Recessive Dystrophic Epidermolysis Bullosa and Pregnancy

Boria, Félix; Maseda, R.; Martín-Cameán, María; Calle, María de la; Lucas, R. de

doi:10.1016/j.adengl.2018.11.004

Cited by 6 publications

(25 citation statements)

References 13 publications

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“… 17 , 18 , 19 Depending on the inheritance pattern (autosomal recessive, autosomal dominant) the risk assessment for the pregnancy can be estimated and prenatal testing offered. 11 , 18 , 19 , 20 , 21 , 22 , 23 , 24 , 25 , 26 Couples at reproductive risk of severe forms of EB may wish to pursue prenatal or preimplantation genetic testing, accounting for family preferences, religious/cultural beliefs and national regulations. Carrier screening of an unaffected and unrelated partner may be offered according to the individual situation and national regulations, after genetic counselling.…”

Section: Recommendationsmentioning

confidence: 99%

“…R15 ↓↓ Avoid vaginal birth if: there is a specific ‘routine’ obstetric indication for caesarean section; there are extensive EB‐related genital blistering/wounds, or vaginal stenosis; 9 , 18 , 19 , 20 , 40 , 48 if a baby is in a breech presentation at term, an external cephalic version is not recommended, owing to shearing force that would be placed on the woman’s abdominal skin; delivery by caesarean section would be recommended. …”

Section: Recommendationsmentioning

confidence: 99%

“…there are extensive EB‐related genital blistering/wounds, or vaginal stenosis; 9 , 18 , 19 , 20 , 40 , 48 …”

Section: Recommendationsmentioning

confidence: 99%

“…Some women will have strong intention to breastfeed their newborn, while others may be concerned about possible nipple blistering. Breastfeeding among women with EB has been reported, 9 , 12 , 18 , 24 , 29 , 42 , 44 , 48 , 54 although nipple blistering is common and may limit ongoing efforts. 12 , 29 , 42 Offer to assist with positioning of the baby and adequate latching on to the areola, which may reduce subsequent blistering.…”

Section: Recommendationsmentioning

confidence: 99%

See 3 more Smart Citations

Recommendations on pregnancy, childbirth and aftercare in epidermolysis bullosa: a consensus‐based guideline*

et al. 2021

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Section: Recommendationsmentioning

confidence: 99%

Section: Recommendationsmentioning

confidence: 99%

“…there are extensive EB‐related genital blistering/wounds, or vaginal stenosis; 9 , 18 , 19 , 20 , 40 , 48 …”

Section: Recommendationsmentioning

confidence: 99%

Section: Recommendationsmentioning

confidence: 99%

See 2 more Smart Citations

Recommendations on pregnancy, childbirth and aftercare in epidermolysis bullosa: a consensus‐based guideline*

et al. 2021

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“…An early epidural may be indicated to avoid the risk of an emergent general anesthetic for cesarean delivery, and there are several reports of successful neuraxial anesthesia for both vaginal and cesarean deliveries. [30][31][32][33] There are reports of successful breastfeeding in mothers with EB, 30 and a consultation with a lactation specialist should be offered.…”

Section: Special Considerationsmentioning

confidence: 99%

Anesthetic Management of Adults With Epidermolysis Bullosa

Mittal

Goodnough

Bushell

et al. 2021

Anesthesia &Amp; Analgesia

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Epidermolysis bullosa (EB) is a group of rare, inherited diseases characterized by skin fragility and multiorgan system involvement that presents many anesthetic challenges. Although the literature regarding anesthetic management focuses primarily on the pediatric population, as life expectancy improves, adult patients with EB are more frequently undergoing anesthesia in nonpediatric hospital settings. Safe anesthetic management of adult patients with EB requires familiarity with the complex and heterogeneous nature of this disease, especially with regard to complications that may worsen during adulthood. General, neuraxial, and regional anesthetics have all been used safely in patients with EB. A thorough preoperative evaluation is essential. Preoperative testing should be guided by EB subtype, clinical manifestations, and extracutaneous complications. Advanced planning and multidisciplinary coordination are necessary with regard to timing and operative plan. Meticulous preparation of the operating room and education of all perioperative staff members is critical. Intraoperatively, utmost care must be taken to avoid all adhesives, shear forces, and friction to the skin and mucosa. Special precautions must be taken with patient positioning, and standard anesthesia monitors must be modified. Airway management is often difficult, and progressive airway deterioration can occur in adults with EB over time. A smooth induction, emergence, and postoperative course are necessary to minimize blister formation from excess patient movement. With careful planning, preparation, and precautions, adult patients with EB can safely undergo anesthesia.

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Management of epidermolysis bullosa simplex in pregnancy: A case report

Shah

Kumaraswami

Mushi

2019

Case Reports in Women's Health

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Epidermolysis bullosa (EB) encompasses a group of diseases characterized by extreme fragility of skin and mucous membranes, resulting in blister formation following minimal injury. There are 4 types of EB, with epidermolysis bullosa simplex (EBS) being the most common. We report our experience with the care of a parturient woman diagnosed with EBS. There is little literature on pregnancy in women with this condition. Special precautions are necessary during diagnostic and therapeutic interventions to avoid bullae formation or exacerbation of existing lesions. Frictional or shearing forces are typically more damaging than compressive forces. Multidisciplinary planning was done for our patient to ensure uneventful labor and delivery. Elective induction of labor was started at 40 weeks of gestation. She eventually underwent a cesarean delivery after failed trial of labor. We present this case to highlight the obstetric and anesthetic implications of caring for a parturient with EBS.

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Recessive Dystrophic Epidermolysis Bullosa and Pregnancy

Cited by 6 publications

References 13 publications

Recommendations on pregnancy, childbirth and aftercare in epidermolysis bullosa: a consensus‐based guideline*

Recommendations on pregnancy, childbirth and aftercare in epidermolysis bullosa: a consensus‐based guideline*

Anesthetic Management of Adults With Epidermolysis Bullosa

Management of epidermolysis bullosa simplex in pregnancy: A case report

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