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1.
Tomonobu Kanasugi Akihiko Kikuchi Atsushi Matsumoto Miyuki Terata Chizuko Isurugi Rie Oyama Akimune Fukushima Toru Sugiyama 《Congenital anomalies》2013,53(2):95-97
We report a rare case of a monochorionic twin gestation after intracytoplasmic sperm injection (ICSI) in which one of the fetuses had VACTERL association. A 27‐year‐old woman became pregnant by ICSI and was found to have monochorionic twin fetuses. One fetus was noted to have the following anomalies: a multicystic, dysplastic left kidney with a hydroureter, and a dilated colon. A normal‐sized stomach and normal amount of amniotic fluid were observed during the prenatal period with no other anomalies. The postnatal examination revealed hypospadias, and anal, esophageal, and duodenal atresia; thus, a diagnosis of VACTERL association was established. Although the prenatal diagnosis of this disorder is a challenge, even in a singleton, some of the characteristic features observed during antepartum ultrasonography may be a clue to the diagnosis, especially in a twin pregnancy after ICSI. 相似文献
2.
Nobuhiro Suzumori Yukio Hattori Saori Kaneko Yoshikatsu Suzuki Mayumi Sugiura‐Ogasawara 《Congenital anomalies》2009,49(4):274-275
A 22‐year‐old pregnant woman noticed a rapid increase of abdominal growth, uterine tenderness and irregular contraction, for which she hospitalized at 25 weeks of gestation. An ultrasound examination demonstrated a single fetus with normal anatomy and massive hydramnios. Serial therapeutic amniocentesis was performed for relief of maternal symptoms and indomethacin compress was initiated. Both the maternal and amniotic fluid IgM were positive for cytomegalovirus (CMV). Maternal compress indomethacin was discontinued at 32 weeks. Cesarean section was performed due to fetal distress at 34 weeks of gestation. A female infant was delivered and the neonatal examination was within normal limits with urine culture positive for CMV. At 1 year of age the child was developing normally with normal hearing and no clinical sequelae of intrauterine CMV infection. We postulate that serial and large volume reduction of amniotic fluid by amniocentesis and compress indomethacin in our case interrupted the natural course and provided sufficient time for the fetus to recover from the acute phase of viral infection. 相似文献
3.
Akgun Oral Ibrahim Caner Murat Yigiter Mecit Kantarci Hasim Olgun Naci Ceviz Ahmet Bedii Salman 《Pediatrics international》2012,54(3):361-364
Background: The VACTERL association (VA) is the non‐random co‐occurrence of vertebral anomalies, anal atresia, cardiovascular malformations, tracheoesophageal fistula and/or esophageal atresia, renal anomalies, and/or limb anomalies, and is referred to by the first letters of its components. Studies investigating the clinical characteristics of VA patients and probing of the observed current six component types are limited, and none of them is focused on neonates. We investigated the clinical characteristics of our patients diagnosed as having VA in the newborn period. Methods: We retrospectively reviewed the neonates whose final diagnosis was VACTERL association. Presence of at least three components of previously reported six anomalies was accepted as VACTERL association. Sex, birthweight, gestational age, postnatal age, anomalies of the systems that are included in VA, and the other features were recorded. Results: There was a male predominance (14/11) of 28 patients; and there were three patients with ambiguous genitalia. The most common observed VACTERL component was vertebral anomalies (n= 26), followed by anal atresia (n= 19), tracheoesophageal fistula/esophageal atresia (n= 17), renal anomalies (n= 15), limb anomalies (n= 15) and cardiac anomalies (n= 14). The most frequent combination was VCTL (n= 4). Fifteen (57%) patients had non‐VACTERL anomalies and the most frequent of these was ambiguous genitalia (n= 3). Conclusion: VA patients may have different clinical characteristics in different populations, and clinicians may miss some component features if the patients are evaluated after the neonatal period. 相似文献
4.
Sina Haeri Simone Hernandez Ruano Leila M. S. Farah Raquel Joffe Rodrigo Ruano 《Congenital anomalies》2013,53(2):89-91
The aim of this study was to test if prenatal cytogenetic diagnosis can be performed on fetal urine in fetal lower urinary tract obstruction. In this retrospective cohort study of fetuses with lower urinary tract obstruction (LUTO) over a 4‐year period at one institution, cytogenetic evaluation was attempted on fetal urine samples as well as amniotic fluid specimens. A total of 11 cases, ranging in gestational age from 15 to 25 weeks, underwent amniocentesis and vesicocentesis. Traditional cytogenetic evaluation was successfully completed in amniotic fluid and fetal urine samples in all 11 cases (100%). The karyotype was normal in seven (64%), trisomy 21 in two (18%), Trisomy 13 in one (9%), and partial chromosome 4 deletion in one (9%). Traditional cytogenetic evaluation can be successfully performed on fetal urine samples in cases of lower urinary tract obstruction. 相似文献
5.
High‐mobility group box‐1 release into fetal circulation from umbilical cord tissue and amniotic epithelium in fetal ischemia
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Toshihiko Nakamura Toshirou Yoshioka Shingo Yamada Taku Miyasho Nana Sakakibara Daishuke Hatanaka 《Pediatrics international》2016,58(7):631-634
We report the case of a baby with low birthweight born by emergency caesarean section at 33 weeks 2 days' gestation due to placental abruption. High‐mobility group box‐1 (HMGB‐1) and interleukin‐17 concentration in the umbilical cord blood were high at 55.7 ng/mL and 50.7 pg/mL, respectively. On immunostaining of umbilical cord and amniotic epithelium, HMGB‐1 was identified in the nuclei of vascular endothelial cells and cytoplasm of the surrounding cells in the umbilical cord. This suggests that, in the present case of placental abruption and subsequent ischemic placenta and fetus, the high level of HMGB‐1 observed was due to the release of HMGB‐1 into the umbilical cord blood from the vascular endothelial cells of the umbilical cord. 相似文献
6.
The goal of this study was to describe a novel pattern of genitourinary (GU) anomalies in VACTERL association,which involves congenital anomalies affecting the vertebrae,anus, heart, trachea and esophagus, kidneys, and limbs.We collected clinical data on 105 patients diagnosed with VACTERL association and analyzed a subset of 89 patients who met more stringent inclusion criteria. Twenty-one percent of patients have GU anomalies, which are more severe (but not more frequent) in females. Anomalies were noted in patients without malformations affecting the renal, lower vertebral, or lower gastrointestinal systems. There should be a high index of suspicion for the presence of GU anomalies even in patients who do not have spatially similar malformations. 相似文献
7.
Yoshitaka Seki Masami Matsushita Akihiko Kimura Hiroshi Nishiura Kumiko Aoki Takahiro Inokuchi Tatsuki Mizuochi Takao Kurosawa Yukuo Kimura Toyojiro Matsuishi 《Pediatrics international》2011,53(6):1028-1033
Background: Large amounts of unusual bile acids are synthesized by the fetal liver in late gestation. These compounds are mostly transferred from fetus to mother, although some are excreted into the amniotic fluid. We investigated the role of placental transfer of bile acids in fetal bile acid metabolism, particularly with respect to the unusual bile acids (1β‐hydroxylated and ketonic bile acids). Methods: We measured concentrations of bile acids in umbilical cord blood and urine of newborn infants, and in perinatal maternal serum and urine, using gas chromatography‐mass spectrometry. Serum and urine specimens from healthy non‐pregnant women were used as controls. Results: In newborn infants at delivery, cord blood and urine contained mostly primary and 1β‐hydroxylated bile acids, respectively. We also detected large amounts of ketonic bile acids in their urine, and the urinary concentration of total bile acids was elevated. Main maternal bile acids at 30 and 35 weeks of gestation and at delivery were 1β‐hydroxylated bile acids. After delivery, main bile acids changed from 1β‐hydroxylated bile acids to primary bile acids (P < 0.03), which also predominated in healthy non‐pregnant women. Conclusion: Fetally synthesized unusual bile acids were transported from fetus to mother. Pregnant women appear to excrete these bile acids into the urine, lowering both fetal and maternal serum bile acid concentrations. 相似文献
8.
408 kb 15q11.2 microduplication by array comparative genomic hybridization in a fetus presenting with exomphalos,micrognathia, tetralogy of Fallot and normal karyotype: A genetic counseling dilemma in paternal carrier status
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Gabriele Tonni Maria Bellotti Marcella Palmisano Viola Alesi Marta Bertoli Maria Paola Bonasoni 《Congenital anomalies》2015,55(1):65-70
Exomphalos may be associated with chromosomal abnormalities and syndromes. Severe exomphalos (herniation of liver, midgut and spleen) associated with increased nuchal translucency was seen at first trimester screening test. Karyotype by chorionic villus sampling showed normal male fetus. Follow up scan at 16 and 18 weeks of gestation confirmed the severe exomphalos and detected micrognathia and tetralogy of Fallot. Array comparative genomic hybridization (a‐CGH) further demonstrated a 408 kb 15q11.2 microduplication, with the father‐to‐be as healthy carrier. This is the first case of an association between 15q11.2 micorduplication and fetal sonographic anomalies. Genetic counseling for estimation of recurrent risk of congenital anomalies is discussed. 相似文献
9.
Tadao Okada Fumiaki Sasaki Kazutoshi Cho Shouhei Honda Satsuki Naito Gentarou Hirokata Satoru Todo 《Pediatrics international》2008,50(4):576-580
Background: The aim of the present study was to retrospectively determine the clinical factors affecting the outcome after birth in prenatally diagnosed sacrococcygeal teratomas (SCT). Methods: Six cases of prenatal SCT were identified from January 1985 until August 2005. A retrospective review of case‐notes and pathological reports was carried out. Clinical data during the perinatal period, operative findings, postoperative complications and follow up were evaluated in the patients with prenatally diagnosed SCT. Results: SCT presented as type I in two neonates and type III in four between 22 and 33 weeks’ gestation. Fetal intervention was not performed for any fetus. Five of six were delivered by cesarean section and the other was delivered vaginally due to small tumor size. Patients were born at between 29 and 39 weeks’ gestation and weighed from 1840 to 3500 g. All patients with type III SCT presented with related diseases, including bilateral hydronephrosis, neurological deficit of the communicating peroneal nerve such as paralytic talipes equines, bladder or bowel dysfunction, high‐output cardiac failure, or fetal hydrops in one of a set of fraternal twins. A baby with high‐output cardiac failure and fetal hydrops underwent urgent cesarean section at 29 weeks’ gestation and died 8 days after birth despite intensive care due to multi‐organ failure. In five cases, surgery was successful with good outcomes maintained at follow‐up of between 8 months and 14 years. Conclusions: Detailed ultrasound should be performed to rule out associated anomalies, and determine the presence or absence of hydrops in prenatally diagnosed SCT. Fetal hydrops, orthopedic impairment such as lower extremity weakness and swelling, and urinary incontinence are important clinical factors affecting the outcome after birth in prenatally diagnosed SCT. In particular, the present study indicated that the association of a fraternal twin and fetal hydrops makes it very difficult to treat SCT perinatally. 相似文献
10.
Jessica Diaz Blanche Chavers Srinath Chinnakotla Priya Verghese 《Pediatric transplantation》2019,23(2)
In this single‐center retrospective study, we analyzed kidney transplant outcomes in nine pediatric patients with VACTERL [vertebral defects, anal atresia, cardiac defects, tracheoesophageal fistula, renal anomalies, limb abnormalities] association—making this the largest study of its kind. Of 743 pediatric kidney transplant recipients at our center (1980‐2017), nine had documented diagnoses of VACTERL association. All nine had congenital anorectal malformations and renal anomalies, five had vertebral defects, and one had a bifid thumb and tracheoesophageal fistula. Renal anomalies included dysplasia (n = 6), aplasia (n = 3), and horseshoe kidney (n = 2). Congenital lower urinary tract anomalies included neurogenic bladder (n = 6), obstructive uropathy (n = 4), anovesicular fistula (n = 1), rectourethral fistula (n = 1), and posterior urethral valves (n = 1). Age at transplant ranged from 1.2 to 15 years (mean, 7.3; standard deviation [SD], 5.5); 6 (67%) were male, and 3 (33%) were female; 6 (67%) had a living related donor, and 3 (33%) had a deceased donor. The overall graft survival rate was 78% (range, 1.5 to 25.2 years; mean, 10.5; SD, 8.9). One month post‐transplant, one recipient died with a functioning graft. At 3.7 years post‐transplant, one graft failed because of recurrent pyelonephritis. Post‐transplant urologic complications included pyelonephritis (n = 6), vesicoureteral reflux (n = 5), and graft hydronephrosis (n = 4). We conclude that pediatric patients with VACTERL association can be safely transplanted—careful patient selection with vigilance and intervention for pre‐ and post‐transplant urologic complications is essential. 相似文献
11.
A 2-day-old male infant, born of a non-consanguineous marriage and uneventful pregnancy was found to have anomalies of vertebral,
anal, cardiac, tracheo-esophageal, radial and limb (VACTERL) association. The striking feature was the simultaneous occurrence
of two rare limb defects of right upper and lower limb in the baby who also had imperforate anus and ventricular septal defect.
These limb defects were-meromelia of the right upper limb (due to transverse deficiency of right humerus and absence of all
the bony elements distally), and a short right lower limb due to co-existence of proximal femoral hypoplasia and fibular hemimelia.
We could not trace the co-existence of these rare skeletal defects in any case with VACTERL association in the existing English
literature, as was observed by us. The simultaneous occurrence of the defects involving distant anatomic sites supports the
hypothesis of ‘axial mesodermal dysplasia’ in our patient, rather than ‘caudal regression syndrome’, as is popularly held
in patients with anorectal malformation (ARM). Further, it points to occurrence of an early embryonic insult, probably taking
place at blastogenic stage, when the developing embryo can be considered a polytopic development field. However, in absence
of antenatal history suggestive of exposure to a known teratogen and a chromosomal analysis, it appears that the spectrum
of anomalies in this neonate might have resulted secondary to early amniotic leak and temporary oligohydramnios. 相似文献
12.
Albuquerque CA Smith KR Saywers TE Johnson C Cock ML Harding R 《Early human development》2002,68(2):119-126
BACKGROUND: Oligohydramnios, a severe reduction in the volume of amniotic fluid, is associated with fetal lung hypoplasia but underlying processes are unclear. Studies in sheep suggest that oligohydramnios may lead to lung hypoplasia by causing increased flexion of the fetal spine, but this has not been demonstrated in the human, which has a different uterine anatomy. AIMS: Our aims were to quantify spinal flexion in the human fetus and to determine the relationship between oligohydramnios and the degree of spinal flexion. SUBJECTS AND METHODS: In 35 pregnancies, we used ultrasonography to assess amniotic fluid volume and to image the fetal spine so as to provide an index of mean spinal radius between the upper thoracic and lumbar spine. In 17 pregnancies with evidence of oligohydramnios resulting from premature rupture of membranes (mean +/- SEM gestation 28.8 +/-1.4 weeks), the mean radius of fetal spinal curvature between the upper thorax and sacrum was compared with values from 18 control fetuses with normal amounts of amniotic fluid (at 28.9 +/- 1.6 weeks). RESULTS: In each fetus, the spine could be imaged and the mean radius of curvature calculated. Oligohydramnios was associated with a significantly increased degree of fetal spinal flexion compared to controls. CONCLUSION: In human pregnancy, oligohydramnios is associated with increased flexion of the fetal spine, which is likely due to reduced uterine volume. This could contribute to the development of fetal lung hypoplasia, fetal immobility and other fetal anomalies. 相似文献
13.
Communicating bronchopulmonary foregut malformations (CBPFMs) are unusual congenital structures composed of a segment of lung
tissue connected to the foregut. We present what we believe is the first reported case of identical twins concordant for CBPFM
who are discordant for the VACTERL association. Their nonfunctional lung tissue was successfully removed and the fistulae
were corrected, and they are expected to live normal life spans. We review the literature concerning these malformations and
the proposed theories of their etiology. This case report of concordance in identical twins suggests that a possible genetic
component to CBPFMs cannot be ruled out. The discordance for the VACTERL association implies that the etiology is most likely
multifactorial. 相似文献
14.
目的 分析新生儿VACTERL联合征的临床表现及影像学特点,提高对本病的认识和诊断水平.方法 选择2009年1月至2012年11月在本院放射科检查的食管闭锁和肛门闭锁患儿,按照VACTERL联合征的诊断标准,回顾X线影像资料,并分析患儿临床表现及影像学特点.结果 研究期间在本院放射科进行检查的食管闭锁和肛门闭锁患儿共38例,其中4例食管闭锁患儿符合VACTERL联合征的诊断.1例食管闭锁并气管食管瘘、胸椎半椎畸形、右侧第11肋骨缺如、多指畸形;1例食管闭锁并先天性心脏病(房间隔缺损、动脉导管未闭)、右心房右心室扩大、双手拇指畸形;1例食管闭锁并气管食管瘘、双侧第13肋骨、脊柱裂、左肾积水、右肾缺如;1例食管闭锁并气管食管瘘、先天性心脏病(室间隔缺损、动脉导管未闭)、左手赘指畸形.结论 新生儿VACTERL联合征临床和影像表现具有一定特点,临床和影像学检查结合可以做出该诊断. 相似文献
15.
Keckler SJ St Peter SD Valusek PA Tsao K Snyder CL Holcomb GW Ostlie DJ 《Pediatric surgery international》2007,23(4):309-313
The VACTERL complex refers to anomalies of the bony spinal column (V), atresias in the gastrointestinal tract (A), congenital heart lesions (C), tracheoesophageal defects (TE), renal and distal urinary tract anomalies (R) and limb lesions (L). The incidence of each of these components has not been precisely quantified in the recent literature and the full array of anomalies within each systemic class of the VACTERL complex has not been well described. Therefore, we reviewed our most recent 20-year experience of patients born with esophageal atresia to comprehensively delineate and accurately describe the type and incidence of associated lesions. A retrospective review was then conducted on all patients diagnosed with esophageal atresia between 1985 and 2005. Patient demographics recorded included gestational age, weight and gender. The specific types of lesions were carefully cataloged. The outcome measure recorded was survival. One hundred and twelve patients were diagnosed with esophageal atresia were identified during the study period. The gestational age range was 28-41 weeks with an average of 36.5 weeks. Average birth weight was 2,557 g (range 1,107-3,890). A male predominance was seen with 62 males and 50 females. The overall survival was 92.9%. The categorical breakdown of anomalies were vertebral (24.1%), atresia (14.3%), cardiac (32.1%), tracheoesophageal fistula (95.5%), urinary (17.0%), skeletal (16.1%) and other (10.8%). VACTERL anomalies are common in patients with esophageal atresia, however, they appear to have little impact on overall survival. 相似文献
16.
E Kelly S Newell K Brownlee S Farmery C Cullinane W Reid P Jackson S Gray J Primrose M Lagopoulos 《Archives of disease in childhood. Fetal and neonatal edition》1997,76(3):F158
AIMS—To determine whether epidermal growth factor (EGF) or the related transforming growth factor α (TGFα) may have a role in the developing human stomach; to substantiate the presence of EGF in human liquor in the non-stressed infant and whether EGF in amniotic fluid is maternally or fetally derived.METHODS—The temporal expression and localisation of EGF, TGFα, and their receptors during fetal and neonatal life were examined in 20 fetal and five infant stomachs. Simultaneously, samples of amniotic fluid and fetal urine from 10 newborn infants were collected and assayed for EGF by radioimmunoassay.RESULTS—EGF immunoreactivity was not noted in any of the specimens examined. In contrast, TGFα immunoreactivity was shown in mucous cells from 18 weeks of gestation onwards. EGF receptor immunoreactivity was seen on superficial mucous cells in gastric mucosa from 18 weeks of gestation onwards. The median concentration of EGF was 30 and 8.5 pg/ml in amniotic fluid and fetal urine, respectively, suggesting that EGF is not produced by the fetus.CONCLUSIONS—This study adds weight to the hypothesis that swallowed EGF, probably produced by the amniotic membranes, and locally produced TGFα, may have a role in the growth and maturation of the human stomach. 相似文献
17.
ABSTRACT Regarding radial ray deficiency, several reports suggest that preaxial limb anomalies occur frequently, while postaxial limb anomalies and cleft hand (split–hands) are rarely associated with VACTERL. We describe a rare clinical case of VACTERL with cleft hand and a number of visceral anomalies. 相似文献
18.
Mortell A O'Donnell AM Giles S Bannigan J Puri P 《Pediatric surgery international》2004,20(4):276-282
Background The VACTERL association is a spectrum of clinical conditions, including esophageal atresia (EA) and tracheoesophageal fistula (TEF), which affects approximately 1 in 5,000 live human births. The administration of intraperitoneal Adriamycin to pregnant rats reliably induces anomalies, such as EA and TEF, in their offspring, in what is known as the Adriamycin rat model (ARM). In affected embryos the presence of gross notochord abnormalities is commonly found, with typical features being ectopic ventral branches and adherence of the notochord to the foregut. Fibronectin (FN) is an extracellular matrix (ECM) glycoprotein present on most cell surfaces, in extracellular fluids and in plasma. FN is involved in various functions, including cell adhesion, cell motility and wound healing. Previous studies in rats have shown that a single dose of Adriamycin can produce an appreciable rise in FN levels in various organs such as kidney and heart. We hypothesised that Adriamycin administration could promote upregulation of FN expression contributing to increased gut–notochord adherence and the development of abnormal ventral notochordal branching in the ARM. This study was designed to investigate FN expression in ARM embryos.Methods Adriamycin (1.75 mg/kg) was administered intraperitoneally to pregnant rats on days 7,8 and 9 of gestation (E7, E8 and E9 respectively). Control animals were given saline. Embryos recovered on E10–E14 were fixed, embedded in paraffin and sectioned. Immunohistochemistry using an anti-FN rabbit polyclonal antibody was performed.Results FN expression in both Adriamycin and control embryos on E10, E11 and E12 was comparable. However, the levels of FN expression in Adriamycin embryos on E13 and E14 were significantly greater in embryos with abnormal notochords than in equivalent control embryos.Conclusion Adriamycin-induced increased expression of FN, in the ARM, may contribute to abnormal notochord development leading to the VACTERL association. 相似文献
19.
Vasopressin concentration in amniotic fluid as an index of fetal hypoxia: mechanism of release in sheep 总被引:1,自引:0,他引:1
R I Stark S S Daniel M K Husain U M Sanocka A B Zubrow L S James 《Pediatric research》1984,18(6):552-558
Hypoxia is a potent stimulus to the release of vasopressin in fetal sheep and, in turn, plasma concentrations of the hormone correlate inversely with fetal oxygenation. Because the fetal kidney contributes to vasopressin clearance, we propose that measurement of increased amounts of vasopressin in amniotic fluid would be indicative of fetal hypoxia. We therefore measured concentrations of vasopressin in amniotic fluid under resting conditions, during and after fetal hypoxia, and with intravenous and intra-amniotic administration of vasopressin in 15 chronically instrumented fetal lambs 111-141 d gestation. In the resting state mean (+/- SE) vasopressin concentrations in amniotic fluid (1.6 +/- 0.3 pg . ml-1) did not differ from those in maternal (1.4 +/- 0.4 pg . ml-1) or fetal (1.8 +/- 0.2 pg . ml-1) plasma. After exposure of the ewe to 10% O2 or partial occlusion of the umbilical cord, vasopressin concentrations in fetal plasma increased significantly (P less than 0.001) to 200 +/- 59 pg . ml-1 with a delayed increase in amniotic fluid concentrations (P less than 0.03) to 15.8 +/- 4.5 pg . ml-1. This rise in concentration of vasopressin in amniotic fluid was sustained for at least 24 h and levels at that time were highly correlated with peak plasma concentrations (r = 0.83, P less than 0.001). Intravenous infusion of vasopressin into the fetus was accompanied by an equally significant (P less than 0.02) and sustained increase of vasopressin in amniotic fluid. After intraamniotic injection of vasopressin, levels remained increased for at least 24 h.(ABSTRACT TRUNCATED AT 250 WORDS) 相似文献
20.
The present case report describes two patients with a novel combination of VACTERL (vertebral, anorectal, cardiac, tracheoesophageal, renal, limb), neural tube defect and crossed renal ectopia. Though cases of VACTERL associated with crossed renal ectopia have been described, the present case report is the first to describe its combination with neural tube defect. The cases reported here are significant because central nervous system manifestations are scarce in VACTERL syndrome. The role of sonic hedgehog pathway has been proposed in VACTERL association and neural tube defects. Axial Sonic hedgehog signaling has also been implicated in the mediolateral positioning of the renal parenchyma. With this knowledge, the etiopathogenesis of this novel combination is discussed to highlight the role of sonic hedgehog signaling as a point of coherence. 相似文献