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Conservative Treatment Ofgiant Omphalocele*

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Conservative Treatment Ofgiant Omphalocele* Arch Dis Child: first published as 10.1136/adc.38.198.130 on 1 April 1963. Downloaded from Arch. Dis. Childh., 1963, 38, 130. CONSERVATIVE TREATMENT OF GIANT OMPHALOCELE* BY F. SOAVE From the Department ofPaediatric Surgery, Istituto G. Gaslini, Genoa, Italy Omphalocele is a congenital malformation charac- cases. Many, such as harelip, palatoschisis, extro- terized by the failure of the abdominal walls to join phia of the bladder, meningocele, inguinal hernia together on the middle line as a consequence of a and talipes, do not require immediate treatment. developmental defect. On the other hand, it is of the utmost importance The diameter of the resulting opening may range that the presence of more severe anomalies such as from a few centimetres to the almost total absence atresia of the intestine, duplication, caecal intussus- of the abdominal wall. ception, malrotation, patent omphalomesenteric The diameter of the defect usually ranges from ductus, or trans-diaphragmatic hernia is investi- 3 to 8 cm. Naturally, the wider the opening, the gated. more completely do the intestine and liver, covered These malformations may be associated with only by the thin amniotic sac and peritoneum, omphaloceles of any size. Whatever the size of protrude from the abdomen. the hernia, there is always a disproportion between The sac is so transparent that the abdominal the volume of the organs contained in it and the organs contained in it can easily be recognized. capacity of the abdominal cavity into which this mass must be replaced, thus giving rise to a difficult Incidence surgical problem. Omphalocele is a rare condition, with an incidence Size of Omphalocele and Indications for Treatment copyright. of one case in every 6,000 births. The obstetrician Prognosis. In mild cases of omphalocele in which must avoid rupturing the amniotic sac, as occurs in the diameter of the defect in the abdominal wall is about 20 % of cases, because this is almost invariably less than 3 to 4 cm. and only a small part of the followed by secondary infection of the peritoneum. liver and part of the intestine protrude into the sac, The hernia should be disinfected with alcohol the surgical problem is not severe and the mal- and covered with a sterile dressing. The newborn formation can be corrected surgically. as soon as infant should then be transferred possible When the defect is small, the only problem is to http://adc.bmj.com/ to the Department of Paediatric Surgery because the join together the fasciae of the recti abdominis sooner the severity of the defect is investigated and muscles and the abdominal wall solidly after having the most suitable treatment selected, the less excised the amniotic sac. danger is there of contaminating the thin avascular membrane. Two-step Treatment. Cases of omphalocele of There can be no doubt that this represents an larger size, in which the diameter of the defect is emergency, because the air that the infant swallows 4 to 6 cm. or more, usually cannot be treated in one as soon as it starts breathing fills the intestine and step because the fasciae of the recti abdominis on September 26, 2021 by guest. Protected gradually distends it and it therefore becomes cannot be brought together. In such cases the increasingly difficult to reduce it into the abdominal two-step method is used. With this technique the cavity. Furthermore, unless a dressing is immedi- amniotic tissue is preserved in order to prevent the ately placed over the sac, it may become infected, formation of adhesions between the abdominal giving rise to mechanical ileus and peritonitis. organs and skin. Unless other malformations incompatible with The two-step method was described by Williams life are present, every possible attempt must be in 1930 and first successfully applied by Gross in made to cure the malformation. 1948. This technique has since been universally adopted. Associated Anomalies. In our experience, asso- It has many advantages, but also a number of ciated malformations are present in at least 55 % of drawbacks, and cannot always be applied. In fact, the amount of cutaneous tissue available is not * A paper read at a meeting of the British Association of Paediatric Surgeons in London, September 1962. always abundant and, in such cases, the suture 130 Arch Dis Child: first published as 10.1136/adc.38.198.130 on 1 April 1963. Downloaded from CONSERVATIVE TREATMENT OF GIANT OMPHALOCELE 131 is under considerable tension, with consequent Thus, the process which failed to take place, on considerable increase in endo-abdominal pressure account of a developmental defect of the walls of caused by the partly reduced abdominal organs, the abdomen during the tenth to twelfth weeks of distended by the intestinal gases; these displace the foetal life, occurs partly and incompletely. diaphragm upwards, thus obstructing the return During this process of healing by second intention, blood flow to the heart in the vena cava inferior lasting from two to three months (according to the and vena porta, with the consequent occurrence of size ofthe omphalocele), the abdominal cavity begins cyanosis, dyspnoea, and even irreversible shock. to form and to enclose the organs which it had It should be remembered that compression of formerly failed to contain. the intestine can cause perforation and peritonitis. Thus, also the liver (which almost always pro- These complications must be carefully considered trudes, to a variable extent, into the amniotic sac) in newborn infants with a large omphalocele con- forms its own site, and no inflammatory processes taining most of the liver. In such cases, provided due to compression and causing perivisceritis (as the sac has not been injured, the writer prefers the observed with the two-step surgical method) occur. conservative method, with which excellent ana- At about the end of the third month, the whole tomical and clinical results have been obtained in surface of the abdomen is covered with skin (see four cases (Figs. 5 and 6). Fig. 4), and the child must be wrapped with a soft elastic bandage of suitable tension. Conservative Method When a newborn infant with omphalocele in Our Clinical Experience. Our experience of which the diameter of the abdominal defect is more conservative treatment of omphalocele, proposed by than 4 cm. (Fig. 1) is transferred to our department, Ahlfeld in 1899 and successfully applied by Grob in surgery is discarded in favour of the following 1957 and Cunningham in 1956, began in 1956. procedure: the umbilical cord is clamped and cut as This technique was adopted after the Gross method short as possible; the entire surface of the amniotic had failed in three cases of large-sized omphalocele; sac and the abdominal wall are disinfected with these cases died during the first 24 to 48 post- alcohol and are then swabbed with a 2% mercuro- operative hours of shock caused by compression copyright. chrome solution; a sterile protective dressing is of the intestine and thoracic organs because only applied. The patient is then placed in a warm an inadequate amount of skin tissue could be bed under a protective arch which prevents the mobilized to cover the uninjured amniotic sac and blankets from contaminating the sac. This anti- consequently the suture was always excessively septic treatment is continued for a few days until taut. the thin amniotic tissue necroses and becomes In our experience, the amniotic sac disinfected covered with a thick squamous crust which protects and treated with 2 % mercurochrome does not the sac from the risk of possible infections or become contaminated and does not perforate; http://adc.bmj.com/ perforation. the gradual process of epithelization from the The base of the omphalocele is then protected borders inward favours cicatricial retraction of the by means of a ring-shaped wad of cotton wool skin and this covers the defect over a period of wrapped with a bandage, in order to fix it in the some weeks without danger of compression and required position and to prevent sudden movements. shock; this is associated with the gradual replace- Appropriate antibiotic treatment must naturally be ment of the abdominal organs into the abdominal instituted upon admittance. Granulation tissue is cavity, until they can be replaced surgically with on September 26, 2021 by guest. Protected gradually formed under the crust, from the borders plastic surgery of the abdominal walls as soon as of the sac inwards (see Fig. 2), and is then sub- this seems feasible. stituted by epithelial tissue as the dry layer drops off As indicated in the Table, 25 newborn infants (see Fig. 3). with omphalocele of different sizes were transferred Sometimes a strong-smelling corpusculated serous to our department from Liguria and other Regions secretion oozes out from under the crust. during the period 1951-1960. Of these 25 patients, As epithelization proceeds from the borders five were not treated or operated upon because of towards the centre of the defect, cicatrization and the presence of other associated malformations and subsequent retraction occur in the tissues over the of the severe condition of the babies, due to rupture defect, and this is associated with the gradual of the sac (in utero). All five died. replacement of the intestine and liver in the abdomi- Eight infants with small omphaloceles (2 to 4 cm.) nal cavity, without risk of respiratory or cardiac were operated on immediately. Three died during complications, or shock. the post-operative period. Arch Dis Child: first published as 10.1136/adc.38.198.130 on 1 April 1963. Downloaded from 132 ARCHIVES OF DISEASE IN CHILDHOOD FIG. 2. copyright. http://adc.bmj.com/ : . I FIG. 3, FIG. 5. FIG. 1.-A 15-hour-old female child with large omphalocele (9 cm.).
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