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Background: Intranasal corticosteroids (INCS) are frequently used to treat OSA syndrome (OSAS) in children. However, their efficacy has not been rigorously tested., Research Question: Do INCS result in improved OSAS symptoms, polysomnography findings, behavior, and quality of life compared with placebo?, Study Design and Methods: In this randomized, double-blind, placebo-controlled trial, children with OSAS aged 5 to 12 years (N = 134) were randomized 2:1 to receive 3 months of INCS or placebo. Children in the INCS arm were then re-randomized to receive 9 months of INCS or placebo. Polysomnography, symptoms, and neurobehavioral findings were measured at baseline, 3 months, and 12 months. The primary outcome was change in obstructive apnea hypopnea index (OAHI) at 3 months, available for 122 children. The secondary outcome was OAHI change at 12 months, available for 70 children., Results: Median (interquartile range) age and OAHI at baseline for the entire group were 7.9 (6.3 to 9.9) years and 5.8 (3.6 to 9.7) events per hour. OAHI changes at 3 months (-1.72 [-3.91 to 1.92] events per hour) and 12 months (-1.2 [-4.22 to 1.71] events per hour) were not different between the two groups (P = not significant). OSAS symptoms and neurobehavioral results did not differ between the INCS and placebo groups at 3 and 12 months. The 38 children who received INCS for 12 months reported a significant OAHI decrease from 7.2 (3.62 to 9.88) events per hour to 3.7 (1.56 to 6.4) events per hour (P = .039)., Interpretation: In children with OSAS, treatment with INCS did not result in significant polysomnography, neurobehavioral, or symptom changes at 3 and 12 months of treatment. Twelve months of INCS treatment resulted in a statistically significant but not clinically relevant OAHI reduction., Clinical Trial Registration: ClinicalTrials.gov; No.: NCT02180672; URL: www., Clinicaltrials: gov., (Copyright © 2022 American College of Chest Physicians. Published by Elsevier Inc. All rights reserved.)

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.)

We report four individuals from two unrelated consanguineous families with 3MC syndrome. In the first family, chromosome microarray data revealed that the two affected sisters, born to first-cousin parents, shared a unique homozygous C-terminal deletion in the COLEC11 gene. Two affected brothers from a second family, also born to first-cousin parents, shared a region of homozygosity that included the second gene known to cause the 3MC syndrome, MASP1. We discuss the diagnostic approach of craniofacial disorders born to consanguineous parents and highlight a literature search and reference a helpful dysmorphology solution powered by FDNA (Facial Dysmorphology Novel Analysis) technology.

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.

Objective: The objective of the study was to evaluate whether the current screening regimen of measuring amniotic fluid alpha-fetoprotein (AF-AFP) at the time of amniocentesis and reflex acetylcholinesterase testing vs ultrasound alone to detect neural tube and ventral wall defects offers improved diagnostic accuracy and cost benefit., Study Design: A retrospective chart review on all patients who had amniocentesis performed at 1 center over the past 11 years was performed. Those with an elevated AF-AFP were compared with those whose AF-AFP was within normal limits. Ultrasound findings and outcomes were reviewed in all cases to assess whether neural tube defects (NTDs) or ventral wall defects (VWDs) were missed by AF-AFP or ultrasound screening. A cost-benefit analysis was then performed., Results: Of 6232 women who underwent amniocentesis between January 2002 and December 2012, 81 had an elevated AF-AFP with or without a positive acetylcholinesterase (AChE). Of these 81 women, 13 had NTDs and 5 had VWDs. The sensitivity of the detailed ultrasound was 100% in detecting NTDs and VWDs, whereas that of the AF-AFP ranged from 22% to 77%, with the inclusion of AChE. The total expenditure for AF-AFP in our sample set (n = 6232 amniocentesis at $76.00 per AF-AFP) was $473,632, and all NTDs and VWDs were detected by ultrasound. Translated to a national laboratory (>42,447 samples/year), the cost savings in 2011 alone would be $3,225,972., Conclusion: Given the accuracy of high-resolution ultrasound in the detection of both NTDs and VWDs, measuring AF-AFP and AChE as a reflex-screening test is not a cost-effective approach., (Copyright © 2014 Mosby, Inc. All rights reserved.)

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.

Human omphalocele is a congenital defect of the abdominal wall in which the secondary abdominal wall structures (muscle and connective tissue) in an area centered around the umbilicus are replaced by a translucent membranous layer of tissue. Histological examination of omphalocele development and moreover the staging of normal human abdominal wall development has never been described. We hypothesized that omphalocele is the result of an arrest in the secondary abdominal wall development and predicted that we would observe delays in myoblast maturation and an arrest in secondary abdominal wall development. To look for evidence in support of our hypothesis, we performed a histological analysis of normal human abdominal wall development and compared this to mouse. We also conducted the first histological analysis of two human specimens with omphalocele. In these two omphalocele specimens, secondary abdominal wall development appears to have undergone an arrest around Carnegie Stage 19. In both specimens disruptions in the unidirectional orientation of myofibers were observed in the external and internal obliques, and rectus abdominis but not in the transversus abdominis. These latter findings support a model of normal abdominal wall development in which positional information instructs the orientation of myoblasts as they organize into individual muscle groups., (Copyright © 2012 Wiley Periodicals, Inc.)

The lectin pathway of complement is an important component of innate immunity. Its activation has been thought to occur via recognition of pathogens by mannan-binding lectin (MBL) or ficolins in complex with MBL-associated serine protease (MASP)-2, followed by MASP-2 autoactivation and cleavage of C4 and C2 generating the C3 convertase. MASP-1 and MASP-3 are related proteases found in similar complexes. MASP-1 has been shown to aid MASP-2 convertase generation by auxiliary C2 cleavage. In mice, MASP-1 and MASP-3 have been reported to be central also to alternative pathway function through activation of profactor D and factor B. In this study, we present functional studies based on a patient harboring a nonsense mutation in the common part of the MASP1 gene and hence deficient in both MASP-1 and MASP-3. Surprisingly, we find that the alternative pathway in this patient functions normally, and is unaffected by reconstitution with MASP-1 and MASP-3. Conversely, we find that the patient has a nonfunctional lectin pathway, which can be restored by MASP-1, implying that this component is crucial for complement activation. We show that, although MASP-2 is able to autoactivate under artificial conditions, MASP-1 dramatically increases lectin pathway activity at physiological conditions through direct activation of MASP-2. We further demonstrate that MASP-1 and MASP-2 can associate in the same MBL complex, and that such cocomplexes are found in serum, providing a scenario for transactivation of MASP-2. Hence, in functional terms, it appears that MASP-1 and MASP-2 act in a manner analogous to that of C1r and C1s of the classical pathway.

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.

We report a case of gastroschisis in which a paraumbilical band was found at the right margin of the abdominal wall defect and extended into the antimesenteric side of the small intestine. The band consisted of 2 thin cords. Microscopically, 1 band showed a fibrous tissue, and the other 1 revealed a unique vascular structure resembling the vitelline artery and vein, suggesting that the paraumbilical band represents a remnant of the yolk stalk that failed to be incorporated into the umbilical stalk. The origin of the paraumbilical band and an associated pathogenetic hypothesis of gastroschisis are discussed.

Background: Since 30 years, we have attempted to repair gastroschisis as early as possible, often even in the delivery room. We examined 12 recent years of patient records to evaluate the effect of immediate repair and other factors on the outcome of gastroschisis., Methods: We reviewed the medical records of patients presenting with gastroschisis (87) at the Children's Hospital of Michigan between 1998 and 2009. Data were evaluated specifically to determine the effect of the place of repair [obstetric hospital ("DR") vs. children's hospital ("OR")], the time of repair [less than an hour after delivery ("IR") or more than one hour ("ER")], and the type of repair [primary fascial repair and skin closure ("PR") vs. staged repair ("SR")]., Results: Patients in the PR group were more likely to spend one week or less on MV (66% in PR vs. 11% in SR, p<0.01). Patients in the DR group were more likely to spend 2 weeks or less on TPN, as were patients in the PR group (51% in PR vs. 17% in SR, p<0.01). Patients in the PR group were more likely to stay in hospital for less than 3 weeks, but the IR and ER groups had almost same hospital stay. Major associated anomalies were present in 19 patients (29%). These patients and those with little or no peel tended to outperform those with peel in each of our outcome measures., Conclusion: Repair immediately after delivery is beneficial in terms of achieving primary closure of the defect, leading to shorter times on assisted ventilation and parenteral nutrition, and shorter hospital stays., (© Georg Thieme Verlag KG Stuttgart · New York.)

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.)

Prune-belly sequence (PBS) usually results from early urethral obstruction. In rare cases, PBS seems to be due to a faulty primary development of the parietal mesenchyme leading to underdevelopment of the abdominal wall musculature, and disorganization of the smooth muscles in the urinary tract. We report on two patients with segmental, unilateral wall musculature deficiency associated with homolateral agenesis of ribs. One patient also had hemivertebrae and the other one ipsilateral diaphragmatic eventration and aplasia cutis. This combination of anomalies may represent a localized deficiency in the development of somitic mesoderm mesenchyme during early embryogenesis., (© 2010 Wiley-Liss, Inc.)

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.

Prune Belly Syndrome is a rare congenital anomaly characterized by deficient anterior abdominal wall musculature, bilateral cryptorchidism, bilateral megaureters and often unilateral or bilateral vesico-ureteric junction obstruction. The report of prune belly syndrome in the adult is scanty. We report a case of prune belly syndrome in a 24 year old Nigerian who presented with 3 year history of recurrent right loin pain. Examination showed wrinkled abdominal skin, bilateral undescended testes and an hypoplastic rectus abdominis, below the umbilicus. Further evaluation revealed enlarged bladder, bilateral megaureters and right intra-abdominal testis. A diagnosis of Prune Belly Syndrome was made. The challenges in the diagnosis and management of this rare condition are highlighted in this presentation.

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.

Central and lateral abdominal wall defects are probably distinct and likely arise from different pathogenetic mechanisms. An autopsy study was done using data from a total of 45 central and lateral abdominal wall defect cases to evaluate if they are indeed separate entities and to suggest possible mechanisms involved in their formation. Central defects were found to be statistically different from lateral defects; malformations that co-existed with central defects were mainly bilateral and internal and also involved "inferior" organs in relation to fetal-embryonal blood flow. Patients with lateral defects were more often female, and their coexistent defects were usually unilateral and external, with only 1 defect occurring in an "inferior" organ. These results indicate mechanisms of a vascular perfusion deficit for the majority of the central defects and of external disruption for the lateral defects.

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.

The acute abdomen in the newborn provides challenging problems from many aspects, not only with regard to diagnosis, resuscitation and treatment, but also now with prenatal management. Most conditions are uncommon and treatment in specialist centres enables concentration of appropriate resources and expertise. Co-morbidity is common, particularly in the preterm or low birth weight infant. A multi-disciplinary team of surgeons, anaesthetists, neonatologists, radiologists, cardiologists, obstetricians, nurses, physiotherapists and other health professionals experienced in dealing with extremely small infants will provide the best outcome. The infant should be resuscitated and, as soon as conditions permit, transferred to a specialist surgical centre with intravenous fluids, gastric tube drainage and circulatory, respiratory and general support as needed. This involves close liaison within healthcare networks and readily available patient transfer facilities. Surgery itself should be carried out in a theatre fully equipped for neonatal surgery. A gentle touch is essential because of the fragility of the tissues, and painstaking care should be taken with blood loss.

Background: There are two approaches to close gastroschisis. Primary closure (PC) is reduction and fascial closure; silo closure (SC) places viscera in a preformed-silo and reduces the contents over time. We have shifted from PC to SC. This study compared the outcomes of these two techniques., Methods: Records of babies with gastroschisis from 1994-2004 were reviewed. Closure type, ventilator days, days to full-feeds, hospital days, complications, and mortality were recorded., Results: Twenty-eight patients underwent PC; 20 patients had SC. Differences in ventilator days, days to full-feeds, and hospital days were not statistically significant. Nine PC patients developed closure-related complications vs. none in SC (P < 0.05). Eight PC vs. two SC patients had non-closure-related complications (P < 0.05). Four PC vs. zero SC patients developed necrotizing enterocolitis (P < 0.05). Five PC vs. one SC patients had ventral hernia (P < 0.05). No patient died., Conclusion: PC resulted in higher incidence of reclosure, non-closure-related complications, and necrotizing enterocolitis. Consequently, we recommend SC as the preferred treatment.

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.

Msx1 and Msx2 genes encode the homeodomain transcription factors. Several gene knockout mice and expression studies suggest that they possess functionally redundant roles in embryogenesis. In this study, we revealed that Msx1 and Msx2 were expressed during ventral body wall formation in an overlapping manner. Msx1/Msx2 double-mutant mice displayed embryonic abdominal wall defects with disorganized muscle layers and connective tissues. These findings indicate that Msx1 and Msx2 play roles in concert during embryonic ventral abdominal wall formation., (Copyright (c) 2005 Wiley-Liss, Inc.)

We present the ultrasonographic diagnosis at the 19th gestational week in a case of Prune-belly syndrome which consists in abdominal wall distension with deficiency of the abdominal wall musculature, urinary tract abnormalities (specially mega bladder), and in males, cryptorchidism. The pathologic examination found the patient devoid of striated muscles anterior abdominal wall, hypertrophy of the urinary bladder, and urethral atresia. The Prune-belly is accessible to antenatal diagnosis and the variable prognosis does not always justify abortion.

The Pentalogy of Cantrell (PC) is a rare association of defects involving the lower sternum, abdominal wall, diaphragm, pericardium and heart. We report two rare cases of the PC (variant form), showing fatal progression. Case 1 only survived two hours because of severe cardio-respiratory failure. Physical examination showed midline abdominal and thoracic defects, ectopic heart, pericardial defect, diaphragmatic defect, bilateral undescended testis, scoliosis, and adherence between left upper limb and trunk. In addition, the autopsy revealed diaphragmatic agenesia, intraabdominal testis, bilateral lung hypoplasia and lymphocytic meningitis. Case 2 only survived 15 minutes. In addition to the physical findings, including lower sternal defect, ectopic heart, epigastric omphalocele and scoliosis, the autopsy showed left diaphragmatic agenesia, pericardial agenesia, bilateral lung hypoplasia, deformed rib cage, anterior thoracic myeloschisis, adreno-hepatic fusion, left renal agenesia, meckel diverticulum and multiple accessory spleens. When comparing with other cases of PC, the concurrence of bilateral intraabdominal testis and lymphocytic meningitis in case 1, and adreno-hepatic fusion, anterior myeloschisis, meckel diverticulum, multiple accessory spleens, and renal agenesia in case 2 have not been described previously.

The prenatal diagnosis of laparoschisis may be established by ultrasound early in the end of the first pregnancy trimester. The visualisation of the abdominal wall defect may predict a good fetal prognosis in the absence of the other anomalies and may establish the most favorable moment of birth. Identification of several risk factors for this malformation could lead to the decrease of its incidence.

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.

Gastroschisis, one of the more common congenital abdominal wall defects, results in herniation of fetal abdominal viscera into the amniotic cavity. This article discusses theories about gastroschisis etiology, in utero diagnostic tools, delivery options, and postdelivery care. Included are detailed considerations regarding immediate interventions after delivery to support the infant's thermal and fluid management needs and to protect the exposed bowel. Surgical options and postoperative care issues and complications are reviewed, as are respiratory distress and vena cava compression from increased abdominal pressure, nutritional support, and interventions related to the prevention of infection. Giving birth to an infant with gastroschisis is an upsetting experience for parents. Evidence suggests, however, that with today's advances in neonatal care and nutrition and with meticulous attention, the survival rate for infants born with gastroschisis can be excellent.

The frequent use of prenatal diagnostic techniques including ultrasound and maternal serum alpha-fetoprotein has increasingly led to detection of abdominal wall defects before birth. This prenatal detection creates the opportunity to influence neonatal outcome by alteration in management of pregnancy or delivery. The optimal management of an individual fetus depends on careful prenatal assessment of the abdominal wall defect, combined with experience and knowledge of the natural history for that particular lesion. A multidisciplinary approach to the fetus can improve neonatal outcome. Careful assessment for other structural anomalies and karyotype analysis should be performed. Delivery at a high-risk perinatal center should be encouraged. Currently, there is no convincing evidence to support routine cesarean section for most abdominal wall defects.

Authors analyzed the type and the number of treated congenital malformations in 544 newborns operated between 1992-2001 in the Department of Paediatric Surgery in the Institute of Mother and Child. The patients were divided in the following groups: digestive tract defects, abdominal wall and diaphragm defects, neural tube defects, urinary track defects, craniofacial and brain defects and others anomalies occurring rarely. Most of the operations were preformed in the first 48 hours of life. Since 1995 special newborn transport, early cardiac surgery and neonatal intensive care have been introduced. Total mortality of operated newborns and death in particular groups were analyzed. The implemented elements caused a decrease in mortality from 36 to 13 percent. In the authors' opinion improvement in treatment results is due to earlier diagnosis and better understanding of pathophysiology of the defects, introduction of noninvasive pre- and postnatal diagnostics and establishment of centres specialized in neonatal surgery and intensive care.

Survival for newborns with congenital abdominal wall defects (primarily omphalocele and gastroschisis) has improved, but controversy remains regarding etiology, anatomy and embryology, the role of prenatal diagnosis and mode of delivery, and initial management. A number of recent studies have added to our knowledge and understanding of several of these topics, while several others have raised questions regarding traditional initial management of these infants. Continued improvement in the survival of these infants can be anticipated with further understanding of the in utero and antepartum diagnosis and management of infants with these common congenital abnormalities.

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.

In the last two to three decades, increasing rates of gastroschisis but not of omphalocele have been reported from different parts of the world. The present study represents a register containing 469 children born with abdominal wall defects based on data retrieved from 20 birth cohorts (1970-89) in three nationwide registries. A tentative estimate of the completeness as regards identification of liveborn and stillborn infants is a minimum of 95% and 90% respectively. All cases were reclassified to 166 cases of gastroschisis, 258 of omphalocele and 16 of gross abdominal wall defect. The average point prevalence at birth of gastroschisis was 1.33 per 10 000 live and stillbirths. During the first decade, an increase in prevalence occurred culminating in 1976, followed by a decrease reaching its initial value in 1983 and then a new increase. Overall, no significant linear trend could be demonstrated for the entire period. The average point prevalence at birth for omphalocele was 2.07 and for gross abdominal wall defect 0.12 per 10 000 live and stillbirths with no significant change in the period. The geographical distribution of gastroschisis and omphalocele showed no difference per county.

The prune-belly syndrome (PBS) usually is described as a deficiency of the anterior abdominal muscle involving bilateral cryptorchidism and urinary tract malformations. In this report, we will present an eleven-month-old boy with PBS associated with a complex cardiac anomaly. A bilateral bidirectional Glenn shunt was performed with the diagnosis of isolated dextrocardia, single ventricle, pulmonary atresia, incomplete A-V septal defect, hemiazygos continuity, persistent right superior vena cava, patent ductus arteriosus-dependent pulmonary blood flow. The patient required special consideration for postoperative pulmonary care.

Objective: To determine whether there is sufficient evidence to support cesarean delivery over vaginal delivery in women carrying a fetus with an abdominal wall defect., Data Sources: An OVID MEDLINE search of English language abstracts using medical subject heading terms "gastroschisis," "omphalocele," and "fetal abdominal wall defects" was performed. The generated list of articles was supplemented by a review of their bibliographies and the bibliographies of obstetric texts., Study Selection: A total of 27 peer-reviewed observational studies were identified, and 15 were included in the meta-analysis. Our primary inclusion criterion was the reporting of neonatal outcomes for infants with abdominal wall defects who delivered vaginally and who delivered by cesarean section. Studies were excluded if they were a case series or if neonatal outcomes could not be ascertained from the data presented in the manuscript., Tabulation, Integration, and Results: Standard meta-analytic techniques were applied to assess the question of whether cesarean delivery improves neonatal outcomes in infants with abdominal wall defects. There was no significant relationship between mode of delivery and the rate of primary fascial repair (random effects model: pooled relative risk [RR] 1.22, 95% confidence interval [CI] 0.99, 1.51), neonatal sepsis (random effects model: pooled RR 0.70, 95% CI 0.30, 1.62), or pediatric mortality (random effects model: pooled RR 1.14, 95% CI 0.59, 2.21). Additionally, there was no significant relationship between mode of delivery and time until enteral feeding or length of hospital stay., Conclusion: The available data do not provide evidence to support a policy of cesarean delivery for infants with abdominal wall defects.

With the routine use of fetal imaging studies during prenatal care, increased numbers of unusual intrauterine events are now detected. Prenatal closure of the abdominal defect in gastroschisis is an example. We report a 34 5/7-week stillborn who had prenatal closure of a ventral abdominal wall defect, which had been seen earlier on fetal ultrasound examination. Two ultrasound examinations performed at 15 1/7 weeks and 19 1/7 weeks showed a mass of exteriorized bowel that herniated through the abdominal defect, just to the right of the umbilical cord. At 30 1/7 weeks, no exteriorized bowel was seen, but thickened and dilated intraabdominal bowel was identified. No abdominal defect or exteriorized bowel was found at autopsy. There was a severely dilated proximal jejunum with the absence of the rest of the small intestine and the right side of the colon. The remaining left side of the colon was small and blind proximally. Six similar isolated examples have been reported since 1991. Prenatal closure of an abdominal defect was associated with long-segment atresia of the midintestine in each case. We believe that the spontaneous closure of this abdominal defect was associated with atresia and resorption of exteriorized bowel. It is likely some of the cases of long-segment atresia may in fact be associated with closed gastroschisis.

The aim of this study was to describe the prevalence at birth of two abdominal wall defects (AWD), omphalocela and gastroschisis and to identify possible etiologic factors. The AWD came from 265,858 consecutive births of known ouome registered in the registry of congenital malformations of Strasbourg for the period 1979 to 1998. Request information on the child, the pregnancy, the parents and the family was obtained for cases and for controls. Hundred five cases with AWD were analysed, 55.2 % were omphalocele and 44.8 % were gastroschisis. The mean prevalence rate for omphalocele was 2.18 per 10,000 and for gastroschisis 1.76 per 10,000. Associated malformations were found in 74.1 % of omphalocele compared with 53.2 % of gastroschisis; 29.3 % of fetuses with omphalocele had an abnormal karyotype, 44,8 % had a recognizable syndrome, association or an unspecified malformation pattern; 51.0 % of fetuses with gastroschisis had additional malformations that were not of chromosomal origin, but 1 case. Antenatal ultrasound examination was able to detect 39 (67.2 %) cases of omphaloceles and 27 (57.4 %) cases of gastroschisis. In 30 (51.7 %) cases of omphalocele and in 7 (14.9 %) cases of gastroschisis parents opted for termination of pregnancy. The overall survival rate was 14 (24.1 %) for omphalocele and 30 (63.8 %) for gastroschisis. Weight, length and head circumference at birth of infants with AWD were less than those of controls. The weight of placenta of infants with AWD was not different from the weight of placenta of controls. Gastroschisis was associated with significantly younger maternal age than omphalocele. Pregnancies with AWD were more often complicated by threatened abortion, oligohydramnios and polyhydramnios. Mothers of children with AWD took more often medication during pregnancy than mothers of controls.