www.elsevier.com/locate/jpedsurg

Management of giant omphalocele with rapid creation ofabdominal domain

Robert Fogliaa,*, Alex Kaneb, Devra Beckerb, Jose Asz-Sigalla, George Mychaliskaa

aDivision of Pediatric Surgery, Department of Surgery, Washington University School of Medicine,

St Louis Children’s Hospital, St Louis, MO 63110, USAbDivision of Plastic Surgery, Department of Surgery, Washington University School of Medicine, St Louis Children’s Hospital,

St Louis, MO 63110, USA

0022-3468/$ – see front matter D 2006

doi:10.1016/j.jpedsurg.2005.12.013

Presented at the 57th Annual Meetin

American Academy of Pediatrics, Washi

* Corresponding author.

E-mail address: fogliar@wustl.edu (

Index words:Abdominal wall defect;

Giant omphalocele;

Tissue expander;

Peritoneal domain

AbstractBackground: The management of giant omphaloceles (GO) can be quite difficult when there is absence

of abdominal domain. Coverage with delayed closure has been described. We present a technique to

create an adequate peritoneal domain.

Methods: This is a retrospective review of our experience using an intraperitoneal tissue expander

(IPTE) to create adequate abdominal domain in 2 patients with GO.

Results: In 2 children with unrepaired GO, an IPTE was placed into the pelvis and was inflated to the

target volume over 3 to 4 weeks. At the definitive operation for the closure of the abdominal defect, the

IPTE was removed, allowing reduction of all the viscera into the peritoneal cavity and achieving

complete abdominal wall closure. The patients are now 1 year postoperative, each having a normal

abdomen and enjoying normal growth and development.

Conclusion: Intraperitoneal tissue expander placement can create the needed domain over several weeks

in GO. Its use in 2 cases was associated with satisfactory complete abdominal wall closure in short

order. This technique should be considered as a treatment option.

D 2006 Published by Elsevier Inc.

inal wall defects, such as use of prosthetic material as a fascial bridge under the skin,

The management of abdom

omphalocele or gastroschisis, has evolved over the past

4 decades. If primary fascial closure cannot be easily

achieved, the use of a silo technique with staged closure is

standard. However, the management of a giant omphalocele

(GO), defined as an omphalocele with a fascial defect greater

than 6 cm and with most of the liver in an extraperitoneal

position, remains problematic. Many strategies have been

used, such as Gross’s [1] description of skin-flap closure, the

Published by Elsevier Inc.

g of the Section on Surgery of the

ngton, DC, October 7-9, 2005.

R. Foglia).

sequential sac ligation, techniques to encourage epithelial-

ization of the omphalocele membrane, silo application with

gradual reduction of the extraperitoneal viscera (EPV), and

sequential clamping without prosthetic material [2-5]. These

techniques all describe coverage of the viscera with delayed

closure. Others have described the relative advantages of

nonoperative initial management in treating GO [6,7].

Fundamental to the problem with GO is the disproportion

between the amount of EPV and the diminutive size of the

peritoneal cavity. Gross aptly described the abdominal

viscera as having blost its right of domicile in the peritoneal

cavity.Q We felt that the creation of an adequate peritoneal

domain was the primary aim of treatment. Our effort

Journal of Pediatric Surgery (2006) 41, 704–709

Management of giant omphalocele with rapid creation of abdominal domain 705

was focused on identifying a technique to increase

peritoneal domain to the point that the viscera could be

reduced in one operation. Most of the described techniques

often required multiple-staged operations over a protracted

period to gradually reduce the EPV as the peritoneal cavity

was progressively increased in size. We report a novel

technique using an intraperitoneal tissue expander (IPTE) to

create peritoneal domain. It was used successfully in 2 pa-

tients, a newborn and a 4-year-old child who was previously

treated nonoperatively.

ig. 2 Computed tomography scan showing the EPV and the

iminutive peritoneal cavity.

1. Case reports

We treated 2 patients with GO, both with virtually the

entire liver extraperitoneal along with other abdominal

viscera, and minimal peritoneal domain. Both patients were

treated with the use of an IPTE to create adequate peritoneal

domain before visceral reduction and abdominal wall closure.

GH, a 4-year-old, 13-kg girl was born with a constellation

of anomalies including a left congenital diaphragmatic

hernia, an associated pulmonary hypoplasia and pulmonary

hypertension, an atrial septal defect, no inferior vena cava,

and a GO. She underwent repair of the diaphragmatic hernia

shortly after birth. No attempt was made to repair the GO

because of its massive size, the disproportion between the

EPV and the peritoneal cavity, and her associated problems.

A gastrostomy tube was placed into the stomach through a

lower left quadrant incision. She was hospitalized for the first

6 months of life. She moved to our community at 3.5 years of

age and had a soccer ball–sized unrepaired GO (Fig. 1). In

addition, she refused all oral intake and received all nutrition

via the gastrostomy button. A multidetector computed

tomography (CT) scan showed the relative volumes of the

EPV and the peritoneal cavity (Fig. 2). The volume of the

EPV was calculated from the computed axial tomography

scan and was found to be 535 mL.

In addition to the strikingly small peritoneal domain, we

faced additional problems because of her oral aversion and

gastrostomy tube placement directly through the abdominal

wall musculature, which we wished to advance to achieve

abdominal wall closure. Speech pathology consultation was

obtained, and over 3 months, the patient’s oral aversion was

Fig. 1 The GO and its relative size in comparison to the abdomen.

Fd

resolved, and she would drink up to 240 mL at a time, eat

solid food, and no longer require a gastrostomy. We could

now use a strategy to increase peritoneal domain.

At her first operation, carried out through a small lower

left quadrant incision at the gastrostomy site, the gastrostomy

button was removed, the gastrotomy closed, and an IPTE

was placed in the pelvis. This consisted of a 10 � 12–cm

rectangular tissue expander (PMT Corporation, Chanhassen,

Minn) with a nominal fill capacity of 600 mL and 50%

overfill capacity, allowing a total capacity of 900 mL. The

IPTE catheter came out of the abdomen through a small stab

wound in the lower abdomen. The patient was hospitalized

for 4 days, and we began to gradually add volume to the

IPTE. On an outpatient basis, over the next 4 weeks, the

IPTE gradually was filled with saline to 900 mL of volume.

We chose this as approximately 50% more than the

calculated EPV volume (Fig. 3).

At the second operation, 1 month after IPTE placement,

the omphalocele membrane was excised and the incision was

extended superiorly and inferiorly in the midline. The IPTE

was deflated and removed. The peritoneal cavity had been

expanded sufficiently to allow for reduction of all of the liver

into the peritoneal cavity along with the remainder of the

EPV, consisting of multiple loops of bowel. The abdominal

wall musculature could not be approximated without ten-

sion. Therefore, a small 2-cm-wide by 7-cm-long Alloderm

strip (Life Cell Corp, Branchburg, NJ) was used to complete

the fascial closure. The skin and subcutaneous tissue easily

closed (Fig. 4). The potential problem with reducing the liver

and the possibility of kinking or obstructing venous drainage

did not materialize.

The patient’s postoperative course was uneventful. She

was extubated on the first postoperative day, was eating on

the fifth postoperative day, and was discharged home on the

14th postoperative day.

BC, a 3.4-kg newborn female, had a 10-cm GO with all

of the liver extraperitoneal along with a portion of the small

bowel. At the initial operation, a Silastic silo was sewn

circumferentially to the abdominal wall fascia to cover the

EPV. The child remained intubated and received total

Fig. 3 Abdominal CT scan showing the expansion of the peritoneal cavity and the IPTE filled with 900 mL of saline.

R. Foglia et al.706

parenteral nutrition. Over the next 5 days, it was apparent

that the EPV could not be reduced because of its large

volume relative to the small peritoneal domain. The patient

underwent a second operation to place an IPTE in the pelvis.

The IPTE was inflated to 300 mL of volume over 20 days.

The next day, the silo and IPTE were removed (Fig. 5). The

liver and other EPV were completely reduced but left an 8 �7 fascial defect. A Silastic sheet was sewn circumferentially

to the fascia. This sheet was gradually imbricated over

22 days, decreasing the short or horizontal axis of the

abdominal wall muscular defect from 7 to 2 cm (Fig. 6). The

Fig. 4 Appearance of the abdominal wall immediately after

repair.

sheet was then removed, and complete abdominal wall

closure was achieved without tension.

Both patients have been followed 1 year and have had no

complications. They are eating well and are enjoying normal

growth and development.

2. Discussion

The outcomes of infants born with abdominal wall

defects have improved tremendously in the past 40 years

Fig. 5 The EPV after silo removal and at the beginning of the

definitive repair. The IPTE catheter is seen in the lower part of the

abdomen.

Fig. 6 Viscera reduced and the fascial defect bridged with

Silastic sheeting (8 � 7 cm) and imbricated in 2 stages.

Management of giant omphalocele with rapid creation of abdominal domain 707

because of medical and surgical advances. The development

of neonatal intensive care units, infant ventilators, and

hyperalimentation has dramatically improved survival. The

use of prosthetic material to assist in closure of the abdominal

wall was a turning point in the treatment of these children.

Before 1965, reports would often cite a mortality rate of 90%

for infants born with a gastroschisis. Schuster [2] first

described the use of prosthetic sheeting using artificial

material to bridge the abdominal muscle wall defect and then

to mobilize skin flaps in the fashion of Gross to cover the

sheeting. Pickett, Allen, and Wesselhoeft [cited in 8,9]

reported various techniques of using prosthetic material either

to bridge the fascial defect, but not to cover the prosthetic

material with skin, or to use the prosthetic material as a silo

to allow for coverage of the EPV and subsequent staged

closure. These techniques resulted in excellent outcomes for

most of the children with gastroschisis or omphalocele.

The management of the child with a GO, either a neonate

or an older child, who has never been repaired or has had a

failed repair, is complex and remains daunting. Patients with

particularly small peritoneal domain are especially challeng-

ing. Options that are available and have been used include

(1) short-term silo reduction (ideal if the peritoneal domain is

adequate), which may take several weeks followed by fascial

closure or closure with prosthetic material; (2) long-term silo

reduction over 2 to 6 weeks followed by closure; (3) a staged

reduction closing skin flaps over the omphalocele membrane

followed by delayed closure of the ventral hernia 6 to

12 months later; and (4) the use of external compression

before repair to facilitate delayed closure [10-12].

The fundamental problem with the GO is the insufficient

peritoneal domain in most of these infants. Silo placement

and staged closure allows for the gradual reduction of the

EPVwith increasing intra-abdominal pressure causing a slow

increase in peritoneal volume. Most of the aforementioned

techniques involve a series of operations over several weeks

to months to slowly and gradually increase intraperitoneal

domain. Often, the patients may not be able to be fed enterally

during this period, and there are potential complications with

silo dehiscence, infection, and compromise of blood flow to

visceral organs. Furthermore, in patients with GO, there is a

potential problem because of angulation or obstruction of

hepatic venous drainage through the suprahepatic inferior

vena cava [13,14]. Recognition of these problems is essential

in the operative treatment of these patients.

We chose to focus on a method to increase peritoneal

domain without using the viscera as the mechanism to

achieve an increase in intra-abdominal pressure. One of us

(AK) has had experience with tissue expansion. He had

identified that there was a case report of one patient in the

Netherlands with an omphalocele where a tissue expander

had been placed intraperitoneally [15]. He felt that this

might be advantageous in these patients with GO because

the peritoneal domain could be increased without using the

viscera as the source of pressure. The use of an IPTE has

several advantages: (1) one can precisely control the amount

of expansion; (2) it is less invasive than gradually

constricting or compressing the silo where the viscera are

used to exert increased intra-abdominal pressure; (3) and

with the information from the multidetector CT scan, one

can calculate the EPV volume and therefore make a

reasonable projection of how much volume the tissue

expander should be inflated.

This technique has worked well for us both in a newborn

and an older child. In the latter patient, the ability to have

her extubated and eating so quickly after the definitive

procedure is in contrast to the often prolonged postoperative

course and the need for a series of operative procedures

using a number of the other techniques in similar patients.

In the newborn, although we could reduce all of the

viscera into the peritoneal cavity proper, we could not

achieve abdominal wall closure. The gradual imbrication of

the Silastic sheet allowed us to slowly reduce the size of the

defect before abdominal wall closure. In the older child, a

small Alloderm patch was needed to bridge the fascia at

closure. These 2 points speak to the fact that surgical

judgment is necessary regarding how much abdominal wall

tension is acceptable. In the former case, the defect was so

large that we were reticent to use Alloderm for fascial

approximation. In the older patient, we felt that a small

Alloderm segment would function very well and could be

left permanently in place.

R. Foglia et al.708

References

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[7] Burge MD, Glasson MI. The conservative management of exompha-

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an initial management of giant omphaloceles. J Pediatr Surg

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[12] Sander S, Elicevik M, Unal M. Elastic bandaging facilitates primary

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Discussion

Judson Randolph, MD (Nashville, TN): Chairman Lang-

ham, Chairman Smith, I hope everybody recognizes that

the importance of this paper is inversely proportional to

its length. This is an important concept for us. I’d like the

leave of the chairs to mention for a moment where we’ve

come from. You remember that Dr Gross developed flaps

of skin to go over the omphalocele way back in the 40s.

This worked for omphaloceles that were intact. It didn’t

work for giant omphaloceles, it didn’t work for gastro-

schisis, it didn’t work for ruptured omphaloceles, and we

ended up with defects that looked much like the first

patient that was shown. Sam Schuster, was the inventor

of the idea of plastic sewn to the rectus muscle, and then

skin closed over it. He insisted on the skin closure over

the plastic. That led to a series of operations, but

ultimately some dramatic success. It was Michel Gilbert

in Miami who first showed that the plastic didn’t need to

be covered by skin.

And shortly thereafter, Allen and Wrenn in Memphis,

Larry Pickett from New Haven, and Connie Wesselhoeft

and I in Washington all dealt with the plastic that could

be left outside the skin, and so began the long saga with

plastic pouches and then silos, and as you know, silos

were gradually improved. There was Dennis Shermeta

who had a preformed silo that could be rolled down,

Biemann Otherson, Chairman Smith’s partner, showed

that a double lumen silo would accommodate pressure

that could be pushed down. All of these things have

helped us with the problems such as were shown on these

slides. But I think that the idea that we can improve the

inner cavity of the abdomen is exciting, and I will bet

you that in just this audience, in the next year or two,

there will be 10 or 20 or even 25 patients who will

benefit from this concept.

I wish you would take just a moment to tell us a

little more about the insertion of the tissue expander.

Thank you.

R. Foglia, MD (response): Dr Randolph, thank you very

much for your comments. Essentially, what you can do if

you have an older child is to use the CT scan which will

allow you to calculate the volume of viscera that’s outside

of the peritoneal cavity. We simply took that volume,

added 50% to it, and chose an expander which could be

filled to that volume. The expander can be folded up fairly

easily so you make a small incision and put it in the

peritoneal cavity. You want to put it down in the pelvis.

You then begin to inflate through the catheter and increase

the volume over a number of weeks. In the newborn with

the giant omphalocele, a silo initially was sewn to the

fascia. When it was apparent that a typical staged closure

could not be performed easily, we chose to use an

expander. We could easily slip an expander down into

the pelvis bringing it out through a separate incision, much

the way you bring out a Tenckhoff catheter. So it’s

relatively straightforward.

Agostino Pierro, MD (London, UK): I very much enjoyed

this paper and congratulations for this treatment. This is a

difficult category of patients. We use a different strategy

that we reported in the May issue of this year’s JPS.

Twelve babies, repaired at birth with giant omphaloceles

with a defect between 8 and 15 cm, so in the same

category that you presented. What we’ve done is a series

of reductions of the silo. The key issue is that we use a

Prolene mesh and we suture it to the edges of the fascia

leaving a flat surface so that there is an ingrowth of tissue

into the mesh. The time that we needed to fully close the

abdomen was a median of 26 days. We felt that we

augmented the abdominal cavity by reducing progres-

sively, with a limited duration of technique and avoided

Management of giant omphalocele with rapid creation of abdominal domain 709

the use of a foreign body, such as a tissue expander. Did

you have any problem with breathing in your neonate,

because some of these babies have pulmonary hypoplasia

and don’t tolerate high intra-abdominal pressures or

elevation of the diaphragm?

R. Foglia, MD (response): There is no question that this

occurs in a number of children. You’ll have a chest in

some of these children which looks like a canine chest

or a Gothic window type thorax, and that’s especially

problematic. Here, I think one of the advantages we

found with the expander was that with instilling fluid in,

if there was a problem for example, you could simply

take the fluid out. So you really have a way to

accurately put fluid in or take it out, and it’s almost

like playing poker with somebody. You can raise the

amount, and if you find it’s too much, you can back it

off a little bit. The technique you’re describing sounds

like is a very good one too. I think that it’s something

that as pediatric surgeons, we should have as many

things as we can in our armamentarium that we can use

in taking care of these children.

Agostino Pierro, MD (London, UK): With our technique, we

could release the pressure by releasing the tack that we’ve

done on the Prolene mesh, but the key issue is the Prolene

mesh, and the ingrowth of the Prolenemesh into the fascia.

Arthur Cooper, MD (New York, NY): Congratulations on an

outstanding contribution. I rise primarily to call the

attention of the section to the technique described by my

colleague, Dr Barbara Barlow, here about 15 years ago,

on external reduction using an external wrap for intact

omphaloceles. We’ve continued to use that technique

over the last 15 years, and we have not needed to use a

silo for anything but the very largest omphaloceles.

Thanks to you we now have a technique to use with those

children as well.

R. Foglia, MD (response): Thanks.

Michael A. Skinner, MD (Durham, NC): This is a very

interesting technique. What was the reason for placing the

Silastic on for a period of time before putting the expander

in?Were you concerned that if you just put the expander in

with the omphalocele sac intact, that it would just stretch

the sac instead of stretching the abdominal cavity?

R. Foglia, MD (response): The short answer is that actually

one of the other surgeons had put the Silastic on first and

then I became involved afterwards, and the technique we

were looking at was evolving at the same time. So it was

a case of probably the right approach at an opportune

time. Typically with a giant omphalocele if he had

chosen not to let the thing just epithelialize, we would

cover it with Silastic and see if it could be reduced. Over

that first 5 days or so, it was apparent that we weren’t

going to get anywhere. We needed to look at doing

something else, so that’s why we chose to put the

expander in after the silo had been placed.

Michael A. Skinner, MD (Durham, NC): So, for next time,

are you just going to make an incision and place the

expander and defer repairing the omphalocele until you

have established an increased abdominal domain?

R. Foglia, MD (response): I think that’s what we’re looking

at doing next. We have another child right now who is

much like the other one, it’s a 5-year old who has a

number of other issues, so we have that one sort of in the

wings, but I think with the newborn, we would consider

using this a priori. Yes.