CHAPTER 48 Joong Shin Park Department of Obstetrics and Gynecology, Seoul National University College of Medicine, Seoul National University Hospital, Seoul, Korea Figure 48.1 Congenital pulmonary airway malformation at presentation. Figure 48.2 Associated findings of fetal hydrops (left: ascites, right: anasarca). Figure 48.3 Aggravation of fetal hydrops at 24+5 weeks of gestation. Figure 48.4 In utero treatments of thoracoamniotic shunting. Hydrops fetalis is usually defined as the accumulation of excessive fluid in at least two fetal extravascular compartments and body cavities including scalp edema, body wall edema, pericardial effusion, pleural effusions, and ascites. Classically, it has been divided into two main entities based on etiology: immune (related to maternal alloimmunization to red cell antigens) and non‐immune (related to a diversity of other causes). With the widespread use of Rhesus immune globulin for the prevention of RhD alloimmunization, most cases of hydrops were nonimmune [1]. Non‐immune hydrops may be caused by a variety of conditions such as cardiovascular disorders, chromosomal abnormalities, infectious diseases, twin‐to‐twin transfusion syndrome, metabolic disorders, hematologic disorders, lymphatic dysplasia, structural anomalies(just like this scenario for CPAM), or tumors. However, in one third of the cases, the etiology is unknown. Development of fetal hydrops associated with CPAM is caused by an impaired venous return because of compression of the vena cava or heart [2]. Polyhydramnios can be developed by decreased fetal swallowing amniotic fluid or by increased secretion of amniotic fluid by the CPAM. In about 10% of the cases, additional extra‐pulmonary abnormalities may be found, such as cardiac failure, renal dysfunction, central nervous abnormalities, and gastrointestinal defects [3]. CPAM is a pulmonary mass derived from proliferation of bronchial structures, with an incidence of between 1 : 250 000 and 1 : 35 000 in livebirths [4–6]. The prognosis is highly variable, depending on the presence of fetal hydrops or the size of the mass [7]. The large mass size and secondary sequelae, including mediastinal shift, pulmonary hypoplasia, polyhydramnios, and hydrops are associated with adverse outcomes [8]. In the presence of large dominant cyst and hydrops, percutaneous aspiration or thoracoamniotic shunting can be considered [9]. Although still controversial, treatment of choice after birth is complete resection of the CPAM even in asymptomatic neonates, because of the risks of infection and occult malignant transformation [10]. And the long term outcome of infants with CPAM following resection is usually excellent. In order to address the issues of most relevance to your patient and to help in searching the literature for the evidence regarding these issues, you should structure your clinical questions as recommended in Chapter 1. Hydrops fetalis is nonspecific condition, as the final stage of a wide variety of disorders. With the widespread use of Rhesus immune globulin for the prevention of RhD alloimmunization, most remaining cases of hydrops fetalis are nonimmune. Nonimmune hydrops fetalis has a multifactorial cause as follows; cardiovascular, hematologic, chromosomal, syndromic, lymphatic dysplasia, inborn errors of metabolism, infectious, thoracic, urinary tract malformations, extra thoracic tumors, placental (twin to twin transfusion syndrome), gastrointestinal, and idiopathic. Understanding of the underlying disorder in cases with hydrops fetalis is very important for adequate management. However, the direct mechanisms responsible for generating hydrops fetalis are still unclear. The generalized edema of hydrops fetalis may particularly result from high interstitial fluid accumulation by low plasma oncotic pressure, high central venous pressure and reduced lymphatic flow owing to multi‐organ failure in various pathologic conditions [11]. A large number of studies have investigated the etiologic classification in cases with nonimmune hydrops fetalis. Several systemic reviews and observational studies have addressed various causative disorders of the nonimmune hydrops fetalis and their percentage among the causes (Table 48.1). Although cardiovascular disorders including cardiac structural anomalies and rhythmic disorders are the most common cause of nonimmune hydrops fetalis, there are considerable hydropic cases with unknown etiology (idiopathic). After exclusion of the main causes of hydrops fetalis, hereditary disorder should be considered in unexplained cases. Metabolic disease, including lysosomal storage disease, is found in 1–2% of all nonimmune hydrops fetalis cases [26, 27]. The diagnosis of metabolic disease can be performed using cultured amniotic fluid cells for specific metabolites. In the South‐East Asian population, alpha‐thalassemia is common etiology of nonimmune hydrops fetalis [15, 16]. Table 48.1 Distribution of cases with nonimmune hydrops fetalis in relation to etiologic classification
Hydrops fetalis
Background
Clinical questions
Critical appraisal of the literature
Study
No. of cases
Cardiovascular
Hematologic
Chromosomal
Syndromic
Lymphatic
Metabolic
Infectious
Thoracic
Urinary
Placental
Miscellaneous
Idiopathic
Hansmann et al. [12]
402
71
39
47
18
89
11
23
9
31
64
100%
17.7%
9.7%
11.7%
4.5%
22.1%
0.0%
2.7%
5.7%
2.2%
0.0%
7.7%
15.9%
Machin [13]
1345
370
163
172
17
19
61
132
53
86
93
179
100%
28%
12%
13%
0%
1%
1%
5%
10%
4%
6%
7%
13%
McCoy et al. [14]
82
19
4
13
9
3
11
5
18
100%
23%
5%
16%
11%
0%
0%
4%
13%
0%
6%
0%
22%
Anandakumar et al. [15]
100
23
23
10
9
2
5
4
24
100%
23%
23%
10%
9%
0%
0%
2%
5%
0%
4%
0%
24%
Yang et al. [16]
78
15
25
5
2
2
2
7
20
100%
19%
32%
6%
0%
3%
0%
3%
3%
0%
0%
9%
26%
Lallemand et al. [17]
94
13
31
4
1
15
3
3
8
7
9
100%
14%
0%
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