Tratamiento quirúrgico de macroglosia grave en el síndrome de Beckwith-Wiedemann: reporte de un caso

Abstract

Arch Argent Pediatr 2018;116(2):e341-e345 / e341Presentación de casos clínicos RESUMEN El síndrome de Beckwith-Wiedemann es una enfermedad congénita, poco frecuente, caracterizada por presentar macroglosia, defectos de la pared abdominal, hemihipertrofia, onfalocele, hipoglucemia neonatal, hernia umbilical, hepatomegalia, anomalías cardíacas, entre otros. La macroglosia se presenta en el 90% de los casos y genera problemas en la masticación, deglución, fonación y respiración, que ocasionan un cierre de la vía aérea superior. La opción terapéutica de elección es la glosectomía parcial. Se presenta a un paciente pediátrico de dos meses de nacido, con síndrome de BeckwithWiedemann y obstrucción de la vía aérea por macroglosia grave. En los antecedentes médicos, se reportaron cardiopatías congénitas, comunicación interauricular, conducto arterioso persistente, epilepsia sintomática, falla renal, hipoglicemia, traqueotomía y gastrostomía por el colapso de la vía aérea y disfagia. Se realizó la técnica quirúrgica de glosectomía de reducción anterior, con resultados favorables. Palabras clave: macroglosia, lengua, glosectomía, síndrome de Beckwith-Wiedemann. ABSTRACTBeckwith-Wiedemann syndrome is a rare congenital condition, characterized by presenting macroglossia, defects of the abdominal wall, hemihypertrophy, omphalocele, neonatal hypoglycemia, umbilical hernia, hepatomegaly, cardiac abnormalities, among others. Macroglossia occurs in 90% of cases, causing a problem in chewing, swallowing, phonation and breathing, resulting in a closure of the upper airway. The therapeutic option of choice is partial glossectomy. We present a 2-month-old pediatric patient with Beckwith-Wiedemann syndrome and area blockage due to severe macroglossia; in the medical history, congenital heart disease, interatrial communication, persistent ductus arteriosus, symptomatic

Effective Collaboration in the Surgical Management of Macroglossia in Beckwith–Wiedemann Syndrome

“…7 In our presented case, concerns raised by the patient's mother, including a large tongue, drooling, and speech difficulties, underscored the need for surgical intervention to enhance the child's condition and facilitate more effective speech therapy. 5,7 Led by the plastic surgeon, the surgical approach used the keyhole technique, combining anterior wedge resection, and central tongue reduction. Preserving the lateral neurovascular bundles, this successful procedure significantly improved speech capabilities and overall quality of life.…”

“…3,5 In this context, the plastic surgeon plays a pivotal role not only in executing surgical procedures but also in the comprehensive management of the patient's condition, as exemplified in our case report. [5][6][7][8]10 Macroglossia, a characteristic feature of BWS, often leads to functional impairments in speech, swallowing, and chewing, with potential psychological impact due to social stigmatization. 7 In our presented case, concerns raised by the patient's mother, including a large tongue, drooling, and speech difficulties, underscored the need for surgical intervention to enhance the child's condition and facilitate more effective speech therapy.…”

“…[5][6][7][8]10 Macroglossia, a characteristic feature of BWS, often leads to functional impairments in speech, swallowing, and chewing, with potential psychological impact due to social stigmatization. 7 In our presented case, concerns raised by the patient's mother, including a large tongue, drooling, and speech difficulties, underscored the need for surgical intervention to enhance the child's condition and facilitate more effective speech therapy. 5,7 Led by the plastic surgeon, the surgical approach used the keyhole technique, combining anterior wedge resection, and central tongue reduction.…”

“…Preserving the lateral neurovascular bundles, this successful procedure significantly improved speech capabilities and overall quality of life. [6][7][8]10 The multifaceted roles of plastic surgeons extend beyond surgery, encompassing collaboration with other specialists in the multidisciplinary team to develop personalized treatment plans optimizing function, aesthetics, and overall well-being. 4 Active involvement in postoperative care ensures smooth recovery and successful integration of surgical interventions within the broader management plan.…”

“…Initially referred at age 5, the patient underwent simultaneous dental rehabilitation and tongue reduction surgery using the keyhole technique, preserving lateral neurovascular bundles. [5][6][7][8] Under general anesthesia with nasal intubation, the surgical procedure began. Strategic markings on the dorsal tongue outlined the resection area, and using the keyhole technique, the tongue's length and width were reduced.…”