CTEV WITH MEGA URINARY BLADDER: A CASE REPORT

Congenital talipes equino varus (CTEV), also known as congenital club foot is one of the common developmental deformities occurring at ankle, subtalar and metatarsal joints. Three elements comprise this deformity- equinus, inversion of foot and adduction of the forefoot relative to the hind-foot and equinus. The incidence of the deformity is 1 per 1000 live births. 20% of cases are associated with distal arthrogryposis, congenital myotonic dystrophy, myelomeningocele, amniotic band sequence and genetic syndromes such as trisomy 18 or chromosome 22q11 deletion syndrome, in the remaining cases the deformity is isolated and the exact etiology is unknown. We are presenting a case of aborted male fetus of 20 weeks with congenital talipes equino varus in left foot with enlarged urinary bladder. The purpose of this report is to document the association of CTEV with enlarged urinary bladder.

Amniotic Band Sequence Syndrome – A Rare Disorder

Amniotic bands sequence is a inherited diseases distinguished by craniofacial, body wall, and limb oddities that may be in relation with foetal-placental fibrous bands. Its pervasiveness has been delineated to range from 0.19 to 8.1 per 10 000 births [1]. It is a customary state prospectively alike with a variety of different confinements’ disability. The deformities arise in the wake of the pompous bodily part have formed usually in early evolution. The required cause of amniotic disruption complex is undisclosed .The three most usual decorative design are constriction ring syndrome signalized by one or more limbs being high-flown; the limb-body- wall complex; and amniotic band syndrome characterized by abnormalities of the head and face (craniofacial abnormalities), inadequacies of the brain and genuine deformation of the appendages [2]. The condition in few cases might be considered prior to nativity (antepartum), unlikely to change upon the sequels of explicit detailed imaging practices, like fetal USG, which may give away the feature inadequacies [3]. Here we present a case of pregnant woman admitted in our Obstetrics and gynecological department with her ultrasonography report indicative of amniotic band and further discussed about antenatal diagnosis, diagnostic and therapeutic approach, prognosis and genetic counselling of amniotic disruption complex.

Prenatal Diagnosis and Postnatal Treatment in a Case of Abdominal Obstruction and Polyhydramnios Caused by Amniotic Band Sequence

Background: Amniotic band sequence (ABS) is a rare congenital disorder resulting from the entanglement of fetal parts by fibrous bands that may cause disruptions, deformations, or malformations that can range from mild to life threatening conditions. Prenatal diagnosis is usually based on ultrasounds visualization on amniotic bands attached to fetal parts, possibly causing fetal defects. Case Presentation: A 19-year-old woman with an unremarkable clinical history and a low-risk pregnancy was referred to our Fetal Therapy Unit for a suspected fetal clubfoot at 22 weeks gestational age. A chorioamniotic separation, together with unilateral clubfoot was diagnosed. Due to a high risk of premature rupture of membranes a decision was made not to perform amniocentesis for genetic investigation. At prenatal follow up progressive polyhydramnios developed with a preterm spontaneous rupture of the membranes at 34 weeks. After caesarean section – due to breech presentation – an amniotic band was observed at the abdominal level causing a circular skin lesion, constriction, and a sub-occlusive intestinal status. Moreover, a left clubfoot with amputation of distal phalanx of the second toe and a right foot amputation of 2nd and 3rd toes were evident. The neonate underwent abdominal plastic surgery on the second day of life with complete recovery. Following the postnatal diagnosis of ABS, we re-examined a stored fetal 3D ultrasound volume acquired at 22 weeks and, focusing on the fetal surface rendering, we could notice the deep abdominal skin furrow that was evident at birth. Discussion: This case represents an unusual antenatal presentation of an amniotic band sequence that escaped prenatal diagnosis with a progressive polyhydramnios as an indirect sign of fetal bowel obstruction caused by an abdominal constricting band. In the presence of chorioamniotic separation and additional ominous ultrasound findings, it is advisable to consider the possibility of an ABS.

Early Surgical Intervention in Amniotic Band Sequence for Upper Extremity Motor Nerve Palsies: A Case Report

Urgent surgical intervention for amniotic band sequence (ABS) is currently indicated for concerns of vascular compromise and progressive lymphedema. Peripheral motor nerve palsies are rare, and reports of surgical intervention in these cases describe persistent motor dysfunction. We report band release and ulnar, median, and radial nerve decompression in a 1-week-old with a severe upper extremity constriction band and signs of ulnar nerve motor dysfunction. A literature review on nerve exploration and outcomes of patients with motor nerve palsy from ABS was performed. Early evidence of ulnar motor function was observed at 5.5-month follow-up. Previous reports of nerve decompression for upper extremity constriction bands with motor nerve palsy document poor recovery after interventions beginning at 3 months of age. In this case, band release and nerve decompression were undertaken at 7 days of age, and we observed early motor recovery. This finding suggests that very early surgical intervention in the neonate may facilitate nerve recovery in appropriate candidates.

Diagnostic Clue in a Neonate with Amniotic Band Sequence

Amniotic band syndrome (ABS) is a set of congenital malformations that mainly affect the limbs and more rarely the skull, face, chest, and abdomen. Two main hypotheses have been proposed to explain the nature of the disorder: an intrinsic and extrinsic factor. We report a newborn with ABS presenting with several malformations involving both hands and foot. In this case, the malformative event localized at the hands and right foot without involvement of any other internal organs and is asymmetric which leads us to suppose the extrinsic factor as cause of the ABS.

Prenatal diagnosis of the pronounced form of amniotic band sequence in the I trimester of gestation

The case of early prenatal diagnosis of multiple congenital malformations of the fetus caused by amniotic bands is presented. Ultrasound examination at 13 weeks of gestation revealed exencephaly, ectopia of the heart, fixed position of the fetus, short umbilical cord, generalized soft tissue edema, amniotic bands. A pathological examination based on the detected amniotic fibrous bands covering the head, trunk and umbilical cord of the fetus confirmed the diagnosis. It was identified that the fetus additionally had: anophthalmia, dysplasia of the auricles, atresia of the auditory canals, dysplasia of the upper and lower jaws, cleft lip, agenesis of the sternum, gastroschisis, contracture of the right elbow joint.

Inherited cause of in utero digital malformations

Amniotic band sequence (ABS) is common birth defect of incompletely understood origin. Here we describe a case of ABS in a child with paternally inherited Ehlers-Danlos syndrome, vascular type (vEDS). This is the third reported instance of ABS associated with paternally inherited vEDS in the medical literature. The two main theories of ABS formation are the extrinsic and intrinsic. The extrinsic theory states that placental tears form fibrous cords that wrap around the fetus; the intrinsic states that poor vascularisation in the fetus leads to necrosis of distal extremities. We believe this case supports extrinsic theory as it shows that as an amnion weakened by vEDS in fetal components is associated with ABS.