RESUMO

Spina bifida is the most common congenital central nervous system anomaly, resulting in lifelong neurologic, urinary, motor, and bowel disability.1 Its most frequent form is myelomeningocele, characterized by spinal cord extrusion into a sac filled with cerebrospinal fluid.1 We report the case of a 28-year-old pregnant female with no comorbidities. At 16 weeks of pregnancy, fetal ultrasound presented ventriculomegaly, cerebellar herniation, and lumbar myelomeningocele. At 22 weeks, intrauterine surgical correction was performed (Video 1). A minihysterotomy spanning approximately 3 cm was performed. The defect was opened, and the neural placode was dissected and released. This was followed by the isolation of the peripheric dura, which was molded into a tube and closed with watertight suture. Finally, the minihysterotomy was sutured and the skin was closed. The pregnancy followed its course with no complications, and the child was born at term with the lesion closed and no necessity of intensive care. Recent studies have demonstrated that infants who undergo open in utero myelomeningocele repair have better neurologic outcomes than those who are treated after birth.1,2 However, maternal morbidity is nonnegligible with the classical open surgery.2 Peralta et al2 propose a modification of the classic 6.0- to 8.0-cm hysterotomy in which the same multilayer correction of the spinal defect is performed through a 2.5- to 3.5-cm hysterotomy. This modification, called minihysterotomy, has been successfully performed outside of its creation center and was associated with reduced risks of preterm delivery and maternal, fetal, and neonatal complications.2,3.

Assuntos

Hidrocefalia , Meningomielocele , Disrafismo Espinal , Adulto , Feminino , Humanos , Recém-Nascido , Gravidez , Encefalocele/complicações , Feto/cirurgia , Hidrocefalia/cirurgia , Hidrocefalia/complicações , Meningomielocele/diagnóstico por imagem , Meningomielocele/cirurgia , Meningomielocele/complicações , Disrafismo Espinal/diagnóstico por imagem , Disrafismo Espinal/cirurgia , Disrafismo Espinal/complicações

RESUMO

BACKGROUND: Craniosynostosis are cranial deformities resulting from the early closure of 1 or more sutures. Concomitant facial changes are complex and usually result from the involvement of multiple sutures, which may lead to restriction of cranial growth and brain expansion, ocular compression, and breathing difficulties. Surgical techniques to correct syndromic craniosynostosis have improved over time, considerably reducing the rate of complications of this procedure. OBJECTIVE: To describe in detail (step-by-step) and with pertinent anatomic considerations the technique of monobloc frontofacial advancement using internal distractors. METHODS: We describe the monobloc frontofacial advancement technique with the use of internal distractors, which we use in patients with primary syndromic craniosynostosis (Apert, Crouzon, and Pfeiffer) who have major facial hypoplasia and secondary respiratory repercussions. To illustrate this technique, the procedure was performed in 2 cranial models: an adult artificial acrylic skull of normal morphology for better evidence of anatomic repairs and a 3-dimensional printed infant skull from a tomography file obtained from a child diagnosed with Apert syndrome. RESULTS: The benefits of osteogenic distraction and better surgical timing for each procedure are presented. We presented the changes and details of osteotomies performed during the procedure, as well as anatomic details and care regarding the pterygomaxillary dysjunction. CONCLUSION: Monobloc frontofacial distraction is a procedure with widely demonstrated aesthetic and functional results, and this detailed step-by-step description may improve familiarity with the anatomic landmarks of the procedure and provide a better dynamic understanding of the distraction process.

Assuntos

Acrocefalossindactilia , Disostose Craniofacial , Craniossinostoses , Acrocefalossindactilia/diagnóstico por imagem , Acrocefalossindactilia/cirurgia , Adulto , Criança , Disostose Craniofacial/diagnóstico por imagem , Disostose Craniofacial/cirurgia , Craniossinostoses/diagnóstico por imagem , Craniossinostoses/cirurgia , Ossos Faciais/cirurgia , Humanos , Lactente , Osteotomia/métodos

RESUMO

BACKGROUND: Pituicytomas originate from pituicytes, modified glial cells derived from ependymal lineage that are found in the stalk and posterior lobe of pituitary gland. The clinical presentation is similar to other pituitary tumors and imaging exams may suggest pituitary adenoma. The diagnostic is based on histopathological analysis. Surgical treatment can be performed by transsphenoidal approach with good results. The prognostic is good after total tumor resection. CASE DESCRIPTION: We describe here the case of a 17-year-old patient with a history of persistent headache and visual disturbances. Magnetic resonance imaging demonstrated an enhancing solid sellar mass suggestive of pituitary adenoma. The intrasellar mass was resected through a transsphenoidal approach and the diagnosis of pituicytoma was made after histopathological analysis. CONCLUSION: Pituicytomas are rare tumors of the neurohypophysis derived from pituicytes. Their clinical presentation resembles that of non-functional pituitary adenomas, but these two types of tumors are histologically well distinct.