Omphalocele is labeled as giant when the diameter of the abdominal wall defect is larger than 5 cm and when the sac contains the liver 1. The prognosis of giant omphalocele (GO) depends on the presence of other associated major structural and/or chromosomal abnormalities, which are now detected antenatally 2–19.
The treatment of GO is difficult, primarily because of the disproportion between the large volume of the omphalocele and the small volume of the abdominal cavity. The management of GO presents a major challenge to pediatric surgeons.
We report two cases of GO that required delayed closure. In both cases, Hyalomatrix was used to prepare for skin grafting to repair GO 1,2.
Several studies have reported the use of Hyalomatrix for skin reconstruction in partial deep dermal burns, in scalp reconstruction, and in skin reconstruction at other sites 20–22.
Patients and methods
Our study included two cases of GO: one was 10 cm in diameter and the other was 18 cm in diameter at the time of surgery.
The case was a full-term, male baby born by lower segment cesarean section on 9 March 2010. The baby had a GO of 10×10 cm in size that was associated with congenital feet anomalies (congenital talipes equines) and microcephaly, which was diagnosed later on as craniosynostosis.
A silo bag was applied immediately after birth and the bowels were then gradually reduced by squeezing the bag; this was carried out daily for 10 days. It only resulted in partial reduction of the bowels. Hence, the child was taken back to the operation theater on 22 March 2010. The silo bag was removed and a Gortex mesh (Gore Medical Co., USA) was applied.
Postoperatively, the patient had heavy pseudomonas infection in the wound, which mandated change of the mesh; this was performed on 19 April 2010. This time a dual mesh was applied.
On 3 May 2010, the mesh was removed and wound debridement was performed followed by application of Hyalomatrix (Anika therapeutics, Abano teme, Italy); the Hyalomatrix was left on the wound for 2 weeks.
On 17 May 2010, skin graft was performed. The skin was meshed 1 : 1.5.
On 27 May 2010, patient was discharged from hospital after complete healing of the wound. He has ventral hernia, which awaits repair (Figs 1–6).
The case was a 32-week-old preterm male baby, delivered by lower segment cesarean section on 6 October 2010 due to breech presentation. The baby was a twin to a normal male baby.
Omphalocele was diagnosed during antenatal care.
After initial resuscitation, the child was taken for surgery; intraoperative evaluation showed that the patient has very small abdominal cavity, very thin recti muscles, and very thin muscles of the diaphragm with deficient areas that partly exposed the lungs. The omphalocele was huge containing liver, colon, and stomach.
A silo bag was sutured to the edges of the fascia after repair of the diaphragmatic defects.
Daily squeezing of the silo bag failed to reduce the bowels completely; hence, the silo bag was removed and a Gortex mesh was applied on 18 October 2011.
The mesh was changed on 27 October 2011; on 30 November 2011, Hyalomatrix was applied after removal of the Gortex mesh and conservative debridement of the granulation tissue that was formed as a reaction to the Gortex application.
On 21 December 2011, the patient was taken to the operation theater and removal of the outer silastic layer of the Hyalomatrix was performed; the dermis-like layer was already formed on the defect of 18×18 cm (324 cm2) in size. The patient’s thigh circumference was 100 cm2 (about 1/3 the size of the defect); hence, meshing 1 : 3 was performed for the skin grafts. The skin graft was applied over the defect and a tie-over dressing was performed using silver dressing (Aquacell Silver; Conva Tec., UK). The skin graft survived well.
Extubation of the child was carried out 1 week postoperatively, but he kept on desiderating and needed reintubation and assisted ventilation.
On 17 February 2012, the patient suffered a cardiac arrest. Resuscitation was not successful and the patient died. The primary reason for death was respiratory failure (Figs 7–15).
Yamagishi et al.14 reported the management of one case of ruptured GO that was first treated with application of a silo bag for 3 weeks followed by application of vicryl mesh for 2 weeks, and finally meshed split thickness skin graft was applied. After 2 weeks, the skin was fully reconstructed. The child died at the age of 8 months because of respiratory insufficiency. Yamagishi et al.14 considered their steps of management as a standard protocol for the treatment of GO and gastroschisis.
In our study, we used silo bag for 3 weeks, followed by application of Gortex mesh for 2 weeks then application of Hyalomatrix, and finally meshed split thickness skin graft.
What is Hyalomatrix?
The main role of hyaluronic acid in wound healing is as follows:
- stimulation of the migration of phagocytotic cells;
- stimulation of the proliferation of fibroblasts and keratinocytes; and
- promotion of angiogenesis 20.
Esterification of hyaluronic acid with benzyl alcohol is used to obtain Hyaff – an ester of hyaluronic acid used in Hyalomatrix production 21.
When Hyaff comes into contact with exudates, it jellifies and creates an environment favorable to wound healing and to the orderly deposition of collagenous fibers 22.
Hyalomatrix is a skin substitute composed of two layers:
- The inner layer is a three-dimensional dermis-like matrix consisting of fibers of a hyaluronic acid ester called Hyaff. It is in the form of scaffold, which lies under the outer layer.
- The outer layer is a flexible and transparent elastomeric film that operates as a semipermeable barrier to external agents. The transparency of the elastomeric film makes it possible to monitor the evolution of the underlying wound without removing the medication.
We used our technique in two patients:
- The first patient is still alive at more than 2 years of follow-up, and he was operated upon for craniosynostosis. Now, he is ready for abdominal wall reconstruction and hernia closure.
- The second patient died at an age older than 3 months because of respiratory failure.
From our experience with the above-mentioned two cases, we reached a conclusion that, for operative treatment of major omphalocele and to avoid abdominal compartment syndrome, we can consider the steps used in these two cases (silo followed by application of Gortex mesh, which will initiate formation of granulation tissue, followed by application of one of the dermal substitute (Hyalomatrix) that will initiate a dermis-like layer on which thin split thickness skin graft is applied as a last stage) as another alternative of treatment of these very complex cases that will end in full-skin reconstruction.
The resulting ventral hernia could be corrected at a later time when the abdominal cavity is fully developed.
The morbidity and mortality of the GO depends mainly on the other chromosomal anomalies and associated syndromes, mainly related to pulmonary hypoplasia.
Conflicts of interest
There are no conflicts of interest.
1. Charlesworth P, Ervine E, McCullough M.Exomphalos major: the Northern Ireland experience.Pediatr Surg Int2009;25:77–81.
2. Khan AN, MacDonald S, Thomas N, Cohen HL, Mohan HC, Coombs BD.Omphalocele. Available at: http://emedicine.medscape.com/article/404182-overview
3. Greenwood RD, Rosenthal A, Nadas AS.Cardiovascular malformations associated with omphalocele.J Pediatr1974;85:818–821.
4. Gilbert WM, Nicolaides KH.Fetal omphalocele: associated malformations and chromosomal defects.Obstet Gynecol1987;70:633–635.
5. Benacerraf BR, Saltzman DH, Estroff JA, Frigolett FD jr.Abnormal karyotype of fetuses with omphalocele: prediction based on omphalocele contents.Obstet Gynecol1990;75:317–319.
6. Nyberg DA, Fitzsimmons J, Mack LA, et al..Chromosomal abnormalities in fetuses with omphalocele. Significance of omphalocele contents.J Ultrasound Med1989;8:299–308.
7. Mann L, Ferguson-Smith MA, Desai M, et al..Prenatal assessment of anterior abdominal wall defects and their prognosis.Prenat Diagn1984;4:427–435.
8. Salihu HM, Pierre-Louis BJ, Druschel CM, Kirby RS.Omphalocele and gastroschisis in the State of New York, 1992–1999.Birth Defects Res A Clin Mol Teratol2003;67:630–636.
9. Mann S, Blinman TA, Douglas Wilson R.Prenatal and postnatal management of omphalocele.Prenat Diagn2008;28:626–632.
10. Adam AS, Corbally MT, Fitzgerald RJ.Evaluation of conservative therapy for exomphalos.Surg Gynecol Obstet1991;172:394–396.
11. Pereira RM, Tatsuo ES, Simoes e Silva AC, Guimarães JT, Paixão RM, Lanna JC, Miranda ME, et al..New method of surgical delayed closure of giant omphaloceles: Lazaro da Silva’s technique.J Pediatr Surg2004;39:1111–1115.
12. De Ugarte DA, Asch MJ, Hedrick MH, Atkinso JB.The use of tissue expanders in the closure of a giant omphalocele.J Pediatr Surg2004;39:613–615.
13. Moazam F, Rodgers BM, Talbert JL.Use of Teflon mesh for repair of abdominal wall defects in neonates.J Pediatr Surg1979;14:347–351.
14. Yamagishi J, Ishimaru Y, Takayasu H, Otani Y, Tahara K, Hatanaka M, et al..Visceral coverage with absorbable mesh followed by split-thickness skin graft in the treatment of ruptured giant omphalocele.Pediatr Surg Int2007;23:199–201.
15. Gross RE.A new method for surgical treatment of large omphalocele.Surgery1948;24:277–283.
16. Allen RG, Wrenn EL Jr.Silon as a sac in the treatment of omphalocele and gastroschisis.J Pediatr Surg1969;4:3–8.
17. Patkowski D, Czernik J, Baglaj SM.Active enlargement of the abdominal cavity – a new method for earlier closure of giant omphalocele and gastroschisis.Eur J Pediatr Surg2005;15:22–25.
18. Baird R, Gholoum S, Laberge JM, Puligandla P.Management of a giant omphalocele with an external skin closure system.J Pediatr Surg2004;39:1111–1115.
19. Pereira RM, Tatsuo ES, Simões e Silva AC, Guimarães JT, Paixão RM, Lanna JC, Miranda ME.New method of surgical delayed closure of giant omphaloceles: Lazaro da Silva’s technique.J Pediatr Surg2008;43:246–250.
20. Gravante G, Delogu D, Giordan N, Morano G, Montone A, Esposito G.The use of Hyalomatrix PA in the treatment of deep partial thickness burns.J Burn Care Res2007;28:269–274.
21. Dessy LA, Mazzocchi M, Rizzo MI, et al..Scalp reconstruction using dermal induction template.In Vivo2013;27:153–158.
22. Perrot P, Delliere V, Brancati A, Duteille F.Hyalomatrix PA in skin substitutes about 10 cases.Ann Chir Plast Esthet2011;56:107–111.