Encefalopatía por Kernicterus. serie clínica / Kernicterus (bilirubin encephalopathy): case reports

Introducción: La ictericia es común en los recién nacidos (RN). Niveles de bilirrubina a partir de 20 mg/dL (en RN de término) pueden causar parálisis cerebral coreoatetósica, hipoacusia sensorioneural, trastornos de la mirada y displasia del esmalte dental, cuadro clínico conocido como kernicterus. Objetivo: Describir 5 casos de kernicterus controlados en una Unidad de Neurología, entre los años 2002-2012. Casos clínicos: Se presentan 5 niños con edades gestacionales entre 35 y 39 semanas, con peso de nacimiento rango 2.580 y 4.250 g y niveles de bilirrubina entre 24 y 47 mg/dL. Dos RN estaban en su domicilio cuando iniciaron la encefalopatía aguda. Todos se trataron con fototerapia y en 3 casos se realizó además exanguineotransfusión. La edad del diagnóstico de kernicterus fluctuó entre los 12 días y 10 años (3 pacientes se diagnosticaron en etapa neonatal) con una resonancia magnética que demostró impregnación de ganglios basales. Todos evolucionaron con trastornos del movimiento de severidad variable. En 3 pacientes se diagnosticó hipoacusia sensorioneural y en dos hubo trastornos de la mirada. Los test psicométricos evaluaron retraso cognitivo en 3 pacientes y desarrollo normal en los restantes. Conclusión: El kernicterus en una enfermedad devastadora que aún está presente en la realidad nacional. Es una causa de parálisis cerebral prevenible, por lo cual es necesario educar a los padres, población y equipo de salud para la detección precoz y tratamiento oportuno de la hiperbilirrubinemia neonatal.

Introduction: Jaundice is common in newborn babies (NB). Bilirubin levels of 20 mg/dL or higher may cause choreoathetoid cerebral palsy, sensorineural hearing loss, eye disorders and enamel dysplasia in term infants; clinical picture compatible with kernicterus. Objective: To describe five cases of kernicterus treated at a Neurology Unit between 2002 and 2012. Case reports: Five cases of babies with gestational ages between 35 and 39 weeks, birth-weight ranging from 2580 to 4250 grams and bilirubin levels between 24 and 47 mg/dL are presented. Two infants were at home when acute encephalopathy developed, all were treated with phototherapy and 3 of them underwent exchange transfusion. The age of diagnosis of kernicterus was between 12 days to 10 years; three patients were diagnosed in neonatal period through MRI that revealed basal ganglia impregnation. All patients evolved presenting movement disorders of varying severity. Three of them were diagnosed with sensorineural hearing impairments and two presented eye disorders. Psychometric tests showed cognitive delay in three patients and normal development in the remaining children. Conclusion: Kernicterus in a devastating disease present in the national reality. It is a preventable cause of cerebral palsy; therefore, it is necessary to educate parents, population and health care professionals about neonatal hyperbilirubinemia early detection and treatment.

[Elastic stable intramedullary nailing as alternative therapy for pediatric Monteggia fractures]. / Die elastisch-stabile Marknagelung als Therapiealternative in der Versorgung kindlicher Monteggia-Frakturen.

Forearm fractures are some of the most common injuries in childhood. Monteggia fractures and Monteggia equivalents are rare injuries of the proximal forearm, but they are important because of their special biomechanics. Many authors have reported good results with excellent function if the Monteggia injuries are diagnosed primarily and treated by an axial reposition of the ulna and by an exactly repositioned proximal radioulnar joint. In our investigation, some clinical cases of pediatric Monteggia fractures were operated upon using the technique of elastic stable intramedullary nailing (ESIN). In correct indications, ESIN could be used as a minimally invasive therapeutic alternative to plate osteosynthesis for treating pediatric Monteggia injuries.

A randomized trial of the long-term effects of digital ocular compression in the late postoperative period.

PURPOSE: To determine whether digital ocular compression is a viable technique to lower intraocular pressure in patients at least 3 months after trabeculectomy. PATIENTS AND METHODS: A 6-month prospective, randomized, controlled, single-masked trial of 29 patients who underwent a trabeculectomy at the Glaucoma Service of Wills Eye Hospital. Patients were assigned to two groups: ocular compression or cheekbone compression (control group). The ocular compression group performed compression to the operated eye three times a day in the pattern of 10 seconds of pressure, 5 seconds of rest, and 10 seconds of pressure. Pressure was applied with the index finger through the closed lid to the center of the cornea. Pressure was steady and firm, but not painful. No massaging was performed. The cheekbone compression group applied pressure to the zygomatic arch with an identical style and frequency. RESULTS: At 6 months, the change in mean intraocular pressure for the ocular compression group was 0.25 mm Hg compared with -0.44 mm Hg for the control group (P = 0.7). A few patients in both groups experienced large swings in intraocular pressure and mild to moderate discomfort. CONCLUSION: Ocular compression had little to no success in the long-term management of increased intraocular pressure in the late postoperative period in this study.

Reversal of optic disc cupping after glaucoma surgery analyzed with a scanning laser tomograph.

OBJECTIVE: To detect and quantitate changes in optic nerve morphology after glaucoma surgery using the Heidelberg Retina Tomograph (HRT, Heidelberg Instruments, Heidelberg, Germany). DESIGN: Nonconsecutive observational case series. PARTICIPANTS AND INTERVENTION: The authors prospectively enrolled 21 adult patients undergoing incisional glaucoma surgery for progressive glaucoma damage. Quantitative analysis of the optic nerve head by scanning laser tomography and automated perimetry were performed before and after glaucoma surgery. MAIN OUTCOME MEASURES: Changes in optic nerve parameters were subjected to linear regression analysis with respect to percent of postoperative reduction of intraocular pressure (IOP), as well as with respect to age, refraction, preoperative cup:disc ratio, and change in visual field parameters. RESULTS: Seventeen patients had pre- and postoperative images suitable for analysis. Mean IOP at the time of image acquisition before surgery was 30.5+/-12 mm Hg, and after surgery 11.8+/-5.2 mm Hg (mean follow-up, 26+/-7 weeks). Eleven of 13 (85%) patients having IOP reduction of greater than 40% showed improvement in optic disc parameters. All four patients with less than 25% reduction in IOP showed worsening of most parameters. Changes in optic disc parameters were highly correlated with percent IOP reduction and with age. The parameters in which change most strongly correlated with percent change of IOP were cup area, rim area, cup:disc ratio, and mean cup depth (each, P<0.005). The age of the patient correlated highly with change in maximum cup depth (P<0.005). Refraction and clinically determined cup:disc ratio correlated poorly with changes in measured optic disc parameters. Clinical improvement in visual fields was correlated with the degree of improvement of cup:disc ratio (P = 0.025). CONCLUSION: Most patients showing a 40% lowering of IOP after glaucoma surgery show improved optic nerve morphology as measured by the HRT. The amount of improvement correlated highly with the percent reduction of IOP.

A color Doppler analysis of nifedipine-induced posterior ocular blood flow changes in open-angle glaucoma.

PURPOSE: This pilot study used color Doppler imaging to investigate the effects of nifedipine on the posterior ocular blood flow of patients with glaucoma progression at normal intraocular pressures. PATIENTS AND METHODS: Eighteen patients, 11 men and seven women with a median age of 61.7 years, were imaged before and 6 weeks after the initiation of 30 mg of sustained-release nifedipine (Procardia XL; produced by either Pfizer or Pratt) daily. RESULTS: There was no statistically significant change in the blood velocity of the ophthalmic artery, central retinal artery, and main nasal and temporal short posterior ciliary arteries after treatment with nifedipine. CONCLUSION: The routine use of nifedipine in patients with normal tension glaucoma progression is not supported by this study.

Simple radiographic evaluation of ileoanal pouch volume.

PURPOSE: Total colectomy and mucosal protectomy with ileal reservoir and anal pull-through is used in the treatment of ulcerative colitis or familial polyposis, with one complication being frequent bowel movements. A simple radiographic test to predict frequency of bowel movements and measure spasticity was evaluated. METHODS: Fourteen patients underwent evaluation after ileal reservoir and anal pull-through J-pouch construction. Barium sulfate suspension was instilled into the pouch via the anus in the standing position until reflux flowed into the small intestine proximal to the pouch and patients felt the urge to defecate. Total volume infused (VOLtot), volume to reflux (VOLrflx), and volume voided (VOLvoid) were measured. RESULTS: VOLvoid and the "voiding efficiency" (VOLvoid/VOLtot) correlated significantly with stool frequency (R=-0.744, P<0.002 and R=-0.754, P<0.002, respectively). Time from operation was correlated with VOLvoid and stool frequency (R=-0.723, P<0.003 and RO.573, P<0.032, respectively). CONCLUSIONS: The addition of quantitative measurements to this radiographic test gives useful information about pouch performance. Furthermore, the data imply that spasticity, as measured by voiding quantum and efficiency, rather than actual pouch volume is a major determinant of bowel movement frequency.