Ventral Body Wall Research Articles

Mutations that prevent phosphorylation of the BMP4 prodomain impair proteolytic maturation of homodimers leading to early lethality in mice.

Bone morphogenetic protein4 (BMP4) plays numerous roles during embryogenesis and can signal either as a homodimer, or as a more active BMP4/7 heterodimer. BMPs are generated as inactive precursor proteins that dimerize and are cleaved to generate the bioactive ligand and inactive prodomain fragments. In humans, heterozygous mutations within the prodomain of BMP4 are associated with birth defects. We studied the effect of two of these mutations (p.S91C and p.E93G), which disrupt a conserved FAM20C phosphorylation motif, on ligand activity. We compared the activity of BMP4 homodimers or heterodimers generated from BMP4, BMP4S91C or BMP4E93G precursor proteins in Xenopus embryos and found that these mutations reduce the activity of BMP4 homodimers but not heterodimers. We generated Bmp4 S91C and Bmp4 E93G knock-in mice and found that Bmp4 S91C/S91C mice die by E11.5 and display reduced BMP activity in multiple tissues including the heart at E10.5. Most Bmp4 E93G/E93G mice die before weaning and Bmp4 -/E93G mutants die prenatally with reduced or absent eyes, heart and ventral body wall closure defects. Mouse embryonic fibroblasts (MEFs) isolated from Bmp4 S91C and Bmp4 E93G embryos show accumulation of BMP4 precursor protein, reduced levels of cleaved BMP ligand and reduced BMP activity relative to MEFs from wild type littermates. Because Bmp7 is not expressed in MEFs, the accumulation of unprocessed BMP4 precursor protein in mice carrying these mutations most likely reflects an inability to cleave BMP4 homodimers, leading to reduced levels of cleaved ligand and BMP activity in vivo. Our results suggest that phosphorylation of the BMP4 prodomain is required for proteolytic activation of BMP4 homodimers, but not heterodimers.

Ventral body wall closure: Mechanistic insights from mouse models and translation to human pathology.

The ventral body wall (VBW) that encloses the thoracic and abdominal cavities arises by extensive cell movements and morphogenetic changes during embryonic development. These morphogenetic processes include embryonic folding generating the primary body wall; the initial ventral cover of the embryo, followed by directed mesodermal cell migrations, contributing to the secondary body wall. Clinical anomalies in VBW development affect approximately 1 in 3000 live births. However, the cell interactions and critical cellular behaviors that control VBW development remain little understood. Here, we describe the embryonic origins of the VBW, the cellular and morphogenetic processes, and key genes, that are essential for VBW development. We also provide a clinical overview of VBW anomalies, together with environmental and genetic influences, and discuss the insight gained from over 70 mouse models that exhibit VBW defects, and their relevance, with respect to human pathology. In doing so we propose a phenotypic framework for researchers in the field which takes into account the clinical picture. We also highlight cases where there is a current paucity of mouse models for particular clinical defects and key gaps in knowledge about embryonic VBW development that need to be addressed to further understand mechanisms of human VBW pathologies.

The sequence of acrania–exencephaly–anencephaly (AEAS)- An infrequent case report

Acrania–exencephaly–anencephaly sequence has an incidence of 3.6–5.4 for 10,000 live births and has been reported in literature. Exencephaly, described here is a defect of the neural tube which occurs due to the absence of closure of the neural fold. The main diagnostic ultrasound features include that are characterized by acrania, decreased size of cranial pole in comparison with the chest, irregular cranial surface, with increased amniotic fluid echogenicity due to the damaged brain tissue. Associated with amniotic band syndrome, Pentalogy of Cantrell, limb anomalies and ventral body wall defects. It is incompatible with life. Conducting programs training the budding neuro-sonographers about the knowledge in detection, diagnosis of NTD according to the Carnegie Classification is crucial to look forward in pathogenesis and application in the clinical scenario.

Decoding the dilemma of antenatal ventral body wall defects: A case report and literature review

We are presenting the case of a rare fetal polymalformative syndrome known as the limb body wall complex (LBWC) and its antenatal diagnosis. The level II Anomaly ultrasound of a primigravida female detected exencephaly, thoracoabdominoschisis, club foot, kyphoscoliosis, fetoplacental adherence, and a deficient umbilical cord, classically described in LBWC. It is imperative for radiologists to be familiar with this rare fatal entity of sporadic occurrence and to differentiate it from other non-lethal counterparts for appropriate maternal counseling and timely termination of pregnancy.

Open Approach to the Transversus Abdominis Plane in Horses: A Cadaver Feasibility Study.

The study's objective was to evaluate the feasibility and dispersion of an open approach to the transversus abdominis plane (TAP) block in eight adult equine cadavers. A ventral midline incision was made, starting 2 cm cranial to the umbilicus and extending 25 cm cranially. In total, 0.5 mL/kg of new methylene blue (NMB) was injected per horse, divided into six injections. Using an 18 g, 8 cm Tuohy needle, three injections were made per side. The needle was guided blindly into the TAP using palpation. A 60 mL syringe was attached directly to the needle, depositing ~0.08 mL/kg at each site. The time to complete the injections was recorded for each cadaver. Following injection, the ventral body wall was dissected to determine if the dye was present within the TAP space as well as to measure the extent of the dispersion of the dye, the cranial to caudal extent, and the width of the dye's spread. Complete deposition of NMB into the TAP (six of six sites) was achieved in 5/8 horses. The median time needed to perform all the injections was 263 s. Increased adiposity (retroperitoneal fat) was associated with unsuccessful injections. This approach to the TAP was easily and quickly performed, though less successful in horses with increased retroperitoneal fat and increased BCS.

Ectopia Cordis with Omphalocele in First Trimester Pregnancy: A Case Report of Incomplete Type of Pentalogy of Cantrell

Ectopia Cordis (EC) is a rare congenital malformation in which the heart is located partially or totally outside the thoracic cavity. It results from the failure of migration of the lateral mesoderm into the midline. The four main ectopic positions are cervical, thoracic, thoracoabdominal and abdominal. EC can manifest as an isolated deformity or as part of a broader group of ventral body wall defects affecting the abdomen, thorax, or both. Pentalogy of Cantrell is a well known association that comprises EC, omphalocele (typically supraumbilical), congenital diaphragmatic hernia, sternal cleft and congenital heart disease. A 27-year-old primigravida came for a routine antenatal ultrasound at 13 weeks of gestation. There was no family history of congenital anomalies, genetic abnormalities, or exposure to teratogenic agents. The ultrasound showed a single foetus corresponding to 13 weeks of gestation with an anterior thoracic defect and an extrathoracic heart, along with partial herniation of the liver near the midline in the epigastric region. These findings were confirmed by foetal Magnetic Resonance Imaging (MRI). An unfavourable prognosis for the foetus was explained to the parents, and medical termination of pregnancy was performed. Therefore, prenatal ultrasonographic diagnosis of EC should be followed by a thorough search for associated defects, as the prognosis may vary.

Analyzing the factors that contribute to the development of embryological classical type of bladder exstrophy.

Bladder exstrophy is a rare congenital condition of the pelvis, bladder, and lower abdomen that opens the bladder against the abdominal wall, produces aberrant growth, short penis, upward curvature during erection, wide penis, and undescended testes. Exstrophy affects 1/30,000 newborns. The bladder opens against the abdominal wall in bladder exstrophy, a rare genitourinary condition. This study is vital to provide appropriate therapy choices as a basis to improve patient outcomes. This study may explain bladder exstrophy and provide treatment. Epispadias, secretory placenta, cloacal exstrophy, and other embryonic abnormalities comprise the exstrophy-spades complex. The mesenchymal layer does not migrate from the ectoderm and endoderm layers in the first trimester, affecting the cloacal membrane. Embryological problems define the exstrophy-aspidistra complex, which resembles epimedium, classic bladder, cloacal exstrophy, and other diseases. Urogenital ventral body wall anomalies expose the bladder mucosa, causing bladder exstrophy. Genetic mutations in the Hedgehog cascade pathway, Wnt signal, FGF, BMP4, Alx4, Gli3, and ISL1 cause ventral body wall closure and urinary bladder failure. External factors such as high maternal age, smoking moms, and high maternal body mass index have also been associated to bladder exstrophy. Valproic acid increases bladder exstrophy risk; chemicals and pollutants during pregnancy may increase bladder exstrophy risk. Bladder exstrophy has no identified cause despite these risk factors. Exstrophy reconstruction seals the bladder, improves bowel function, reconstructs the vaginal region, and restores urination.

Caudal ventral midline herniation in a foal

SummaryThis report outlines the diagnosis, surgical treatment and successful outcome following the treatment of a foal presented with a congenital abdominal hernia. A focal ventral abdominal swelling, enlarging since birth, starting just caudal to the intact umbilicus and extending caudally over the prepuce was observed. Once in dorsal recumbency, the hernia was found to originate from a defect of the abdominal wall in the linea alba region. A simple closed herniorrhaphy with inversion of the large peritoneal sac into the abdomen was performed. Post‐operative complications included fever with the development of an abscess at the surgical site. This was diagnosed and drained with ultrasound guidance. Based on culture and sensitivity testing of the abscess exudate, antibiotic therapy was adapted and the foal made a full recovery. A clinical examination undertaken 1 year later revealed complete healing of the linea alba. Herniation due to a muscle wall default has not been previously reported in foals. This article reviews the commonly observed causes of herniation (umbilical and inguinal hernias). However, a third type of congenital ventral body wall hernia should be considered for the differential diagnosis of swelling of the preputial area in foals.

SPECC1L: a cytoskeletal protein that regulates embryonic tissue dynamics.

Many structural birth defects occur due to failure of tissue movement and fusion events during embryogenesis. Examples of such birth defects include failure of closure of the neural tube, palate, and ventral body wall. Actomyosin forces play a pivotal role in these closure processes, making proteins that regulate actomyosin dynamics a priority when studying the etiology of structural birth defects. SPECC1L (sperm antigen with calponin homology and coiled-coil domains 1 like) cytoskeletal protein associates with microtubules, filamentous actin, non-muscle myosin II (NMII), as well as membrane-associated components of adherens junctions. Patients with SPECC1L mutations show a range of structural birth defects affecting craniofacial development (hypertelorism, cleft palate), ventral body wall (omphalocele), and internal organs (diaphragmatic hernia, bicornuate uterus). Characterization of mouse models indicates that these syndromic mutations utilize a gain-of-function mechanism to affect intra- and supra-cellular actin organization. Interestingly, SPECC1L deficiency appears to affect the efficiency of tissue dynamics, making it an important cytoskeletal regulator to study tissue movement and fusion events during embryonic development. Here we summarize the SPECC1L-related syndrome mutations, phenotypes of Specc1l mouse models, and cellular functions of SPECC1L that highlight how it may regulate embryonic tissue dynamics.

Muscles of the sex organs of Panagrolaimus detritophagus (Chromodorea: Rhabditida: Panagrolaimidae)

Summary The sex organs are important for nematode systematics, but the associated muscles are rarely used as phylogenetic characters. This research aimed to study the musculature of Panagrolaimus detritophagus, using confocal microscopy. The ring-shaped anal sphincter is pierced by a biconical X-structure and connected by two pairs of processes to the subventral stomato-intestinal muscles and dorsal body wall. The female has two pairs of vaginal dilators that are connected to the proximally invaginated vaginal sphincter and subventral body wall. The anal depressor is a pair of dorsoventral muscles. The male has 7-8 pairs of caudal diagonal muscles located beneath the ventral body wall muscles, followed by five pairs of postcloacal oblique muscles. The paired posterior longitudinal muscles are connected to the cloacal opening and tail tip. The paired anterior retractors of the spicule connect the spicule capitula to the lateral body wall; a pair of spicule-gubernacular protractors runs from spicule capitula to the anterior part of gubernaculum, and paired ventral protractors run from spicule capitula to anterior cloacal lip. Two pairs of gubernaculum erectors are attached to the mid-gubernaculum, and the unpaired gubernaculum protractor extends ventrally to attach to the body wall at the posterior cloacal lip. The sex muscle pattern is of the Rhabditidae-Cephalobidae type with a 15-character code for phylogenetic reconstructions: 63313-22234-42243.

Epidermal growth factor receptor signaling protects epithelia from morphogenetic instability and tissue damage in Drosophila.

Dying cells in the epithelia communicate with neighboring cells to initiate coordinated cell removal to maintain epithelial integrity. Naturally occurring apoptotic cells are mostly extruded basally and engulfed by macrophages. Here, we have investigated the role of Epidermal growth factor (EGF) receptor (EGFR) signaling in the maintenance of epithelial homeostasis. In Drosophila embryos, epithelial tissues undergoing groove formation preferentially enhanced extracellular signal-regulated kinase (ERK) signaling. In EGFR mutant embryos at stage 11, sporadic apical cell extrusion in the head initiates a cascade of apical extrusions of apoptotic and non-apoptotic cells that sweeps the entire ventral body wall. Here, we show that this process is apoptosis dependent, and clustered apoptosis, groove formation, and wounding sensitize EGFR mutant epithelia to initiate massive tissue disintegration. We further show that tissue detachment from the vitelline membrane, which frequently occurs during morphogenetic processes, is a key trigger for the EGFR mutant phenotype. These findings indicate that, in addition to cell survival, EGFR plays a role in maintaining epithelial integrity, which is essential for protecting tissues from transient instability caused by morphogenetic movement and damage.

Y-27632 Impairs Angiogenesis on Extra-Embryonic Vasculature in Post-Gastrulation Chick Embryos

Y-27632 inhibits Rho-associated coiled-coil-containing protein kinase (ROCK) signaling, which is involved in various embryonic developmental processes, including angiogenesis, by controlling actin cytoskeleton assembly and cell contractility. Administration of Y-27632 impairs cytoskeletal arrangements in post-gastrulation chick embryos, leading to ventral body wall defects (VBWDs). Impaired angiogenesis has been hypothesized to contribute to VBWDs. ROCK is essential in transmitting signals downstream of vascular endothelial growth factor (VEGF). VEGF-mediated angiogenesis induces gene expressions and alterations of the actin cytoskeleton upon binding to VEGF receptors (VEGFRs). The aim of this study was to investigate effects of Y-27632 on angiogenesis in post-gastrulation chick embryos during early embryogenesis. After 60 h incubation, embryos in shell-less culture were treated with Y-27632 or vehicle for controls. Y-27632-treated embryos showed reduced extra-embryonic blood vessel formation with impaired circulation of the yolk sac, confirmed by fractal analysis. Western blot confirmed impaired ROCK downstream signaling by decreased expression of phosphorylated myosin light chain. Interestingly, RT-PCR demonstrated increased gene expression of VEGF and VEGFR-2 1 h post-treatment. Protein levels of VEGF were higher in Y-27632-treated embryos at 8 h following treatment, whereas no difference was seen in membranes. We hypothesize that administration of Y-27632 impairs vessel formation during angiogenesis, which may contribute to failure of VWB closure, causing VBWDs.

LE SYNDROME DE LIMB BODY WALL COMPLEX A PROPOS DUN CAS ET REVUE DE LA LITTERATURE

Limb body wallcomplex (LBWC) is a rare poly malformative syndrome of unknownorigin, comprising major anomalies of the ventral body wall (thoracoabdominal) associatedwithother anomalies, includingthose of the limbsthatmay range frompositionaldeformity to agenesis, unusualcraniofacial malformations, and a variety of visceral anomalies thatinclude the heart, lungs, and genitourinary system. Twophenotypes are describeddepending on the feto-placentalattachment. We report a case of LBWC diagnosed post partum in a stillbornwith the following malformations: a large anterioromphalocele, spina bifida withmeningocele, and severescoliosis. The lowerlimbsappearedasymmetricalwithbilateral club feetassociatedwithpolydactyly. The stillbirthadheres to the placenta by a very short cord of 3 cm comprising 2 arteries and a vein.

Prenatal diagnosis of acrania/exencephaly/anencephaly sequence (AEAS): additional structural and genetic anomalies.

To analyse additional structural and genetic anomalies in fetuses with acrania/exencephaly/anencephaly sequence (AEAS). A retrospective analysis of 139 fetuses with AEAS diagnosed between 2006 and 2020 in a single tertiary referral ultrasound department. The median gestational age at diagnosis decreased from 15weeks in 2006 to 13weeks in 2020 (- 0.21 per each year; p = 0.009). In 103 fetuses, the defects were limited to the neural tube (NTD) (74.1%), in 36 fetuses (25.9%), there were additional structural non-NTD anomalies. The most common were ventral body wall defects present in 17.8% (23/139), followed by anomalies of the limbs (7.2%; 10/139), face (6.5%; 9/139) and heart (6.5%; 9/139). Genetic anomalies were diagnosed in 7 of the 74 conclusive results (9.5%; 7/74; trisomy 18, n = 5; triploidy, n = 1; duplication of Xq, n = 1). In univariate logistic regression models, male sex, limb anomalies and ventral body wall defects significantly increased the risk of genetic anomalies (OR 12.3; p = 0.024; OR 16.5; p = 0.002 and OR 10.4; p = 0.009, respectively). A significant number of fetuses with AEAS have additional structural non-NTD anomalies, which are mostly consistent with limb body wall complex. Genetic abnormalities are diagnosed in almost 10% of affected fetuses and trisomy 18 is the most common aberration. Factors that significantly increased the odds of genetic anomalies in fetuses with AEAS comprise male sex, limb anomalies and ventral body wall defects.

A Novel Description of Breast Anatomy Indicates the Need for New Surgical Approaches

Mastectomy remains a major treatment for breast cancers and prophylaxis for carriers of the BRCA genes, yet the amount of tissue removed is variable across surgical approaches. The anatomical and surgical literatures provide conflicting and incomplete descriptions of breast gross anatomy. In order to clarify this anatomy, we dissected 6 male and 15 female human cadaveric specimens. We found that the mammary glandular tissue is constrained to a membrane‐bound, central structure referred to as the corpus mammae in the surgical literature, and not dispersed throughout the breast as described in the anatomical literature, consistent with other epidermally derived glands of the body. The major fasciae of the human ventral body wall, including the superficial fatty Camper’s fascia and the deeper membranous Scarpa’s fascia, both contribute to the structure of the breast. Based on these findings, we propose that during embryological development the mammary gland grows dorsally from the integument, pushing Camper’s and Scarpa’s fasciae ahead of it. When the mammary gland reaches the rigid thoracic wall, the gland grows laterally, pulling along Camper’s fascia to create the fat of the breast. Ventrally Scarpa’s fascia becomes a double layer that creates the shape of the breast and dorsally Scarpa’s forms a circummammary ligament that 1) stabilizes the breast against the thoracic wall and 2) is continuous with Scarpa’s fascia on the rest of the ventral body wall at the inframammary fold. The suspensory ligaments of the breast represent the typical, albeit thickened, septations between compartmentalized fat lobules found consistently throughout the human body, and do not attach to the skin. Instead, these ligaments attach to Scarpa’s fascia. We propose replacing surgical approaches that remove the entire breast with more targeted procedures focused on removing the gland only. Since the glandular material is constrained to the corpus mammae, in low‐grade cancers and prophylaxis surgeons can utilize fascial planes to remove only the gland while protecting the rest of the breast. Such a procedure could allow the patient to maintain most of the breast, thereby potentially reducing physical and emotional trauma.

Limb Morphology in the Macaroni Penguin (Eudyptes chrysolophus) Exhibits Adaptations for Both Sub‐aqueous Flying and Locomotion on Land

Requiring both wing‐propelled swimming and rugged overland locomotion, Macaroni penguins possess highly specialized morphology in both limbs. We dissected both the wing and hindlimb of one female and one male macaroni penguin. The distal wing exhibits very reduced musculature, and only retains very small brachialis and dorsal ulno‐metacarpal muscles. The elbow and metacarpal joints exhibit significantly decreased mobility compared to other birds. Proximal extrinsic wing muscles extend long tendons into the distal wing to control the dorso‐ventrally flattened flipper. Two sesamoid bones at the elbow joint provide a main attachment point for the proximal musculature, and probably contribute to the stiffening of the wing during underwater propulsion. The enlarged and extended sternum houses an extensive pectoralis muscle, responsible for the downstroke, which comprises nearly the entire ventral body wall. Supracoracoideus, found anatomically deep to pectoralis major and responsible for the upstroke, weighed about half of the pectoralis muscle, unlike most birds, where supracoracoideus weighs only about one‐fifth. An enlarged supracoracoideus increases the strength of the upstroke against viscous water. The tendons of latissimus dorsi cranialis and caudalis pass through a fibrous trochlea extending from the triceps complex to attach to the humerus, and possibly increases wing forces when moving the wing caudally. Unlike the sparse wing musculature, extensive leg musculature comprises about half of the dorsal body wall. The sartorius muscle, the most cranial hindlimb muscle, lies cranial to the caudal edge of the scapula, and cranial to the caudal margin of the sternum. Despite this, penguins exhibit restricted mobility during locomotion on land including apparent reduced range of motion in the knee joint, which contributes to their signature waddle. In addition, both Macaroni penguin specimens possess a unique muscle we have named the adductor tibialis. This muscle extends from the tibia of one hindlimb, across the midline where it slightly connects to the sternum, and attaches to the tibia of the contralateral limb, effectively tying the hindlimbs together. This permanent adduction probably helps create a streamlined figure while swimming to create faster speeds. In contrast to the knee, the penguin can greatly extend and flex the tarsus during locomotion. The considerable extensor system in the tarsus helps the penguin cross different terrains while walking, as well as give them the ability to jump.

Combined pentalogy of Cantrell with ectrodactyly and surgical implant‐free repair of a sternal cleft and supraumbilical hernia in an adult cat

AbstractA 20‐month‐old, British shorthair cat was presented for treatment of a ventral thoracic wall defect. Physical examination showed cleft sternum with supraumbilical body wall defect, an immediately palpable heart under the skin between the cleft halves and a unilateral forelimb ectrodactyly. Survey radiographs and sonographic examination revealed ectopia cordis associated with the sternal cleft, a supraumbilical abdominal hernia, ventral diaphragmatic defect and cardiac abnormalities. Radiography of the dysostotic limb confirmed ectrodactyly. Surgery involved reconstruction of the ventral body wall defects using a transversus abdominis muscle flap supported by an omental flap. Clinical, imaging and intraoperative findings supported the diagnosis of complete pentalogy of Cantrell. The cat recovered from surgery uneventfully and periodic follow‐ups to 17 months after surgery confirmed a good outcome.

Double-layered two-directional somatopleural cell migration during chicken body wall development revealed with local fluorescent tissue labeling.

The thoracic ventral body wall consists of the rib, the sternum, the intercostal muscles, and the connective tissues surrounding them. The ribs and the intercostal muscles are derived from the somite. The connective tissues are derived from the somatic layer of the lateral plate mesoderm, somatopleure. The lateral growth of the somatopleure forms the primary ventral body wall. The migration of somitic cells into the somatopleure generates the secondary body wall. As the migrating behavior of the somatopleural cells during secondary body wall formation is still unclear, we investigate here the migratory behavior of the somatopleural cells in the thorax during chicken ventral body wall development by labeling the thoracic somatopleural cells one-somite-wide by DiI labeling or gene transfection of the enhanced green fluorescent protein and observe their distribution assisted by the tissue-clearing technique FRUIT. Our labeling experiments revealed the rostral migration of the somatopleural cells into a deep part of the thoracic body wall in embryonic day 6.5 chickens. For embryonic day 8.5 chickens, these deep-migrating somatopleural cells were found around the sternal ribs. Thus, we identified the double-layered two-directional migrating pathways of the somatopleural cells: the rostral migration of the deep somatopleural cells and the lateral migration of the superficial somatopleural cells. Our findings imply that the rostral migration of deep somatopleural cells and the lateral migration of superficial ones might be associated with the developing sternal ribs and the innervation of the thoracic cutaneous nerves, respectively.

Organization of dermal glands and characteristic of secretion in the freshwater mite, Limnesia maculata (O. F. Müller, 1776) (Acariformes, Limnesiidae).

The presence of dermal glands is a synapomorphy in the freshwater mites-the large branch of the hyporder Parasitengonina. Dermal glands of the mite Limnesia maculata (O. F. Müller, 1776) (Acariformes, Limnesiidae) were studied using light and electron microscopy (TEM and SEM) for the first time. Two types of dermal glands were recognized in adult mites-14 pairs of the uniformly organized common dermal glands scattered throughout the entire body volume, and one pair of the characteristic so-called idiosomal dermal glands centrally located and stretched along the ventral body wall, supposedly corresponding to the epimeroglandularia 4. The common dermal glands are composed of a single large alveolus with the basally located columnar epithelium. An intra-alveolar lumen is positioned above the epithelium and packed with a secretion in the form of long curved electron-dense bands. These bands are directed to the excretory opening and released to the outside in the form of strongly coiled bands. The cytoplasm of the common dermal glands is filled with short RER cisterns and shows few characteristic Golgi bodies with round dense secretory granules at the distal pole. In contrast, a large elongated sac represents the idiosomal dermal glands having a relatively thin secretory epithelium on the periphery. The cuboidal epithelial cells contain areas with tightly packed RER cisterns and weakly recognized Golgi bodies. The apical cell surface bears irregular short microvilli. The large intra-alveolar lumen always contains an electron-dense secretion with myriads of small lighter particles. This kind of secretion was never seen released through the opening. The excretory opening of the common and the idiosomal dermal glands shows differences in its organization but basically is represented by a cuticular "bell" narrowing to the opening and showing a particular kind of valves at the inner side of the flaps of the orifice.

OEIS Syndrome: Omphalocele, Exstrophy of the Cloaca, Imperforate Anus, and Spinal Defects

OEIS Syndrome: Omphalocele, Exstrophy of the Cloaca, Imperforate Anus, and Spinal Defects