RESUMEN
Abdominal lymphatic malformations (LM) are rare congenital malformations of the lymphatic system, representing only 2% of all LM in newborns. They may arise from intra-abdominal solid organs (such as the liver, pancreas, kidneys, spleen, adrenal glands, and gastrointestinal tract), mesentery, omentum, and retroperitoneum. Mesenteric LM are the most commonly seen, with retroperitoneal LM being the second most common. Fetal abdominal LM could be associated with karyotypic or other abnormalities, including skin edema, hydrops fetalis, and polyhydramnios, and prenatal diagnosis and perinatal counseling for these LM are important. Prenatal ultrasound (US) and magnetic resonance imaging (MRI) have led to an increased diagnosis of abdominal LM and improved monitoring and intervention postnatally. This article provides an overview of fetal abdominal LM, including the prenatal diagnoses, differential diagnoses, comprehensive illustrations of the imaging findings, treatments, and fetal outcomes.
Asunto(s)
Abdomen/embriología , Anomalías Linfáticas/diagnóstico , Diagnóstico Prenatal/métodos , Abdomen/diagnóstico por imagen , Diagnóstico Diferencial , Femenino , Humanos , Anomalías Linfáticas/embriología , Imagen por Resonancia Magnética , Embarazo , Ultrasonografía PrenatalRESUMEN
Objective: To assess perinatal and long-term follow-up outcomes of fetal cervical masses diagnosed by three-dimensional (3D) magnetic resonance imaging (MRI) and 3D virtual models.Methods: This retrospective cohort study evaluated 15 pregnant women (age, 21-38 years) at 29-40 weeks of singleton gestation, whose fetuses exhibited congenital oral and cervical masses. These women were referred to our facility because of suspected fetal malformations on routine obstetric ultrasound, and the cases were confirmed, excluded, or complemented by MRI. Demographic data and perinatal and long-term follow-up outcomes were assessed.Results: Cervical masses were predominant in females (3:2), and the most frequent diagnosis was lymphatic-venous malformation (71%). The masses were cystic in 53.3% of the cases and solid in 46.7%. The esophagus and trachea were displaced in 46.6% of the cases. Associated malformations were diagnosed in 13.3% of the cases. There was complete agreement between prenatal MRI and postnatal diagnoses. Among the newborns, 40% had complications and 46.6% were admitted to the neonatal intensive care unit. Two infants died from complications due to epignathus. Surgical resection was performed in 33.3% of the cases, including complete resection in 26.6%. Sclerotherapy was administered to 53.3% of the cases, with complete remission achieved in 50% of these cases.Conclusion: Cervical masses diagnosed in the prenatal period had good postnatal outcomes except for cases of epignathus, which were associated with high mortality. MRI demonstrated the relationship between cervical masses and adjacent organs and allowed 3D virtual reconstruction of the airways. There was complete agreement between the prenatal diagnosis of cervical masses on MRI and postnatal diagnosis. Surgical treatment was effective in most cases, and sclerotherapy was satisfactory in cases with intrathoracic components.
Asunto(s)
Neoplasias de Cabeza y Cuello/diagnóstico por imagen , Anomalías Linfáticas/diagnóstico por imagen , Imagen por Resonancia Magnética/métodos , Teratoma/diagnóstico por imagen , Adulto , Femenino , Edad Gestacional , Neoplasias de Cabeza y Cuello/embriología , Humanos , Imagenología Tridimensional , Recién Nacido , Anomalías Linfáticas/embriología , Masculino , Embarazo , Estudios Retrospectivos , Teratoma/embriología , Ultrasonografía PrenatalRESUMEN
PURPOSE: The natural history of prenatally diagnosed lymphatic malformations (LM) remains unknown. The ability to predict growth of a lesion is important to prenatal counseling and any future prenatal intervention. We describe the prenatal growth patterns of LMs as they relate to gestational age, anatomical location, and postnatal management. METHODS: A retrospective review of fetuses prenatally diagnosed with an LM who were followed with serial ultrasounds from 2003 to 2014 was performed with attention to the growth of the lesion as indicated by the lesion volume ratio (LVR). RESULTS: Thirty patients with LM had serial ultrasound measurements between 19 and 39weeks gestation. The LVR increased in 53%, decreased in 23%, and remained stable in 23% of fetuses from the initial to the final ultrasound. Unlike other locations that demonstrated both positive and negative growth profiles, axillary lesions only demonstrated increased growth. Lesions with positive growth increased throughout gestation (peak LVR at 35±3weeks). Twenty-four patients had postnatal interventions, including surgical resection, sclerotherapy, and surgery + sclerotherapy. CONCLUSION: LMs have variable prenatal growth profiles. The majority of lesions, especially axillary LMs, will continue to grow throughout gestation and will not reach a growth plateau until the end of gestation. LEVEL OF EVIDENCE: Level III (Retrospective cohort study).
Asunto(s)
Anomalías Linfáticas/embriología , Axila , Femenino , Estudios de Seguimiento , Edad Gestacional , Humanos , Recién Nacido , Anomalías Linfáticas/diagnóstico por imagen , Anomalías Linfáticas/terapia , Embarazo , Estudios Retrospectivos , Escleroterapia , Ultrasonografía PrenatalRESUMEN
Lymphatic malformations in the neck can present as large fetal neck masses causing airway obstructions with potential perinatal demise and can pose a therapeutic challenge. We present a rare case of prenatally diagnosed large fetal neck mass with features of lymphatic malformation with intralesional hemorrhage of uncertain origin. Postnatal evaluation showed a complex cystic-solid lesion eroding through the skin with an open wound that made it clinically hard to differentiate from a teratoma. Given that malignancy could not be completely ruled out, surgery was favored. Final pathology showed a complex lymphatic malformation with intralesional hemorrhage, despite having no associated capillary, venous or arterial malformations.
Asunto(s)
Anomalías Linfáticas/patología , Enfermedades Linfáticas/patología , Cuello/patología , Adulto , Femenino , Hemorragia , Humanos , Recién Nacido , Anomalías Linfáticas/embriología , Enfermedades Linfáticas/embriología , Enfermedades Linfáticas/cirugía , Cuello/embriología , Cuello/cirugía , Embarazo , Resultado del Tratamiento , Ultrasonografía PrenatalRESUMEN
We describe a 12-year-old girl with a retroperitoneal lymphatic malformation (LM) and horseshoe kidney. The imaging characteristics of the lesions are reported. Retroperitoneal LM coexisting with horseshoe kidney is extremely rare. We hypothesized that they might share the similar etiologic factors. Imaging examinations are helpful in the definition of the 2 lesions and the relationship between them, but no characteristic findings are available to diagnose retroperitoneal LM before surgery. Surgical excision is ideal to treat LM, and the prognosis is good. Although asymptomatic horseshoe kidney need not be treated, it is important for patients to receive regular follow-up because of the propensity for various complications.