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Congenital hypoplasia of the abdominal wall muscles following fetal ascites due to parvovirus B19 infection

2010; Wiley; Volume: 37; Issue: 4 Linguagem: Inglês

10.1002/uog.8904

1469-0705

G. Macé, G. Audry, A. Cortey, Anh Quoc Nguyen, L. Slaïm, Vanina Castaigne, Cathérine Garel, B. Carbonne,

Prenatal Screening and Diagnostics

Maternofetal parvovirus B19 infection may be a cause of fetal hydrops through fetal anemia or myocarditis and heart failure1, 2. We report a case of fetal hydrops with subsequent postnatal muscular hypoplasia and major persistent abdominal distension. A 25-year-old woman, gravida 2 para 1, was referred at 22 weeks' gestation for fetal hydrops detected at routine ultrasound scan. Findings included abundant fetal ascites and moderate pericardial effusion. Abdominal circumference was 203 mm (99th centile). Middle cerebral artery peak systolic velocity, at 56 cm/s, was > 1.5 multiples of the median. Serology testing was positive for parvovirus B19 immunoglobulin-M. Fetal anemia was confirmed by blood sampling (hemoglobin 4.4 g/dL) and an in-utero transfusion restored hemoglobin to 14 g/dL. Parvovirus infection was confirmed by polymerase chain reaction. Paracentesis was performed at 25 weeks' gestation for persistent abundant ascites. Labor was induced at 41 + 6 weeks and a female infant weighing 3710 g was delivered vaginally with a 5-min Apgar score of 10. The neonate's abdomen was enlarged but without significant ascites. Subsequent follow-up showed hypoplasia of the anterior and lateral abdominal wall muscles visible during crying as two lateral subumbilical oval projections (Figure 1). Distension continued at 1 and 2 years. Computed tomography showed hypoplasia, mainly of the internal oblique and transverse muscles of the abdomen (Figure 2). The rectus abdominis muscle was affected to a lesser degree. Clinical examination of the infant at 1 year of age, showing bilateral projection of the abdominal wall (visible during crying). Computed tomography image (axial view) of the abdomen obtained at 2 years of age, at the level of the iliac wings. On the right, internal oblique (1) and transverse (2) muscles of the abdomen are clearly distinguished. The internal oblique muscle is very thin. On the left, only one of these two muscles is visible and also appears thin, as does the rectus abdominis muscle (3). According to embryological studies, diastasis recti abdominis would result from disruptive phenomena before the fusion of myotomes during the eighth week, while muscular hypoplasia should result from later phenomena. Several genetic disorders such as prune belly syndrome and Poland syndrome may be involved in abdominal muscle defects3, 4. In our case, the anomaly was isolated and followed mechanical distension associated with massive ascites. The severity of ascites, its persistent duration for at least 1 month and the early gestational age at onset—somewhere between 12 and 22 weeks—might suggest a purely mechanical explanation. However, no previous case of muscle hypoplasia in the context of fetal ascites has been reported, including after major parietal distension5 or fetal hydrops. Other factors might predispose to or aggravate muscle fragility. Several genes are involved in myogenesis6. None of these has known transcriptional or post-transcriptonal modification due to parvovirus, but some genetic predispositions of weakness or diastasis of the rectus abdominalis muscle exist7, in which excess intra-abdominal pressure may be pathogenic. Parvovirus infection has been found to be associated with juvenile dermatomyositis and extreme muscle weakness8. Parvovirus-related myositis and fasciitis with infectious necrosis have also been described9, but parvovirus-related primary damage of the peripheral nerve has not been previously described, although parvovirus infections can cause damage to the fetal central nervous system10. Finally, ischemia due to vascular compression might produce this type of damage, similar to that seen during the formation of gastroschisis. A combination of the above mentioned factors could be responsible for muscle hypoplasia. Studies are necessary to assess whether parvovirus B19 may be directly involved in alterations of myogenesis in order to support a non-mechanical explanation for abdominal muscle hypoplasia. Considering the possible multifactorial etiology, there is no evidence that peritoneal–amniotic shunting could improve parietal development in such cases. G. Macé*, G. Audry , A. Cortey , A. Nguyen*, L. Slaim , V. Castaigne*, C. Garel , B. Carbonne , * Department of Obstetrics and Gynecology, Hopital Saint Antoine, Paris, France, Department of Pediatric Surgery, Hopital Trousseau, Paris, France, Centre National de Reference en Hemobiologie Perinatale, Hopital Saint Antoine, Paris, France