The Rare Congenital Anomaly Omphalocele: A Review of 25 years of Management

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Date
2017
Authors
Pitre, Chantalle
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Abstract
Objective: The objective of this study was to: 1. Evaluate the various techniques chosen for management of small versus giant omphalocele & the complications that arose for each 2. Compare the above outcomes with current published literature Study Design: Cases of omphalocele that underwent evaluation between January 1991 and December 2016 at Health Sciences Centre (HSC) Children’s Hospital were identified. Inclusion criteria was restricted to live births with a plan for postnatal repair and patients who had their initial treatment for the defect at HSC hospital. Results: Among the 28 cases, 14 patients were categorized as having a small omphalocele. Of these 14 patients, 11 underwent primary fascial closure, 1 had a staged closure, and 2 were treated with a delayed closure technique. No complications occurred among the small omphalocele patients. Fourteen patients were categorized as having a giant omphalocele. Of these 14 patients, 2 underwent primary fascial closure, 8 had a staged closure, and 4 were treated with a delayed closure technique. Of the 2 patients who underwent primary fascial closure, 1 developed an infection at the surgical site and respiratory failure. Three of the 8 patients treated with staged closure developed various combinations of inferior vena cava thrombosis, metabolic acidosis, acute renal failure, respiratory failure and infection at the surgical site. Delayed closure did not result in any complications. Conclusion: Primary fascial closure is the preferred technique for small omphalocele repair at HSC Children’s Hospital and in current published literature. Primary fascial closure is an excellent technique for small omphalocele as it requires only one procedure and few complications arise. Staged closure is the preferred technique for giant omphalocele reduction at HSC Children’s Hospital and in current published literature. This technique is often chosen as it produces less complications than primary fascial closure in giant omphalocele. Delayed closure was less frequently employed for giant omphalocele despite having the lowest complication rates of all three techniques. Key Words: Omphalocele, small, giant, primary, staged, delayed, silo, mesh, skin flap Limitations: Omphalocele documentation - description versus measurement. Giant omphalocele definition. Unknown eviscerated contents. Small sample size. Purpose: The purpose of this study was to relay to physician assistants current information about the treatment of omphalocele patients treated in Manitoba, and to provide a comparison to treatment in current literature. The results of this study will help physician assistants educate their patients about what to expect in regards to epidemiology, embryology, diagnostics, co-morbidities, post-natal management, treatments and potential complications.
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omphalocele
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