Frequency of perigraft hygroma after open aortic reconstruction.

OBJECTIVE: The incidence of perigraft hygroma (PGH) development after aortic reconstruction remains poorly defined and its clinical relevance is questionable. This study was designed to establish the incidence of and determine the risk factors associated with PGH formation and its outcomes. METHODS: Patients who underwent open aortic reconstruction for either aneurysmal or occlusive disease with an expanded polytetrafluoroethylene (ePTFE) or polyester graft from 2004 to 2018 were retrospectively reviewed (n = 262). Only those who had follow-up imaging 3 or more months after repair were included. Patients with mixed graft types were excluded. PGH was defined as a perigraft fluid collection of 30 mm or greater in diameter with a radiodensity of 30 or fewer Hounsfield units on computed tomography at a minimum of 3 postoperative months. Analysis was conducted between patients with and without PGH. RESULTS: One hundred forty patients met the inclusion criteria: 88 were treated with ePTFE and 52 with polyester grafts. Twenty-three patients (16.4%) were found to have radiologic evidence of PGH. PGH developed more frequently in patients with ePTFE (21/88 [23.9%]) compared with those with polyester grafts (2/52 [3.8%]) (P = .002). Mean PGH size was 63.5 ± 36.4 mm (range, 33-153 mm) and the average time to PGH detection 27.7 months (range, 3-112 months). Baseline characteristics were similar between the groups. Patients who developed PGH had larger aneurysms, more often received ePTFE grafts, had larger graft diameters, and had bifurcated grafts. The overall mortality was 32.1% at a mean follow-up of 5.2 years. The 5-year mortality rates were similar between patients with and without PGH (26.1% vs 18.8%; P = .41). Of the 23 patients with PGH, 4 (all with ePTFE) presented with symptoms related to the PGH. The average size of symptomatic and asymptomatic PGH were 11.5 and 4.8 cm, respectively. Mortality rates overall were similar between those with and without symptoms (50.0% vs 36.8%; P = .99). CONCLUSIONS: Nearly one-quarter of aortic reconstructions with ePTFE are associated with PGH formation compared with 4% with polyester. Clinically significant PGH-related symptom development occurs in 20%. Patient education and close surveillance are warranted. Manufacturer's device modification is needed.

Fetal cystic hygroma: the importance of natural history.

OBJECTIVES: To evaluate the natural history of cystic hygroma (CH) in fetal and neonatal periods to enrich parental counselling. Ultrasonographic characteristics, associated syndromes, chromosomal anomalies, fetal cardiac pathology and life after birth were considered. STUDY DESIGN: From May 1985 to September 2010, 207 pregnancies were seen the authors' centre with suspected vascular-lymphatic fetal malformation: 156 of them had CH. Cases were followed up by telephone interview to determine fetal and neonatal outcomes. Chi-squared test was used for statistical analysis. RESULTS: Among the 156 cases of CH, the condition was septated in 75% of cases, associated with other pathologies in 74%, and retronuchal in 88%. Intrauterine regression was seen in 36% of cases, with complete disappearance in 77%. The karyotype was normal in 55% of cases. Follow-up was completed in 85 cases and revealed 54 spontaneous abortions (63%) and 31 live births (37%). Amongst these, 21 out of 31 children had a favourable outcome (68%). A negative embryo-fetal outcome was significantly associated with CH being associated with other pathologies, such as hydrops, retronuchal position and altered karyotype. Spontaneous regression or resolution of CH was associated with live births. CONCLUSIONS: The management of pregnancies with a diagnosis of fetal CH requires knowledge of natural history of the malformation for appropriate parental counselling.

Autopsy findings in fetuses with cystic hygroma: a literature review and our center's experience.

PURPOSE OF INVESTIGATION: To report our experience of autopsied cases of fetal cystic hygroma (CH) and discuss the role of fetal autopsy in genetic counseling. METHODS: A review of autopsy reports at our institution revealed 18 cases of fetal CH over a 10-year period (from 2000 to 2010). The clinical data, results of cytogenetic analysis and prenatal ultrasound findings were also retrieved and compared to the autopsy findings. RESULTS: Fetal death was due to intrauterine death in eight cases, therapeutic abortion in eight cases and spontaneous abortion in two cases. Cytogenetic analysis was available in 12 cases, and the results showed an abnormal karyotype in seven cases (5 cases of Turner syndrome and 2 cases of trisomy 21). The mean size of CH was 5.4 cm. Other malformations or findings suggestive of the cause of fetal death were diagnosed in 10/18 cases (55.6%). The most common autopsy findings were hydrops and central nervous system anomalies. The autopsy findings were in agreement with the prenatal ultrasound findings in 13/18 cases (72.2%), while in five cases (27.8%) additional findings were detected during autopsy. The most common placental abnormalities were infarcts and calcifications. CONCLUSION: In addition to prenatal diagnostic studies, fetal autopsy and pathologic examination of fetal and placental tissues may help to establish the exact cause of death and disclose important information as to the presence of various fetal malformations or placental abnormalities.

First-trimester cystic hygroma: relationship of nuchal translucency thickness and outcomes.

OBJECTIVE: To estimate the relationship between nuchal translucency thickness and abnormal karyotype, major congenital anomaly, perinatal loss, and composite abnormal outcome in fetuses with first-trimester nuchal cystic hygroma. METHODS: We performed a retrospective cohort study of first-trimester fetuses with ultrasound-diagnosed nuchal cystic hygroma collected over a 10-year period. RESULTS: There were 944 first-trimester fetuses with nuchal cystic hygroma. A karyotype abnormality occurred in 54.9% (400 of 729) of fetuses. A major congenital anomaly occurred in 28.8% (61 of 212) of fetuses with a normal karyotype. Perinatal loss occurred in 39% (115 of 295) of fetuses not electively terminated. Overall, an abnormal outcome occurred in 86.6% (543 of 627) of fetuses. After adjusting for potential confounders, every 1-mm increase in nuchal translucency thickness increased the odds of an abnormal karyotype by 44% (adjusted odds ratio [OR] 1.44, 95% confidence interval [CI] 1.29-1.60, P<.001), the odds of major congenital anomaly by 26% (adjusted OR 1.26, 95% CI, 1.08-1.47, P=.003), the odds of perinatal loss by 47% (adjusted OR 1.47, 95% CI 1.07-2.02, P=.019), and the odds of a composite abnormal outcome by 77% (adjusted OR 1.77, 95% CI 1.15-2.74, P=.01). CONCLUSION: First-trimester nuchal cystic hygroma is associated with high rates of karyotype abnormality, major congenital anomaly, perinatal loss, and abnormal outcome. As the thickness of the nuchal translucency increases, the odds of abnormal karyotype, major congenital anomaly, perinatal loss, and abnormal outcome increase.

[Increased nuchal translucency and cystic hygroma in the first trimester: prenatal diagnosis and neonatal outcome]. / Hyperclarté nucale et hygroma cervical au premier trimestre de la grossesse: diagnostic prénatal et devenir néonatal.

OBJECTIVE: A prospective study of pregnancy outcome in fetuses with increased nuchal translucency above the 95th centile (group NT) or cystic hygroma (group CH) at 10 to 14 weeks of gestation was performed. PATIENTS AND METHODS: Maternal and fetal data (nuchal translucency, caryotype, pregnancy outcome) and infant follow-up of 223 fetuses with first trimester nuchal translucency thickness (183 NT and 40 CH) were analysed. RESULTS: The measurement of nuchal translucency thickness shows a significant difference between group CH and NT (7.4+/-2.9 mm compared 3.7+/-0.8 mm). Chromosomal abnormalities were present in 55% (22/40) in group CH, with 9 cases/22 (40.9%) of Turner syndrome, compared with 14.2% (26/183) in group NT with trisomy 21 in 15 cases/26 (57.7%) (P<0.05). The rate of unfavourable outcome of pregnancy (spontaneous abortion, elective termination of pregnancy, serious structural anomalies) was 80% (32/40) in group CH compared with 18% (33/183) in group NT (P<0.05). In chromosomally normal pregnancies, the rate of fetus with no visible serious structural anomalies was 44.4% (8/18) in group CH compared with 93% (146/157) in group NT (P<0.05). DISCUSSION AND CONCLUSION: Our data show ultrasonographic evaluation of the fetal nuchal translucency thickness at the first trimester is actually indispensable. Neonatal outcome and malformation rate in fetuses with increased nuchal translucency or cystic hygroma are different, even with normal karyotype.

First-trimester septated cystic hygroma: prevalence, natural history, and pediatric outcome.

OBJECTIVE: To estimate prevalence, natural history, and outcome of septated cystic hygroma in the first trimester in the general obstetric population, and to differentiate this finding from simple increased nuchal translucency. METHODS: Patients at 10.3-13.6 weeks of gestation underwent nuchal translucency sonography as part of a multicenter clinical trial. Septated cystic hygroma cases were offered chorionic villi sampling for karyotype, and targeted fetal anatomical and cardiac evaluations. Survivors were followed up for fetal and long-term pediatric outcome (median 25 months, range 12-50 months). Cases of septated cystic hygroma were also compared with cases of simple increased nuchal translucency. RESULTS: There were 134 cases of cystic hygroma (2 lost to follow-up) among 38,167 screened patients (1 in 285). Chromosomal abnormalities were diagnosed in 67 (51%), including 25 trisomy-21, 19 Turner syndrome, 13 trisomy-18, and 10 others. Major structural fetal malformations (primarily cardiac and skeletal) were diagnosed in 22 of the remaining 65 cases (34%). There were 5 cases (8%) of fetal death and 15 cases of elective pregnancy termination without evidence of abnormality. One of 23 (4%) normal survivors was diagnosed with cerebral palsy and developmental delay. Overall, survival with normal pediatric outcome was confirmed in 17% of cases (22 of 132). Compared with simple increased nuchal translucency, cystic hygroma has 5-fold, 12-fold, and 6-fold increased risk of aneuploidy, cardiac malformation, and perinatal death, respectively. CONCLUSION: First-trimester cystic hygroma was a frequent finding in a general obstetric screening program. It has the strongest prenatal association with aneuploidy described to date, with significantly worse outcome compared with simple increased nuchal translucency. Most pregnancies with normal evaluation at the completion of the second trimester resulted in a healthy infant with a normal pediatric outcome.

The EXIT procedure: experience and outcome in 31 cases.

BACKGROUND: The EXIT (ex utero intrapartum treatment) procedure, although initially designed for reversal of tracheal occlusion in fetuses with congenital diaphragmatic hernias (CDH), has been adapted to treat a variety of fetal conditions. METHODS: A retrospective chart review of all consecutive EXIT procedures since 1996 was conducted. RESULTS: Thirty-one women underwent the EXIT procedure, with an average maternal age of 29 years (range, 20 to 38), and average gestational age of 34 weeks (range, 29 to 40). The indication was airway obstruction from fetal neck mass in 13, and reversal of tracheal occlusion from in utero clipping in 13. Singular indications included an EXIT-to-ECMO (extracorporeal membrane oxygenation) procedure for a fetus with CDH and a cardiac defect (n = 1), congenital high airway obstruction syndrome (CHAOS, n = 1), resection of a very large congenital cystic adenomatoid malformation of the lung (CCAM) on uteroplacental bypass (n = 1), unilateral pulmonary agenesis (n = 1), and thoracoomphalopagus conjoined twins. The mean duration on uteroplacental bypass (from uterine incision to umbilical cord clamping) was 30.3 plus minus 14.7 minutes (range, 8 to 66). No fetus experienced hemodynamic instability during uteroplacental bypass as recorded by fetal heart rate (FHR), pulse oximeter, and fetal echocardiography, except for one instance of reversible bradycardia from umbilical cord compression. The mean FHR and fetal saturation were 153.0 plus minus 38.5 beats per minute and 71.2% plus minus 19.9%, respectively. Five fetuses required a tracheostomy. Only 1 death occurred during an EXIT procedure because of inability to secure the airway secondary to extensive involvement by a lymphangioma. The average cord pH and pCO(2) were, respectively, 7.20 plus minus 0.11 and 63.2 plus minus 14.6. Two maternal complications occurred: bleeding from a hysterotomy site and dehiscence of an old hysterotomy scar noticed at a subsequent cesarean section. The average maternal blood loss was 848.3 plus minus 574.1 mL. CONCLUSION: The EXIT procedure was used successfully to ensure uteroplacental gas exchange and fetal hemodynamic stability during a variety of surgical procedures performed to secure the fetal airway or ensure successful transition to postnatal environment.

In utero evaluation and the long-term prognosis of living infants with cystic hygroma.

OBJECTIVE: The aim is to evaluate the natural course and the long-term prognosis of cystic hygroma following live birth. METHODS: Of 39 cases, 12 were excluded because of either artificial termination or intrauterine treatment. 27 cases were retrospectively classified into 2 groups based on the outcome, and the clinical profiles were compared. The long-term prognosis was analyzed for live births. RESULTS: 5 (18.5%) and 22 (81.5%) cases resulted in live birth and fetal demise, respectively. Both the frequency of hydrops fetalis and the maximum size of the cyst were more significantly associated with a poor prognosis (p < 0.01). Of 5 live births, 3 cases with chromosomal or structural abnormalities were handicapped. CONCLUSIONS: Hydrops fetalis and cyst size are poor prognostic factors, and fetal chromosome and structural malformations should be taken into account when predicting the long-term prognosis.

Cervical cystic hygroma: pre-, intra-, and post-operative morbidity and mortality in Zaria, Nigeria.

The management of extensive cystic hygromas in the cervical region (CCH) presents difficult challenges. A retrospective study of 41 children with CCH treated over 24 years in Nigeria was carried out; there were 28 boys and 13 girls with an age range of 3 days to 10 years (median 6.5 months). Thirty-three (80%) presented with 34 life-threatening complications including infection in 11 (27%), rapid increase in cyst size in 10 (24%), respiratory obstruction in 7 (17%), and intracystic hemorrhage in 6 (15%). Complete excision was possible in only 14 of 34 (41%) patients, and injuries to neighboring structures occurred in 6 (18%) (facial nerve 2, recurrent laryngeal nerve 1, internal jugular vein 1, parotid duct 1, pharynx 1). Postoperatively, 8 (24%) patients developed 9 complications. Wound infections occurred in 5 incompletely-excised cysts and 2 patients had respiratory obstruction. One patient with a wound infection developed edema of the tongue lasting several days and drainage was prolonged (> 6 weeks) in 1. Five patients died, 3 from respiratory obstruction and 1 each from wound and chest infection. Four patients (12%) developed a recurrence within 5 years of surgery. The pre-, intra-, and post-operative morbidity were high in this series. Although complete excision is the ideal treatment for CCH, this need not be pursued if neighboring structures are liable to injury. When cysts are incompletely excised, antibiotic prophylaxis is necessary as the incidence of wound infection is high.

Prenatally diagnosed cystic lymphangioma in infants.

BACKGROUND/PURPOSE: Prenatally diagnosed cystic lymphangioma (CL) is often associated with chromosomal anomalies, hydrops fetalis, and, in the case of cervical CL, occasionally respiratory distress just after birth. Often it is difficult to treat prenatally diagnosed CL because of the large size and associated symptoms. METHODS: Between 1988 and 1997, 11 cases of prenatally diagnosed CL were treated. Five pregnancies were terminated electively and one aborted spontaneously (nondelivered, non-D group), and another five delivered (delivered, D group). Thirteen infants nondiagnosed prenatally were also seen in the same period (prenatally nondiagnosed, without pre-D group). RESULTS: In the non-D group, massive CLs were diagnosed by ultrasound scan between 16 and 20 weeks' gestation. They occurred in the head and neck in all six cases, and four had associated pleural effusions or hydrops. In the D group, CL occurred in the face and neck in four cases. Three were born by cesarean section with neonatologists and surgeons standing by, then transferred to our hospital immediately after birth. Respiratory distress appeared in two cases; tracheostomy was carried out in one case. One patient with a huge CL in the chest and abdominal wall necessitated partial resection after OK-432 sclerotherapy. In the without pre-D group, there was CL in the face or neck in 10 cases, abdominal wall in two cases, and chest wall in one case. Respiratory distress was observed in three cases of cervical CL, and tracheostomy was required in two cases. One patient with hypoxic brain damage sequela died of pneumonia afterward. CONCLUSIONS: Prenatal diagnosis permits planned delivery, prompt postnatal resuscitation, and improve prognosis. Infants with massive CL diagnosed early in pregnancy have other associated anomalies as well as hydrops fetalis, and a poor outcome or difficult management can be expected.

Linfangioma cavernoso, diagnóstico prenatal, pronóstico y manejo / Cavernous lymphagioma, prenatal diagnosis, prognosis and management

Se presenta un raro caso de linfangioma cavernoso localizado en tórax, abdomen, región lateral derecha de cuello y brazo derecho. Representa el primer caso reportado de linfangioma cavernoso en nuestra institución. Se hacen comentarios acerca del diagnóstico prenatal, pronóstico y manejo

Cytogenetic evaluation of cystic hygroma associated with hydrops fetalis, oligohydramnios or intrauterine fetal death: the roles of amniocentesis, postmortem chorionic villus sampling and cystic hygroma paracentesis.

BACKGROUND: Pregnancies complicated by fetal cystic hygroma in the second and third trimesters are often associated with hydrops fetalis, oligohydramnios or intrauterine fetal death which may make genetic assessment more difficult. We investigated the roles of amniocentesis, postmortem chorionic villus sampling and cystic hygroma paracentesis in cytogenetic evaluation of cystic hygroma under such circumstances. METHODS: Thirty-five fetuses of cystic hygroma associated with hydrops fetalis, oligohydrammos, or intrauterine fetal death were studied. All fetuses were delivered at Mackay Memorial Hospital, Taipei, Taiwan between January, 1987 and July, 1995. Data collected included maternal age, prenatal sonograms, gestational age at diagnosis, fetal karyotypes and diagnostic procedures. RESULTS: Of 35 fetuses, all had hydrops fetalis, 19 had suffered IUFD at the time of diagnosis, and 10 had severe oligohydramnios. Cytogenetic studies were performed via amniocentesis, postmortem chorionic villus sampling, or cystic hygroma paracentesis. Successful karyotyping was achieved in 32 fetuses and the karyotype of 45,X was found in 24 fetuses. In cases with IUFD, successful karyotyping rates on cells from amniotic fluid, chorionic villi and cystic hygroma fluid were 88.9% (8 of 9), 69.2% (9 of 13) and 20% (1 of 5), respectively, whereas, in cases with living hydropic fetuses, successful karyotyping was achieved in 12 of 12 amniotic fluid and 5 of 5 cystic hygroma fluid samples. CONCLUSIONS: Amniocentesis is a better method for cytogenetic evaluation of fetal cystic hygroma associated with intrauterine fetal death than postmortem chorionic villus sampling and cystic hygroma paracentesis. However, in the case with a living hydropic fetus and oligohydramnios, cystic hygroma paracentesis appears to be a practical alternative for cytogenetic assessment.

Cystic hygroma: comparison between prenatal and postnatal diagnosis.

Antenatal diagnosis of congenital malformation often leads to opportunities for intervention in the pregnancy that may have a profound effect on the outcome of the fetus. The accuracy of the diagnostic technique is a critical factor in judging the appropriateness of such interventions. This article reports the current accuracy of prenatal ultrasound diagnosis of cystic hygroma (CH) in a region with a population of 3.5 million and an annual birth rate of 52,000. During a 32-month period (to December 1994), 113 diagnoses of CH were reported to the South East Thames Regional Congenital Malformation Registry. Eighty-nine diagnoses were made prenatally and 24 were made postnatally (at delivery or postmortem). The number of confirmed cases of CH was 52 (46% total diagnoses). The prenatal diagnoses were established at a mean gestational age of 19 weeks. Fifty-six (63%) pregnancies with a prenatal diagnosis of fetal CH were terminated; in only 25% of these was the presence of CH confirmed after termination. Overall, of the 89 prenatal diagnoses, 28 (32%) were confirmed, 45 (50%) were incorrect, and 16 (18%) could not be confirmed or refuted. Only 24% of fetuses with a prenatal diagnosis of CH were live born. The survival rate at 1 year for the live-born infants with CH in this series was 88%. The diagnosis of fetal CH has a recognised association with chromosomal abnormalities, and consequently may lead to termination of the pregnancy. A potential error rate of almost 70% in the prenatal ultrasound diagnosis of this condition requires clinicians to adopt a more cautious approach to the management of the fetus with "cystic hygroma."