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170
Congenital Anomalies 2015; 55, 170
doi:10.1111/cga.12108
LETTER TO THE EDITOR
Caudate lobe of the liver as the only content of the umbilical cord hernia
Umbilical cord hernia (UCH) is the least common of abdominal wall defects, wherein there exists an amniotic sac only containing intestinal loops protruding through a defect with a diameter less than 4 cm (Klein 2012). A 2600 gram female newborn was referred. Her physical examination revealed an umbilical cord hernia with a diameter of 3.5 cm. There was no associated anomaly. Bowel ischemia was suspected because of purple color and urgent surgical repair was planned (Fig. 1a). When the sac was opened, there was no bowel in it. Instead, caudate lobe of the liver was the only content of the sac (Fig. 1b). The liver was dissected free from the sac and repositioned into the abdomen. She fed orally on postoperative second day, and was discharged on the sixth day uneventfully. The well-known facts about UCH are that it is the least common of abdominal wall defects, its diameter is less than 4 cm, a membrane covers it, the only content is midgut, and it is able to be repaired primarily (Haas et al. 2011; Klein 2012). When bowel ischemia is suspected because of dark purple color of the sac, the management should be done immediately. We did so in the present case; however, we were faced with an unusual content – “caudate lobe of the liver”. There are very few reports revealing liver content of UCH in the literature. It has been reported that the accessory lobe of the liver or gallbladder has been in the sac (Festen et al. 1988; Elmasalme et al. 1995). Nearly the whole liver appearing in the UCH has also been reported very rarely (Zameer et al. 2012). The present case is the first report of UCH including only the caudate lobe of the liver besides these cases. This unusual and rare content should be kept in mind in cases with umbilical cord hernia that is dark in color to avoid causing fatal liver damage.
Fig. 1 (a) The purple color appearance of the umbilical cord hernia and the operation scene. (b) The partially freed sac and the caudate lobe of the liver in the sac.
Correspondence: Özlem Boybeyi, MD, Assistant Professor of Pediatric Surgery, Hacettepe University, Medical Faculty, Department of Pediatric Surgery, Ankara, Turkey. Email: [email protected] Received January 27, 2015; revised and accepted February 24, 2015.
© 2015 Japanese Teratology Society
DISCLOSURE None declared. Özlem Boybeyi1, Ismail Ozmen2, Yasemin Dere Gunal2, Mustafa Kemal Aslan2, and Didem Aliefendioglu3 1 Department of Pediatric Surgery, Faculty of Medicine, Hacettepe University, Ankara, 2Department of Pediatric Surgery, Faculty of Medicine, 3Department of Pediatrics, Division of Neonatology, Faculty of Medicine, Kırıkkale University, Kırıkkale, Turkey
REFERENCES Elmasalme F, Aljudaibi A, Matbouly S, Hejazi N, Zuberi MSH. 1995. Torsion of an accessory lobe of the liver in an infant. J Pediatr Surg 30:1348–1350. Festen C, Severijnen RSVM, vd Staak FHJM. 1988. Gallbladder embedded in an accessory liver lobe in umbilical cord hernia. J Pediatr Surg 23:978– 979. Haas J, Achiron R, Barzilay E, Yinon Y, Bilik R, Gilboa Y. 2011. Umbilical cord hernias, prenatal diagnosis and natural history. J Ultrasound Med 30:1629–1632. Klein MD. 2012. Congenital defects of the abdominal wall. In: Coran AG, Adzick NS, Krummel TM, Laberge JM, Shamberger RC, Caldamone AA, editors. Pediatric surgery. 7th edn. Philedelphia: Elsevier. p 973– 984. Zameer MM, Mahalik S, Kanojia R, Rao KLN. 2012. Entire liver as the only content of hernia of the umbilical cord. Hernia 16:605–606.
170
Congenital Anomalies 2015; 55, 170
doi:10.1111/cga.12108
LETTER TO THE EDITOR
Caudate lobe of the liver as the only content of the umbilical cord hernia
Umbilical cord hernia (UCH) is the least common of abdominal wall defects, wherein there exists an amniotic sac only containing intestinal loops protruding through a defect with a diameter less than 4 cm (Klein 2012). A 2600 gram female newborn was referred. Her physical examination revealed an umbilical cord hernia with a diameter of 3.5 cm. There was no associated anomaly. Bowel ischemia was suspected because of purple color and urgent surgical repair was planned (Fig. 1a). When the sac was opened, there was no bowel in it. Instead, caudate lobe of the liver was the only content of the sac (Fig. 1b). The liver was dissected free from the sac and repositioned into the abdomen. She fed orally on postoperative second day, and was discharged on the sixth day uneventfully. The well-known facts about UCH are that it is the least common of abdominal wall defects, its diameter is less than 4 cm, a membrane covers it, the only content is midgut, and it is able to be repaired primarily (Haas et al. 2011; Klein 2012). When bowel ischemia is suspected because of dark purple color of the sac, the management should be done immediately. We did so in the present case; however, we were faced with an unusual content – “caudate lobe of the liver”. There are very few reports revealing liver content of UCH in the literature. It has been reported that the accessory lobe of the liver or gallbladder has been in the sac (Festen et al. 1988; Elmasalme et al. 1995). Nearly the whole liver appearing in the UCH has also been reported very rarely (Zameer et al. 2012). The present case is the first report of UCH including only the caudate lobe of the liver besides these cases. This unusual and rare content should be kept in mind in cases with umbilical cord hernia that is dark in color to avoid causing fatal liver damage.
Fig. 1 (a) The purple color appearance of the umbilical cord hernia and the operation scene. (b) The partially freed sac and the caudate lobe of the liver in the sac.
Correspondence: Özlem Boybeyi, MD, Assistant Professor of Pediatric Surgery, Hacettepe University, Medical Faculty, Department of Pediatric Surgery, Ankara, Turkey. Email: [email protected] Received January 27, 2015; revised and accepted February 24, 2015.
© 2015 Japanese Teratology Society
DISCLOSURE None declared. Özlem Boybeyi1, Ismail Ozmen2, Yasemin Dere Gunal2, Mustafa Kemal Aslan2, and Didem Aliefendioglu3 1 Department of Pediatric Surgery, Faculty of Medicine, Hacettepe University, Ankara, 2Department of Pediatric Surgery, Faculty of Medicine, 3Department of Pediatrics, Division of Neonatology, Faculty of Medicine, Kırıkkale University, Kırıkkale, Turkey
REFERENCES Elmasalme F, Aljudaibi A, Matbouly S, Hejazi N, Zuberi MSH. 1995. Torsion of an accessory lobe of the liver in an infant. J Pediatr Surg 30:1348–1350. Festen C, Severijnen RSVM, vd Staak FHJM. 1988. Gallbladder embedded in an accessory liver lobe in umbilical cord hernia. J Pediatr Surg 23:978– 979. Haas J, Achiron R, Barzilay E, Yinon Y, Bilik R, Gilboa Y. 2011. Umbilical cord hernias, prenatal diagnosis and natural history. J Ultrasound Med 30:1629–1632. Klein MD. 2012. Congenital defects of the abdominal wall. In: Coran AG, Adzick NS, Krummel TM, Laberge JM, Shamberger RC, Caldamone AA, editors. Pediatric surgery. 7th edn. Philedelphia: Elsevier. p 973– 984. Zameer MM, Mahalik S, Kanojia R, Rao KLN. 2012. Entire liver as the only content of hernia of the umbilical cord. Hernia 16:605–606.
