Journal of Pediatric Surgery (2006) 41, 1850 – 1853
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Traction-compression-closure for exomphalos major Antonino Morabitoa,*, Anthony Owenb, Adrian Bianchia a
Neonatal Surgical Unit, St Mary’s Women and Children’s Hospital, Manchester M13 9WL, UK Pediatric Surgical Unit, Sheffield Children’s Hospital, Sheffield S10 2TN, UK
b
Index words: Anterior abdominal wall development; Midline abdominal wall anomalies; Exomphalos; Delayed closure; Aesthetics
Abstract Purpose: We present our experience with traction-compression-closure (TCC) for exomphalos major (EM) to achieve a safe and embryologically correct midline supraumbilical aesthetic closure with preservation of the umbilicus. Methods: Nineteen neonates with EM were paralyzed and ventilated. The abdominal domain was increased by upward cord traction to assist liver-bowel reduction by gravity and sac ligation, followed by circumferential elastic body binder compression. The supraumbilical abdominal wall anomaly cicatrized spontaneously or was closed surgically as a midline scar, with preservation of the umbilicus. Results: Over 7 years (1998-2004), 19 patients with EM were treated by TCC, 18 of whom survived. The patients’ median gestational age was 36 weeks (range, 24-40 weeks); their median birth weight was 2312 g (range, 890-3000 g). The median time to reduction was 4 days (range, 3-5 days), whereas that to full enteral feeds was 6 days (range, 4-6 days). Mechanical ventilation for 7 days (range, 6-8 days) was not associated with any morbidity, and the time to home discharge was 11 days (range, 8-12 days). Five patients did not require any surgery. There was no episode of sac rupture or infection. Conclusion: Abdominal expansion by vertical cord traction followed by compression reduction (TCC) under muscle relaxation and ventilation is time well spent toward a safe and aesthetic midline abdominal wall closure without tension for EM. D 2006 Elsevier Inc. All rights reserved.
In England and Wales, exomphalos major (EM) has a prevalence of 0.77 per 10,000 live births [1], with an overall mortality of 34% largely determined by associated cardiac and chromosomal anomalies [2]. Although most children with EM will survive [3,4], immediate closure at birth may be hazardous because of major visceroabdominal disproportion. Liver and bowel reduction followed by abdominal wall closure can be achieved using local tissue or prosthetic materials [5-7]. Alternatively, closure may be achieved after topical application of various chemicals [8-11]. The major
* Corresponding author. Tel.: +44 161 276 6542; fax: +44 161 276 6854. E-mail address:
[email protected] (A. Morabito). 0022-3468/$ – see front matter D 2006 Elsevier Inc. All rights reserved. doi:10.1016/j.jpedsurg.2006.06.044
factors relevant to survivors are their quality of life and the cosmetic appearance of the abdominal wall, including the umbilicus [10,12-14]. We present our experience with traction-compression-closure (TCC) for EM to achieve a safe and embryologically correct midline supraumbilical aesthetic closure with preservation of the umbilicus.
1. Methods Over 7 years, from 1998 to 2004, 33 neonates with EM were managed by 6 surgeons. Exomphalos major was defined as a supraumbilical midline abdominal wall defect containing all or a major portion of the liver and variable bowel content. The diameter of the anomaly was not a
Traction-compression-closure for exomphalos major
1851 excluded from the study. Two of the remaining 21 neonates, managed by 2 surgeons, were excluded from TCC because of sac detachment at delivery. Selection considerations for TCC were an intact or repairable sac, the general condition and respiratory status of the child, and associated anomalies significant to survival. After exclusions and selection, the study group consisted of a cohort of 19 patients with a median gestational age of 36 weeks (range, 24-40 weeks) and a median birth weight of 2312 g (range, 890-3000 g). Patient demographics, time to reduction and closure, length of paralysis and ventilation, time to full feeds, length of stay, complications, and survival were analyzed.
1.1. Traction After stabilization, the children were paralyzed and ventilated. The abdominal wall was stretched by vertical cord traction against the baby’s weight such that the child’s back is just lifted off the bed (Fig. 1A). A soft paraffin dressing was wrapped around the sac to prevent desiccation. Enteral feeding can be commenced during this phase.
1.2. Compression Progressive reduction was initially achieved by sac compression from above. Because the sac contents gradually accommodated within the abdominal cavity, it was not uncommon for the liver to detach from the sac by the third to fifth day. Once the liver and bowel have entered the abdominal space, cord traction was replaced by downward compression with a circumferential elastic body binder (Fig. 1B). A series of gauze swabs placed sequentially beneath the binder maintained reduction and downward compression. This allowed neonates to gradually acclimatize to the additional abdominal contents and further increased the abdominal domain.
1.3. Closure
Fig. 1 A, The neonate’s back is suspended just off the bed by vertical cord traction and the sac is ligated above the contents using cotton tape to achieve gradual reduction. B, Once the sac contents are reduced, cord traction is replaced by downward compression using a circumferential elastic body binder. C, Aesthetic appearance of the umbilicus after nonsurgical closure.
criterion in diagnosis. Twenty-nine patients had an intact sac, whereas 4 had a ruptured sac. Four surgeons always applied a prosthetic silo, and 12 patients were therefore
Once full reduction and acclimatization have been achieved, the supraumbilical anomaly was closed as a midline scar. The residual sac was dissected and a layered closure was undertaken apposing the fascia whenever possible and always preserving the umbilicus. Careful dissection was necessary at the xiphisternum to avoid injury to the hepatic veins, which lie superficially, and to allow the liver an optimal intraabdominal position without venous impairment. Umbilical aesthetics may be improved with a purse-string suture of absorbable monofilament material. Some children may not require surgery, the anomaly having cicatrized spontaneously after full reduction (Fig. 1C).
2. Results Of 33 neonates with EM, 19 (11 boys and 8 girls) were treated by TCC and 18 survived. One neonate, born at 24 weeks’ gestation and weighing 890 g, died of respiratory distress syndrome associated with severe prematurity.
1852 Seven patients did not undergo any surgery during the neonatal phase, and all healed spontaneously (without ventral hernia in 5 and with ventral hernia in 2). Of the 11 neonates who underwent surgical abdominal closure during the neonatal period, 7 had an uncomplicated fascial and skin closure. Three children had skin closure only as the first part of a staged procedure. Two had delayed fascial closure of the residual ventral hernia when they were 4 years old. The fourth child developed respiratory distress during surgical closure, and so a prosthetic silo was applied. This was subsequently removed with successful uneventful aesthetic fascial and skin closure. Among the 18 survivors, the median time to reduction was 4 days (range, 3-5 days), the median time to full enteral feeds was 6 days (range, 4-6 days), the median time receiving mechanical ventilation was 7 days (range, 6-8 days), and the median time to discharge receiving full oral feeds was 11 days (range, 8-12 days). There was no ventilator-related morbidity; neither was there any sac infection or detachment during TCC. There has been no readmission for wound infections, feeding difficulties, and intestinal obstruction. The median follow-up was 33 months (range, 7-70 months). All survivors have demonstrated weight and height increases along the normal centiles as well as mental development appropriate for their chronological age. All retain their umbilicus with an acceptable upper abdominal midline scar, none of which has required revision.
3. Discussion Exomphalos major presents as a supraumbilical midline anomaly extending from the xiphisternum to the umbilicus. There is often an intact sac formed of an outer layer of amnion and an inner layer of peritoneum, containing all or a large portion of the liver and a variable amount of bowel. The umbilical cord usually inserts into the apex of the sac [15]. The costal margin is widely splayed and the anterior diaphragm is split in the midline such that the inferior vena cava and hepatic veins lie more superficially. We did not find the diameter of the anomaly to be a relevant factor in the diagnosis of EM. The globular shape and size of the extruded liver, often on a mesentery, may present a problem at reduction. Staged repair has been advocated [6-8,10,13,16-19] to avoid liver injury and abdominal compartment syndrome. Skin cover of the defect can be achieved conservatively over several weeks, allowing the sac to form an eschar and to epithelialize [8-11]. Alternatively, closure with large lateral skin flaps [5] has been suggested, although this extreme measure is rarely necessary. Both methods leave large ventral hernias necessitating delayed surgical repair [7,10]. Management is directed at ensuring survival and closure of the midline supraumbilical anomaly with minimal
A. Morabito et al. morbidity, respecting normal anatomy and long-term aesthetics. Sac ligation or clamping and external compression have previously been described in small series with some success [16-19]. We have incorporated both sac ligation and external compression with cord traction using a natural silo of available autologous tissue, providing optimal protection for the liver and bowel during reduction to increase the abdominal domain. Once all contents lie comfortably within the abdominal space, the anomaly is closed surgically as a layered midline supraumbilical scar, always preserving the umbilicus. During our study, we have attempted to clarify the exclusion and selection criteria. Although an intact membrane is an asset, membrane rupture amenable to repair is not a contraindication. The diameter of the base and the degree of extruded content were only relevant in determining the ease of reduction and eventual closure, with larger lesions being less likely to close spontaneously. Therefore, the only real considerations for TCC were the child’s general condition and respiratory status, any associated anomaly having a bearing on survival, and the child’s tolerance of graded membrane compression. We believe that this ongoing study demonstrates that abdominal expansion by upward cord traction combined with organ reduction by membrane compression under muscle relaxation is time well spent toward a safe abdominal wall closure without tension. Although parenteral nutrition was part of the management plan, all children were able to tolerate enteral feeding and no child was discharged home fed intravenously. The long-term results of the neonates in terms of normal bowel and liver function as well as aesthetic appearance of the abdominal wall are much appreciated by the parents and the children, who today demand a high aesthetic standard. Despite increasingly successful management, it must be emphasized that exomphalos should not be regarded lightly. It remains to be a dangerous life-threatening condition that can severely tax surgeons’ ingenuity and expertise.
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