Mandible fracture in a child with osteogenesis imperfecta on bisphosphonates. Open versus closed treatment? A case report.

Fractures in the pediatric population are less common than in adults. Facial fractures represent only 4%-5% of total trauma in infants. Osteogenesis imperfecta is a group of genetic disorders where multiple fractures can occur even in early years of life. Long-term treatment with bisphosphonates is currently used in these children in order to increase bone strength and to alleviate symptoms. This paper reports a rare case of a traumatic fracture of the mandible in a 4-year-old child with osteogenesis imperfecta type I. This is an unusual complication in children, even in this group of patients. Open reduction and rigid fixation are not often described as the best treatment. As there are controversies about the subject, a literature review and discussion are presented.

Sarcoma mieloide intraoral: forma poco común de debut de la leucemia aguda mieloide / Intraoral myeloid sarcoma. An uncommon debut of acute myeloid leukemia

La leucemia aguda mieloide (LAM) es una neoplasia maligna con manifestaciones sistémicas, pudiendo presentar lesiones a nivel intraoral. Es el cáncer más frecuente en la infancia y constituye la primera causa de muerte por cáncer en la edad pediátrica. El sarcoma mieloide es un tumor maligno extramedular compuesto por células precursoras mieloides inmaduras. Este tumor se asocia fundamentalmente con la leucemia mieloide aguda y síndromes mielodisplásicos y, generalmente, debuta después del diagnóstico de la enfermedad primaria. La aparición del sarcoma mieloide dentro de la cavidad oral como manifestación inicial de la LAM es extremadamente rara (3-6 % de los casos según las series). Se presenta el caso de una lactante de 19 meses de edad que llevaron a Urgencias por fiebre y una tumoración gingival en el cuarto cuadrante refractaria a tratamiento antibiótico. Se solicitó un estudio ecográfico para valorar la existencia de un absceso, donde se identificó una lesión que, tras realizar una biopsia para estudio anatomopatológico, resultó ser un sarcoma mieloide. La paciente fue diagnosticada de LAM y se inició tratamiento quimioterápico. Tras tres ciclos, actualmente la paciente se encuentra libre de enfermedad

Acute myeloid leukemia (AML) is a malignant neoplasm with systemic manifestations, which may present intraoral lesions. It is the most common cancer in childhood and the leading cause of death from cancer in pediatric population. Myeloid sarcoma is a malignant extramedullary tumor composed of immature myeloid precursor cells. This tumor is mainly associated with AML and myelodysplastic syndromes and, generally, shows up after the diagnosis of the primary disease. The appearance of myeloid sarcoma within the oral cavity as an initial manifestation of AMK is extremely rare (3-6% of cases according to the series). We present a case of a 19-month infant who was taken to the Emergency Department due to fever and a gingival lesion that did not respond to antibiotic treatment. An ultrasound study was requested to assess the presence of an abscess. However, a lesion was identified and after performing a biopsy, pathologist found it was a myeloid sarcoma. The patient was diagnosed with AML and chemotherapy treatment was started. After three cycles of treatment, the patient is currently free of disease

Screening for symptoms of obstructive sleep apnea in children with severe craniofacial anomalies: assessment in a multidisciplinary unit.

OBJECTIVE: To assess the incidence of airway obstruction symptoms and the presence of obstructive sleep apnea in children with severe craniofacial anomalies by a proactive screening program using a standard questionnaire and cardiorespiratory polygraphy. PATIENTS AND METHODS: Children with severe craniofacial anomalies referred to our paediatric airway unit from February 2001 to June 2011 were eligible to be included in this retrospective, single centre study. Symptoms of airway obstruction were proactively investigated using the shorter version of the Pediatric Sleep Questionnaire (PSQ). Obstructive sleep apnea was assessed by means of cardiorespiratory polygraphy. Demographic data and reason for referral were also recorded. Primary outcomes were the prevalence of symptoms of airway obstruction and OSA. RESULTS: 44 children (24 girls) with severe craniofacial anomalies (15 Crouzon, 13 Apert, 9 Goldenhar, 5 Treacher-Collins, 2 Pfeiffer) were included, at a mean age of 5 years (range 8 months to 14 years). Reason for referral was routine follow up in 30 patients and overt OSA symptoms and signs in the remaining 14. PSQ results showed symptoms of airway obstruction in 82% of patients, being snoring the most frequent symptom (64.1%) followed by apneas (33.3%). Polygraphic studies showed inconclusive results in 8 children (18.2%), normal apnea-hypopnea index (AHI) in 16 (36.4%), mild obstructive sleep apnea in 9 (20.4%), moderate in 4 (9.1%) and severe obstructive sleep apnea in 7 (15.9%). CONCLUSIONS: Children with craniofacial anomalies have a high prevalence of symptoms of airway obstruction and obstructive sleep apnea that support a proactive screening strategy in this highly selected population.

The role of bronchoscopy in the management of patients with severe craniofacial syndromes.

PURPOSE: The purpose of this study is to assess the incidence of airway anomalies in children with severe craniofacial syndromes and to establish the role of bronchoscopy in the care of these patients. METHODS: Consecutive children with craniofacial syndromes, including both bony deformities of the skull and face, in which a bronchoscopy was performed between 1995 and 2010 were retrospectively reviewed. RESULTS: Thirty-six patients (22 boys, 14 girls; mean age, 39 months) were studied. Craniofacial synostosis was present in 21 patients (Crouzen syndrome, 11; Apert syndrome, 7, Pfeiffer syndrome, 3) and craniofacial dysostosis in 15 (Goldenhart syndrome, 8; Treacher Collins syndrome, 7). In 30 patients (83.3%), bronchoscopy was performed because of respiratory symptoms (apneic episodes, 22; respiratory distress, 13; stridor, 6; cyanosis, 1) and, in the remaining 6 (asymptomatic children), during guided tracheal intubation before a surgical procedure. Airway anomalies were found in 69.4% of patients (70% in symptomatic patients). Management consisted of tracheotomy in 13 patients, adenoidectomy/tonsillectomy in 13, glossopexy in 5, antireflux surgery in 3, and supraglottoplasty in 2. CONCLUSIONS: Airway anomalies occurred in 70% of children with severe craniofacial syndromes and respiratory symptoms. Bronchoscopy should be performed routinely in this selected group of patients, and the entire airway must be examined. Treatment should be tailored to each individual patient.

Lipoescultura facial: técnica quirúrgica y revisión bibliográfica / Facial liposculpture: surgical technique and literature review

Esta revisión presenta la infiltración de grasa autóloga como un procedimiento de gran utilidad para el relleno de las partes blandas faciales con objetivos estéticos o reconstructivos. Se ha realizado una revisión sobre los procedimientos de lipoescultura de la literatura publicada en PubMed. Se describen la técnica de Coleman, la técnica subdérmica, y la infiltración intramuscular, así como sus principales modificaciones, y los agentes estudiados y usados para incrementar la tasa de mantenimiento del injerto graso(AU)

This review shows that autologous fat grafting is useful for filling the soft tissues of the face for cosmetic and reconstructive purposes. The literature on liposculpture techniques published on PubMed was reviewed. The Coleman technique, subdermal technique, and intramuscular infiltration are described, in addition to their primary modifications, and the agents used to improve fat graft maintenance were studied(AU)

Spontaneous subperiosteal hematoma of the orbit.

Orbital subperiosteal hemorrhage is rare, with most cases occurring in young males as a result of direct facial or orbital trauma. The authors present a case of a spontaneous subperiosteal hematoma of the orbit in a 4-year-old girl.

Tratamiento ortopédico prequirúrgico de la fisura palatina con la filosofía de Latham / Presurgical orthopedic treatment of cleft palate with Latham´s philosophy

El tratamiento ortopédico prequirúrgico de la fisura palatina es untema controvertido debido a que no está claro si los efectos estéticos son o noduraderos y a que tampoco están claros los efectos que este tratamiento producesobre la futura oclusión. Existen diferentes modalidades de tratamiento.Se presentan dos casos clínicos tratados con la filosofía de Latham

Surgical orthopedic treatment of cleft palate continues to be a controversialtheme due to the fact that it is not clear if it has lasting estheticeffects and to the controversy that exists with regard to its effects on dentalocclusion. There are different treatment modalities. We present two cases treatedwith Latham´s philosophy