Resolution of hydrops fetalis in congenital cystic adenomatoid malformation after prenatal steroid therapy

Background/Purpose: Development of hydrops fetalis in fetuses with congenital cystic adenomatoid malformations (CCAM) is a significant risk factor for fetal or neonatal demise. In rare cases, resolution of CCAM has occurred, presumably owing to lesion maturation or involution. Steroid therapy, utilized for lung maturity, has been postulated to accelerate this process. The natural history of hydropic fetuses with CCAM after receiving steroid therapy is presented. Methods: The authors prospectively followed up with 3 patients who had antenatally diagnosed CCAM and nonimmune hydrops fetalis with predicted mortality. All patients declined or were not candidates for fetal intervention and were treated with standard prenatal betamethasone to increase lung maturity. Results: Three fetuses had CCAM and nonimmune hydrops fetalis diagnosed prenatally. After a course of prenatal steroids during the second trimester, all 3 patients had resolution of their hydrops and were delivered at term without respiratory distress. Conclusions: Nonimmune hydrops fetalis in fetuses with congenital cystic adenomatoid malformation is a harbinger for fetal demise. The resolution of hydrops in these patients after receiving steroid therapy is an interesting and compelling observation. Because the mechanism of this process is speculative, further studies are needed to elucidate the relationship between antenatal steroids and maturation of congenital cystic adenomatoid malformation. J Pediatr Surg 38:508-510.     

Prenatal steroids for microcystic congenital cystic adenomatoid malformations

Objective

The purpose of this study is to evaluate the effect of prenatal steroid treatment in fetuses with sonographically diagnosed congenital cystic adenomatoid malformations (CCAMs).

Methods

This was an institutional review board-approved retrospective review of 372 patients referred to the University of California, San Francisco (UCSF), for fetal CCAM. Inclusion criteria were (1) a predominately microcystic CCAM lesion sonographically diagnosed at our institution, (2) maternal administration of a single course of prenatal corticosteroids (betamethasone), and (3) no fetal surgery. CCAM volume-to-head ratio (CVR), presence of hydrops, mediastinal shift, and diaphragm eversion were assessed before and after administration of betamethasone. The primary end points were survival to birth and neonatal discharge.

Results

Sixteen patients with predominantly microcystic CCAMs were treated with prenatal steroids. Three were excluded because of lack of follow-up information. All remaining fetuses (13/13) survived to delivery and 11/13 (84.6%) survived to neonatal discharge. At the time of steroid administration, all patients had CVR greater than 1.6, and 9 (69.2%) also had nonimmune hydrops fetalis. After a course of steroids, CVR decreased in 8 (61.5%) of the 13 patients, and hydrops resolved in 7 (77.8%) of the 9 patients with hydrops. The 2 patients whose hydrops did not resolve with steroid treatment did not survive to discharge.

Conclusion

In high-risk fetal patients with predominantly microcystic CCAM lesions, betamethasone is an effective treatment. This series is a pilot study for a prospective randomized trial comparing treatment of CCAM with betamethasone to placebo.     

Prenatal diagnosis and outcome of fetal lung masses

Aim

The purpose of this study is to evaluate the accuracy of prenatal diagnostic features, particularly congenital cystic adenomatoid malformation volume ratio (CVR), in predicting outcomes for fetuses with lung masses.

Methods

The records and imaging features of all fetuses referred to the Texas Children's Fetal Center with a fetal lung mass between July 2001 and May 2010 were reviewed retrospectively. Data collected included gestational age (GA) at diagnosis, fetal magnetic resonance imaging findings, CVR, mass size, nature of fetal treatment, surgical findings, pathology, and outcome. Data were analyzed for predicting development of hydrops or the need for fetal therapy using receiver operating characteristic curves.

Results

Of 82 fetuses (41 male) evaluated for a lung mass, 53 (65%) were left-sided (1 bilateral), and the mean (SD) GA at diagnosis was 21.5 (4.3) weeks. Seventy-three fetuses underwent fetal magnetic resonance imaging at a mean (SD) GA of 26.1 (4.6) weeks. Thirteen fetuses (16%) had fetal treatment. Four fetuses with hydrops underwent open fetal surgical resection, and 3 survived. Six fetuses with large lung masses and persistent mediastinal compression near term underwent ex-utero intrapartum therapy-to-resection procedures, and 3 fetuses with hydrops underwent serial thoracentesis. Congenital cystic adenomatoid malformation volume ratio correlated strongly with the development of hydrops and the need for fetal therapy with an area under the receiver operating characteristic curve of 0.96 (P < .0001) and 0.88 (P < .0001), respectively. Of 18 fetuses with a CVR greater than 2.0 compared with 2 (3%) of 60 with a CVR of 2.0 or less, 10 (56%) required fetal intervention (P < .0001).

Conclusion

Congenital cystic adenomatoid malformation volume ratio correlates strongly with the development of fetal hydrops and the need for fetal intervention. A threshold value of 2.0 yields the most powerful statistical results.     

Fetal cystic adenomatoid malformation: prenatal diagnosis and natural history

We studied the natural history and pathophysiology of congenital cystic adenomatoid malformation (CCAM) detected prenatally by ultrasound in twelve fetuses. Two types of fetal CCAM can be distinguished by gross anatomy, ultrasound findings, and prognosis. Microcystic lesions are usually associated with fetal hydrops and have a poor prognosis (five cases with one survivor). Antenatal diagnosis, maternal transport, and immediate thoracotomy after birth allowed the first reported survival of a newborn with a large microcystic CCAM. Macrocystic lesions are not usually associated with hydrops and have a favorable prognosis (five of seven survived). We conclude that fetuses with hydrops are at high risk for fetal or neonatal demise without intervention. Fetuses with CCAM but without hydrops have a good chance for survival with maternal transport, planned delivery, and immediate neonatal resuscitation and surgery.     

Esophageal ligature in experimental gastroschisis

Background/Purpose: Recently, the authors have shown that in human fetuses suffering from gastroschisis, there is an amniotic fluid inflammatory response and that amniotic fluid exchange designed to disrupt the inflammatory loop seems to have a favorable impact on outcome. The authors, therefore, designed in the fetal sheep a model of gastroschisis in which amnioinfusion significantly improved the deleterious process. They hypothesized that regurgitation and presence of digestive enzyme in the amniotic fluid triggers and maintins the process of inflammation. Methods: To test this hypothesis, the authors used their model of gastroschisis in the fetal lamb combined with esophageal ligation and compared it with gastroschisis with or without amnioinfusion. Results: Of 34 fetuses operated on at midgestation (days 70 through 80), 11 died in utero or were stillborn, 8 had gastroschisis and amnioinfusion, 8 had gastroschisis and no amnioinfusion, and 7 had gastroschisis and esophageal ligation. There were 9 control fetuses. Fetuses were killed at day 145 by cesarean section. Extraabdominal bowels with fibrous peel were processed for histologic examination. Thickness of bowel muscularis (micrometers) was 82.7 [plusmn] 19 for controls, 159 [plusmn] 56 for the nonamnioinfused fetuses, 126 [plusmn] 21 for the amnioinfused fetuses (P = .001), and 240 [plusmn] 225.8 for fetuses with esophageal ligature combined with gastroschisis. The same results were obtained for thickness of serous fibrosis and plasma cell infiltration. Assay of amniotic fluid ferritin, lipase, and protein showed that only amnioinfusion lowered ferritin and protein to levels similar to those of controls, thus, illustrating its preventive effect on inflammation and that esophageal ligature did not prevent digestive enzyme presence in the amniotic fluid. Conclusion: In this model of gastroschisis in the fetal sheep, ligature of the esophagus, which was supposed to protect the extruded bowel by preventing oral regurgitation of digestive enzymes and by creating a relative hydramnios, did not improve the inflammatory and deleterious process, which is best prevented by amnioinfusion. J Pediatr Surg 37:1160-1164.     

Meconium dependence of bowel damage in gastroschisis

Background/Purpose: Increasing evidence of physiologic in utero defecation supports the hypothesis that bowel damage in gastroschisis may be meconium dependent. In this study, the author investigated the role of meconium on parameters of bowel damage in a fetal rat model of gastroschisis. Methods: Pregnant rats underwent laparotomy at 18 1/2 days gestational age (GA). There were 4 experimental groups of 11 fetuses each; the G_M group consisted of fetuses with isolated gastroschisis and was considered to have moderate meconium contamination of the amniotic fluid (MCAF); the G_L group consisted of fetuses with gastroschisis and anal ligation, performed to prevent MCAF; the G_H group consisted of fetuses with gastroschisis and colon perforation, performed to increase MCAF; and the Sham group consisted of sham operated controls. All fetuses were harvested by cesarean section at 21 1/2 days GA, and the fetal intestine was assessed for peel, intestinal length, intestinal weight per unit length, and histologic appearance. Results: The authors achieved the following fetal survival rates: G_M group, 91% (10 of 11); G_L group, 78% (7 of 9, the ligation was not successful in 2 fetuses); G_H group, 82% (9 of 11). Sham group, 100% (11 of 11). Intestinal length was decreased in fetuses with gastroschisis, and this reduction was related directly to the grade of MCAF (Sham, 18.4 [plusmn] 0.6; G_L, 11.5 [plusmn] 0.5; G_M, 10.2 [plusmn] 0.6; G_H, 9.1 [plusmn] 0.6 cm; P [lt ] .01). In contrast, intestinal weight per unit length increased in fetuses with gastroschisis, and this increase was related directly to the grade of MCAF (Sham, 7.8 [plusmn] 0.5; G_L, 9.4 [plusmn] 0.5; G_M, 11.3 [plusmn] 0.5; G_H, 16.9 [plusmn] 0.7 mg/cm; P [lt ] .01). In comparison with the G_M group, the degree of peel coverage and bowel adherence were increased markedly in the G_H group, whereas the fetuses of the G_L group had neither peel nor bowel adherence. Conclusions: All bowel damage parameters were affected by MCAF supporting the hypothesis that bowel damage in gastroschisis is at least partially dependent on meconium exposure. Further research is required to clarify other factors that contribute to bowel damage and to identify risk factors that may allow prenatal identification of severely affected fetuses.     

Cystic anomalies of biliary tree in the fetus: is it possible to make a more specific prenatal diagnosis?

Background/Purpose: Biliary tree cystic malformations (BCM) occur rarely. Current progress in ultrasonography makes it possible to identify them prenatally. The aim of this study was to analyze retrospectively prenatal ultrasound patterns of 5 consecutive subhepatic cysts in an effort to differentiate the BCM before birth and onset of symptoms. Methods: The prenatal ultrasound patterns of 5 consecutive BCM were correlated with the postnatal diagnosis. Results: Case 1 showed a hyperechogenic cyst that underwent enlargement during fetal life; the diagnosis at birth was an obstructed choledochal cyst (CC). In cases 2 and 3, an anechoic cyst with small, stable dimensions during pregnancy was identified; a biliary atresia (BA) type III-C was confirmed at birth. A slightly hyperechogenic cyst with no increase in size at repeated prenatal scans, was observed in cases 4 and 5; a CC and a cystic lesion at the confluence of the hepatic ducts (without obstruction) were diagnosed postnatally, respectively. Conclusions: The ultrasound scan pattern and size allow to make a prenatal differential diagnosis of BCM. Anechoic, small cyst in the hepatic hilum is highly suspicious for BA. Large, echoic or enlarging cyst is strongly suggestive of [ldquo ]obstructed[rdquo ] CC. Echoic, small and stable cyst is more suggestive of [ldquo ]unobstructed[rdquo ] CC. J Pediatr Surg 37:1191-1194.     

Deficient motor innervation of the sphincter mechanism in fetal rats with anorectal malformation: a quantitative study by fluorogold retrograde tracing

Background/purpose: Deficiency of motoneuron innervation to the sphincter mechanism has been described in patients with anorectal malformation. Whether this event is primary or secondary remains unclear.Methods: The authors quantified the motoneuron innervation of the sphincter mechanism by Fluorogold (FG) retrograde tracing experiment in fetal rats with anorectal malformation. Anorectal malformation was induced in rat fetuses by ethylenethiourea (ETU). Serial longitudinal sections encompassing the whole width of lumbosacral spinal cord were examined. The number of FG-labelled motoneurons were scored and compared between male fetuses with or without malformation in the ETU-fed group and normal controls.Results: The number of FG-labelled motoneurons in the fetuses without defect, with imperforate anus (IA), with neural tube anomalies (NTA), with combined IA and NTA, and normal controls were determined to be (mean ± SEM) 109.13 ± 37.88, 55.05 ± 25.85, 48.20 ± 30.34, 54.43 ± 28.55, and 135.22 ± 28.78, respectively. FG-labelled motoneurons in the fetuses with IA, NTA, and combined IA and NTA are significantly fewer than that in fetuses without defects (P < .05) and in normal controls (P < .005).Conclusions: These findings suggest that defective motoneuron innervation to the sphincter mechanism is a primary anomaly that coexists with the alimentary tract anomaly in anorectal malformation during fetal development. The intrinsic neural deficiency is an important factor likely to contribute to poor postoperative anorectal function despite surgical correction of anorectal malformation.     

Congenital adenomatoid malformation of the lung: current management and prognosis

A retrospective 10-year review of congenital adenomatoid malformation (CAM) included 10 cases diagnosed in utero by ultrasound and 13 cases that presented postnatally. Two prenatally diagnosed cases were aborted because of associated lethal anomalies. All remaining patients underwent resection. Up to one third of all cases, whether diagnosed prenatally or postnatally, were asymptomatic. Resection is recommended to avoid respiratory distress, infection, or associated malignancy. There were 5 nonsurvivors, including 2 therapeutic abortions and 3 who died postoperatively. All had either polyhydramnios or ascites. All patients who died postoperatively had a respiratory arrest at birth and underwent immediate lobectomy. All died on the first day of life after a brief period and were found to have associated pulmonary hypoplasia. One had undergone a prenatal transthoracic cyst aspiration at 34 weeks gestation in an attempt to allow lung growth and prevent premature labor. Prenatal ultrasound findings suggestive of poor prognosis included polyhydramnios, ascites, mediastinal shift, and noncystic type III CAM. However, there is a spectrum of severity of CAM. The lesion can either regress and be asymptomatic at birth, or it can progress to cause either fetal death from hydrops or neonatal death caused by associated pulmonary hypoplasia. These findings should be considered in prenatal counseling for CAM.     

Characteristics of congenital cystic adenomatoid malformations associated with nonimmune hydrops and outcome

Background/Purpose

In fetuses with congenital cystic adenomatoid malformations of the lung (CCAMs), hydrops fetalis and large masses are associated with poor outcomes. This study attempts to (1) determine sonographic features (in addition to large size) that correlate with hydrops and (2) characterize the features that correlate with outcome among hydropic fetuses.

Method

Charts and sonograms of fetuses with large, unilateral CCAMs were retrospectively reviewed. Mass features evaluated included laterality, macrocystic/microcystic, cystic/solid predominance, degree of mediastinal shift, retrocardiac component, diaphragm eversion, polyhydramnios, and mass-thorax ratio (MTR). Features of hydrops included degree of ascites, scalp and integumentary edema, pleural/pericardial effusion, and placentomegaly.

Results

Thirty-six fetuses with large CCAMs were studied: 27 with and 9 without hydrops. Three sonographic features were significantly associated with hydrops: MTR of at least 0.56, cystic predominance of mass, and eversion of hemidiaphragm. Of 27 fetuses with hydrops, 10 (37%) demonstrated all 3 features compared with none in those without hydrops (P = .04). All 9 nonhydropic fetuses were expectantly managed, and 100% survived. In the hydropic group, none of the expectantly managed fetuses survived, and 10 (43%) of the 21 fetuses who underwent fetal intervention survived.

Conclusion

Three features of large CCAMs were significantly associated with hydrops: MTR, cystic predominance, and diaphragm eversion. Identification of these features will allow clinicians to accurately predict which fetuses may warrant closer follow-up and possible treatment.     

Management of the fetus with a cystic adenomatoid malformation

Prenatal detection and serial sonographic study of fetuses with congenital cystic adenomatoid malformation (CCAM) now makes it possible to define the natural history of this lesion, determine the pathophysiologic features that affect clinical outcome, and formulate management based on prognosis. In a series of 22 prenatally diagnosed cases, the overall prognosis depended on the effects of the lung mass and the consequent physiologic derangement: A large mass may cause mediastinal shift, hypoplasia of normal lung tissue, polyhydramnios, and cardiovascular compromise leading to fetal hydrops. Two types of CCAM were distinguished by gross anatomy, ultrasonographic findings, and prognosis. Microcystic lesions (cysts < 5 mm; solid appearance) are often associated with fetal hydrops and have a poor prognosis. Macrocystic lesions (single or multiple cysts > 5 mm) are not usually associated with hydrops and have a more favorable prognosis. Four initially large lesions partially disappeared on serial prenatal sonography, suggesting that improvement can occasionally occur during fetal life. The finding that fetuses with hydrops are at high risk for fetal or neonatal demise led to fetal surgical resection of the massively enlarged pulmonary lobe (fetal lobectomy) in two cases. Fetuses with CCAM but without hydrops have a good chance for survival with maternal transport, planned delivery, and immediate neonatal resuscitation and surgery.

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Resumen La detección prenatal y el estudio sonográfico seriado de fetos con malformación adenomatosa quística congénita (MAQC) actualmente permite definir la historia natural de esta lesión, determinar las características patofisiológicas que afectan el resultado clínico y formular un manejo basado en pronóstico. En una serie de 22 casos diagnosticados en el período prenatal, el pronóstico global dependió de los efectos de las masas pulmonares y la consecuente alteración fisiológica: una gran masa puede producir desviación mediastinal, hipoplasia del tejido pulmonar normal, polihidramnios y compromiso cardiovascular resultante en hidrops fetal. Los dos tipos de MAQC pudieron ser distinguidos por la anatomía macroscópica, los hallazgos ultrasonográficos y el pronóstico. Las lesiones microquísticas (quiste <5 mm; apariencia sólida) generalmente están asociadas con hidrops fetal y tienen un pronóstico pobre. Las lesiones macroquísticas (quistes únicos/múltiples >5 mm) usualmente no están asociados con hidrops y exhibieron un pronóstico más favorable. Cuatro lesiones inicialmente de gran tamaño desaparecieron subsiguientemente bajo observación con sonografía seriada prenatal, lo cual sugiere que ocasionalmente se presenta mejoría en el curso del desarrollo fetal. El hallazgo de que los fetos con hidrops conllevan un alto riesgo de muerte fetal o neonatal ha llevado a la resección quirúrgica prenatal del lóbulo pulmonar de volumen masivo (lobectomía fetal) en dos casos. Los fetos con MAQC pero libres de hídrops tienen una buena probabilidad de sobrevivir mediante el transporte materno, un parto programado y resucitación y cirugía neonatales inmediatas.

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Résumé L'étude échographique répétée des foetus ayant la maladie kystique (adénomateuse) congénitale du poumon (MKACP) et la détection prénatale ont contribué à augmenter nos connaissances de l'histoire naturelle de la lésion, ont aidé à préciser les données physiopathologiques qui peuvent influencer l'évolution clinique et déterminer une démarche thérapeutique basée sur le pronostic. Dans cette série de 22 cas, diagnostiqués avant la naissance, le pronostic était basé sur l'effet de masse pulmonaire et les conséquences physiologiques: une masse importante peut en effet être responsable d'une déviation du médiastin, de l'hypoplasie des tissus pulmonaires normaux, d'une hydramnios, et d'une décompensation cardiovasculaire dont le résultat global est une anasarque foetale. Selon les données anatomiques macroscopiques, échographiques et pronostiques, on peut distinguer deux types de MKACP. Les kystes microscopiques (kystes < 5 mm, allure solide) sont souvent associés à une anarsaque foetale et un mauvais pronostic. Les lésions macroscopiques (kystes solitaires ou multiples > 5 cm) ne sont pas habituellement associés à une anasarque et le pronostic est généralement plus favorable. Dans cette série, quatre lésions larges initialement, ont diminué de taille au cours de la surveillance échographique, suggérant qu'une amélioration spontanée est toujours possible pendant la vie foetale. En raison du mauvais pronostic associé à une anasarque foetale avec mort foetale ou néonatale, on a été amené à pratiquer une résection chirurgicale élargie des lobes pulmonaires dans deux cas. Les foetus avec MKACP mais sans anasarque ont de bonnes chances de survie à condition d'entourer la maternité, la naissance et les premiers jours de l'enfant avec des soins chirurgicaux et de réanimation adaptés.

     

Congenital diaphragmatic hernia and hydrops: a lethal association?

Background/Purpose: Nonimmune hydrops in the fetus is a finding that often portends death. The association and prognosis of fetuses with congenital diaphragmatic hernia (CDH) and hydrops is not known. Methods: A retrospective review of all prenatally diagnosed cases and referrals of CDH was performed. Variables analyzed included gestational age at diagnosis and delivery, side of hernia, presence of associated anomalies and hydrops, and neonatal outcome. Results: Since 1993, 474 prenatal referrals for CDH have been made. One hundred seventy-five were evaluated; 15 fetuses had hydrops (9%). Five patients had CDH, hydrops, and associated lethal anomalies. In the remaining 10 patients, 6 of the diaphragmatic defects were right-sided and 4 were left-sided. All except one had a major portion of the liver herniated into the chest. Six fetuses had prenatal intervention. Five neonates died shortly after birth. There were 5 long-term survivors; all received prenatal intervention. Conclusions: The association of CDH and hydrops is rare but often results in fatality. Hydrops appears to be associated with liver in the hernia, right-sided lesions, and lethal anomalies. Fetal intervention can be performed successfully in patients with CDH and hydrops, and may improve long-term survival rate in this group.     

Defining hydrops and indications for open fetal surgery for fetuses with lung masses and vascular tumors

PurposeThe aim of this study was to identify the most accurate prenatal predictors of outcomes and need for fetal surgery for fetuses with high-risk lung masses and vascular tumors.MethodsThe records of all fetuses with high-risk lung mass (congenital cystic adenomatoid malformation-volume ratio > 1.6 or findings of hydrops) and vascular tumor evaluated between July 2001 and March 2011 were reviewed retrospectively. Hydrops was defined as accumulation of fluid in 2 or more compartments.ResultsOf fetuses with high-risk lung mass, hydrops was identified in 46% (11/24). Fetuses with hydrops and an abnormal echocardiogram (n = 8) demonstrated poor survival without fetal surgery (13%) compared with 100% survival in fetuses with hydrops and a normal echocardiogram (n = 3; P = .02). Of 21 fetuses with vascular tumor (11 sacrococcygeal and 8 cervical teratomas; 2 hemangioendotheliomas), hydrops was identified in 29% and an abnormal echocardiogram in 57%. All fetuses with hydrops had an abnormal echocardiogram and either died (n = 5) or required fetal surgery (n = 1). However, all fetuses with abnormal echocardiograms alone (n = 7) survived without fetal intervention.ConclusionsFor fetuses with lung mass, an abnormal echocardiogram in the setting of hydrops is the best predictor of mortality and need for fetal surgery. For fetuses with vascular tumor, hydrops in the setting of high-output physiology best predicts demise and need for fetal surgery.     

Large fetal congenital cystic adenomatoid malformations: growth trends and patient survival

Purpose

The prognosis for fetuses with large congenital cystic adenomatoid malformations (CCAMs) remains uncertain. This study examined the natural history of large fetal CCAMs managed expectantly at a major referral center.

Methods

A 5-year retrospective review was conducted on fetuses diagnosed with a thoracic lesion (n = 59). Large CCAMs were identified on prenatal imaging and followed longitudinally. Perinatal outcomes were assessed.

Results

Twelve (20.3%) fetuses had large CCAMs in the absence of other congenital anomalies. Peak CCAM size occurred at 25.3 ± 3.6 weeks' gestation. Serial magnetic resonance volumetry demonstrated a trend toward decreasing CCAM mass volume relative to thoracic cavity volume over time. Overall, 6 patients, including 3 with signs of early hydrops, showed a marked regression of their lesions relative to estimated fetal weight. Five fetuses required an emergent intervention postnatally, including extracorporeal membrane oxygenation support (n = 1), cyst aspiration (n = 1), and lung resection (n = 5). Overall survival was 75%, with severe hydrops before 30 weeks seen in all 3 deaths.

Conclusion

Large fetal CCAMs tend to peak in size at 25 weeks' gestation and are characterized by in utero diminution relative to overall fetal growth. The prognosis for most fetuses with large CCAMs remains quite favorable under careful perinatal management.     

Mainstem bronchial atresia: a lethal anomaly amenable to fetal surgical treatment

Purpose

The purpose of this study was to review the unique imaging characteristics, prenatal course, and outcomes for fetuses with mainstem bronchial atresia (MBA).

Methods

The records of all patients referred for a fetal lung malformation from 2001 to 2012 and the medical literature were reviewed to identify cases of MBA.

Results

Of 129 fetuses evaluated, 3 were diagnosed prenatally with right-sided MBA. The first had a CCAM-volume ratio (CVR) of 9, hydrops, mirror syndrome, and preterm delivery of a nonviable fetus. The second (CVR 2.6) had ascites, preterm delivery at 34-weeks, and neonatal demise. The third fetus (CVR 5.7) presented with hydrops at 21-weeks, prompting fetal pneumonectomy. Postoperatively, hydrops resolved, and the contralateral lung grew dramatically, but preterm delivery occurred 3 weeks later. Ventilation could not be sustained, and the infant died. Four similar cases of MBA were in the literature, all right-sided. Two fetuses with hydrops delivered at 25-weeks and died immediately. One pregnancy was terminated. One fetus underwent pneumonectomy at 24-weeks but died intraoperatively.

Conclusion

MBA is a rare and lethal lesion that must be distinguished from other right-sided lung masses. Fetal pneumonectomy can be performed with resolution of hydrops and compensatory contralateral lung growth, but remains limited by complications of preterm birth.     

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 [plusmn] 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 [plusmn] 38.5 beats per minute and 71.2% [plusmn] 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 pCO₂ were, respectively, 7.20 [plusmn] 0.11 and 63.2 [plusmn] 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 [plusmn] 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.     

A case of thoraco-amniotic shunt for congenital cystic adenomatoid malformation

Congenital cystic adenomatoid malformation (CCAM) is a congenital pulmonary anomaly, which may lead to fetal hydrops, pulmonary hypoplasia, and fetal or neonatal death. Recently, diagnosis and surgery for fetus have been improved. We experienced a case of CCAM, classified in Stocker class I, with a single cyst of about 5 cm in diameter. A 32 year-old pregnant woman had a fetus diagnosed as CCAM at 25 th gestational week. The thoraco-amniotic shunt placement using a modified double pig tail catheter was performed at 27 th gestational week under general anesthesia with 1% isoflurane in oxygen 2 l x min(-1) and nitrous oxide 4 l x min(-1), and 100 microg of fentanyl. Fetal movement was suppressed during surgery. This fetal therapy was effective and the cyst disappeared after surgery. The baby was delivered with caesarian section under spinal anesthesia with 0.5% bupivacaine 2.5 ml. On the next day, right lung lobectomy was accomplished under general anesthesia with fentanyl 0.2 mg and pancuronium 6.0 mg. Anesthesia with isoflurane, nitrous oxide, and fentanyl was useful for the fetal surgery of thoraco-amniotic shunt placement. Consequently, caesarian section and lung lobectomy were completed successfully.     

Surgical implications of urachal remnants: Presentation and management

Background/Purpose: The abdominal manifestations of urachal remnants often prompt referral to the pediatric general surgeon. The purpose of this study was to evaluate the authors[apos ] management of this anomaly. Methods: The authors performed a retrospective review of patients presenting to their institution with urachal remnants between 1984 and 2001. Clinical and radiographic details of presentation, management, and outcomes are described. Results: Twenty-six patients presented at a median age of 4 years (range, 2 days to 12 years), 16 were boys, and 18 required inpatient care. Eleven (42%) presented with infection, 7 (27%) with clear drainage, 3 (12%) with umbilical polyps/granulation, 3 (12%) with pain, one (4%) with recurrent urinary tract infections, and one (4%) with an asymptomatic punctum. One had an associated anomaly (hypospadias). Urinalysis and urine cultures did not correlate with infection. Ultrasound scan was diagnostic in greater than 90% of cases. Overall, 20 patients underwent primary cyst excision, and 6 underwent incision and drainage (I[amp ]D) with delayed excision. Five patients underwent primary excision while infected, and 2 had postoperative complications (wound infection and urine leak). All 6 patients who underwent 2-stage procedure initially presented with infection, and none had complications. Conclusions: Persistent urachal remnants can present at any age with a variety of clinical manifestations. Ultrasound scan is a reliable diagnostic tool. Additional diagnostic studies generally are not warranted. Simple excision of noninfected lesions is appropriate. In cases of acute infection, initial I[amp ]D with delayed cyst excision may be preferable to avoid unnecessary complications. J Pediatr Surg 38:798-803. [copy ] 2003 Elsevier Inc. All rights reserved.     

Amniotic fluid beta-endorphin: a prognostic marker for gastroschisis?

Purpose: The aim of this work was to study amniotic fluid [beta ]-endorphin as a potential predictor for postnatal morbidity in gastroschisis. Methods: Beta-endorphin was assayed in 43 amniotic fluid samples from 13 pregnant women with fetal gastroschisis undergoing diagnostic amniocentesis or therapeutic amnioinfusion and compared with 33 controls. Within the gastroschisis group, the authors investigated the relationship between postnatal morbidity and the peak value of amniotic fluid [beta ]-endorphin (AFBE). Results: Ten AFBE values in 6 cases of gastroschisis were above the upper limit of the 95% confidence interval derived from controls. Postnatal morbidity was significantly higher when peak AFBE exceeded 10 [mu ]g/L (n = 4 pregnancies) compared with below 5 [mu ]g/L (n = 9 pregnancies), as shown by mean duration of mechanical ventilation (15.2 v 3 days; P = .01), of parenteral feeding (77 v. 18.7 days; P = .04), and of hospitalization (84 v 32.2 days; P = .04). There was no statistically significant association between postnatal morbidity markers and prenatal dilation of fetal bowel. Conclusions: The most severe cases of gastroschisis are associated with high levels of AFBE. The authors speculate that this fetal hormonal response could result from stress or pain caused by prenatal bowel damage.     

Spectrum of intrapartum management strategies for giant fetal cervical teratoma

Background/Purpose: The management of the fetus with a large neck mass that obstructs the airway remains a clinical challenge. The authors review their experience with giant fetal cervical teratoma and discuss options for management. Methods: A retrospective review of all patients referred since 1994 for prenatal management of a fetal neck mass was performed. Variables examined included gestational age at diagnosis and delivery, size and location of the neck mass, presence of fetal hydrops, associated anomalies, management methods, operating time, and outcome. Results: Seven patients were identified with a prenatal diagnosis of giant cervical teratoma. Four patients had fetal hydrops; of these, 2 died in utero of hydrops, and a third fetus underwent elective termination. The remaining hydropic and previable fetus underwent fetal surgery for resection of the mass. The 3 nonhydropic patients underwent ex utero intrapartum treatment (EXIT) procedures for airway control. Endotracheal intubation was possible in one patient, and one received a tracheostomy. In the third fetus, neither intubation nor tracheostomy were possible, and resection of the neck mass was performed on placental support. There were no deaths in the surgical group. Conclusions: The management of fetal giant cervical teratoma includes a spectrum of options. For the rare previable fetus with hydrops, fetal resection may be indicated. In patients with airway obstruction, EXIT procedure provides the luxury of time to obtain airway control either by intubation, tracheostomy, or, if necessary, tumor resection on placental support. J Pediatr Surg 38:446-450.