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Indian Dermatology Online Journal 2020
PubMed: 32055521
DOI: 10.4103/idoj.IDOJ_506_18 -
Plastic and Reconstructive Surgery.... Jul 2019Congenital amniotic band syndrome (ABS) is a complex disorder characterized by a spectrum of rare congenital anomalies, with constriction bands as their hallmark...
Congenital amniotic band syndrome (ABS) is a complex disorder characterized by a spectrum of rare congenital anomalies, with constriction bands as their hallmark manifestation. Constriction bands about the thorax, abdomen, or pelvis are rare. There is no consensus in the classification criterion for constriction bands of the thorax, abdomen, or pelvis in the literature. We present the first true pelvic constriction band in a newborn female with ABS. This case adds valuable information to the literature on ABS. The authors propose a classification criterion to further classify constriction bands of the thorax, abdomen, and pelvis using accepted and reliable anatomical landmarks.
PubMed: 31942360
DOI: 10.1097/GOX.0000000000002336 -
Revista Chilena de Pediatria Oct 2019To describe the experience of using the 3D-printed prosthetic hand Cyborg Beast in ado lescents of Teletón Santiago with congenital hand amputation and their main...
OBJECTIVE
To describe the experience of using the 3D-printed prosthetic hand Cyborg Beast in ado lescents of Teletón Santiago with congenital hand amputation and their main caregivers who partici pated in a case study to evaluate the functionality of the prosthetic hand.
CLINICAL CASE
Qualitative and descriptive research of case studies using semi-structured interviews with five adolescents with congenital hand amputation and their main caregivers. The information was transcribed and ana lyzed through open coding. Participants visualize the prosthesis as an opportunity for them by asso ciating it with normality. They also identify positive and negative characteristics, emphasizing in the latter. In addition, they described positive and negative effects produced when using the prosthesis, highlighting that the use of the prosthesis allows them to talk about their condition. Finally, im provement proposals for the prosthesis are presented, defining that the prototype must be corrected and change the age of the target population.
CONCLUSIONS
The study is the first to investigate a little explored topic, allowing to provide information regarding the subjective experience of adolescents who use a prosthesis that currently has great media importance. The study participants reported dif ficulties in using the hand prosthesis, either due to materiality and design aspects. The prosthesis did not meet the expectations of use and esthetic.
Topics: Adolescent; Adult; Aged; Amniotic Band Syndrome; Artificial Limbs; Caregivers; Hand; Humans; Interviews as Topic; Middle Aged; Printing, Three-Dimensional; Prosthesis Design; Young Adult
PubMed: 31859738
DOI: 10.32641/rchped.v90i5.1125 -
Medicine Dec 2019The significant ultrasonic characteristics of amniotic band syndrome (ABS) are the malformations of fetal affected parts and the band-like echoes in amniotic cavity....
RATIONALE
The significant ultrasonic characteristics of amniotic band syndrome (ABS) are the malformations of fetal affected parts and the band-like echoes in amniotic cavity. This article first suggests that the fetal hand adhered to umbilical cord with restricted movement provides some values in the diagnosis of ABS in early gestational weeks especially when the fetal malformation is not obvious and amniotic band is thin and fine.
PATIENT CONCERNS
Two pregnant women had no discomfort and underwent routine ultrasound examination at 11 to 14 gestational weeks.
DIAGNOSIS
Only the fetal hand adhered to umbilical cord with restricted movement was detected during the first ultrasound examination at 11∼14 gestational weeks, and the floating band-like echos were detected in the amniotic cavity with follow-up examinations 2 to 3 weeks later. Both of the 2 fetus were diagnosed as ABS by ultrasound INTERVENTIONS:: The two pregnant women underwent the prenatal counseling and were recommended closely follow-up and further examination.
OUTCOMES
Two fetuses died in utero between 17 and 19 weeks. After induction of labor, it was found that the hands and umbilical cord of the fetuses were wrapped by amniotic bands, which was proved pathologically as ABS.
LESSONS
The adhesion of the fetal hand and umbilical cord is an important ultrasonic sign suggesting ABS with poor prognosis in early pregnancy. We hope that this study can provide some guidance for the early diagnosis of ABS during 11 to 14 week's ultrasound examination.
Topics: Adult; Amniotic Band Syndrome; Early Diagnosis; Fatal Outcome; Female; Fetus; Gestational Age; Humans; Pregnancy; Tissue Adhesions; Ultrasonography, Prenatal; Umbilical Cord
PubMed: 31852110
DOI: 10.1097/MD.0000000000018302 -
Journal of Medical Case Reports Dec 2019De Morsier syndrome, or septo-optic dysplasia, is a rare, heterogeneous, complex condition with a highly variable phenotype. It is characterized by optic nerve...
INTRODUCTION
De Morsier syndrome, or septo-optic dysplasia, is a rare, heterogeneous, complex condition with a highly variable phenotype. It is characterized by optic nerve hypoplasia, pituitary gland hypoplasia, and midline brain abnormalities, including absence of septum pellucidum and corpus callosum dysgenesis. Diagnosis is made clinically by the presence of any two or more features from the clinical triad.
CASE PRESENTATION
We report a case of a premature African newborn male baby born to nonconsanguineous parents who presented to our institution with agenesis of the septum pellucidum, unilateral optic nerve hypoplasia, and pituitary stalk hypoplasia. However, he had intact central endocrine function. He also presented with limb defects due to constricting amniotic band syndrome. Other dysmorphic features were low-set ears, microcephaly, and bilateral talipes equinovarus. He otherwise had a normal neurological examination result. Over time, he had an adequate weight gain and was managed by a multidisciplinary team.
CONCLUSION
De Morsier syndrome still represents a diagnostic challenge, despite advances in neuroimaging and genetic studies, due to the heterogeneous nature of the disorder. This case adds to existing knowledge on the vascular pathogenesis of septo-optic dysplasia.
Topics: Amniotic Band Syndrome; Hand Deformities, Congenital; Humans; Hypopituitarism; Infant, Newborn; Male; Prognosis; Septo-Optic Dysplasia; Surgery, Plastic; Tomography, X-Ray Computed; Treatment Outcome
PubMed: 31839004
DOI: 10.1186/s13256-019-2306-2 -
BMC Pregnancy and Childbirth Nov 2019To evaluate the incidence and outcomes of septostomy in twin-to-twin transfusion syndrome (TTTS) after fetoscopic laser therapy.
BACKGROUND
To evaluate the incidence and outcomes of septostomy in twin-to-twin transfusion syndrome (TTTS) after fetoscopic laser therapy.
METHODS
A retrospective analysis of TTTS postlaser septostomy between 2005 and 2018 was performed. Postlaser septostomy was diagnosed using both (1) a free-floating intertwin membrane flap visible on ultrasound examination and (2) the rapid equalization of amniotic fluid maximum vertical pocket in the donor and recipient amniotic sacs observed after laser therapy. Perinatal survival, neonatal brain image anomaly, gestational age at operation and birth, incidence of premature rupture of membranes (PROM) within 3 weeks after operation, pseudoamniotic band syndrome, and cord entanglement were evaluated.
RESULTS
In the 159 TTTS cases included, 12 had postlaser septostomy. Relative to the group without septostomy, the septostomy group had a lower total fetal survival rate (54.2% vs 73.6%, p = 0.041), an earlier mean gestational age at delivery (27.8 vs 34.4 weeks, p = 0.009), a higher risk of PROMs within 3 weeks after operation (33.3% vs 5.4%, p = 0.004), a higher cord entanglement rate (16.7% vs 0%, p = 0.005), and a higher brain image anomaly rate (23.0% [3/13] vs 5.0% [11/218], p = 0.035). After considering the severe Quintero stages (stage III and IV), postlaser septostomy was the only variable [p = 0.003, odds ratio = 5.1] to predict neonatal brain image anomaly. Postlaser septostomy combined with severe Quintero stages could predict PROMs within 3 weeks after laser therapy [p = 0.001, odds ratio = 14.1 and p = 0.03, odds ratio = 5.4, respectively] and delivery before the gestational age of 28 weeks [p = 0.017, odds ratio = 4.5 and p = 0.034, odds ratio = 2.3, respectively]. The risk of pseudoamniotic band syndrome was not increased by postlaser septostomy in this case series.
CONCLUSIONS
Postlaser septostomy in TTTS was associated with poorer fetal survival and more adverse perinatal outcomes even after considering severe Quintero stages before laser therapy. Efforts should be made to prevent septostomy during laser therapy, and septostomy as the primary method to treat TTTS is not advisable.
Topics: Adult; Amniotic Band Syndrome; Amniotic Fluid; Female; Fetal Membranes, Premature Rupture; Fetofetal Transfusion; Fetoscopy; Humans; Incidence; Laser Therapy; Odds Ratio; Postoperative Complications; Pregnancy; Pregnancy Complications; Pregnancy, Twin; Retrospective Studies; Survival Rate; Treatment Outcome
PubMed: 31675920
DOI: 10.1186/s12884-019-2555-5 -
Orthopaedics & Traumatology, Surgery &... Feb 2020Congenital pathologies of the forefoot encompass two broad entities with vastly different treatments and prognosis: malformations, which occur during the embryonic... (Review)
Review
Congenital pathologies of the forefoot encompass two broad entities with vastly different treatments and prognosis: malformations, which occur during the embryonic period and cause anatomical defects, and deformations, which occur during the fetal period on a foot that is configured normally. These deformities are more easily cured when they occur later during the fetal period. When the anomaly is bilateral, a genetic origin must be considered. There are two main entities under the term "deformity": metatarsus adductus and skewfoot (aka "Z"-foot or serpentine foot). Within malformations are brachydactyly (transverse defects), longitudinal defects, syndactyly, polydactyly, clinodactyly and macrodactyly. Among other forefoot abnormalities are hallux valgus, which rarely presents in congenital form, and for which conservative treatment is sometimes sufficient. Also in this group are sequelae of amniotic band constriction, forefoot anomalies secondary to the treatment of congenital pathologies (talipes equinovarus and congenital vertical talus) and nail-related pathologies (ingrown toe nail and incorrect nail position).
Topics: Child; Foot Deformities, Congenital; Global Health; Humans; Incidence; Metatarsal Bones; Radiography
PubMed: 31648997
DOI: 10.1016/j.otsr.2019.03.021 -
Medicina (Kaunas, Lithuania) Aug 2019Perineal groove is a well-defined clinical entity that belongs to a broader group of anorectal malformations. It is characterized by a non-epithelialized mucous membrane...
Perineal groove is a well-defined clinical entity that belongs to a broader group of anorectal malformations. It is characterized by a non-epithelialized mucous membrane that appears as an erythematous sulcus in the perineal midline, extending from the posterior vaginal fourchette to the anterior anal orifice. The defect is gradually cicatrized, unless there are complications like infection, defecation disorders, trauma, and bleeding. The differential diagnosis includes several other conditions like trauma, infection, irritant dermatitis, lichen sclerosis, and ulcerated hemangioma. Since it is a rare malformation, it is often misdiagnosed and its presence often elicits unnecessary diagnostic workup and intervention. In this respect, neonatologists, dermatologists, or pediatric surgeons may under- or overestimate it. We report four cases of perineal groove out of 2250 live births at a Greek Maternity Hospital between September 2016 and April 2019. The "high" incidence of perineal groove cases in our Department allowed us to familiarize with this rare defect and minimize our clinical interventions.
Topics: Amniotic Band Syndrome; Anal Canal; Anorectal Malformations; Diagnosis, Differential; Female; Greece; Hospitals, Maternity; Humans; Infant, Newborn; Perineum; Vagina
PubMed: 31443289
DOI: 10.3390/medicina55080488 -
Seminars in Plastic Surgery Aug 2019Scarless healing has long been the holy grail for plastic surgery. While historically fetal surgery has tempted plastic surgeons with the allure of scarless correction... (Review)
Review
Scarless healing has long been the holy grail for plastic surgery. While historically fetal surgery has tempted plastic surgeons with the allure of scarless correction of congenital abnormalities, the risks far outweighed the benefits and these interventions never materialized. Current advances in fetal surgery with minimally invasive fetoscopic surgery have made these early fetal interventions safer, leading to expanding applications. While the plastic surgeon's role is limited as of yet, this article provides a review of the history of fetal surgery and the advances that may become relevant to the future plastic surgeon.
PubMed: 31384237
DOI: 10.1055/s-0039-1693431 -
Ultrasound in Obstetrics & Gynecology :... Jan 2020
Topics: Amniotic Band Syndrome; Carbon Dioxide; Female; Fetofetal Transfusion; Humans; Insufflation; Laser Coagulation; Pregnancy; Pregnancy Trimester, Second; Prenatal Care; Ultrasonography, Prenatal
PubMed: 31290581
DOI: 10.1002/uog.20396