Anicteric Gallbladder Rupture with Elevated Bile Acids in Abdominal Effusion in a Dog with Cholecystitis.

Anicteric gallbladder rupture has been rarely described in veterinary medicine, and, generally, it has been related to gallbladder wall necrosis secondary to gallbladder mucocele. A 5 yr old, male, neutered Labrador retriever presented for acute onset anorexia, lethargy, and vomiting. Cholecystitis was diagnosed based on the ultrasonographic findings and bactibilia, and, consequently, medical treatment was established. Despite improvement of the patient, a focal ultrasound of the hepatobiliary tract was performed 72 hr after admission for reassessment, revealing gallbladder wall thickening and abdominal effusion. Intracellular bacteria were present in nondegenerated neutrophils, and the effusion was categorized as septic exudate, compatible with septic peritonitis. Exploratory laparotomy confirmed an anicteric gallbladder rupture potentially secondary to cholecystitis and/or previous cholecystocentesis. The patient was not icteric the day of the surgery, serum bilirubin was within normal limits, abdominal fluid bilirubin concentration was below that of serum, and no bile pigment was detected; however, bile acids were significantly higher in the abdominal effusion compared with the serum concentration. This case describes an anicteric gallbladder rupture in a dog with concomitant cholecystitis and raises the question about the sensitivity of bile acid evaluation as a tool for diagnosis of gallbladder rupture and bile peritonitis in dogs.

Ocular impairment as the first and only manifestation of Bardet-Biedl syndrome: A case report.

Bardet-Biedl syndrome is a ciliopathy mainly associated with retinal dystrophy, renal dysfunction, post-axial polydactyly, obesity, cognitive deficit and hypogonadism. The symptoms associated with retinal dystrophy do not usually appear until the first decade of life, so the diagnosis is usually delayed. Ocular involvement may be the initial form of manifestation of this syndrome, it may even be the only one, so it should be taken into account in the differential diagnosis of amblyopia in a child who does not improve despite correct compliance with treatment. A case of low visual acuity in a pediatric patient is presented as an initial manifestation that leads to the diagnosis of Bardet-Biedl Syndrome, and which is also the only symptom that the patient presents to date, despite being a multisystem disease.

Childhood-Onset Non-Infectious Uveitis in the "Biologic Era". Results From Spanish Multicenter Multidisciplinary Real-World Clinical Settings.

OBJECTIVE: To characterize and describe clinical experience with childhood-onset non-infectious uveitis. STUDY DESIGN: A multicenter retrospective multidisciplinary national web-based registry of 507 patients from 21 hospitals was analyzed. Cases were grouped as immune disease-associated (IMDu), idiopathic (IDIu) or ophthalmologically distinct. Characteristics of juvenile idiopathic arthritis-associated (non-HLA-B27-related) uveitis (JIAu), IDIu, and pars planitis (PP) were compared. RESULTS: IMDu (62.3%) and JIAu (51.9%) predominated in young females; and IDIu (22.7%) and PP (13.6%) in older children, without sex imbalance. Ocular complications occurred in 45.3% of cases (posterior synechiae [28%], cataracts [16%], band keratopathy [14%], ocular hypertension [11%] and cystoid macular edema [10%]) and were associated with synthetic (86%) and biologic (65%) disease-modifying antirheumatic drug (DMARD) use. Subgroups were significantly associated (p < 0.05) with different characteristics. JIAu was typically anterior (98%), insidious (75%), in ANA-positive (69%), young females (82%) with fewer complications (31%), better visual outcomes, and later use of uveitis-effective biologics. In contrast, IDIu was characteristically anterior (87%) or panuveitic (12.1%), with acute onset (60%) and more complications at onset (59%: synechiae [31%] and cataracts [9.6%]) and less DMARD use, while PP is intermediate, and was mostly bilateral (72.5%), persistent (86.5%) and chronic (86.8%), with more complications (70%; mainly posterior segment and cataracts at last visit), impaired visual acuity at onset, and greater systemic (81.2%), subtenon (29.1%) and intravitreal (10.1%) steroid use. CONCLUSION: Prognosis of childhood uveitis has improved in the "biologic era," particularly in JIAu. Early referral and DMARD therapy may reduce steroid use and improve outcomes, especially in PP and IDIu.

Icare-Pro Rebound Tonometer Versus Hand-held Applanation Tonometer for Pediatric Screening.

PURPOSE: To compare intraocular pressure (IOP) measurements obtained using the new rebound tonometer Icare-Pro (Icare, Tiolat Oy, Helsinki, Finland) and the hand-held version of the Goldmann applanation tonometer (Perkins; Clement Clarke, Haag-Streit, Harlow, United Kingdom) in healthy children during clinical practice. METHODS: In this prospective study, three IOP measurements were made using each tonometer in a single session, starting with the Icare-Pro. Participants were 173 non-anesthetized patients aged 1 to 16 years. Measurements were made in both eyes but only data for the right eye were entered in the analysis. Central corneal thickness, anterior chamber depth, and axial length were also measured in each patient. Data were compared by determining interclass correlation coefficients (ICCs) for each tonometer and representing the differences detected as Bland-Altman plots. RESULTS: Good linear correlation was observed between IOP readings obtained using the Perkins and Icare-Pro tonometers (r = 0.87, P < .001), although the Icare-Pro readings were slightly higher (mean IOP difference: 0.26 ± 1.58 mm Hg, P = .037). The 95% limits of agreement between the two methods were 2.8 to -3.4 mm Hg. The ICC was 0.857 (95% confidence interval: 0.810 to 0.893), indicating good agreement. For both tonometers, a low but significant correlation was detected between IOP and central corneal thickness or age. However, no correlation of IOP was found with axial length or anterior chamber depth. CONCLUSIONS: Pediatric IOP measurements determined using the new Icare-Pro rebound tonometer showed good correlation with those obtained using the hand-held Perkins applanation tonometer in a routine clinical examination with no need for general anesthesia. [J Pediatr Ophthalmol Strabismus. 2018;55(6):382-386.].

[Optic neuropathy in biotinidase deficiency]. / Neuropatía óptica por déficit de biotinidasa.

CLINICAL CASE: We report a case of a 12 year old male with vision loss (0.1 in both eyes). He also had sensorineural hearing loss (cochlear implant), asthma, dermatitis and alopecia. He was diagnosed with retrobulbar optic neuropathy, and was started on a treatment of intravenous corticosteroids, showing no improvement. A screening for congenital metabolopathies revealed a biotinidase deficiency, and treatment with biotin achieved a rapid clinical improvement. DISCUSSION: When faced with an optic neuropathy in a child, we must always look for a biotinidase deficiency, because biotin therapy is the only treatment that achieves a clinical improvement.

[Superior oblique sharpening in Brown's syndrome "plus". Results]. / Afilamiento del oblicuo superior en el síndrome de Brown "plus". Resultados.

PURPOSE: To study the results found in patients with congenital and constant Brown's Syndrome who were surgically treated with superior oblique sharpening technique. METHOD: Nine patients were selected among nineteen cases diagnosed of Brown's Syndrome in our Department for 4 years. Indications for surgery were vertical deviation in primary position and/or torticollis. The procedure of choice was the superior oblique sharpening in all cases, associated to its recession in 3 patients. RESULTS: The limitation of elevation in adduction was notably marked in 100% of the cases, improving in all of them, except in one patient (11.1%) after surgery. The preoperative hypotropia presented in 7 cases (77.8%) was resolved in 5 (71.42%) after the intervention. The initial torticollis had the same incidence than the hypotropia (77.8%: 7/9), but it completely resolved in 3 cases, and improved in 3 other with this technique. Binocular vision was recovered in 2 patients. Overall, 2 out of the 9 cases (22.22%) did not clinically improve. Intraoperative and postoperative complications were not observed, except a temporary superior oblique palsy that resolved without surgery. CONCLUSIONS: The superior oblique sharpening is a good technique which may improve the Brown Syndrome patients with primary position deviation and/or torticollis.

[Treatment of sixth nerve palsy of traumatic or tumor etiology using botulinum toxin]. / Tratamiento de las parálisis del VI par de etiología traumática y tumoral mediante toxina botulínica.

PURPOSE: To study the treatment of sixth nerve palsies of traumatic or tumoral etiologies using botulinum toxin. The factors and possible influences are analysed. METHODS: 35 patients with unilateral sixth nerve palsy are studied, 21 presenting traumatic (group I) and 14 with tumoral etiology (group II). They have been treated with botulinum toxin into the medial rectus muscle, using topical anesthesia. In group I the mean preoperative deviation was 59 diopters, in group II it was 37 diopters. The follow-up time, the initial deviation, the dose, the number of injections, the colateral effects and the final results are analyzed. RESULTS: In group I, the mean number of injections was 1.7, and the dose 10.23 international units, success was achieved in 38% of the patients, better results are obtained when treatment is injected within six months after traumatism, when less initial deviation and better initial lateral muscle function are present. In group II, the mean number of injections was 1.5 and the dose 8.21 international units, success was achieved in 57%, no influence among these factors was found. CONCLUSIONS: We consider useful to treat the traumatic palsy with botulinum toxin within six months after traumatism. The results depend on the initial deviation and the previous lateral muscle function. It is also useful as a chronic treatment and as a diagnosis procedure.

Afectación ocular como primera y única manifestación del síndrome de Bardet-Biedl: presentación de un caso / Ocular impairment as the first and only manifestation of Bardet–Biedl syndrome: A case report

El síndrome de Bardet-Biedl (SBB) es una ciliopatía que se asocia principalmente a distrofia retiniana, disfunción renal, polidactilia posaxial, obesidad, déficit cognitivo e hipogonadismo. Los síntomas vinculados a la distrofia retiniana no suelen aparecer hasta la primera década de vida, por lo que la detección tiende a retrasarse. La afectación ocular puede ser la forma inicial de manifestación de este síndrome, incluso puede ser la única, por lo que se debería tener en cuenta en el diagnóstico diferencial de una ambliopía en un niño que no mejora a pesar del correcto cumplimiento del tratamiento. Se presenta un caso de baja agudeza visual (AV) en una paciente pediátrica como manifestación inicial que lleva al diagnóstico del SBB y que es, además, el único síntoma que exhibe hasta la fecha, a pesar de tratarse de una enfermedad multisistémica.(AU)

Bardet–Biedl syndrome is a ciliopathy mainly associated with retinal dystrophy, renal dysfunction, post-axial polydactyly, obesity, cognitive deficit and hypogonadism. The symptoms associated with retinal dystrophy do not usually appear until the first decade of life, so the diagnosis is usually delayed. Ocular involvement may be the initial form of manifestation of this syndrome, it may even be the only one, so it should be taken into account in the differential diagnosis of amblyopia in a child who does not improve despite correct compliance with treatment. A case of low visual acuity in a pediatric patient is presented as an initial manifestation that leads to the diagnosis of Bardet–Biedl syndrome, and which is also the only symptom that the patient presents to date, despite being a multisystem disease.(AU)

Neuropatía óptica por déficit de biotinidasa / Optic neuropathy in biotinidase deficiency

Caso clínico: Varón de 12 años de edad que presenta disminución de la agudeza visual (0,1 en ambos ojos). Antecedentes personales: hipoacusia neurosensorial (implante coclear), asma, dermatitis y alopecia. Es diagnosticado de neuropatía retrobulbar, iniciándose tratamiento con corticoides i.v., sin apreciarse mejoría. El estudio de metabolopatías congénitas demuestra un déficit de biotinidasa, observándose una rápida mejoría clínica y al iniciar el tratamiento con biotina. Discusión: Ante un cuadro de neuropatía óptica en un niño hay que descartar el déficit de biotinidasa, ya que la biotina es el único tratamiento que consigue una mejoría clínica (AU)

Afilamiento del oblicuo superior en el síndrome de Brown 'plus'. Resultados / Superior oblique sharpening in Browns syndrome 'plus'. Results

Objetivo: Estudiar los resultados obtenidos en los pacientes con síndrome de Brown congénito intervenidos mediante la técnica del afilamiento del oblicuo superior. Método: De los 19 casos diagnosticados de síndrome de Brown en nuestra sección durante un período de 4 años (enero de 1992 y diciembre de 1995), 9 enfermos fueron seleccionados e intervenidos por presentar hipotropía en posición primaria de la mirada y/o tortícolis. En 3 casos se asoció una retroinsercción del oblicuo superior Resultados: La limitación de la elevación en aducción mejoró en todos los casos excepto en uno (11,1 por ciento). La hipotropía prequirúrgica presente en 7 enfermos (77,8 por ciento) se resolvió posteriormente en 5 (71,42 por ciento). El tortícolis inicial con una incidencia del 77,8 por ciento (7/9) desapareció por completo en 3, y mejoró en otros 3. La visión binocular se recuperó en 2 casos. Un mal resultado se obtuvo en un 22,22 por ciento (2/9) por persistir el tortícolis y la hipotropía. No se observaron complicaciones intraoperatorias ni postoperatorias, salvo una paresia transitoria del oblicuo superior que no requirió cirugía. Conclusiones: El afilamiento del tendón del oblicuo superior es una técnica eficaz en el tratamiento del síndrome de Brown en aquellos pacientes que presentan una alteración vertical en posición primaria de la mirada y/o tortícolis (AU)

Tratamiento de las parálisis del VI par de etiología traumática y tumoral mediante toxina botulínica / Treatment of the sixth nerve palsy by a traumatic or tumoral etiology using botulinum toxin

Objetivo: Valorar los resultados obtenidos en las parálisis del sexto nervio craneal de las citadas etiologías tratadas mediante toxina botulínica, y analizar los factores que pueden influir. Métodos: Se estudian 35 ojos diagnosticados de parálisis unilateral del sexto nervio, 21 de etiología traumática (grupo I) y 14 de etiología tumoral (grupo II). Todos los casos eran unilaterales. El tratamiento consistió en la inyección de toxina botulínica en el recto medial del ojo afecto, con anestesia tópica. En el grupo I, la desviación media era de 59 dioptrías, y en el grupo II de 37 dioptrías. Analizamos la desviación inicial, el inicio del tratamiento, la dosis y número de inyecciones, los efectos colaterales, el resultado final y el tiempo de seguimiento. Resultados: En el grupo I, el número medio de inyecciones fue de 1,7 y la dosis media de 10,23 unidades internacionales, con un éxito del 38 por ciento, obteniéndose mejores resultados si se inicia el tratamiento antes de los seis meses, cuanto menor es la desviación inicial y cuanto mejor es la función inicial del recto lateral. En el grupo II, el número medio de inyecciones fue de 1,5 y la dosis media de 8,21 unidades internacionales, con un éxito del 57 por ciento, sin que ninguna de las variables anteriormente citadas influyan. Conclusiones: En las parálisis de etiología traumática consideramos útil inyectar toxina antes de seis meses, el resultado dependerá de la función inicial del recto lateral y del ángulo de desviación inicial. También puede ser empleada como ayuda diagnóstica y como tratamiento crónico en aquellos pacientes que no pueden ser intervenidos por su patología de base (AU)