11/43. Mermaid syndrome with amniotic band disruption.An association of Amniotic Band Disruption Sequence and Mermaid syndrome in a newborn having multiple congenital anomalies is being reported. The newborn had aberrant string like tissues attached to the amputed fingers and toes. Adhesions of amniotic bands had disrupted the fetal parts especially anteriorly in the midline, causing multiple anomalies. Apart from these features of Amniotic Band Disruption Sequence, the newborn had complete fusion of the lower limbs by cutaneous tissue, a characteristic of Mermaid syndrome (Sirenomelia). Associated malformations were anal stenosis, rectal atresia, small horseshoe kidney, hypoplastic urinary bladder and a bicomuate uterus. The single umbilical artery had a high origin, arising directly from the aorta just distal to the celiac axis, which is unique to sirenomelia. Theories put forward regarding the etiopathogenesis of both the conditions are discussed.- - - - - - - - - - ranking = 1keywords = single umbilical artery, single umbilical, umbilical artery, umbilical, artery, single (Clic here for more details about this article) |
12/43. An interesting rare case of sirenomelia.In sirenomelia the caudal tissues of the foetus fail to develop because of the "stealing" of blood by a vitelline vascular shunt from the distal aorta during embryogenesis interfering with foetal development. A 25 years old 2nd gravida delivered one full term premature grossly asphyxiated baby at BS Medical College on 24-10-1998. The examination of the dead foetus revealed no genitalia, no anal nor urethral opening and having a single lower limb with the single thigh and rudimentary leg. Skiagram of the foetus corroborated it to be a case of syrenomelia.- - - - - - - - - - ranking = 2.1139851344571E-5keywords = single (Clic here for more details about this article) |
13/43. Sirenomelia.Sirenomelia is a rare congenital anomaly with incidence of 1.5-4.2 per 100,000 births. Vascular steal phenomenon, posterior axial mesodermal defect or teratogenic defects have been implied in its pathogenesis. The authors present two cases with associated upper limb involvement and vertebral defects and complete absence of one lower limb bones in one case. autopsy revealed abnormalities in internal organs. Both cases were associated with single umbilical artery and severe oligohyramnios. Sirenomelia should be suspected in antenatal period in cases presenting with severe oligohydramnios and intrauterine growth retardation for a early diagnosis and appropriate management of pregnancy.- - - - - - - - - - ranking = 1keywords = single umbilical artery, single umbilical, umbilical artery, umbilical, artery, single (Clic here for more details about this article) |
14/43. Fetal bilateral renal agenesis, phocomelia, and single umbilical artery associated with cocaine abuse in early pregnancy.BACKGROUND: Maternal cocaine abuse in pregnancy is associated with complications such as intrauterine growth retardation, abruptio placentae, and preterm delivery. CASE: We report what is, to our knowledge, the first published observation of fetal bilateral renal agenesis associated with a vascular disruption syndrome comprising upper limb reduction defect and a single umbilical artery following maternal cocaine abuse in early pregnancy. CONCLUSIONS: This constellation in a fetus aborted at 18 weeks extends the spectrum of complications possibly associated with cocaine abuse in pregnancy.- - - - - - - - - - ranking = 5keywords = single umbilical artery, single umbilical, umbilical artery, umbilical, artery, single (Clic here for more details about this article) |
15/43. The role of color and power Doppler ultrasound in the prenatal diagnosis of sirenomelia.Sirenomelia is a rare congenital abnormality characterized by a variety of anomalies involving the lower limbs, severe oligohydramnios, bilateral renal agenesis, anorectal atresia and aberrant fetal vasculature. The prenatal diagnosis and differentiation from isolated bilateral renal agenesis is severely limited by the accompanying oligohydramnios that hinders visualization of fetal anatomy. We present three prospectively assessed cases of sirenomelia, two of which had bilateral renal agenesis, and all of which had a single umbilical artery derived from the aberrant vasculature that accompanies the syndrome.- - - - - - - - - - ranking = 1keywords = single umbilical artery, single umbilical, umbilical artery, umbilical, artery, single (Clic here for more details about this article) |
16/43. Sirenomelia apus with vestigial tail.Sirenomelia is an exceptionally rare congenital malformation characterized by complete or near complete fusion of lower limbs. A newborn with clinical features of sirenomelia including fused lower limbs in medial position, absent fibula, anal atresia, complete absence of urogenital system (bilateral renal agenesis, absent ureters, urinary bladder, absent internal and external genitalia), a single umbilical artery and a vestigial tail is reported. association of vestigial tail with sirenomelia is not described in the literature.- - - - - - - - - - ranking = 1keywords = single umbilical artery, single umbilical, umbilical artery, umbilical, artery, single (Clic here for more details about this article) |
17/43. Two cases of a fetus with sirenomelia sequence.We report two cases of a fetus with sirenomelia sequence which showed oligohydramnios and a single umbilical artery. The first case was of a single fetus with symelia apus and only one leg. prenatal diagnosis of this case was possible. The second case was of a dichorionic-diamniotic twin pregnancy in which one fetus had symelia dipus with two fused lower extremities. prenatal diagnosis of the condition was not made. In both cases, the fetuses died shortly after birth from respiratory distress due to severe pulmonary hypoplasia. Absence of urinary tract, imperforate anus, and spine deformity were confirmed in both cases. Although prenatal diagnosis of symelia dipus seems difficult, this condition must be considered in a fetus with severe oligohydramnios.- - - - - - - - - - ranking = 1.0000105699257keywords = single umbilical artery, single umbilical, umbilical artery, umbilical, artery, single (Clic here for more details about this article) |
18/43. Cavovarus foot deformity with multiple tarsal coalitions: functional and three-dimensional preoperative assessment.In rare instances, tarsal coalition leads to cavovarus foot deformity, although the pathologic mechanism leading to this deformity is not clear. This article reports a case of a 14-year-old boy presenting a severe cavovarus deformity of the right foot with talocalcaneal and calcaneonavicular coalitions, and a mild cavus deformity of the left foot with a single talocalcaneal coalition. Computed tomography and postoperative histologic analysis demonstrated a synostosis between talus and calcaneus and a fibrous calcaneonavicular coalition with partial ossification. Instrumented gait analysis revealed a limited range of ankle plantar flexion and increased external rotation of the ankle. Associated skeletal malformations including incomplete hemimelia of the forearm and scoliosis raised the possibility of a teratologic condition, but neurologic examination, spinal magnetic resonance imaging, and nerve conduction velocities were normal. The progressive ossification of combined coalitions during growth of the foot may have been one factor leading to this complex foot deformity. The fine-wire electromyogram showed normal tibialis anterior and posterior muscle activity. Small soft tissue tears in the sinus tarsi may have led to a mild reflexive increase of the muscle tone and tendon shortening, which pulled the forefoot into adduction and the heel into varus, and raised the medial arch. Mechanical alterations of the ankle appear secondary to the heel varus and to the progressive deformity of the talus. Three-dimensional computed tomography reconstruction and gait analysis appeared to be helpful additional parameters to understanding the pathomechanics of this complex foot deformity and for preoperative planning of triple arthrodesis.- - - - - - - - - - ranking = 1.0569925672285E-5keywords = single (Clic here for more details about this article) |
19/43. Multidisciplinary surgical approach to a surviving infant with sirenomelia.Sirenomelia is an extremely complex and rare malformation with different degrees of lower-extremities fusion associated with gastrointestinal, musculoskeletal, vascular, cardiopulmonary, and central nervous system malformations. In the English literature, there are only 5 reports of infants surviving with this condition. In our case, a 2540-g female infant was born with normal vital signs, no facial dysmorphism, and a complete soft tissue fusion of the lower limbs, from perineum to ankles. Radiologic examinations revealed an intestinal atresia and a single pelvic kidney, with a unique ureter, 2 femurs, 2 tibias, 2 fibulas, and 2 feet (simpus dipus). At 7 months of age, a multidisciplinary surgical team achieved complete separation of the lower limbs, with independent vascular and nerve supplies. At the time of this writing, the infant was 28 months old and had a regular growth curve. Many future reconstructive surgeries have been planned to achieve an acceptable quality of life for this infant.- - - - - - - - - - ranking = 1.0569925672285E-5keywords = single (Clic here for more details about this article) |
20/43. Sirenomelia of an intracytoplasmic sperm injection conceptus: a case report and review of mechanism.Postmortem dissection of the 18-week male conceptus, product of intracytoplasmic sperm injection (ICSI), demonstrated the hallmark findings of sirenomelia. The lower legs were fused and the left knee was rotated medially. Internal organs showed hypoplastic lungs, a multicystic kidney, and unilateral ureteral hypoplasia. The vitelline artery was absorbed, in a classic fashion, into the umbilical artery and communicated with the aorta at a point proximal to the iliac arteries. The tributaries distal to this point were hypoplastic. This finding is consistent with previously documented cases of sirenomelia and is thought to be the pathogenetic mechanism resulting in a vascular steal from the lower extremities. A rare finding was the presence of a penis on the dorsal side just below a perforate anus. In this case report, we discuss the pertinent clinical history and autopsy findings. A brief review of the mechanism thought to give rise to sirenomelia is provided. To our knowledge, this is the first reported case of sirenomelia in an ICSI conceptus.- - - - - - - - - - ranking = 0.046323983226606keywords = umbilical artery, umbilical, artery (Clic here for more details about this article) |
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