BRONGO S

D'ANDREA, FRANCESCO

GRELLA E

GRELLA R

NICOLETTI GF

Archivio della ricerca - Università degli studi di Napoli Federico II

HEPATIC OMPHALOCELE IN AN ADULTFrancesco D’Andrea, Sergio Brongo, Elisa Grella, Roberto Grella and Giovanni Francesco NicolettiFrom the Plastic Surgery Department, “Second University of Naples’’, Naples, ItalyScand J Plast Reconstr Surg Hand Surg 2004; 38: 236–239Abstract. The finding of an untreated omphalocele in adulthood is extremely rare. We report the case of a 29-year-old patient, who presented to us with a congenital defect of the abdominal wall and protrusion of underlyingviscera.Key words: abdominal wall defects, omphalocele, staged abdominal wall reconstruction.Correspondence to: Professor Francesco D’AndreaVia Campiglione 18, IT-80122 Naples, Italy. (E-mail:francesco.dandrea@unina2.it)Accepted 15 May 2003An omphalocele is a congenital defect that affects thedevelopment of the abdominal wall in the umbilicalregion with a hernial type sac of variable size. It hasan incidence of between 1/3000 to 1/10 000 live births(1, 3, 11, 13, 17). It is generally treated surgically atbirth, either as an emergency or as a planned procedure,depending on the specific case and on the malfor-mations associated with it. The type of treatmentdepends on the size of the omphalocele and on thegeneral condition of the patient, from single-intervention techniques to multistage procedures.CASE REPORTA 29-year-old woman presented with an umbilicalhernia that forced her to wear a surgical trusspermanently.On examination she had a congenital malformationof the abdominal wall with protrusion of underlyingviscera, which we considered to be an omphalocele thathad been partially treated in childhood. However, thelack of specific clinical documentation and absence offamily who would have been able to help with thehistory did not permit a clear understanding of eitherthe course of the illness or the previous therapeuticinterventions.ExaminationClinical examination of the abdominal region showedan elliptical area along the midline where the ab-dominal wall was incompletely formed, which extendedlongitudinally for 18 cm from the xiphoid process tothe hypogastrium and transversely for 9 cm betweenthe two pararectal lines, with no umbilical scar. Theviscera beneath were palpable and covered by a thin,hypotrophic cutaneous layer. The structure of thethoracic arches was visibly asymmetrical, resulting ina difference of position and shape between the twobreasts (Fig. 1a).Computed tomograms of the abdominal regionshowed hypoplasia and diastasis of the rectus ab-dominis muscles in the mesogastric zone; the liver wasenlarged and dysmorphic, lying immediately beneaththe anterior abdominal wall with partial herniationat the superior part of the diastasis. The spleen wasdisplaced in the left iliac fossa and the kidneys,although normal in shape and position, were moderatelyenlarged.No interatrial cardiac or vena caval anomalies werefound. The intestinal loops were distributed irregularly,with the small intestine located in the right quadrantand the large intestine in the left. After contrast mediumhad been given a slowing of intestinal peristalsis wasshown, as well as opacification of the caecum and ofthe ascending colon, which looked to be in the midlinewithin the pelvis. Radiographic examination of thethorax showed serious scoliosis with the convexity tothe right and marked asymmetry of the two costalarches.Diagnosis and treatmentAn omphalocele with no associated anomalies wasdiagnosed, that had been partially treated in childhood 2004 Taylor & Francis. ISSN 0284–4311DOI 10.1080/02844310410026726 Scand J Plast Reconstr Surg Hand Surg 38CASE REPORTJ Plast Surg Hand Surg Downloaded from informahealthcare.com by EBSCO on 05/17/10For personal use only.with cutaneous covering of the hernial sac (GrossTechnique) (12). The defect was repaired by closure ofthe fascia and muscles of the deep layers using a mesh,and the use of tissue expansion to achieve continuity ofthe skin.The operation was done in two stages:During the first stage two rectangular expanders,16 cm by 9 cm with a volume of 500 ml, were placedat the level of the flanks, lateral to the hernial port.After she had recovered she had regular filling of theseexpanders as an outpatient until expansion of 15% oftheir volume capacity had been reached, to obtainsufficient skin for closure of the abdominal defect(Fig. 1b).During the second stage the abdominal wall wasreconstructed. The expanders were removed and thedystrophic cutaneous tissue covering the visceralprotrusion was excised. The peritoneum was carefullydetached from the overlying dermal layers througha midline vertical incision avoiding the xiphoid, soallowing direct exploration of the abdominal visceraand their repositioning within the cavity.The peritoneum was then closed and the abdominalwall reconstructed in layers. The previously savedFig. 1. (a) Preoperative view; the elliptical area iscovered by a thin, extremely hypotrophic cutaneouslayer. The absence of the umbilical scar is evident aswell as the obvious asymmetry of the breasts. (b) Tissueexpansion phase; the expanders are positioned medialto the hernial port during the phase of maximalexpansion. (c) Long term result. Recovery of tone inthe abdominal wall with a completely satisfactoryaesthetic and functional outcome.Scand J Plast Reconstr Surg Hand Surg 38Hepatic omphalocele 237J Plast Surg Hand Surg Downloaded from informahealthcare.com by EBSCO on 05/17/10For personal use only.dermal layer was used to form a first layer, and a meshof polypropylene was positioned over it to cover theentire hernial gap. The two rectus abdominis muscles,which were small and considerably displaced laterallyfrom their points of origin, were identified and isolated.The muscular fascia was sutured medially formingthe third layer. The two rectus abdominis muscleswere then mobilised and sutured medially to reinforcethe reconstruction of the abdominal wall. Continuityof the integument was ensured by bringing theexpanded cutaneous tissue together medially withouttension.The patient was discharged after four days recoveryand is well after four years of follow up. The resultremains satisfactory from anatomical, functional, andaesthetic points of view (Fig. 1c).DISCUSSIONThis case is of interest as it is an unusual pathologicalfinding in adulthood and it has therapeutic problems.The patient was living an apparently normal lifebut the congenital defect of the abdominal wallwith the associated omphalocele was troublesome. Aswell as causing aesthetic problems, the protrusionof viscera covered by a thin layer of skin wasimpinging on everyday life and relationships with theopposite sex, as she was forced to wear a permanentsurgical truss to avoid gastrointestinal complications.Correction therefore had both functional and aestheticobjectives.When planning the treatment, research into thecongenital abnormalities that may be associated withthe omphalocele was of particular importance (includ-ing horseshoe kidney and interatrial septal defects).Specific attention was paid to possible involvement ofthe inferior vena cava, because, as has been reported,these anomalies are frequently associated with, andare determinants of, the development of severe post-operative complications (2, 10).Large defects of the abdominal wall with herniationof the viscera and inadequate availability of coveringsoft tissue, are difficult to treat (5, 6, 14). The basicobjectives are: adequate protection and covering of theabdominal viscera, repair and prevention of visceralprotrusion, and achievement of an acceptable aestheticresult. Numerous methods have been described, de-pending on the presentation. The repair of the deeplayers by plastic reconstruction of fascia and muscles,in association with polypropylene or polytetrafluoro-ethylene meshes and the closure of the integument bydirect approximation of the cutaneous flaps alongthe midline is possible, but only for small defects(1, 15, 17).In the more serious cases such as ours, it is necessaryto use other techniques (7–9, 12, 15–17). The methodwe preferred involved reconstruction of the coveringlayers using tissue expansion and repair of the deeplayers with polypropylene mesh and the closure offascia and muscles.We think that using well vascularised tissue, thatcauses no tension on the sutures, such as expandedtissue (in agreement with Paletta et al. (14), Byrd andHobar (4), and Carlson et al. (5)) reduces complicationslinked to the use of prosthetic mesh and guaranteeslong term stability.In this case the choice was obviously appropriate,allowing both functional and aesthetic objectives tobe reached. Follow-up checks up to four years fromthe first intervention, have shown optimal recovery ofthe tone of the abdominal wall and a satisfactoryaesthetic result that allowed the patient to resume anormal life.REFERENCES1. Alfano C, Grippaudo FR, Tinti A. Congenital defect inthe abdominal wall in an adult: a case report. Scand JPlast Reconstr Surg Hand Surg 2001; 35: 221–223.2. Antoniou EE, Matsuoka S, Mori K, Hayabuchi Y,Kuroda Y. Anomalous inferior vena cava in associationwith omphalocele: a case report. Pediatr Radiol 1995; 25:265–266.3. Baird PA, MacDonald EC. An epidemiologic study ofcongenital malformations of the anterior abdominal wallin more than half a million consecutive live births. Am JHum Genet 1981; 33: 470–478.4. Byrd HS, Hobar PC. Abdominal wall expansion incongenital defects. Plast Reconstr Surg 1989; 84:347–352.5. Carlson GW, Elwood E, Losken A, Galloway JR. Therole of tissue expansion in abdominal wall reconstruction.Ann Plast Surg 2000; 44: 147–153.6. Deiugina MS. A plastic surgery method in vast defects ofthe anterior abdominal wall. Khirurgiia (Mosk) 2001; 3:52–54.7. Dibbell DG Jr, Mixter RC, Dibbell DG Sr. Abdominalwall reconstruction (the “mutton chop’’ flap). PlastReconstr Surg 1991; 87: 60–65.8. Fabian TC, Croce MA, Pritchard FE et al. Plannedventral hernia: staged management for acute abdominalwall defects. Ann Surg 1994; 219: 643–653.9. Gervin AS, Fischer RP. The reconstruction of defects ofthe abdominal wall with split thickness skin grafts.Surg Gynecol Obstet 1982; 155: 413–415.10. Gorenstein A, Meyer S, Shiller M. Inferior vena cavaanomalies associated with giant omphalocele – a pro-posed classification. Z Kinderchir 1983; 38: 380–382.11. Grosfeld JL, Weber TR. Congenital abdominal walldefects: gastroschisis and omphalocele. Curr Probl Surg1982; 19: 157–213.12. Gross RE. New method for surgical treatment of largeomphaloceles. Surgery 1948; 24: 277–282.13. Koivusalo A, Lindahl H, Rintala RJ. Morbidity andquality of life in adult patients with a congenitalScand J Plast Reconstr Surg Hand Surg 38238 F. D’Andrea et al.J Plast Surg Hand Surg Downloaded from informahealthcare.com by EBSCO on 05/17/10For personal use only.abdominal wall defect: a questionnaire survey. J PediatrSurg 2002; 37: 1594–1601.14. Paletta CE, Huang DB, Dehghan K, Kelly C. The use oftissue expanders in staged abdominal wall reconstruc-tion. Ann Plast Surg 1999; 42: 259–265.15. Ramirez OM, Ruas E, Dellon AL. “ComponentsSeparation’’ method for closure of abdominal-walldefects: an anatomic and clinical study. Plast ReconstrSurg 1990; 86: 519–526.16. Smith TR, Frost A. Anomalous inferior vena cava associ-ated with horseshoe kidneys. Clin Imaging 1996; 20:276–278.17. Soper RT, Green EW. Omphalocele. Surg GynecolObstet 1961; 112: 501–504.Scand J Plast Reconstr Surg Hand Surg 38Hepatic omphalocele 239J Plast Surg Hand Surg Downloaded from informahealthcare.com by EBSCO on 05/17/10For personal use only.

Hepatic omphalocele in an adult

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