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Background and objectives : Possible disorders after delivery may interfere with the quality of life. The aim of this study was to ascertain whether abdominal muscles and fasciae differ in women depending on whether they experienced transverse cesarean section (CS) or vaginal delivery (VA) in comparison with healthy nulliparous (NU). Materials and methods : The thicknesses of abdominal muscles and fasciae were evaluated by ultrasound in 13 CS, 10 VA, and 13 NU women (we examined rectus abdominis (RA); external oblique (EO); internal oblique (IO); transversus abdominis (TrA); total abdominal muscles (TAM = EO + IO + TrA); inter-rectus distance (IRD); thickness of linea alba (TLA); rectus sheath (RS), which includes anterior fascia of RS and posterior fascia of RS (P-RS); loose connective tissue between sublayers of P-RS (LCT); abdominal perimuscular fasciae (APF), which includes anterior fascia of EO, fasciae between EO, IO, and TrA, and posterior fascia of TrA). Data on pain intensity, duration, and location were collected. Results : Compared with NU women, CS women had wider IRD ( p = 0.004), thinner left RA ( p = 0.020), thicker right RS ( p = 0.035) and APF (left: p = 0.001; right: p = 0.001), and IO dissymmetry ( p = 0.009). VA women had thinner RA (left: p = 0.008, right: p = 0.043) and left TAM ( p = 0.024), mainly due to left IO ( p = 0.027) and RA dissymmetry ( p = 0.035). However, CS women had thicker LCT (left: p = 0.036, right: p < 0.001), APF (left: p = 0.014; right: p = 0.007), and right IO ( p = 0.028) than VA women. There were significant correlations between pain duration and the affected fasciae/muscles in CS women. Conclusions : CS women showed significant alterations in both abdominal fasciae and muscle thicknesses, whereas VA women showed alterations mainly in muscles. Thinner RA and/or dissymmetric IO, wider IRD, and thicker LCT and APF after CS may cause muscle deficits and alteration of fascial gliding, which may induce scar, abdominal, low back, and/or pelvic pain., Competing Interests: The authors declare no conflict of interest.

Acquired abdominal intercostal hernia (AAIH) is an infrequent occurrence whereby intra-abdominal contents herniate into intercostal space directly from the peritoneal cavity through an acquired defect in the abdominal wall musculature and fascia. These hernias are difficult to diagnose and should always be suspected when a chest wall swelling occur after major or minor trauma. Surgical repair is warranted in symptomatic patients. The majority of AAIHs are repaired through an open approach using tension-free mesh, with significant recurrence risk. Recently, laparoscopic and robot-assisted repairs have been proposed. We discuss a 49-year-old man presented through outpatient setting with a 5-year history of ongoing left subcostal discomfort and a reducible lump. His history included a workplace accident 5 years ago. Contrast-enhanced abdominal CT confirmed AAIH with omentum herniation into the sac. A successful laparoscopic repair with intraperitoneal onlay mesh technique using composite mesh was performed., Competing Interests: Competing interests: None declared., (© BMJ Publishing Group Limited 2018. No commercial re-use. See rights and permissions. Published by BMJ.)

The role of computer tomography (CT), while choosing the abdominoplasty method in the patients with different types of the anterior abdominal wall deformity present. For CT date the anterior abdominal wall deformity type was determined in the accordance to it--the operation volume needed. Depending on the changes degree the patients were divided on 5 groups, and in accordance to the deformity type present a necessary correction was made. The CT application have had permitted to determine the changes degree in the anterior abdominal wall, and to choose a necessary volume of abdominoplasty precisely.

There were results adduced, concerning application of various kinds of alloplasty, done for complex defects of anterior abdominal wall, depending on degree of it tissues deficiency, what was determined, using the method proposed. Application of the method have permitted to estimate objectively the anterior abdominal wall tissues deficiency, and to individualize the choice of alloplasty method for the treatment results improvement.

The mannose-binding lectin associated-protease-3 (MASP-3) is a member of the lectin pathway of the complement system, a key component of human innate and active immunity. Mutations in MASP-3 have recently been found to be associated with Carnevale, Mingarelli, Malpuech, and Michels (3MC) syndrome, a severe developmental disorder manifested by cleft palate, intellectual disability, and skeletal abnormalities. However, the molecular basis for MASP-3 function remains to be understood. Here we characterize the substrate specificity of MASP-3 by screening against a combinatorial peptide substrate library. Through this approach, we successfully identified a peptide substrate that was 20-fold more efficiently cleaved than any other identified to date. Furthermore, we demonstrated that mutant forms of the enzyme associated with 3MC syndrome were completely inactive against this substrate. To address the structural basis for this defect, we determined the 2.6-Å structure of the zymogen form of the G666E mutant of MASP-3. These data reveal that the mutation disrupts the active site and perturbs the position of the catalytic serine residue. Together, these insights into the function of MASP-3 reveal how a mutation in this enzyme causes it to be inactive and thus contribute to the 3MC syndrome.

Purpose: To evaluate the effects of copaiba oil on the correction of abdominal defect treated with the use of polypropylene/polyglecaprone mesh in rats., Methods: A defect in the abdominal wall was created and corrected with polypropylene/polyglecaprone mesh in 36 rats. They were randomly distributed into three groups: control, copaiba by oral administration (gavage) and copaiba oil dip in the mesh. Euthanasia was performed after seven, 14 and 21 post-operative days. The healing process was analyzed regarding the meshes and macroscopic and microscopic aspects., Results: All animals had abdominal adhesions, which were smaller in the copaiba (gavage) group (p<0.05). In microscopy, all animals had an acute inflammation stage and the inflammatory response was best characterized by foreign body-type granulomas around the mesh fragments, which was not found in the mesh fragments within the copaiba dip group. There was a greater area of necrosis and fibrosis in the copaiba dip group compared to the control group (p<0.05). The copaiba (gavage) group had a greater quantity of collagen fibers compared to the control group., Conclusion: Copaiba oil administered by gavage decreased the amount of abdominal adhesions, besides accelerating the process of collagen fibers formation, without damages within the early stages of healing. However, when used by dip directly on the mesh, it had corrosive effects compromising the healing process of the abdominal wall.

The aim of this study follows the detailed evolution of a child diagnosed with prune-belly syndrome. This syndrome is a complex dysplasia, a rare pathology in children, characterized by the triad--the classic--hypo- or aplasia of righteous abdominal, cryptorchidism, abnormality of the urinary tract; also, it can be associated with pulmonary, cardiac, digestive, osteoarticular, and other malformations. Diagnostic criteria and etiopathogeny aspects are presented showing embryopathy and X-linked hereditary transmission theories as the most plausible, as proofed by recent genetic studies. Analyzing therapeutic aspects, it is stressed that medical treatment precedes or follows surgery, which cannot resolve urinary infection unless dysplastic urinary reconstruction is performed. Serious forms of prune-belly syndrome have a development and poor prognosis. Intrauterine and neonatal mortality is 20% and 50% in the first two years of life. The risk of urinary infection and/or lungs burdens the patient's clinical condition, allowing further appreciation on evolution of the disease. For cases solvable by plastic surgical reconstruction, as those who respond to medical therapy, differentiation will be monitored in territory and check-ups by the specialized consulting room from Polyclinic Health Center. Urinary infection relapse danger is permanent, requiring differentiated supervision. These case interest practitioners, by at least two aspects: the rarity of the disease, and complexity of dysplasia constituent, which has serious implications on the body economy.

Distinctive facial features consisting of hypertelorism, telecanthus, blepharophimosis, blepharoptosis, epicanthus inversus, periumbilical defects, and skeletal anomalies are seen in autosomal-recessive Carnevale, Malpuech, Michels, and oculo-skeletal-abdominal (OSA) syndromes. The gene or genes responsible for these syndromes were heretofore unknown. We report on three individuals from two consanguineous Turkish families with findings characteristic of these syndromes, including facial dysmorphism, periumbilical depression, mixed hearing loss, radioulnar synostosis, and coccygeal appendage. Homozygosity mapping yielded an autozygous region on chromosome 3q27 in both families. In one family, whole exome sequencing revealed a missense mutation, MASP1 c.2059G>A (p.G687R), that cosegregated with the phenotype. In the second family, Sanger sequencing of MASP1 revealed a nonsense mutation, MASP1 c.870G>A (p.W290X), that also cosegregated with the phenotype. Neither mutation was found in 192 Turkish controls or 1200 controls of various other ancestries. MASP1 encodes mannan-binding lectin serine protease 1. The two mutations occur in a MASP1 isoform that has been reported to process IGFBP-5, thereby playing a critical role in insulin growth factor availability during craniofacial and muscle development. These results implicate mutations of MASP1 as the cause of a human malformation syndrome and demonstrate the involvement of MASP1 in facial, umbilical, and ear development during the embryonic period., (Copyright © 2010 The American Society of Human Genetics. Published by Elsevier Inc. All rights reserved.)

Cantrell's Pentalogy is a rare condition that consists of defects involving the abdominal wall, lower sternum, anterior diaphragm, pericardium, and heart. In the literature to date, pregnant women with Cantrell's Pentalogy have not been discussed. We performed successful vaginal delivery of a 23-yr-old nulliparous, primigravid woman who had been diagnosed with this condition. Diagnosis was based on cardiac catheterization, angiography, and echocardiogram, and abdominopelvic CT. Vaginal delivery may be an option for women with Cantrell's Pentalogy and may be attempted with caution.

The present work describes specific congenital intestinal accidents found in commercial broiler chickens where the affected individuals have their intestinal loops translocated outside the abdomen. These anomalies have been observed in many embryos that failed to hatch, in newly hatched chicks, as well as in broilers at various stages of growth. It appears that these lesions stem from incomplete closure of the abdominal cavity during the last stages of embryonic development.

We present a case of an anomalous accessory iliacus muscle in the iliac fossa which gets originated from the iliac crest and inserting along with iliopsoas, and appear to compress the L4 root of femoral nerve. During the routine dissection of a male cadaver aged 58 years, we found an accessory iliacus muscle. The L2 and L3 nerve roots joined the L4 root distal to the accessory iliacus muscle. The L4 root of the femoral nerve supplied accessory iliacus muscle. Accessory iliacus muscle might cause tension on the femoral nerve resulting in referred pain to the hip and knee joints and to the lumbar dermatome L4. The clinical significance of this variant muscle and its importance in the femoral nerve entrapment has been discussed.

An unusual hernia is described involving a defect in the right rectus sheath at the junction of its middle and lower third. This was associated with an extension of the sac anteriorly between the right rectus muscle and its overlying sheath.

A case of Limb body wall complex is reported in a preterm, Low birth weight and stillborn neonate. The proposita had abdominal wall defect with evisceration of the organs into an amnio-peritonial sac, a short body stalk, torsion of spine with scoliosis and limb defects. Unusual associations present in this case were absent thymus, interstitial calcification in kidney and a history of exposure to birth control pills during first two months post-conception.

We report on two cases of monozygotic twins discordant for body stalk anomaly, diagnosed prenatally in a multicenter ultrasound screening study at 10-14 weeks of gestation. Ultrasound showed a large abdominal wall defect with most of the abdominal contents and almost half of the body in the celomic cavity, in association with severe kyphoscoliosis and a very short umbilical cord. Both pregnancies were managed expectantly and delivered by Cesarean section. The abnormal babies died soon after birth and autopsy confirmed the sonographic diagnosis. Body stalk anomaly in twins is extremely rare. These are, to our knowledge, the first cases reported on monozygotic twins discordant for this anomaly, indicating that the incomplete twinning theory cannot uniformly explain the pathogenesis of the body stalk in twins.

Aromatization of androgens is a key step in estrogen production, and it regulates the delicate balance between estrogens and androgens in the gonads and sex steroid target tissues. In the present study, we generated transgenic mice (AROM(+)) bearing the human ubiquitin C promoter/human P450 aromatase fusion gene. AROM(+) male mice are characterized by an imbalance in sex hormone metabolism, resulting in elevated serum E(2) concentrations, combined with significantly reduced testosterone and FSH levels, and elevated levels of PRL and corticosterone. AROM(+) males present a multitude of severe structural and functional alterations in the reproductive organs, such as cryptorchidism associated with Leydig cell hyperplasia, dysmorphic seminiferous tubules, and disrupted spermatogenesis. The males also have small or rudimentary accessory sex glands with abnormal morphology; a prominent prostatic utricle with squamous epithelial metaplasia, and edema in the ejaculatory ducts and vas deferens. In addition, the abdominal muscle wall is thin, and the adrenal glands are enlarged, with cortical hyperplasia. Some of the abnormalities, such as undescended testes and undeveloped prostate, resemble those observed in animals exposed perinatally to high levels of exogenous estrogen, indicating that the elevated aromatase activity results in excessive estrogen exposure during early phases of development. Some of the disorders in the reproductive organs, furthermore, can be explained by the fact that AROM(+) males are hypoandrogenic, and have elevated levels of serum PRL and corticosterone. Thus, the AROM(+) mouse model provides a novel tool to investigate the consequences of a prolonged increase in conversion of androgens to estrogens which results in complex hormonal disturbances altering the structure and function of various male reproductive organs.

Cantrell syndrome is characterized by defects that involve the diaphragm, abdominal wall, pericardium, heart, and lower region of the sternum. It is a rare entity, usually diagnosed at birth and accompanied by high mortality due to the complexity and gravity of the anomalies. In this report, we present a 32-year-old male patient, who was diagnosed in infancy but who reached adult age asymptomatic.

A 28-year-old woman was referred at 33 weeks of gestation with suspected fetal intestinal atresia. Sonography showed a large extra-abdominal mass on the right of the normal umbilical cord insertion. Following Cesarean section at 36 weeks and immediate surgical treatment, the malformation was not definable either as an omphalocele or as gastroschisis. This reported case involves a previously undocumented malformation of the fetal abdominal wall described as a 'hernia' of the fetal abdominal wall.

We present a case of partial Cantrell's syndrome with ventricular septal defect, left ventricular diverticulum, dextrorotation of the heart, an anterior diaphragmatic defect, and a midline supraumbilical abdominal wall defect with omphalocele. At the age of 20 months, the patient underwent a successful cardiac surgical procedure. To detect risk factors and to define therapeutic strategies, we analyzed the spectrum and the frequency of malformations described in 153 patients with Cantrell's syndrome. Despite modern surgical standards, Cantrell's syndrome represents a challenge to the surgeon because of the wide spectrum of anomalies, the severity of the abdominal and cardiac malformations, and the high mortality.

Ultrasound examination in a 10-week fetus demonstrated a marked deformity of the lower half of the fetus that was in the celomic cavity, and the upper half of the body was in the amniotic sac. There was abdominal wall defect with herniation of the liver and bowel, the lower limbs were deformed and the umbilical cord was short and thin. These abnormalities were suggestive of body stalk anomaly. The diagnosis was confirmed by pathological examination after termination of the pregnancy at 18 weeks.

In a multicenter project of screening for chromosomal defects by fetal nuchal translucency thickness and maternal age at 10-14 weeks, 14 of 106,727 fetuses examined had body stalk anomaly. The ultrasonographic features were a major abdominal wall defect, severe kyphoscoliosis and a short umbilical cord. In all cases, the upper part of the fetal body was in the amniotic cavity, whereas the lower part was in the celomic cavity. The nuchal translucency thickness was above the 95th centile in ten (71.4%) of the cases, but the fetal karyotype was normal in all 12 fetuses evaluated. The findings suggest that early amnion rupture before obliteration of the celomic cavity is a possible cause of the syndrome.

Two cases of notching in the umbilical artery Doppler waveform in a pregnancy with gastroschisis are reported. The etiology of the abnormal Doppler pattern in relation to the displacement of the fetal stomach in this congenital abnormality is discussed.

Aims: To assess the long term morbidity and quality of life in survivors of gastroschisis., Design: All babies born with gastroschisis between 1972 and 1984 and who survived more than one year were identified. Those who could be traced were questioned about their general health, growth, abdominal symptoms, cosmetic concerns, education, employment, and fertility., Results: Of the 35 patients, two have died, seven could not be traced, and three declined to be interviewed. Twenty three subjects (70% of survivors) with a median age of 16 years (range 12-23 years) responded. Twenty two (96%) were in good health and overall growth was within normal limits. Eight subjects (35%) have had further surgery related to gastroschisis, including two for adhesive small bowel obstruction and three for scar complications. In 13 (57%), absence of an umbilicus caused distress during childhood., Conclusion: Most gastroschisis survivors can eventually expect normal growth and good health. Adhesive bowel obstruction is an uncommon, but potentially late, complication. The umbilicus should be conserved during gastroschisis repair.

Objectives: Analysis of incidence and characteristics of congenital abdominal wall defects, with special reference to the differences between the incidence of gastroschisis and exomphalos (omphalocele)., Design: Retrospective analysis using data from the Office of Population Censuses and Surveys (recoded to differentiate exomphalos and gastroschisis) and the National Congenital Malformation Notification Scheme., Setting: England and Wales, 1987 to 1993., Results: 1043 congenital anterior abdominal wall defects were notified within the seven year study period. Of these, 539 were classified as gastroschisis, 448 as exomphalos, 19 as "prune belly syndrome," and 37 as "unclassified." Gastroschisis doubled in incidence from 0.65 in 1987 to 1.35 per 10,000 total births in 1991, with little further change; the incidence of exomphalos decreased from 1.13 to 0.77 per 10000 births. The overall incidence of notified congenital abdominal wall defects was 2.15 per 10000 total births. Gastroschisis was associated with a lower overall maternal age than exomphalos and with a significantly lower proportion of additional reported congenital malformations (5.0%) than in the cohort with exomphalos (27.4%) (odds ratio 0.14, 95% confidence interval 0.09 to 0.22; P < 0.001). The sex ratio of the two cohorts was the same. The incidence of gastroschisis and exomphalos was higher in the northern regions of England than in the south east of the country., Conclusions: The national congenital malformation notification system showed an increasing trend in the incidence of fetuses born with gastroschisis and a progressive decreasing incidence of exomphalos in England and Wales between 1987 and 1993. Although the reasons for this are likely to be multifactorial, a true differential change seems likely. The observed increase in incidence of gastroschisis relative to exomphalos and the differentiation in maternal age have implications for resource management within the NHS and warrant further epidemiological monitoring. Regional differences may be due to a dietary or environmental factor, which requires further study.

Objective: The authors study reviewed patients who underwent operations for omphalocele and gastroschisis to determine survival, morbidity, and long-term quality of life., Method: Clinical follow-up of 94 patients cared for with omphalocele and gastroschisis during a 10- to 20-year period after birth., Result: Eighty-three patients survived initial treatment. Sixty-one had long-term follow-up. Mean follow-up in the group was 14.2 years. Survival was favorable in the absence of lethal or co-existing major congenital anomalies. Nineteen patients required 31 reoperations, most for abdominal wall hernias and the sequelae of intestinal atresia. Current quality of life was described as favorable (good) in 80% of patients., Conclusions: Survival rate in patients with abdominal wall defects is favorable and deaths occur substantially in patients with co-existing lethal, or multiple, congenital anomalies. Reoperative surgery is necessary principally in those patients who have postclosure abdominal wall hernias, and in those with bowel atresia at birth. Reoperations are not likely to be necessary after school age. Quality of life in survivors is patient-perceived as entirely satisfactory.

A child with phenotypic features of the 9p- syndrome, including metopic craniosynostosis, small ears, abdominal wall defect, and mental retardation, as well as hypopigmentation, was found to have a cytogenetically balanced 3;9 translocation, with breakpoints at 3p11 and 9p23, inherited from his phenotypically normal father. Molecular analysis showed heterozygous deletion of the TYRP (tyrosinase-related protein) locus, as well as loci D9S157, D9S274, D9S268, and D9S267, in the child but in neither parent. FISH analysis of the proband's father indicated that loci deleted in his son, including TYRP, were present on neither the der(3) nor the der(9) translocation products but had been inserted into the long arm of chromosome 8. Therefore, the apparent deletion of these loci in the proband was the result of meiotic segregation of the father's 3;9 translocation chromosomes together with his normal chromosome 8 (not bearing the insertion from 9p23). Neither the deletion of these 9p23 loci from the translocation chromosomes nor their insertion into 8q was detectable by standard chromosome banding techniques. The proband's sister exhibited speech delay, mild facial dysmorphism, and renal malformation, and her karyotype was 46,XX. Molecular analysis showed that she had inherited normal chromosomes 3 and 9, as well as the chromosome 8 with the insertion of 9p23 material, from her father.(ABSTRACT TRUNCATED AT 250 WORDS)

Objective: This study analyzed the factors influencing the postoperative results after the repair of gastroschisis defects during the past 27 years., Summary Background Data: The clinical results after the repair of gastroschisis abdominal defects have improved appreciably during the past 25 years, with the long-term survival rate in most large children's centers currently being approximately 90%. The improvement in survival has been largely attributed to advances in perioperative care, frequent use of parenteral nutrition, and better techniques of surgical repair., Methods: Between 1965 and 1992, 84 infants with gastroschisis underwent surgical repair. The management of 52 infants after 1979 was compared with that of 32 during the previous 14 years. Associated anomalies were present in 29%. The average birth weight was 2412 g. In 31%, primary fascial closure was performed. In another 31% with moderate visceroabdominal disproportion (VAD), a silastic chimney was used initially, and complete repair was performed at a second operation. For 25% who had severe VAD, more than two operative reconstructions were necessary. Seven of 52 infants with moderate VAD underwent initial skin-flap closure and secondary repair within 12 days., Results: Almost all complications (27%) and deaths (4%) occurred in infants with severe VAD and were largely unrelated to associated malformations or birth weight. The length of postoperative mechanical ventilation, need for parenteral nutrition, need for multiple operations, and length of hospitalization were all directly related to the severity of the VAD., Conclusions: Complete repair of gastroschisis at the initial operation is the optimal goal; however, the severity of VAD has permitted this approach in only one third of patients in this study. Delayed repair with a silastic chimney and one or more reconstructive procedures has provided excellent long-term survival with low morbidity and mortality rates. Although skin-flap closure is no longer used initially, this technique has been helpful for the residual defect in infants with severe VAD who have had multiple silon chimney repairs (Applied Biomaterial, Silverdale, WA).

This study aims to establish the usefulness of delivering neonates with gastroschisis in a regional obstetric and neonatal centre without the facility of on site surgery. A retrospective analysis was performed on the notes of 43 consecutive neonates with gastroschisis referred to Birmingham Children's Hospital over a 10 year period. Two groups were compared: those delivered at the regional obstetric centre (n = 9) and those delivered peripherally (n = 34). Both groups underwent postnatal transfer. There were no significant differences with regard to gestational age, birth weight, caesarean section rate, time to operation, and mortality. Primary closure rates were 89% for the regional centre group and 94% for the peripheral hospital group. Mean time to full enteral feeding was 24 days for the regional centre group and 23 days for those delivered peripherally. These data show that good results can be achieved with postnatal transfer. If on site surgery is not available, neonatal services are adequate peripherally, and the transfer distance is not too great, then delivery in a regional obstetric centre with subsequent postnatal transfer offers no advantage.

In order to determine whether the elective delivery of babies with gastroschisis confers advantages in outcome, the case-notes of all babies referred to Bristol with gastroschisis over a 10 year period were reviewed. Various factors were compared between babies born in Bristol and those born elsewhere in the South West region. One neonate from the outborn group died after transfer to Bristol but otherwise there was no evidence that transfer and elective delivery in Bristol conferred any advantage. The important of effective resuscitation of outborn babies before transfer is emphasised and guidelines for resuscitation given.

Forty infants with gastroschisis were referred to two paediatric surgeons during a 13 year period. Overall survival was 90%. Nine patients were transferred in utero and 31 were referred postnatally. Birth weights, gestational ages, and Apgar scores were similar for both groups. Primary closure of the defect was successfully achieved in seven (78%) patients in the prenatally transferred group compared with 17 (55%) in the postnatal group. Significantly less postoperative assisted ventilation, and a trend in favour of early discharge home, were noted after prenatal transfer. Problems arising during postnatal transfer may have contributed to these differences. No major differences resulting from the mode of delivery were identified. Patients treated by primary closure fared significantly better than those undergoing staged repairs with prosthetic material. Prospective randomised studies are required to confirm these findings.

A review of six years' experience with antenatal diagnosis of abdominal wall defects by ultrasound showed its impact to be limited by poor detection rates. Twenty infants with exomphalos and 20 with gastroschisis were recorded but only 25 (63%) were diagnosed antenatally. The ultrasound false negative rate was higher for exomphalos (35%) than for gastroschisis (22%). No difference was detected in the incidence of associated abnormalities, premature gestation, primary closure rate, or mortality between the antenatally and postnatally diagnosed groups for either exomphalos or gastroschisis. Antenatal diagnosis of gastroschisis has little effect on management but allows parental counselling and in utero transfer. The frequency of concomitant abnormalities in exomphalos profoundly affects prognosis and the detection of these is the major role of antenatal diagnosis in this condition. Failure to detect abdominal wall defects by ultrasound may be a reflection of technique or equipment, but some gastroschisis may be of perinatal onset and not detectable antenatally.

A total of 48 infants with abdominal wall defects referred to the South West Regional Neonatal Surgical Centre over a period of six years were reviewed. There were 27 (56%) infants with gastroschisis and 21 (44%) infants with exomphalos. At the regional centre, four pregnancies with gastroschisis were terminated following a prenatal diagnosis, one child was stillborn, and one with exomphalos and trisomy 13 died soon after birth. Of the remaining 42 live births, the one year survival for babies born with a gastroschisis was 95% (21/22) and with an exomphalos was 81% (17/20). All deaths, except one, were due to factors other than the abdominal defect. The effect on outcome of prenatal diagnosis, parental counselling, in utero transfer, mode of delivery, and methods of surgical closure was reviewed.

Based on 14 years' experience with the surgical repair of gastroschisis abdominal wall defects in 32 infants at the UCLA Hospital, certain aspects of care evolved which have served to reduce the overall long-term mortality to 6.2%. The severity of gastroschisis defects appears to be related to the length of time the eviscerated intestine has been exposed to amniotic fluid, and the degree of vascular obstruction to the viscera. In contrast to reports by previous authors recommending a specific operative technique for all infants with this malformation, we believe that choice of the optimal surgical repair depends on the degree of disproportion between the size of the eviscerated intestine and the size of the abdominal cavity. Three of the 32 patients with minimal disproportion underwent primary skin and muscle closure followed by early recovery. Twenty-seven who had primary skin flap closure later underwent secondary ventral hernia repair within six to 12 months. Two of the 32 infants had severe viscerobadominal disproportion and required temporary prosthesis coverage in addition to extensive skin flaps during the primary repair. The low morbidity and mortality following gastroschisis repair are apparently related to these factors: avoiding undue compression of the viscera; early coverage of the contaminated viscera with skin or muscle to minimize infection; careful supportive perioperative management to maintain body heat and provide adequate fluid repletion; and the infusion of intravenous hyperalimentation solutions during the lengthy period of post-operative ileus. Prosthetic materials should be reserved for more complex abdominal wall reconstruction in infants who have severe visceroabdominal disproportion.