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Fryns Syndrome - Case Report. Documentation of Anomalies not Described Previously and Review of the Literature

100%

Bacewicz L., Polnik D., Ganowicz J., Apanasiewicz A., Kaliciński P.

Polish Journal of Surgery

2009

vol. 81

issue 3

149-155

Fryns syndrome is a rare and most often lethal autosomal recessive complex congenital malformation. We report on a case of Fryns syndrome with "coarse facies", broad nasal bridge, dysmorphic low-appearing ears, left cleft lip and palate, left congenital diaphragmatic hernia with lung hypoplasia, distal digital limb hypoplasia of fifth's finger and thumbs, ureter ectopia to the persistent urogenital sinus, bilateral megaureters and hydronephrosis, agenesia of corpus callosum, hernia of the umbilical cord, Meckel's diverticulum, malrotation, choledochal cyst and bilateral inguinal hernia. The persistent urogenital sinus with ectopic ureters and choledochal cyst were not previously described in the literature in association with other features of Fryns syndrome. These malformations add to our knowledge of the phenotype of Fryns syndrome.

Liver Transplantation in Infants with Body Mass Less than 6 KG

59%

Ismail H., Broniszczak D., Apanasiewicz A., Kasprzyk W., Teisserye J., Bacewicz L., Teisserye M., Markiewicz-Kijewska M., Szymczak M., Jezierska E., Kaliciński P.

Liver transplantation (LTx) is the only treatment of fulminant liver failure and end stage liver disease (ESLD).The aim of the study was to assess indications, status at transplantation, surgical techniques, early and late complications and outcome of liver transplantation in children less than 6 kilograms of body weight.Material and methods. Between 1990 and 2008 in the Department of Pediatric Surgery and Organ Transplantation, Children's Memorial Health Institute, there were performed 350 LTx. Within them there were 13 (3.7%) newborn and infants with body weight below 6 kilogram. Retrospective analysis was carried out regarding indications for LTx, status at LTx, surgical techniques, early and late follow-up and outcome.Results. Follow-up of 11 living patients ranges from 5 to 111 months (mean 31.5 months). Patients and graft survival is 84.5%. In all patients liver function is very good or good. Advancement in liver surgery and perioperative care caused that LTx in small recipients became possible and are carried out with good outcome.Conclusion. Encouraging results of liver transplantation in newborn and infants caused that lower age and weight limits for liver recipients are no more significant.

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2 Polish Journal of Surgery

2 2009

2 Apanasiewicz A.

2 Bacewicz L.

2 Kaliciński P.

1 Broniszczak D.

1 Ganowicz J.

1 Ismail H.

1 Jezierska E.

1 Kasprzyk W.

1 Markiewicz-Kijewska M.

1 Polnik D.

1 Szymczak M.

1 Teisserye J.

1 Teisserye M.

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Fryns Syndrome - Case Report. Documentation of Anomalies not Described Previously and Review of the Literature

Liver Transplantation in Infants with Body Mass Less than 6 KG