Surgical Management of Omphalocele: A Plastic Surgeon’s Perspective Brian I. Labow, MD, FACS, FAAP Department of Plastic Surgery Children’s Hospital Boston Harvard Medical School

Introduction Heterogenous population and associated anomalies common Many approaches, techniques and tools No single approach will suit all patients Outcome data based limited by numbers and confounding variables 3

Outline General considerations Tools/techniques Adjuvant procedures Summary

General Comments Usually not an emergency Most cases can be managed with “conventional approaches” Circumstances may mandate change in course…

Clinical Situation? Medically unstable Damage control (e.g. ruptured membrane, silo disruptions) Incomplete reduction Extreme visceral-peritoneal disproportion

Tools and Techniques

Negative-Pressure Wound Therapy NPWT/VAC™ hopefully not necessary! Decrease edema and bacterial colonization accelerate granulation Used with absorbable mesh, biological fascial substitute Bridge to definitive reconstruction (Kilbride et al. J Ped Surg (2006) 41, 212–215)

Tissue-Expansion Mechanical process to increase surface area of adjacent tissues Examples: Growth, Silo, External Skin closure devices Adjunct to flap transfer Progressive process takes time

Tissue-Expanders Tissue expanders require a clean field with minimal inflammation Epidermis thickens, dermis and fat atrophy, muscle thins, angiogenesis Multiple expanders, small, frequent fillings

Tissue-Expanders Subcutaneous, submuscular and intraperitoneal placement all reported Small case series, longest follow-up 3 yrs (Tanenbaum et al. Plas Rec Surg (2007)120,1564–7)

Tissue-Expanders Useful in a subset of patients Additional GA, time and good local tissue conditions required Judgment in rate of expansion Extrusion and infection most frequent complications

Component Separation Relaxing incision(s) separating rectus sheath from ext obliq aponeurosis Autologous tissue, 1-stage Skin deficit? Large experience in adults

Component Separation 1 series of 10 consecutive omphalocele patients (mean age 6.5 months) Van Eijck et al. J Ped Surg (2008) Mean defect size 8 cm Required temporary prosthetic in 1 case Complications in 3 patients (skin necrosis, hematoma, infection) Mean follow-up 2 years, no hernias

Absorbable Mesh Usually a lifeboat Allows egress of fluid, visualization of bowel Used with NPWT Lasts 3-4 months….hernia Cost Vicryl™ 15x 15” $1800* * BCH list price 2013

Non-absorbable, Meshed Allows tissue ingrowth, stronger Higher rate of enterocutaneous fistulae Onlay support Cost e.g. Marlex™ 10x14” $500

Non-absorbable, Non-meshed Temporary use silo construction (e.g. Silastic™) No ingrowth, minimal adhesions Permanent use (e.g. Goretex™) higher hernia rate? Cost* $600 for 10x15”Goretex™ * BCH list price 2013

Biological Materials Variety of freeze-dried, acellular dermal or intestinal products (e.g. Alloderm™, Surgisis™) Inlay graft or onlay above fascia Neovascularized, tissues replaced by native cellular ingrowth

Biological Materials Small series/case reports in pediatric literature (Alaish et al. J Ped Surg (2006) 41, E37–E39) Variable reports in adult abdominal wall reconstruction literature Cost has come down, 5x10” sheet of Alloderm™ ~$1800* * BCH list price 2013

Flaps Local tissues usually sufficient Mobilization wide undermining Can be facilitated with relaxing incisions Zama et al. Br Assoc Plas Surg (2004) 57, 749–753

Flaps Br Assoc Plas Surg (2004) 57, 749–753

Adjunctive Procedures Skin closure: secondary but important part of reconstruction Umbilicoplasty if possible Secondary procedures: hernias, bulges, hypertophic/depressed scar

Adjunctive Procedures