an otherwise healthy mother had a routine ultrasound at 20 weeks , which showed giant omphalocele with liver included in the omphalocele sac .
karyotyping of the amniotic fluid cells showed a normal female karyotype ( 46 , xx ) , -fetoprotein ( afp ) in amniotic fluid was raised ( 38.7 mg / l ; 3 mom  =  20.1 mg / l).these findings were discussed with the parents and it was decided to continue the pregnancy .
the baby was born at 38 weeks and 2 days of gestation by elective cesarean delivery .
the birth weight was 3,500  g and apgar scores were 8/9 at 1 and 5 minutes , respectively .
the omphalocele contained a big part of the liver , almost the whole bowel , and the spleen .
the omphalocele was covered with a sterile petrolatum ( vaseline , smith & nephew , kruisweg , hoofddorp , netherlands ) gauze dressing , which was changed every other day .
the baby expelled meconium shortly after birth and also upon arrival in the neonatal intensive care unit .
feeding was started via nasogastric tube and was well tolerated during the first days of life . on the 6th day after birth ,
, we resected the omphalocele sac ( which was vulnerable by manipulation ) and performed adhesiolysis to appropriately position the spleen and bowel in the abdominal cavity .
the liver remained outside the abdominal cavity . a silo bag ( sb06 , 60-mm ; medicina ltd . , bolton , united kingdom )
was used to temporarily cover the defect and central venous access was obtained in the left subclavian vein ( supplementary fig .
the biggest silo bag available ( 60-mm ) was not big enough to accommodate the liver and was therefore enlarged by attaching a triangular patch made of silastic mesh . because the use of a silo is not optimal for treatment when the liver is situated outside the abdominal cavity , alternative treatment approaches had to be considered . on the 14th day after birth , we removed the silo and placed non  cross - linked intact porcine - derived acellular dermal matrix ( padm ; strattice reconstructive tissue matrix , lifecell corp . , branchburg , new jersey , united states ) at the fascia and sutured it with prolene 30 sutures ( ethicon , somerville , new jersey , united states ) ( fig .
1 ) . a vacuum dressing system was used to cover the padm with a negative pressure of 75  mm hg ( supplementary fig .
the vacuum system was changed in the operation theater on postoperative days 3 ( supplementary fig .
s3 in the online version of the article ) , 9 , and 12 and on the pediatric ward thereafter .
amoxicillin / potassium clavulanate and gentamicin were initiated for prophylaxis against infection and then changed to meropenem .
s4 in the online version of the article ) . 
 completed suturing of the fascia to the porcine - derived acellular dermal matrix ( 14th day after birth ) . on the 26th day of life , the patient developed sepsis from the central venous catheter infection .
central venous system blood cultures were positive and the patient experienced nephritis and renal insufficiency that subsided spontaneously .
enteral feeding was suspended during the sepsis ; however , the patient was tolerating full enteral nutrition by 45 days postnatal age . on the 41st day of life the result of the postnatal array - cgh ( agilent technologies , santa clara , california , united states ) was returned , it showed a maternally inherited 780  kb deletion on chromosome band 3q28 containing part of the tp63 gene , and four more genes that cause autosomal recessive syndromes .
the patient was discharged home at age 2 months and 10 days with the vacuum therapy , which was discontinued 1  week later . after removing the vacuum therapy ,
the patient was bathed daily by her parents and had a sterile petrolatum gauze dressing in place until the wound closed completely ( supplementary fig .
ventral hernia repair was performed when the patient was 2 years and 3 months old ( supplementary fig .
the padm had become integrated into the surrounding fascia and was used to close the abdominal wall ( supplementary fig .
a sample of this padm taken during ventral hernia repair was sent for histological examination .
s8 in the online version of the article ) and experienced no complications through 6 months of follow - up ( fig .
omphaloceles are often associated with other malformations and chromosomal anomalies.1 
 2 the small chromosome 3 deletion in this case ( 1 ) is to our best knowledge ( based on queries in pubmed database and decipher database ) not known to be associated with omphalocele , ( 2 ) contains no genes explicitly associated with omphalocele,3 and ( 3 ) was inherited from a healthy mother.4 therefore , this deletion was considered to be most likely of neutral effect .
the raised amniotic fluid afp was associated with the omphalocele.5 
  giant omphaloceles are not common , which makes it difficult for a single pediatric surgery department to gain extensive treatment experience .
furthermore , most of the very large omphaloceles can be treated conservatively with sterile petrolatum dressing until the epithelialization of the defect results in a large ventral hernia that can be subsequently closed when there is adequate space in the abdominal cavity . in cases where the omphalocele sac has to be removed or when a rupture occurs
the use of a combination of a biological matrix graft and vacuum therapy for treating abdominal wall defects has been previously reported in the medical literature6 
 7 
 8 ; however , the use of padm for this purpose has not yet been described in our knowledge .
strattice is a non  cross - linked porcine , acellular dermal matrix for use in xenograft transplantation , serving as a biological scaffold to support tissue regeneration.9 
 10 it is derived from porcine dermis and processed in a manner to remove the cells and porcine antigens which would provoke an immune response in humans , while retaining all its extracellular matrix ( ecm ) components , its three - dimensional structure and its biomechanical strength . as such when implanted , it acts as a scaffold to support tissue ingrowth .
its ecm components attract in cells and vascularity from the patients adjacent tissue , and over time it is replaced by the patients ' own tissue with all components of the porcine matrix removed , in a regenerative tissue healing process.11 
 12 
  in this patient , the use of padm was successful and showed good long - term biological compatibility , as the histological examination of the collagen showed no difference with the human collagen 2 years after suturing the patch to the fascia ( supplementary fig .
the microscopic examination showed dissections of tissue fragments , irregular collagen bundles containing numerous small vessels , and signs of very mild chronic infection .
based on the histology examination , it was not possible to determine if the sample consisted of porcine collagen tissue or human collagen , even after applying sirius red and verhoeff  van gieson staining .
the padm became integrated with the fascia and was strong enough to be partially used to strengthen closure of the abdominal wall defect .
however , management can be challenging if the omphalocele is ruptured or the sac has to be removed .
we present padm with vacuum therapy as a good alternative in the treatment of giant complicated omphaloceles .
 the management of giant omphaloceles at our department is primarily conservative . 
 however , management can be challenging if the omphalocele is ruptured or the sac has to be removed . 
 we report a case in which a giant omphalocele in a newborn female patient was managed by covering the abdominal defect with non  cross - linked intact porcine - derived acellular dermal matrix ( strattice reconstructive tissue matrix , lifecell corp . , branchburg , new jersey , united states ) sutured to the fascia combined with vacuum therapy . 
Introduction
Discussion
Conclusion
New Insights and the Importance for the Pediatric Surgeon
Supplementary Material