Which centers should be authorized to treat craniosynostosis? A plea for quality and not for quantity.

BACKGROUND: In centers for craniosynostosis surgery, the volume of activity does not necessarily reflect the quality of the treatment. OBJECTIVE: Our aim was to analyze a retrospective series of patients over a period of 6 years in a low-volume craniosynostosis surgery center, and to study indicators that reflect the quality of treatment. PATIENTS AND METHODS: The analysis included all patients who underwent a craniofacial surgery for all forms of craniosynostosis during the period 2012-2017 (annual follow-up for 4 years). Data on the type of synostosis, sex, age, weight, type of surgery, duration of surgery, blood transfusion, postinterventional care, and total length of hospital stay were collected. Medical and surgical complications were recorded using the Leeds classification. RESULTS: Overall, 42 patients (33 male; 23 cases of scaphocephaly, 13 cases of trigonocephaly, 4 cases of coronal plagiocephaly, 1 case of lambdoid plagiocephaly, and 1 case of brachycephaly) underwent craniofacial surgery with a median age of 7.4 months [4.8; 10.4] and a mean weight of 8.40 ± 1.92 kg at surgery. The median hospital stay was 7 days [6;7] with 1 day in the postinterventional care unit for 83% of patients. The global complication rate was 12% (95% CI: 4%-26%) with three minor cutaneous and two major (cardiovascular and septic) complications. CONCLUSION: Complication rates reflect the quality of care in a center that treats craniosynostosis much more than do the number of procedures, mean hospital stay, and blood transfusion rates. It is essential to define new indicators capable of measuring the quality of life linked to surgical procedures and of using them to assess the competence of a center.

Diagnosis of medulloblastoma in pregnant women: To continue with the pregnancy or not? A case report and literature review.

BACKGROUND: Medulloblastoma (MB) is an uncommon and challenging diagnosis in pregnant women, and especially in pregnancy after in-vitro fertilization (IVF). Clinical features are easily misinterpreted and mistaken for other more common gestation-related pathologies. We report the case of a 34-year-old patient with clinical symptoms of intracranial hypertension. MB was diagnosed and operated on during the pregnancy. OBJECTIVE: To conduct a systematic literature review of other cases of MB operated on during pregnancy, and discuss the clinical and surgical management of MB in pregnancy. METHOD: We conducted a systematic literature review according to PRISMA guidelines. RESULTS: In addition to the present case, 9 cases of MB were reported as operated on during viable pregnancy. In one case, medical abortion was decided on before surgical debulking. Pregnancy term was between 8 and 30 weeks. The most common symptoms were headache, nausea and vomiting followed by dizziness. Tumor prognosis after treatment was favorable in 6 cases out of 10 and unfavorable in 4, with 3 cases of recurrence and 3 of death. CONCLUSION: We report the first case of long-term survival after MB in a woman pregnant via IVF. In standard-risk MB, it is possible to carry the pregnancy to term. Vaginal delivery is not contraindicated a priori. Early diagnosis, close clinical and radiological surveillance and surgery are the key factors for better prognosis. Multidisciplinary collaboration is crucial to determine the best timing and treatment.

[Bilateral central femoral dislocation after seizure. Antiprotrusive ring and dual mobility cup as a therapeutic option. Apropos of a case]. / Luxación bilateral central femoral tras crisis convulsiva. Anillo antiprotrusivo y cotilo de doble movilidad como opción terapéutica. A propósito de un caso.

INTRODUCTION: seizures can trigger fractures and dislocations. Injuries depend on the severity, duration and type of seizure. We present a case report of a male patient who presented with a bilateral central dislocation fracture of the hip following an episode of seizure. A case rarely described in the literature with complex and unusual management. CASE REPORT: a 77-year-old man with a history of moderate cognitive impairment suffered a bilateral central dislocation of the hip in the context of a generalized epileptic seizure. Clinically on arrival at the emergency department, the patient presented shortening of the right lower extremity compared to the contralateral, external rotation and joint locking on log roll test in both extremities. An imaging study and clinical optimization were performed prior to surgery. It was performed in two stages. First the left hip on the 8th day of admission, and the right hip on the 15th. In both surgeries the same procedure was performed, with implantation of an antiprotrusive ring and a double mobility cup prosthesis with an uncemented femoral stem. In the immediate postoperative period, the patient did not present any complications associated with the surgery. At 24-month follow-up, the patient performed full weight bearing with a Harris hip score (HHS) of 77 on the right hip and 79 on the left; 12 points on the WOMAC scale. No postoperative complications have occurred so far. CONCLUSIONS: these injuries are uncommon in our daily practice, where multiple options are available to address them. In our patient, the use of arthroplasty and antiprotrusive rings offers advantages over fracture synthesis techniques, such as early mobilization with moderate functional results and few postoperative complications.

INTRODUCCIÓN: las crisis convulsivas pueden desencadenar fracturas y luxaciones. Las lesiones dependen de la severidad, duración y el tipo de crisis. Presentamos un caso clínico de un varón que presentó una fractura luxación central bilateral de cadera tras episodio de crisis convulsiva. Un caso pocas veces descrito en la literatura con un manejo complejo y poco habitual. CASO CLÍNICO: paciente de 77 años con antecedentes de deterioro cognitivo moderado que sufrió una luxación bilateral central de cadera en contexto de una crisis convulsiva generalizada. Clínicamente, a su llegada a urgencias, el paciente presentaba un acortamiento de la extremidad inferior derecha en comparación con la contralateral, rotación externa y bloqueo articular a la realización del log roll test en ambas extremidades. Se realizó estudio de imagen y optimización clínica previo a cirugía. Se realizó en dos tiempos: primero la cadera izquierda al octavo día de ingreso y la cadera derecha al decimoquinto. En ambas cirugías se realizó el mismo procedimiento mediante implantación de anillo antiprotrusivo y prótesis con cotilo de doble movilidad con vástago femoral no cementado. En el postoperatorio inmediato, el paciente no presentó ninguna complicación asociada a la cirugía. En el seguimiento a los 12 meses, el paciente realiza carga completa con un Harris hip score (HHS) de 77 cadera derecha y 79 en la izquierda; 12 puntos en la escala WOMAC. No ha presentado complicaciones postoperatorias hasta el momento. CONCLUSIONES: estas lesiones son poco comunes en nuestra práctica diaria, donde disponemos de múltiples opciones para abordarlas. En nuestro paciente, el empleo de la artroplastía y de anillos antiprotrusivos nos ofrecen ventajas respecto a las técnicas de síntesis de la fractura, como una movilización precoz y evitar desarrollo prematuro de una artrosis postraumática, con resultados buenos, funcionales y pocas complicaciones postoperatorias.

The role of the flexor carpi radialis muscle in scapholunate instability.

PURPOSE: The flexor carpi radialis (FCR) muscle has been suggested to act as a dynamic scaphoid stabilizer. Because the FCR tendon uses the scaphoid tuberosity as a pulley to reach its distal insertion onto the second metacarpal, it has been hypothesized that FCR muscle contraction generates a dorsally directed vector that resists the scaphoid from rotating into flexion. The purpose of the present study was to validate that hypothesis and clarify the role of the FCR as a dynamic scaphoid stabilizer. METHODS: Ten fresh cadaver wrist specimens were tested. A custom-designed testing apparatus was used to hold the forearm and wrist vertically, in neutral forearm rotation. A 6-degree-of-freedom, electromagnetic motion-tracking device, with sensors attached to the scaphoid, triquetrum, capitate, and radius, was used to monitor spatial changes in carpal alignment as a result of isometrically loading the FCR in 5 different wrist positions. RESULTS: In all specimens and all wrist positions, the scaphoid consistently rotated into flexion when the FCR was loaded. It also exhibited variable degrees of pronation or supination, depending on whether the wrist was in flexion or extension. When the wrist was loaded in neutral position, the scaphoid consistently supinated and the triquetrum pronated, these differences being statistically significant (p < .05). CONCLUSIONS: The scaphoid consistently rotated into flexion and supination when the FCR was loaded, while the triquetrum rotated in flexion and pronation. The positive effects of FCR muscle re-education in dynamic scapholunate instabilities can be explained not by this muscle's capability of extending the scaphoid, as has often been hypothesized, but by its ability to induce supination to the scaphoid and pronation to the triquetrum. Such opposite rotations are likely to result in a dorsal coaptation of the scapholunate joint with relaxation of the dorsal scapholunate ligament.

Assessment of Maturational Changes in White Matter Anisotropy and Volume in Children: A DTI Study.

BACKGROUND AND PURPOSE: Anisotropy is a good indicator of white matter fascicle macrostructure and organization but the interpretation of its changes with age remains difficult. The increase of WM fascicle fractional anisotropy with time and its relationship with WM fascicle volume have never been examined during childhood. We studied the maturation of associative WM fascicles during childhood using MR imaging-based DTI. We explored whether the fractional anisotropy increase of the main WM fascicles persists beyond the period of brain growth and is related to WM fascicle volume increase. MATERIALS AND METHODS: In a series of 25 healthy children, the fractional anisotropy and volume of 15 associative WM fascicles were calculated. Several regression linear mixed models were used to study maturation parameters (fractional anisotropy, volume, and total telencephalon volume) considered as dependent variables, while age and sex were independent variables (the variable identifying the different WM fascicles was considered as a repeated measure). RESULTS: In children older than 8 years of age, WM fascicle fractional anisotropy increased with age (P value = .045) but not its volume (P value = .7) or the telencephalon volume (P value = .16). The time course of WM fascicle fractional anisotropy and volume suggested that each WM fascicle might follow a specific pattern of maturation. CONCLUSIONS: The fractional anisotropy increase of several WM fascicles after 8 years of age may not result from an increase in WM fascicle volume. It might be the consequence of other developmental processes such as myelination.

The growth of the posterior cranial fossa in FGFR2-induced faciocraniosynostosis: A review.

BACKGROUND: The growth of the posterior fossa in syndromic craniostenosis was studied in many papers. However, few studies described the pathophysiological growth mechanisms in non-operated infants with fibroblast growth factor receptor (FGFR) type 2 mutation (Crouzon, Apert or Pfeiffer syndrome), although these are essential to understanding cranial vault expansion and hydrocephalus treatment in these syndromes. OBJECTIVE: A review of the medical literature was performed, to understand the physiological and pathological growth mechanisms of the posterior fossa in normal infants and infants with craniostenosis related to FGFR2 mutation. DISCUSSION: Of the various techniques for measuring posterior fossa volume, direct slice-by-slice contouring is the most precise and sensitive. Posterior fossa growth follows a bi-phasic pattern due to opening of the petro-occipital, occipitomastoidal and spheno-occipital sutures. Some studies reported smaller posterior fossae in syndromic craniostenosis, whereas direct contouring studies reported no difference between normal and craniostenotic patients. In Crouzon syndrome, synchondrosis fusion occurs earlier than in normal subjects, and follows a precise pattern. This premature fusion in Crouzon syndrome leads to a stenotic foramen magnum and facial retrusion.

Hydrocephalus and Chiari malformation pathophysiology in FGFR2-related faciocraniosynostosis: A review.

BACKGROUND: Patients with syndromic faciocraniosynostosis due to the mutation of the fibroblast growth factor receptor (FGFR) 2 gene present premature fusion of the coronal sutures and of the cranial base synchondrosis. Cerebrospinal fluid (CSF) circulation disorders and cerebellar tonsil prolapse are frequent findings in faciocraniosynostosis. OBJECTIVE: We reviewed the medical literature on the pathophysiological mechanisms of CSF disorders such as hydrocephalus and of cerebellar tonsil prolapse in FGFR2-related faciocraniosynostosis. DISCUSSION: Different pathophysiological theories have been proposed, but none elucidated all the symptoms present in Apert, Crouzon and Pfeiffer syndromes. The first theory that addressed CSF circulation disruption was the constrictive theory (cephalocranial disproportion): cerebellum and brain stem are constricted by the small volume of the posterior fossa. The second theory proposed venous hyperpressure due to jugular foramens stenosis. The most recent theory proposed a pressure differential between CSF in the posterior fossa and in the vertebral canal, due to foramen magnum stenosis.

Bilateral compartment syndrome in thighs and legs by methanol intoxication: a case report.

Methanol intoxication is infrequent even though it is easily obtainable. One of the complications in locomotor apparatus is the development of a compartment syndrome of the lower extremities. A case is reported of a 49-year-old man with a compartment syndrome in all compartments of both legs and the anterior compartment of both thighs due to methanol intoxication. The patient underwent a bilateral fasciotomy of the legs and thighs. He also had haemodialysis sessions because of acute renal insufficiency. After 4 weeks of haemodialysis, covering of the fasciotomies with cutaneous autograft and rehabilitation treatment, the patient was able to walk on his own again. Early recognition and treatment of compartment syndrome are essential to avoid complications.

[Acetabular revision at 96 years old. Is the age a limiting factor in revision surgery for hip replacement?] / Revisión acetabular a los 96 años. ¿Es la edad un factor limitante en la cirugía de revisión de prótesis de cadera?

This is a case report of a 96 year old woman with pain and functional impotence in her left hip after fall. X rays were performed and loosening of the acetabular component was appreciated. After discussion of treatment options, it was decided to cement an acetabular component for a double mobility head. There was a high rate of medical and surgical complications in revision of hip arthroplasty in elderly patients. However, it can also offer important benefits in terms of independence and quality of life and even increase life expectancy in patients with less comorbidity. In our case after six months of follow-up the patient was able to walk alone using a walker. In the literature review, age does not appear as a limit for the surgical indication. Preoperative medical optimization of the patient as well as shorter operative time and blood loss, are important factors for good results of these cases.

Se presenta el caso clínico de una paciente de 96 años con dolor e impotencia funcional en cadera izquierda tras caída. En las radiografías se aprecia un aflojamiento del componente acetabular. Tras la discusión de las opciones de tratamiento se decide intervenirla colocando un cotilo cementado para cabeza de doble movilidad. La revisión de artroplastía en pacientes ancianos tiene un riesgo considerable. No obstante, también puede ofrecer importantes beneficios en términos de independencia y calidad de vida e incluso aumentar la esperanza de vida en aquellos pacientes con menos comorbilidad. En nuestro caso tras seis meses de seguimiento la paciente es capaz de deambular de forma autónoma con andador. En la revisión bibliográfica la edad no figura como límite para la indicación quirúrgica. Una adecuada optimización preoperatoria del paciente así como una cirugía con el menor tiempo quirúrgico y sangrado son factores destacados para la buena evolución de estos casos.

Luxación bilateral central femoral tras crisis convulsiva. Anillo antiprotrusivo y cotilo de doble movilidad como opción terapéutica. A propósito de un caso / Bilateral central femoral dislocation after seizure. Antiprotrusive ring and dual mobility cup as a therapeutic option. Apropos of a case

Resumen: Introducción: las crisis convulsivas pueden desencadenar fracturas y luxaciones. Las lesiones dependen de la severidad, duración y el tipo de crisis. Presentamos un caso clínico de un varón que presentó una fractura luxación central bilateral de cadera tras episodio de crisis convulsiva. Un caso pocas veces descrito en la literatura con un manejo complejo y poco habitual. Caso clínico: paciente de 77 años con antecedentes de deterioro cognitivo moderado que sufrió una luxación bilateral central de cadera en contexto de una crisis convulsiva generalizada. Clínicamente, a su llegada a urgencias, el paciente presentaba un acortamiento de la extremidad inferior derecha en comparación con la contralateral, rotación externa y bloqueo articular a la realización del log roll test en ambas extremidades. Se realizó estudio de imagen y optimización clínica previo a cirugía. Se realizó en dos tiempos: primero la cadera izquierda al octavo día de ingreso y la cadera derecha al decimoquinto. En ambas cirugías se realizó el mismo procedimiento mediante implantación de anillo antiprotrusivo y prótesis con cotilo de doble movilidad con vástago femoral no cementado. En el postoperatorio inmediato, el paciente no presentó ninguna complicación asociada a la cirugía. En el seguimiento a los 12 meses, el paciente realiza carga completa con un Harris hip score (HHS) de 77 cadera derecha y 79 en la izquierda; 12 puntos en la escala WOMAC. No ha presentado complicaciones postoperatorias hasta el momento. Conclusiones: estas lesiones son poco comunes en nuestra práctica diaria, donde disponemos de múltiples opciones para abordarlas. En nuestro paciente, el empleo de la artroplastía y de anillos antiprotrusivos nos ofrecen ventajas respecto a las técnicas de síntesis de la fractura, como una movilización precoz y evitar desarrollo prematuro de una artrosis postraumática, con resultados buenos, funcionales y pocas complicaciones postoperatorias.

Abstract: Introduction: seizures can trigger fractures and dislocations. Injuries depend on the severity, duration and type of seizure. We present a case report of a male patient who presented with a bilateral central dislocation fracture of the hip following an episode of seizure. A case rarely described in the literature with complex and unusual management. Case report: a 77-year-old man with a history of moderate cognitive impairment suffered a bilateral central dislocation of the hip in the context of a generalized epileptic seizure. Clinically on arrival at the emergency department, the patient presented shortening of the right lower extremity compared to the contralateral, external rotation and joint locking on log roll test in both extremities. An imaging study and clinical optimization were performed prior to surgery. It was performed in two stages. First the left hip on the 8th day of admission, and the right hip on the 15th. In both surgeries the same procedure was performed, with implantation of an antiprotrusive ring and a double mobility cup prosthesis with an uncemented femoral stem. In the immediate postoperative period, the patient did not present any complications associated with the surgery. At 24-month follow-up, the patient performed full weight bearing with a Harris hip score (HHS) of 77 on the right hip and 79 on the left; 12 points on the WOMAC scale. No postoperative complications have occurred so far. Conclusions: these injuries are uncommon in our daily practice, where multiple options are available to address them. In our patient, the use of arthroplasty and antiprotrusive rings offers advantages over fracture synthesis techniques, such as early mobilization with moderate functional results and few postoperative complications.

Personalized mapping of the deep brain with a white matter attenuated inversion recovery (WAIR) sequence at 1.5-tesla: Experience based on a series of 156 patients.

OBJECTIVE: Deep brain mapping has been proposed for direct targeting in stereotactic functional surgery, aiming to personalize electrode implantation according to individual MRI anatomy without atlas or statistical template. We report our clinical experience of direct targeting in a series of 156 patients operated on using a dedicated Inversion Recovery Turbo Spin Echo sequence at 1.5-tesla, called White Matter Attenuated Inversion Recovery (WAIR). METHODS: After manual contouring of all pertinent structures and 3D planning of trajectories, 312 DBS electrodes were implanted. Detailed anatomy of close neighbouring structures, whether gray nuclei or white matter regions, was identified during each planning procedure. We gathered the experience of these 312 deep brain mappings and elaborated consistent procedures of anatomical MRI mapping for pallidal, subthalamic and ventral thalamic regions. We studied the number of times the central track anatomically optimized was selected for implantation of definitive electrodes. RESULTS: WAIR sequence provided high-quality images of most common functional targets, successfully used for pure direct stereotactic targeting: the central track corresponding to the optimized primary anatomical trajectory was chosen for implantation of definitive electrodes in 90.38%. CONCLUSION: WAIR sequence is anatomically reliable, enabling precise deep brain mapping and direct stereotactic targeting under routine clinical conditions.

[Intra cranial meningiomas and long term use of cyproterone acetate with a conventional dose in women. A report of two cases of tumor decrease after treatment withdrawal]. / Méningiomes intracrâniens et utilisation prolongée d'acétate de cyprotérone à dose conventionnelle chez la femme: à propos de deux cas de régression tumorale après arrêt du traitement.

The action of synthetic progestogens, prescribed at a conventional dose in women, for a meningioma, is still poorly understood, and could be related to progesterone receptors. We report two cases illustrating multiple meningiomas with stabilization or tumor reduction after withdrawal of cyproterone acetate originally prescribed for a long term period. We also review the influence of synthetic progestogens on meningiomas, particularly the impact of treatment withdrawal.

Analysis of ulnar variance as a risk factor for developing scaphoid nonunion.

OBJECTIVE: Ulnar variance may be a risk factor of developing scaphoid non-union. METHODS: A review was made of the posteroanterior wrist radiographs of 95 patients who were diagnosed of scaphoid fracture. All fractures with displacement less than 1mm treated conservatively were included. The ulnar variance was measured in all patients. RESULTS: Ulnar variance was measured in standard posteroanterior wrist radiographs of 95 patients. Eighteen patients (19%) developed scaphoid nonunion, with a mean value of ulnar variance of -1.34 (-/+ 0.85) mm (CI -2.25 - 0.41). Seventy seven patients (81%) healed correctly, and the mean value of ulnar variance was -0.04 (-/+ 1.85) mm (CI -0.46 - 0.38). A significant difference was observed in the distribution of ulnar variance (p<.05). These results remained significant after adjusting for age, with an OR of 0.69 (CI 0.49 to 0.95). The patients were categorized into two groups: ulnar variance less than -1mm, and ulnar variance greater than -1mm. It appears that patients with ulnar variance less than -1mm had an OR 4.58 (CI 1.51 to 13.89) with p<.007. DISCUSSION: Desai et al. concluded that radiological features of acute scaphoid fractures cannot be used to predict the likelihood of fracture union. For this reason, the existence of other risk factors was analysed. According to the results of the present study, it can be concluded that patients with scaphoid fracture and ulnar variance less than -1mm have a greater risk of developing scaphoid nonunion, OR 4.58 (CI 1.51 to 13.89) with p<.007.

Incidence and survival of childhood central nervous system tumors: A report of the regional registry of childhood cancers in Auvergne-Limousin.

INTRODUCTION: Central nervous system tumors (CNST) are the most lethal of solid tumors in childhood cancer. PATIENTS AND METHODS: We report incidence and survival data for all CNST (International Classification of Diseases for Oncology third edition, category III or Xa) recorded in children under 15 years of age by the Auvergne-Limousin cancer registry for the period 1986-2009. RESULTS: Annual incidence of all CNST was 3.27 per 100,000 and the male to female ratio was 0.95. Over 45.0% of CNST were glial. Astrocytomas (36.2%) showed the highest incidence for each age group except between 1 and 4 years where embryonal tumors were more common. For all CNST, no significant variation in incidence over time was observed for the evaluated period of 23 years (annual percent change: -0.4%, 95% CI, [-2.8-2.1]). Globally, 5 years overall survival was 67% [59-73] and had increased by more than 16% between 1986-1999 and 2000-2009, mainly due to better survival for astrocytomas, other gliomas, ependymomas and choroid plexus tumors (P=0.01). CONCLUSION: We report that the incidence of CNST in Auvergne-Limousin is similar to that in the literature and did not increase between 1986 and 2009. In addition, 5 years overall survival increased after 1999, especially for surgically treatable tumors.

[Medico-economic analysis of a neurosurgery department at a university hospital]. / Analyse médico-économique d'un service de neurochirurgie en centre hospitalier et universitaire.

OBJECTIVE: Economic and societal constraints require to take into account the economic dimension and medical performance of hospital departments. We carried out a self-assessment study, which we thought could be useful to share with the neurosurgical community. MATERIAL AND METHODS: Care and research activities were assessed from 2009 to 2013. We used institutional and assessment-body parameters in order to describe activities and perform a financial evaluation. It was a retrospective descriptive study based on the guidelines of the DHOS/O4 circular No. 2007/390 of October 29, 2007. RESULTS: The average annual, analytic income statement was +1.39 millions euros, for 63 beds with a 92% occupancy rate, including 6.7 full-time equivalent neurosurgeons (and assistants), for 2553 patients and 1975 surgeries. The average mortality rate was 2.74%. The annual mean length of stay was 6.82 days. Per year, on average 15.6% of patients were admitted in emergency and 76.9% returned home. The annual, act-related-pricing and publication-related incomes represented 77% and 0.6%, respectively of the total funding. Difficulties to find downstream beds for the most severe patients induced 1401 "waiting days" in 2012. CONCLUSION: Medico-economic analysis of a neurosurgery department at a university hospital was useful in order to take into account the care, teaching and research activities, as well as its related financial value.

The edrophonium-Hess screen test in the diagnosis of ocular myasthenia gravis.

The interpretation of the edrophonium (Tensilon) test in the diagnosis of acquired strabismus caused by myasthenia gravis has been problematic because of poorly defined endpoints and unknown sensitivity and specificity. We evaluated the endpoint criteria, dynamics, sensitivity, and specificity of binocular alignment in response to edrophonium by using the Hess screen test in ten normal control subjects, 12 nonmyasthenic patients with acquired strabismus, and in ten patients with acquired strabismus caused by ocular myasthenia gravis. A positive response to the edrophonium-Hess screen test was defined as a 50% or greater reduction in the strabismic deviation at the fixation point associated with maximum deviation within one minute of edrophonium infusion. All myasthenic patients had a 50% or greater reduction in the initial deviation within one minute of edrophonium infusion. Myasthenic patients had a statistically significant reduction in the average deviation up to 150 seconds after edrophonium infusion (P < .05 for all time periods). In contrast, with or without edrophonium infusion, control subjects had a purely horizontal fluctuation in binocular alignment of less than or equal to 2 degrees for the entire four-minute period after edrophonium infusion. None of the 12 nonmyasthenic patients tested positive to the edrophonium-Hess screen test. According to the criterion of positive response as was defined in this study, the test had a high sensitivity and specificity in this sample. These results suggest that clearly defined endpoint criteria make the edrophonium-Hess screen test a sensitive and specific quantitative study for the diagnosis of acquired strabismus caused by myasthenia gravis.

Surgical techniques for repositioning a dislocated intraocular lens, repair of iridodialysis, and secondary intraocular lens implantation using innovative 25-gauge forceps.

PURPOSE: We developed surgical techniques for the sulcus fixation of a posteriorly dislocated or secondarily implanted posterior chamber intraocular lens, repair of an iridodialysis, and the management of a decentered intraocular lens during vitreous surgery using innovative 25-gauge forceps. METHODS: The 25-gauge forceps have a curved shaft, a tip with a distal platform for grasping a suture, and a proximal groove for gripping a haptic. The forceps are inserted through a grooved scleral incision into the plane of the ciliary sulcus, facilitating manipulations such as fastening a suture loop around a haptic, repositing an intraocular lens at the ciliary sulcus, and grasping sutures during repair of a iridodialysis or implantation of a secondary intraocular lens. Seven patients underwent vitreous surgery using the new forceps. RESULTS: In all seven patients the surgical objectives were accomplished with the 25-gauge forceps without postoperative complications such as hemorrhage, hypotony, or lens decentration. CONCLUSIONS: The 25-gauge forceps are innovative instrumentation designed specifically for anterior segment applications during vitreous surgery.

Concomitant lymphangioma and arteriovenous malformation of the orbit.

An 8-year-old girl had an orbital-adnexal lymphangioma and ipsilateral orbital and middle cranial fossa arteriovenous malformations. High-resolution magnetic resonance image scanning, orbital ultrasonography, and digital subtraction angiography were used for diagnosis and preoperative assessment. Complications related to this vascular neoplasm included amblyopia, acute hemorrhage with proptosis, exposure keratitis, cosmetic deformity, and recurrent preseptal cellulitis. The girl was treated with both embolization and orbital surgery for recurrent hemorrhage and proptosis. We postulated that the coexistence of a lymphangioma and arteriovenous malformation represents an unusual and extensive maldevelopment of vascular embryogenesis.

Cinefluoroscopy of the aortic valve: an accurate noninvasive method for assessing critical aortic stenosis in the elderly.

The purpose of this study was to assess whether the degree of fluoroscopic aortic valve calcification could help identify the presence of aortic stenosis in the elderly. To evaluate the diagnostic utility of cardiac fluoroscopy for the detection of aortic stenosis (aortic valve area less than or equal to 1.0 cm 2) in patients 60 years or older, aortic valve area determined by cardiac catheterization was related to the pattern of calcification seen on cinefluoroscopy in 97 patients (mean age of 72 +/- 6 years) with suspected aortic stenosis. Dense circumferential (3+) calcification of the aortic valve was seen on cinefluoroscopy in 56 of 59 patients with an aortic valve area less than or equal to 1.0 cm 2 (sensitivity 95%), compared to 5 of 38 patients with aortic valve area greater than 1.0 cm2 (specificity 87%) (p less than .001). These findings suggest that in patients 60 years of age or older with suspected aortic stenosis, a dense circumferential (3+) aortic valve calcification on cinefluoroscopy is highly predictive of an aortic valve area less than or equal to 1.0 cm2 with a positive predictive accuracy of 91% and a negative predictive accuracy of 91%. Thus cinefluoroscopy may provide an accurate adjunct or alternative to Doppler echocardiography in the assessment of aortic stenosis in the elderly.