Uterine Arteriovenous Malformation: Diagnostic and Therapeutic Challenges.

Uterine arteriovenous malformations are a rare cause of puerperal haemorrhage, but their incidence is increasing due to both improved diagnosis and the more frequent use of uterine surgery in recent years. The use of ultrasound, both B-mode and Doppler, is recommended for diagnosis and follow-up, as it has been shown to be the simplest and most cost-effective method. Endometrial thickening associated with an anechoic and vascular intramiometrial structure is very useful for diagnosis and can help to exclude other causes of dysfunctional bleeding. Pulsed Doppler shows low-resistance vessels and high pulsatility indices with a high peak systolic velocity (PSV). In a healthy myometrium, the vessels have a peak systolic velocity of 9-40 cm/s and a resistance index between 0.6 and 0.8, whereas in the case of AVMs, the systolic and diastolic velocities are 4-6 times higher (PSV 25-110 cm/s with a mean of 60 cm/s and a resistance index of 0.27-0.75 with a mean of 0.41). For treatment, we must individualise each case, taking into account haemodynamic stability, the patient's reproductive wishes, and the severity of the AVM as assessed by its size and PSV.

Gestación gemelar en paciente con epidermolisis bullosa distrófica recesiva severa: revisión de la bibliografía en base a un caso clínico / Twin pregnancy in woman affected by severe recessive dystrophic epidermolysis bullosa: a case report and a literature review

La epidermolisis bullosa distrófica es un grupo de trastornos hereditarios de muy baja prevalencia que se caracterizan por una extrema fragilidad cutánea como consecuencia de una alteración de la cohesión de la unión epidermodérmica. Sin embargo, las manifestaciones clínicas van más allá de las lesiones cutáneas, habiéndose descrito afectación de la mayoría de aparatos y sistemas y siendo frecuente también cierto grado de desnutrición y anemia de origen multifactorial. Presentamos el caso de una paciente de 34 años, secundigesta, con epidermólisis bullosa distrófica recesiva severa, gestante gemelar bicorial y biamniótica, que seguimos durante todo el embarazo y su finalización. La baja prevalencia de la enfermedad hace que el manejo de la gestación suponga un reto para el ginecobstetra. A pesar de que los casos publicados hacen creer que la gestación no modifica el curso natural de la enfermedad, lo cierto es que se recomienda que el embarazo sea seguido por un equipo multidisciplinar. Existen publicados casos en los que se finaliza la gestación por vía vaginal, sin embargo, no existe evidencia suficiente para considerar ésta como la vía de elección.

Dystrophic epidermolysis bullosa is a group of hereditary disorders that has very low prevalence. It is characterized by an extreme cutaneous fragility as a consequence of a cohesion alteration of the epidermis and dermis junction. However, the clinical manifestations also affect other systems and organs, being able to cause malnutrition and anemia. We present the case of a 34-year-old woman affected by severe recessive dystrophic epidermolysis bullosa, with a bichorial and biamniotic twin pregnancy, whom we have followed throughout the pregnancy. The low prevalence of this disease makes the management of the pregnancy a challenge for the obstetrician. Although the published cases suggest that gestation does not modify the natural course of the disease, it is recommended that these pregnancies are monitored by a multidisciplinary team. Some published cases describe vaginal delivery. Nevertheless, it is not clear that this should be the first choice.

Gestación gemelar en paciente con epidermólisis bullosa distrófica / Twin pregnancy in a patient with distrophic bullyus epidermolisis

La epidermólisis bullosa distrófica es un grupo de trastornos hereditarios de muy baja prevalencia que se caracterizan por una extrema fragilidad cutánea como consecuencia de una alteración de la cohesión de la unión epidermodérmica. Sin embargo, las manifestaciones clínicas van más allá de las lesiones cutáneas, habiéndose descrito afectación de la mayoría de aparatos y sistemas y siendo frecuente también cierto grado de desnutrición y anemia de origen multifactorial. Presentamos el caso de una paciente de 34 años, secundigesta, con epidermólisis bullosa distrófica, gestante gemelar bicorial y biamniótica, que seguimos durante todo el embarazo y su finalización. La baja prevalencia de la enfermedad hace que el manejo de la gestación suponga un reto para el tocólogo. A pesar de que los casos publicados hacen creer que la gestación no modifica el curso natural de la enfermedad, lo cierto es que se recomienda realizar un seguimiento del embarazo por un equipo multidisciplinar. Existen publicados casos en los que se finaliza la gestación por vía vaginal, sin embargo, no está claro que sea esta la vía de elección

Dystrophic epidermolysis bullosa is a group of hereditary disorders that has very low prevalence. It is characterized by an extreme cutaneous fragility as a consequence of a cohesion alteration of the epidermis and dermis junction. However, the clinical manifestations also affect other systems and organs, being able to cause malnutrition and anemia. We present the case of a 34-year-old woman affected by dystrophic epidermolysis, with a bichorial and biamniotic twin pregnancy, whom we have followed throughout the pregnancy. The low prevalence of this disease makes the management of the pregnancy a challenge for the obstetrician. Although the published cases suggest that gestation does not modify the natural course of the disease, the truth is that it is recommended to monitor pregnancy by a multidisciplinary team. Some published cases describe vaginal delivery, even though it is not clear that this should be the first choice

Can we predict levator ani muscle avulsion in instrumental deliveries through intrapartum transperineal ultrasound?

Introduction: To determine whether intrapartum translabial ultrasound (ITU) is useful for the prediction of levator ani muscle (LAM) avulsions in instrumental deliveries (vacuum and forceps). Materials and methods: Prospective, observational study, including (1/2016 - 5/2016) 77 nulliparous women, with singleton pregnancies of ≥37 weeks of gestation and with cephalic presentation, who required vacuum or forceps instrumentation to complete the delivery. The ITU parameters evaluated were Angle of Progression (AoP), Progression Distance (PD), Head Direction (HD), and Midline Angle (MLA), both at rest and with maternal push. Evaluation of LAM avulsion was performed at 6 months postpartum with 3-4D transperineal ultrasound. Complete avulsion was defined as an abnormal insertion of LAM in the lower pubic branch identified in all three central slices. Results: Data from 48 nulliparous women were finally included in the study (34 vacuum and 14 forceps). We observed no difference in obstetric parameters between the two study groups (group with avulsion of LAM -14 cases, 29.2% - and group without avulsion of LAM -34 cases, 70.8%). The "LAM avulsion group" had an AoP and a PD of 136.7 ± 22.4 and 43.5 ± 15.6, respectively, versus 141.6 ± 21.3 and 47.2 ± 16.8 recorded in the group without avulsion (NS), respectively. We obtained a ROC curve for AoP and PD with a push of 0.66 (95% CI, 0.28-1.00) and 0.57 (95% CI, 0.39-0.75), respectively. Conclusions: ITU is not a useful technique to predict the occurrence of LAM avulsion in instrumental deliveries with vacuum or forceps.