RESUMEN
BACKGROUND: The ex utero intrapartum treatment (EXIT) is utilized to transition fetuses with prenatally diagnosed airway obstruction to postnatal life. We describe the unique clinical course, diagnosis, treatment, and outcomes of patients with cervical lymphatic malformation (CLM) managed with EXIT. METHODS: Review of fetuses with diagnosed CLM was delivered by EXIT (2001-2018) in a tertiary referral fetal center. Outcomes included survival, tracheostomy at discharge, neonatal course after delivery, and pulmonary hypoplasia. Data are reported as median [range] and rate (%). RESULTS: Out of 45 patients delivered by EXIT, 10 were delivered for CLM: seven had polyhydramnios, one had nonimmune hydrops, five delivered preterm, and three were emergency EXITs. The EXIT time and estimated blood loss were 125 minutes (95, 158) and 900 mL (500, 1500), respectively. Airway was secured in all. There was one neonatal death (day 8) with prematurity, sepsis, and pulmonary hypoplasia. Three out of nine were discharged with a tracheostomy. CONCLUSION: In CLM, close monitoring for structural neck involvement and development of polyhydramnios are important and may be an indication for EXIT as the optimal delivery mode. An experienced multidisciplinary team is a key factor for an effective approach to the obstructed airway in CLM.
Asunto(s)
Anomalías Linfáticas/diagnóstico , Anomalías Linfáticas/terapia , Vasos Linfáticos/anomalías , Cuello/anomalías , Atención Perinatal/métodos , Diagnóstico Prenatal , Adolescente , Adulto , Femenino , Enfermedades Fetales/diagnóstico , Enfermedades Fetales/mortalidad , Enfermedades Fetales/terapia , Humanos , Lactante , Cuidado del Lactante/métodos , Mortalidad Infantil , Recién Nacido , Anomalías Linfáticas/mortalidad , Vasos Linfáticos/cirugía , Masculino , Cuello/patología , Parto/fisiología , Embarazo , Diagnóstico Prenatal/métodos , Estudios Retrospectivos , Adulto JovenRESUMEN
BACKGROUND: Complex lymphatic anomalies are intractable lymphatic disorders, including generalized lymphatic anomaly (GLA), Gorham-Stout disease (GSD), and kaposiform lymphangiomatosis (KLA). The etiology of these diseases remains unknown and diagnosis is confused by their similar clinical findings. This study aimed to clarify the differences in clinical features and prognosis among GLA, KLA, and GSD, in Japanese patients. PROCEDURE: Clinical features, radiological and pathological findings, treatment, and prognosis of patients were obtained from a questionnaire sent to 39 Japanese hospitals. We divided the patients into three groups according to radiological findings of bone lesions and pathology. Differences in clinical findings and prognosis were analyzed. RESULTS: Eighty-five patients were registered: 35 GLA, 9 KLA, and 41 GSD. Disease onset was more common in the first two decades of life (69 cases). In GSD, osteolytic lesions were progressive and consecutive. In GLA and KLA, 18 patients had osteolytic lesions that were multifocal and nonprogressive osteolysis. Thoracic symptoms, splenic involvement, and ascites were more frequent in GLA and KLA than in GSD. Hemorrhagic pericardial and pleural effusions were more frequent in KLA than GLA. GSD had a significantly favorable outcome compared with combined GLA and KLA (P = 0.0005). KLA had a significantly poorer outcome than GLA (P = 0.0268). CONCLUSIONS: This survey revealed the clinical features and prognosis of patients with GLA, KLA, and GSD. Early diagnosis and treatment of KLA are crucial because KLA has high mortality. Further prospective studies to risk-stratify complex lymphatic anomalies and optimize management for KLA are urgently needed.
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Anomalías Linfáticas , Osteólisis Esencial , Encuestas y Cuestionarios , Adolescente , Niño , Preescolar , Supervivencia sin Enfermedad , Femenino , Humanos , Lactante , Japón/epidemiología , Anomalías Linfáticas/diagnóstico por imagen , Anomalías Linfáticas/mortalidad , Anomalías Linfáticas/terapia , Masculino , Osteólisis Esencial/diagnóstico por imagen , Osteólisis Esencial/mortalidad , Osteólisis Esencial/terapia , Radiografía , Tasa de SupervivenciaRESUMEN
OBJECTIVES: To investigate the use of indocyanine green (ICG) lymphography in the diagnosis and assessment of the severity of lymphatic dysfunction in infants and neonates with congenital lymphatic pleural effusion and ascites. STUDY DESIGN: We performed ICG lymphography on 10 neonates and infants with congenital lymphatic pleural effusion and ascites. After the subcutaneous injection of ICG, circumferential fluorescent images of lymphatic drainage channels in the extremities and trunk were identified using an infrared camera system. The lymphographic findings were classifiable into 2 patterns-those showing a linear lymphatic pattern, suggesting normal lymphatic flow, and those showing lymphatic channels with retrograde lymphatic flow (dermal backflow pattern), suggesting an abnormal lymphatic flow. We analyzed the severity of the ICG lymphography findings and the clinical outcomes. RESULTS: Based on the ICG lymphography, the severity of lymphatic dysplasia were classified into 4 categories: mild dysplasia, moderate dysplasia, severe dysplasia, and lymphatic hypoplasia. All cases diagnosed with mild (n = 3) or moderate dysplasia (n = 2) survived, and 2 of the 4 cases diagnosed with severe dysplasia died. The duration of endotracheal intubation ranged from 1 to 17 days (median, 7) in the patients with mild or moderate dysplasia and from 25 to 110 days (median, 77) in those with severe dysplasia. CONCLUSIONS: The ICG lymphographic findings were consistent with the clinical conditions. This imaging technique may be important to the future clinical management of lymphatic dysplasia in neonates and infants.
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Quilotórax/congénito , Ascitis Quilosa/congénito , Colorantes Fluorescentes , Verde de Indocianina , Anomalías Linfáticas/diagnóstico por imagen , Derrame Pleural/congénito , Quilotórax/diagnóstico por imagen , Quilotórax/mortalidad , Ascitis Quilosa/diagnóstico por imagen , Ascitis Quilosa/mortalidad , Femenino , Estudios de Seguimiento , Humanos , Lactante , Recién Nacido , Anomalías Linfáticas/complicaciones , Anomalías Linfáticas/mortalidad , Linfografía/métodos , Masculino , Derrame Pleural/diagnóstico por imagen , Derrame Pleural/mortalidad , Pronóstico , Índice de Severidad de la EnfermedadRESUMEN
OBJECTIVE: To systematically review literature pertaining to head and neck lymphatic malformation treatment and to use individual-level data presented in qualifying case series to describe the reported efficacy, complications, and functional impact of surgery and sclerotherapy. The authors evaluated whether treatment modalities differ by lymphatic malformation stage. DATA SOURCES: The terms lymphangioendothelioma, lymphangioma, cystic hygroma, and malformation were used to perform an Ovid literature search yielding 6292 references. The authors excluded references that did not involve head and neck lymphatic malformation treatment, include at least 5 patients, follow patients 1 year, define treatment modality, and state complications. REVIEW METHODS: The authors summarized data from 1205 patients reported in 41 articles. Individual-level data, in the 29 articles reporting these data, were pooled and tabulated. RESULTS: The absence of within-study comparisons of treatment success, as well as the range of reporting methods, precluded a formal meta-analysis. Most reports were case series (37; 90%). The primary treatment modality was sclerotherapy in 17 (41%), surgery in 16 (39%), or surgery combined with other modalities in 9 (20%). Individual-level data were captured for 283 patients. No treatment modality clearly resulted in superior treatment outcome. Complications were more frequent in surgical series but were reported inconsistently in sclerotherapy studies. CONCLUSION: There is evidence that lymphatic malformation treatment can be done effectively with surgery or sclerotherapy, but further study is necessary to determine which is superior. Standardized guidelines for reporting lymphatic malformation case series do not currently exist, making comparison of treatment outcomes of differing treatment modalities difficult.
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Anomalías Linfáticas/terapia , Terapia Combinada , Humanos , Terapia por Láser/métodos , Anomalías Linfáticas/mortalidad , Anomalías Linfáticas/cirugía , Complicaciones Posoperatorias , Soluciones Esclerosantes/uso terapéutico , Escleroterapia/métodosRESUMEN
Pulmonary lymphangiectasis (PL) is a very rare developmental defect of the lungs, which has previously been reported to have a very poor prognosis. However, recent reports have suggested improved outcomes, possibly as a result of advances in neonatal and pediatric intensive care medicine. We performed a retrospective study on the outcome of children with PL between 1990 and 2008 referred to our tertiary center. Seven patients with histologically proven PL were identified over the 18-year period. Six patients presented in the neonatal period and one patient at 7 months of age, all of them requiring intensive care treatment. Three neonatal patients required extracorporeal membrane oxygenation (ECMO). Six of the seven patients did not survive including all those who received ECMO. Two of the six non-survivors died of other causes than their underlying disease. The only survivor had an antenatal diagnosis of hydrops and required in utero chest drain insertion. Postnatally he was managed with maximal medical treatment for bilateral pleural effusions and persistent pulmonary hypertension. A 7-month follow-up showed this infant to be doing well. In conclusion overall the prognosis of congenital PL remains poor. The one survivor demonstrates that this condition is survivable with aggressive intervention and as current evidence suggests gradual improvement of symptoms may occur over time, and that maximal medical treatment remains warranted.