Idiopathic calcinosis cutis in an infant: The importance of a wait-and-see approach.

A healthy 2-year-old girl presented with multiple asymptomatic subcutaneous nodules on both legs. Histologically demonstrated calcium deposition within the dermis and subcutaneous tissue consistent with calcinosis cutis. Laboratory abnormalities, underlying genetic conditions, and potential triggering factors were ruled out. The lesions resolved over an 18-month period without treatment, emphasizing the importance of the wait-and-see approach in idiopathic cases of calcinosis cutis.

Seudotumor inflamatorio subglótico en un niño de 3 años / Subglottis inflammatory pseudotumour in a 3-year-old child

INTRODUCCIÓN: El cierre quirúrgico de las perforaciones septales sintomáticas a menudo conduce a resultados no satisfactorios. El conocimiento de la irrigación vascular es de suma importancia para el éxito. OBJETIVO: El manejo de las perforaciones septales constituye un reto para el cirujano. Hay descritas una gran variedad de técnicas quirúrgicas, con distintas vías de abordaje. No existen pruebas científicas que avalen un abordaje en concreto. El objetivo de esta revisión es presentar una guía práctica sobre la técnica de elección para cada caso de perforación septal. DISCUSIÓN: La inspección de la mucosa nasal, el tamaño de la perforación, la localización y, sobre todo, el soporte osteocartilaginoso son los pilares para lograr el éxito de la cirugía. Para los colgajos de deslizamiento o rotación de la mucosa del tabique es fundamental haber estudiado previamente si es posible la elevación del mucopericondrio o mucoperiosteo del septum, de lo contrario, el uso de estos colgajos no estaría indicado. Los colgajos de la pared lateral o del suelo nasal son la alternativa. El colgajo pericraneal podría estar indicado en perforaciones totales o casi totales. CONCLUSIÓN: El remanente del septum nasal y el estado del soporte osteocartilaginoso son los factores determinantes en el manejo de las perforaciones septales. Cada caso debe valorarse individualmente y la elección del abordaje se realiza según el tamaño y la localización de la perforación, la calidad de la mucosa, los antecedentes personales, la cirugía previa y la experiencia del cirujano

OBJECTIVE: The management of septal perforations is a challenge for the surgeon. A wide variety of surgical techniques have been described, with different approaches. There is no scientific evidence to support a particular approach. The objective of this review is to present a practical guide on the technique of choice for each case of septal perforation. DISCUSSION: Inspection of the nasal mucosa, the size of the perforation, the location and especially the osteo-cartilaginous support, are the pillars of a successful surgery. For the sliding or rotating flaps of the mucosa of the septum it is essential to know in advance if the elevation of the mucopericondrio or mucoperiosteo of the septum is possible, otherwise the use of these flaps would not be indicated. The flaps of the lateral wall or nasal floor are the alternative. The pericranial flap may be indicated in total or near total perforations. CONCLUSION: The remnant of the nasal septum and status of osteo-cartilaginous support are the determining factors in the management of septal perforations. Each case should be evaluated individually and the approach chosen according to the size and location of the perforation, mucosal quality, personal history, previous surgery and the experience of the surgeon

Alteraciones reversibles en la neuroimagen asociadas al tratamiento con vigabatrina en lactantes con espasmos epilépticos / Reversible alterations in the neuroimages associated with vigabatrine treatment in infants with epileptic spasms

Introducción. La vigabatrina (VGB) es un fármaco de primera línea para el tratamiento de espasmos infantiles. Diversos estudios han hallado anomalías en la resonancia magnética (RM) cerebral, que afectaban particularmente a los ganglios de la base, y especialmente en secuencias de difusión, en lactantes con espasmos que recibían VGB en altas dosis (> 100 mg/kg/día), y se ha observado la desaparición de las lesiones tras la retirada de dicho tratamiento. Casos clínicos. Se presentan dos casos clínicos con inicio de una encefalopatía epiléptica en el primer año de vida y crisis en forma de espasmos infantiles. Ambos recibieron tratamiento con distintos fármacos, entre ellos VGB hasta dosis de 200 mg/kg/día. Con 11 y 28 meses de vida, respectivamente, aparecían imágenes en la RM cerebral con una marcada hiperintensidad de señal en secuencias ponderadas en T2 en ambos pálidos, tálamos, porción posterior del tronco encefálico y núcleos dentados, que asociaban restricción en secuencias de difusión. Ambos disponían de estudios previos de RM, sin alteraciones. Tras excluir una metabolopatía subyacente, se decidió la retirada de la VGB y tres meses después, en una RM de control, se apreció la total reversibilidad de dichas lesiones. Conclusiones. Deben evaluarse con cautela los hallazgos de la RM cerebral en lactantes que reciban VGB en altas dosis para el tratamiento de espasmos. Su aparente efecto citotóxico sobre los ganglios de la base podría simular metabolopatías/ enfermedades mitocondriales. Conocer este efecto de la VGB y sus características típicas en la RM puede evitar pruebas innecesarias, como una biopsia muscular o un nuevo cribado metabólico (AU)

Introduction. Vigabatrin (VGB) is a first-line drug for the treatment of infantile spasms. Recently, several reports claim the existence of abnormalities in magnetic resonance imaging (MRI) (particularly affecting basal ganglia, and visible in T2 and diffusion sequences) in infants with spasms that were receiving high doses of VGB (> 100 mg/kg/day), which appear to be reversible after withdrawal of treatment. Case reports. We present two cases with an epileptic encephalopathy in the first year of life and seizures consisting of infantile spasms. Both were treated with several antiepileptic drugs, including VGB up to a maximum dosage of 200 mg/ kg/day. At the age of 11 and 28 months, respectively, MRI images showed marked signal hyperintensities on T2-sequences on bilateral globus pallidus, thalamus, posterior portion of the brainstem and dentate nuclei, also visible on diffusion sequences. Both had previous unaltered MRI studies. After excluding an underlying metabolic disease, VGB withdrawal is decided, appreciating the reversibility of those lesions in a prospective MRI study, three months later. Conclusions. We must consider and carefully evaluate findings on brain MRI in infants receiving VGB at high doses for treatment of spasms. The apparent cytotoxic effect on basal ganglia could simulate metabolic/mitochondrial diseases. By knowing this effect of VGB and its typical MRI features, unnecessary testing can be avoided in young infants with epileptic encephalopathies, including complex procedures like muscle biopsy or a new metabolic screening (AU)

Pediatric Disseminated Lemierre Syndrome in 2 Infants: Not Too Young for an Ancient Disease.

The Lemierre syndrome is characterized by an oropharyngeal infection, thrombosis of the internal jugular vein, and multiple septic metastases. Fusobacterium necrophorum is a common cause of it. The incidence is 0.6 to 2.3 cases per million with a mortality rate of 4% to 18%. Its fast evolution and the possibility of severe complications require a high index of suspicion for its diagnosis. We present 2 infants with Lemierre syndrome. The younger case, a 6-month-old infant, was fully recovered. In the other side, and despite an aggressive therapy, an 18-month-old infant developed cerebral palsy. We also provide a short literature review with a focus on clinical presentation and differential diagnosis in order to initiate an early and adequate therapy.

Hemicerebellitis: Report of three paediatric cases and review of the literature.

Acute inflammation of a single cerebellar hemisphere (hemicerebellitis) is a rare disorder of unknown origin. The clinical presentation is mainly characterized by headache, ataxia, dysmetria, and vomiting. In addition, some children may develop severe intracranial hypertension. The neuroimaging of hemicerebellitis raises a challenging differential diagnosis, particularly with posterior fossa tumours. Although there is no standard treatment for hemicerebellitis, its outcome is usually favourable. However, ipsilateral hemicerebellar atrophy develops in up to half of cases, and a minority of children may show persisting fine motor and/or neurocognitive sequelae. In this article, we contribute with three new reports and review a total of 35 cases of hemicerebellitis.

Inferior vena cava aneurysm in an infant presenting with a renal mass.

Aneurysm of the inferior vena cava is a rare finding in the pediatric population. We report the case of a 5-month-old infant presenting with anemia, hypertension, and dehydration in the emergency room. A renal mass was found with ultrasound and MRI and a renal tumor was first considered. Histopathologic review of the surgical specimen led to the diagnosis of aneurysmal dilatation of the vena cava.

Gorham-Stout disease and cerebrospinal fluid otorrhea.

OBJECTIVE AND IMPORTANCE: Gorham-Stout disease is a rare entity characterized by vascular proliferation causing local destruction of bone tissue. Owing to its low incidence and variable clinical presentation, the diagnosis requires a high degree of awareness by the clinician. CLINICAL PRESENTATION: We present the case of a 2-year-old boy diagnosed of Gorham-Stout syndrome with involvement of the temporal bone and secondary cerebrospinal fluid (CSF) leakage. INTERVENTION: Because of the CSF leakage, the patient required two surgical interventions. The second intervention included mastectomy and placement of a patch and a lumbar drainage device during 50 days, after which the leakage ceased. CONCLUSION: Gorham-Stout disease is a rare condition that can affect the skull base and even present with CSF leakage.