSE. The fluorescein disappearance test (FDT) is reported to be an objective measure of lacrimal outflow in congenital non-canalisation of the nasolacrimal system. We introduced FDT into our evaluation of children with epiphora to investigate its sensitivity/specificity with regard to symptoms in a prospective study. We also examined the FDT against findings at syringing and probing (S&P) and persistence or resolution of symptoms on follow-up. METHODS. Over a period of 16 months, 88 consecutive children (median age 12 months; range 2.5-192) with epiphora were reviewed and FDT measured at 5 and 10 minutes; 66 FDT were evaluated for inter-observer variation in a masked fashion. S&P were scheduled on the basis of symptoms, abnormal FDT and age "1 year. Normal FDT prompted review. Younger children were observed for natural history and possible resolution with repeat FDT. Equivocal symptoms and normal FDT initiated discharge and telephone review. RESULTS. Inter-observer correlation coefficient = 0.86. Sensitivity/specificity of FDT was 76/76% at 5 minutes and 63/89% at 10 minutes, respectively. In patients under 1 year of age undergoing follow-up (29 patients; 38 eyes) FDT at 10 minutes correctly predicted persistence of symptoms in 65% and resolution in 66% of eyes (follow-up 3-14 months; mean 6); 23 patients (mean age 27 months; range 12-72) underwent S&P with positive surgical findings in 20 (87%). On review, symptoms had improved in 64% eyes (20/31). CONCLUSION. FDT at 5 minutes is an objective measure of symptoms with high inter-observer agreement and agreement with surgical findings. FDT read at 10 minutes may be useful to indicate the persistence or resolution of symptoms and guide patient management.

Orbital abscess masquerading as a rhabdomyosarcoma.

Although orbital cellulitis is the most common cause of acute-onset proptosis with inflammatory signs in a child, the clinician should always be alert to the possibility of rhabdomyosarcoma. We describe an unusual presentation of acute-onset nonaxial proptosis of the left orbit without sinus disease or systemic toxicity in a 6-year-old boy. Our clinical differential diagnosis included orbital cellulitis, metastatic disease, capillary haemangioma, lymphangioma with cyst, ruptured dermoid cyst, and orbital rhabdomyosarcoma. Only after orbital biopsy and subsequent microbiologic confirmation were obtained was a diagnosis of chronic orbital abscess tenable. Features in our patient included paucity of symptoms and signs of inflammation. This case illustrates the difficulty in differentiating a chronic orbital infection from orbital rhabdomyosarcoma on the basis of clinical, laboratory, and orbital imaging findings. Possible causes of this unusual presentation are discussed.

A new test of contour integration deficits in patients with a history of disrupted binocular experience during visual development.

Previous studies have suggested that the integration of orientation information across space is impaired in amblyopia. We developed a method for quantifying orientation-domain processing using a test format that is suitable for clinical application. The test comprises a graded series of cards where each card includes a closed path (contour) of high contrast Gabor signals embedded in a random background of Gabor signals. Contour visibility in both normals and patients with histories of abnormal binocular vision depends jointly on the spacing of elements on the contour as well as background element density. Strabismic amblyopes show significant degradation of performance compared to normals. Small but significant losses in sensitivity were also observed in a group of non-amblyopic strabismus patients. Threshold measurements made with contrast reducing diffusers indicated that the amblyopic loss is not due to the reduced contrast sensitivity of the amblyopic eye. An abnormal pattern of long-range connectivity between spatial filters or a loss of such connectivity appears to be the primary source of contour integration deficits in amblyopia and strabismus.

Binocular fixation pattern and visual acuity in children with strabismic amblyopia.

BACKGROUND: A prospective study was undertaken to compare the binocular fixation pattern and presence of amblyopia in strabismic children. METHODS: Fifty-three children with manifest strabismus and the ability to cooperate with an optotype acuity test were examined. The binocular fixation pattern and logMAR visual acuity were recorded by separate, masked observers under standardized conditions. The binocular fixation pattern was divided into four grades from alternation to uniocular fixation. RESULTS: Patients who freely alternated did not have amblyopia, while those who maintained or preferred fixation with a given eye tended to have amblyopia in the nonpreferred eye. CONCLUSION: The binocular fixation pattern can be rapidly assessed with minimal equipment and training. These findings confirm the usefulness of a graded assessment of the binocular fixation pattern in the detection of amblyopia.

Contour detection threshold: repeatability and learning with 'contour cards'.

Human observers are able to locate contours that are defined solely on the basis of long-range, orientation-domain correlations. The integrity of the mechanisms responsible for second-order contour detection is disrupted by amblyopia (Kovacs et al., 1996; Hess et al., 1997) and it is therefore of interest to develop methods for assessing pediatric patients undergoing treatment for amblyopia. In this study, we have determined the inter-observer and test-retest reliability of a card-based test of second-order contour integration. The magnitude of practice effects was also assessed in both adult and pediatric patient groups. Contour detection thresholds were measured for a closed contour, defined by Gabor patches, embedded in a randomly oriented Gabor-patch background. The visibility of the contour was controlled by varying the density of the background elements. Thresholds, defined in terms of the ratio of contour element spacing to average background spacing were measured with a clinical staircase procedure. Thresholds measured by two observers differed on average by 0.023 +/- 0.075 or about one half the increment between cards. Children and adults showed only small practice effects (0.022 +/- 0.051 vs 0.053 +/- 0.077, respectively) and average unsigned differences between repeated measures were equivalent to approximately 1 card across groups. A card-based test of second-order contour integration produces reliable estimates of contour integration performance in normal and amblyopic observers, including children.

Oscillatory motion but not pattern reversal elicits monocular motion VEP biases in infantile esotropia.

Patients with early disruptions of binocularity show cortical directional asymmetries in their steady state monocular VEP response to oscillatory motion. The VEP directional asymmetry is characterized by significant first harmonic components that show a 180 degrees difference in the response phase between the two eyes. By contrast, the normal response is dominated by even-order response harmonics, although some normal observers also have measurable responses at the first harmonic. Experiments and simulations were conducted to determine if the first harmonic in patients could reasonably be attributed to direction selective mechanisms. A secondary goal was to determine whether the first harmonic response of normals was also due to imbalances in direction selective mechanisms. Monocular steady state VEPs were elicited by oscillating 3 c/deg gratings presented at 6 and 10 Hz in normal observers and observers with infantile esotropia. Responses were also obtained to phase-reversing gratings of the same spatial and temporal frequencies. Phase reversal eliminated the majority of first harmonic responses which were recorded for normal observers to oscillatory motion. However, phase reversal did not elicit the cortical motion asymmetry in infantile esotropia. Modeling results suggest that the first harmonic response to oscillatory motion arises due to non-linearities in both direction selective and non-direction-selective mechanisms, with the latter being dominant in patients with early onset strabismus.

Validation of a handheld automated keratometer in adults.

PURPOSE: To evaluate the Nidek handheld automated keratometer and compare it with the manual Zeiss keratometer. SETTING: St. Paul's Eye Unit, Royal Liverpool University Hospital, Liverpool, United Kingdom. METHODS: Forty-five normal adult volunteers were included in the study. Three sets of randomized keratometry measurements were taken from both eyes of each adult, with both instruments by two independent observers. Readings included powers of the steep and flat meridians and axis of the flat meridian. The mean difference between the two instruments was calculated. The variance of each instrument was calculated for the axis, the steep and flat meridians, and the mean of the two meridians. RESULTS: There was no significant difference between the mean Nidek and Zeiss keratometry readings for the steep (0.015 mm; P = .167) or flat (0.054 mm; P = .069) meridian or axis measurements (P = .172). On repeated measurements, the within-subject and within-group variabilities, calculated separately for each instrument, were significantly less for the Nidek automated keratometer than the Zeiss keratometer (P < .01) when measuring the steep and flat meridians. There was however, a significant increase in axis variability using the Nidek keratometer (range 20 degrees; P < .01). CONCLUSION: The Nidek automated keratometer was accurate, reliable, and easy to use and compared favorably with the manual Zeiss keratometer when measuring corneal curvature. In the handheld mode, the Nidek is not suitable for axis measurements. It has the additional advantage of portability.

Repeatability of the hand-held Nidek auto-keratometer in children.

Measurement of the anterior corneal radius of curvature is important in the pediatric population for proper fitting of contact lenses, calculation of intraocular lens power in patients undergoing cataract surgery, and monitoring changes in shape of the cornea in keratoconus. Because astigmatism in preschool children is mainly the result of corneal asphericity, measurement of the anterior corneal radius of curvature may also be used in screening in children at risk for amblyopia. The aim of this study was to determine the repeatability of the hand-held automated Nidek keratometer (Nidek Co. Ltd, Tokyo, Japan) and its ease of use in children.

Downgaze paresis following severe head trauma in a child.

A 4 1/2-year-old girl developed a downgaze paresis following severe head trauma. Magnetic resonance imaging showed evidence of peri-aqueductal lesions in the rostral midbrain in the region of the rostral interstitial nucleus of the medial longitudinal fasciculus (riMLF). Twenty five weeks following the injury, the downgaze paresis remained unchanged but she developed convergence retraction nystagmus on attempted upgaze. Repeat imaging did not show any change in the lesions in the rostral midbrain. This report provides further evidence for the riMLF in the control of downgaze, and a synkinesis is postulated for the development of the convergence retraction nystagmus.