Hydrops fetalis and pulmonary sequestration

Hydrops Fetalis and Pulmonary Sequestration By Mark G. Evans London, @The development of fetal hydrops in conjunction with intrathoracic pathology has been described, but rarely in association with pulmonary sequestration. The current report presents three cases of antenatally identified nonimmune hydrops fetalis, seen in association with pulmonary sequestrations. In one case, a left-sided chest mass also was defined. One infant was born by emergency cesarean section because of fetal distress at 34 weeks’ gestation; the other two were delivered vaginally at 30 and 36 weeks. Two of the newborns were severely hydropic and required aggressive cardiorespiratory resuscitation that included bilateral chest tubes for massive pleural effusions. The third infant was stable at the time of birth, but thereafter respiratory distress developed, and intubation and ventilation became necessary. Two of the infants had a left-chest mass and the other had a right-sided mass, all identified by chest x-ray. Subsequent ultrasonography showed a chest mass and an identifiable systemic feeding artery in two of the patients. For the third, a specific diagnosis was not made before surgery. After resolution of hydrops, all three infants had successful removal of their intrathoracic pulmonary sequestrations. Two of these were found to be extralobar, and the other was an intralobar sequestration of the left lower lobe. Copyright o 1996 by W.B. Saunders Company lNDEX

WORDS:

Hydrops

fetalis,

pulmonary

sequestration.

P

ULMONARY sequestrations are congenital lesions comprised of nonfunctional lung tissue that does not communicate with the normal tracheobronchial tree and has a systemic arterial blood supply. Such lesions present as respiratory distress in the newborn period or, alternatively, as chronic respiratory infections in older children. With the advent of routine antenatal ultrasonography, pulmonary mass lesions are being identified prenatally with increasing frequency. Hydrops fetalis is most commonly of an immune nature, caused by fetal incompatibility to maternal blood group antigens. It has become increasingly clear that nonimmune fetal hydrops can develop in the presence of a congenital pulmonary mass lesion. Most frequently, this has been described in association with congenital cystic adenomatoid malformation (CCAM), but recent reports also note its association with pulmonary sequestrations. Prenatal ultrasonography makes it possible to identify such cases, and the natural history of hydrops and congenital thoracic lesions has been studied and has led to possible therapeutic recommendations.l Herein we describe three cases of infants who had nonimmune hydrops fetalis, identified antenatally, in association with pulmonary sequestrations. Journa/ofPed/afncSurgery,

Vol31,No6(June),1996:

~~761-764

Ontario

CASE REPORTS

Case 1 A 30-year-old primiparous woman presented during an uncomplicated pregnancy following excessive weight gain. Ultrasonography showed polyhydramnios and a 30-week-gestation fetus with hydropic changes including a large right pleural effusion with significant left-sided mediastinal shift. Two days later, the mother experienced premature rupture of membranes and a spontaneous vaginal delivery ensued. The infant required aggressive resuscitation that included immediate intubation, and bilateral chest tubes were placed. Hemodynamic instability continued, and right-sided pleural effusion persisted. In the presence of a patent ductus arteriosus, ligation was performed. After surgery, the right pleural effusion resolved and a solid intrathoracic mass became apparent. A second thoracotomy was performed, and a right-sided extralobar pulmonary sequestration was removed. The child’s subsequent course was uneventful. The infant continues to do well at 16 months of age.

Case 2 A 26-year-old woman (gravida 5, para 2) presented at 25 weeks’ gestation with sudden weight gain. Ultrasonography at that time showed a fetus with a left-sided chest mass, with significant right mediastinal shift and fetal ascites. Ultrasonography at 30 weeks’ gestation showed increasing hydropic changes in the fetus; however, a subsequent ultrasound scan, at 36 weeks, showed some rmprovement. The woman was induced at 36 weeks’ gestation, and a 3,180-g girl was born vaginally. Respiratory distress developed, and the baby was intubated within a few hours of life. A chest X-ray confirmed the presence of a left-sided chest mass (Fig l), and subsequent ultrasonography showed a solid-appearing mass with a feeding vessel coming from the thoracic aorta (Fig 2). Because of persistent hemodynamic mstability. she was taken to the operating room (on the third day of life) and a left lower lobectomy was performed to remove an intralobar pulmonary sequestration. The infant’s subsequent course was uncomplicated. She continues to do well 8 months later.

Case 3 A 28-year-old primiparous woman presented with a sudden lo-pound weight gain at 34 weeks’ gestation. Ultrasonography showed polyhydramnios, severe fetal hydrops, and minimal fetal movement. Labor was induced, and an attempt at forcep delivery was unsuccessful. A cesarean section was performed because of fetal distress.

From the Division of Paediatnc Surgev, Universlq of Western Ontario and the Children’s Hospital of Western Ontario, London, Ontario. Presented at the 27th Annual Meeting of the Canadian Assouation of Paedratric Surgeons, Montreal, Quebec, September 2-4, 1995. Address reprint requests to Mark G. Evans, MD, FRCSC, Division of Paediam’c Surgery, Children? Hospital of Western Ontario, 800 Commissioners Rd E, London, Ontario. Canada N6C 2V5. Copyright Q I996 by W: B. Saunders Company 0022-3468/9613106-0006$03.00/O 761

MARK

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Fig 1. Chest film shows mediastinal shift.

left-sided

chest

mass

causing

significant

The baby required aggressive cardiorespiratory support including immediate intubation, placement of bilateral chest tubes, and paracentesis. A chest x-ray showed a left-sided chest mass (Fig 3), and ultrasonography showed a systemic arterial blood supply

.&

Fig 3.

Chest

film shows

left-sided

chest

G. EVANS

mass (arrow).

arising from the aorta at the level of the diaphragm (Fig 4). A CT scan confirmed the presence of the left-sided chest mass and a feeding arterial vessel coming from the infradiaphragmatic aorta (Fig 5). The infant had a left thoracotomy and resection of the

c-

;.e-

4a

Fig 2. Ultrasound Doppler study shows

scan shows left-sided systemic artery (arrow)

chest mass (bottom). supplying the mass.

Fig 4. Ultrasound scan shows left-si,ded chest mass (top). study shows systemic artery (arrow) supplying the mass.

26)

Doppler

HYDROPS

FETALIS

Fig 5. CT scan from the abdominal

extralobar later.

AND

shows aorta

pulmonary

PULMONARY

left-sided [arrow).

sequestration,

SEQUESTRATION

chest

and

mass

with

is domg

763

arterial

well

supply

5 months

DISCUSSION

In the past, the diagnosis of pulmonary sequestration often was made in the newborn period, because of the effect of a large intrathoracic lesion on respiratory function. Alternatively, with smaller lesions, older children would present with chronic respiratory infections. With the common use of prenatal ultrasonography, congenital intrathoracic masses are being diagnosed antenatally at an increasing rate.2 The development of hydrops fetalis in association with congenital intrathoracic lesions has been described. Most commonly this is associated with a congenital cystic adenomatoid malformation, particularly the solid (Stocker type III), variant.3 Recently, congenital hydrops has been described in association with fetal pulmonary sequestration.lz4 However, the mechanism by which fetal hydrops occurs is unclear. In the past, it was believed that this likely represented a manifestation of congestive heart failure secondary to a high-output state caused by its anomalous systemic blood s~pply.~ More recently, it

has been suggested that hydrops is secondary to either cardiac compression from large intrathoracic masses or, alternatively, vena caval obstruction owing to the mass itself1 or the associated tension hydrothorax that may accompany such congenital mass lesions.4 This may be particularly true with pulmonary sequestrations, which often are not that large. The associated hydrothorax may be lymphatic in origin, stemming from impaired lymph drainage of the sequestration itself. Two of the newborns in the present series had similar bilateral hydrothoraces that required prompt drainage. The fetus in whom a congenital intrathoracic mass is identified in the absence of any other important congenital anomaly or hydropic changes should be managed conservatively. The mother should be monitored by serial ultrasonography and allowed to carry the infant to term. Arrangements should be made for the infant to be delivered in a tertiary center with immediate access to neonatal and pediatric surgical expertise. The optimal management for the infant with hydrops and an intrathoracic lesion is less clear. The combination of intrathoracic lesion and hydropic changes appears to be associated with a grave prognosis for the fetus. This is particularly true if the underlying lesion is a pulmonary sequestration.4 In a recent review article, Adzick reviewed his experience and suggested a course of management for the fetus with a congenital thoracic mass.’ He states that the overall prognosis with this lesion depends on the size of the lung mass and the secondary physiological derangements that occur. He believes that hydropic changes are a harbinger of neonatal demise. As a consequence, he suggests a role for fetal surgical intervention, particularly in cases of hydropic changes in fetuses under 32 weeks’ gestation. This experience is based primarily on infants that ultimately were shown to have congenital cystic adenomatoid malformations, but Dr Adzick implies that it would be appropriate to extend this experience to infants who have pulmonary sequestrations. In the present study, two of the three fetuses with pulmonary sequestrations had hydropic changes before 32 weeks’ gestation. These two infants were delivered spontaneously, at 30 and 36 weeks’ gestation. The third fetus was identified as having severe hydrops and evidence of distress at 34 weeks’ gestation and thus was delivered by cesarean section at that time. Thereafter, all three infants responded to conventional management that included aggressive cardiorespiratory support, early intubation, and prompt drainage of pleural effusions. Subsequently, semielective resection of the pulmonary sequestrations was performed on all three infants; their postop-

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764

erative courses were uncomplicated, and all three are alive and well. In summary, we report successful outcome for three newborns who had nonimmune hydrops fetalis and pulmonary sequestration. The ability to identify these lesions and the associated hydropic changes prenatally has therapeutic implications that may in-

G. EVANS

elude fetal surgical intervention. However, our study suggests that it may be appropriate to allow these fetuses to reach greater maturity even in the presence of hydrops. All three of the infants were delivered and stabilized using conventional neonatal techniques, which was followed by successful surgical excision.

REFERENCES 1. Adzick NS: Fetal thoracic lesions. Semin Pediatr Surg 2:103108,1993

2. Avni EF, Vanderolst A, Van Gansbeke DV, et al: Antenatal diagnosis of pulmonary tumors. Pediatr Radio1 l&190-195,1986 3. Adzick NS, Harrison MR, Glick PL, et al: Fetal cystic adenomatoid malformation: Prenatal diagnosis and natural history. J Pediatr Surg 20:483-4881985

4. Brus F, Nikkels PGJ, Van Loon AJ, et al: Non-immune hydrops fetalis and bilateral pulmonary hypoplasia in a newborn infant with extralobar pulmonary sequestration. Acta Paediatr 82:416-418,1993 5. Haller JA, Galladay ES, Pickard LR, et al: Surgical management of lung bud anomalies. Ann Thorac Surg 28:33-43,1978