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Liver exstrophy in a neonate with Poland syndrome

2017; Elsevier BV; Volume: 58; Issue: 6 Linguagem: Inglês

10.1016/j.pedneo.2016.11.005

2212-1692

Xenophon Sinopidis, Antonios Panagidis, Vasileios Alexopoulos, Sotirios Tzifas, Gabriel Dimitriou, George Georgiou,

Automotive and Human Injury Biomechanics

A female neonate was delivered by cesarean section at the 34th-week- of-gestation (wg) owing to a right extrathoracic mass, previously diagnosed at the 21st week. An MRI confirmed a herniated lump through a thoracic cleft. The amniotic fluid and karyotype were normal. The oval foramen and the arterial duct were patent. At birth, a mass protruded through an intercostal cleft at the anterior axillary line, with a hypoplastic right hemithorax and supernumerary nipple (Fig. 1A). The upper limbs were normal. A CT confirmed the hepatic origin of the mass (Fig. 1B). A surgical reduction of the herniated segment, and thoracic wall closure were performed. A postoperative MRI indicated a hepatic anatomy adjustment (Fig. 2A). Thoracic asymmetry without a functional deficiency was a remnant of this Poland syndrome (Ps) case at the age of 2 years (Fig. 2B). An aesthetic removal of the lower nipple is the only operation to perform.Fig. 2.A: A follow-up MRI at 6 months: a complete adjustment of the liver parenchyma. There is also a depression of the right thoracic wall, hypoplasia of the right pectoralis major, reduction of the subcutaneous fat, and hypoplasia of the 4th to 6th ribs, all characteristics of the Poland syndrome. B: A clinical follow-up at 2 years of age: hypoplasia of the right pectoralis major muscle producing thoracic asymmetry (a large dark arrow), two nipples (small dark arrows), and the postoperative scar (a white arrow).View Large Image Figure ViewerDownload Hi-res image Download (PPT) A liver exstrophy combined with Ps, and without an upper-limb involvement, is extremely uncommon, with an unknown etiology.1Puder M. Greene A. Mooney D. Hepatic exstrophy complicating Poland's anomaly.J Pediatr Surg. 2002; 37: 1203-1204Abstract Full Text Full Text PDF PubMed Scopus (11) Google Scholar, 2Seifarth F.G. Cruz Pico C.X. Stromberg J. Recinos V.M. Burdjalov V.F. Karakas S.P. Poland syndrome with extracorporeal intercostals liver herniation and thoracic myelomeningocele.J Pediatr Surg. 2012; 47 (e13–7)Abstract Full Text Full Text PDF PubMed Google Scholar A chest wall trauma or deformities have been accredited.2Seifarth F.G. Cruz Pico C.X. Stromberg J. Recinos V.M. Burdjalov V.F. Karakas S.P. Poland syndrome with extracorporeal intercostals liver herniation and thoracic myelomeningocele.J Pediatr Surg. 2012; 47 (e13–7)Abstract Full Text Full Text PDF PubMed Google Scholar Thoracic wall hypoplasia is related to a defect formation. A herniated liver has been erroneously characterized as skin at 28th wg.2Seifarth F.G. Cruz Pico C.X. Stromberg J. Recinos V.M. Burdjalov V.F. Karakas S.P. Poland syndrome with extracorporeal intercostals liver herniation and thoracic myelomeningocele.J Pediatr Surg. 2012; 47 (e13–7)Abstract Full Text Full Text PDF PubMed Google Scholar A diagnosis after the 6th wg favors an arterial hypoplasia hypothesis (a subclavian artery hypoplasia sequence).3Al Faleh K. Al Saadi M. Khalid-Bantuas S. Poland's syndrome with absent limb anomalies.J Clin Neonatol. 2014; 3: 44-46Crossref PubMed Google Scholar A narrow herniated segment may undergo resection, to avoid trauma, incarceration, or torsion.2Seifarth F.G. Cruz Pico C.X. Stromberg J. Recinos V.M. Burdjalov V.F. Karakas S.P. Poland syndrome with extracorporeal intercostals liver herniation and thoracic myelomeningocele.J Pediatr Surg. 2012; 47 (e13–7)Abstract Full Text Full Text PDF PubMed Google Scholar In adult patients with ectopic or accessory liver lobes, a propensity to hepatic carcinogenesis has been described. Nevertheless, no similar alteration of a herniated liver lobe in a child has been documented in the literature.2Seifarth F.G. Cruz Pico C.X. Stromberg J. Recinos V.M. Burdjalov V.F. Karakas S.P. Poland syndrome with extracorporeal intercostals liver herniation and thoracic myelomeningocele.J Pediatr Surg. 2012; 47 (e13–7)Abstract Full Text Full Text PDF PubMed Google Scholar Reduction without hepatectomy has been reported in two cases.1Puder M. Greene A. Mooney D. Hepatic exstrophy complicating Poland's anomaly.J Pediatr Surg. 2002; 37: 1203-1204Abstract Full Text Full Text PDF PubMed Scopus (11) Google Scholar Liver preservation guarantees a quick recovery. After a follow-up period of 2 years the protruding hepatic segment has become undifferentiated from a normal one. Therefore, we suggest minor conservative surgery. There are many operative techniques regarding the treatment of pectoralis major correction in Ps. However, in our case, the problem is only aesthetic, and probably no major surgery will be needed in the future. Periodic examination is mandatory as these cases are rare. The authors have no conflicts of interest relevant to this article.