Aplasia Cutis Congenita

Silberstein, Eldad; Pagkalos, Vasileios A.; Landau, Daniella; Berezovsky, Alexander Bogdanov; Krieger, Yuval; Shoham, Yaron; Levy, Avraham; Rosenberg, Lior; Silberstein, Tali

doi:10.1097/prs.0000000000000638

Cited by 46 publications

(31 citation statements)

References 25 publications

Supporting

Mentioning

Contrasting

Order By: Relevance

“…Non-scalp ACC is often characterized by large, symmetric, and bilateral involvement of the trunk and extremities; however, the majority of these extracranial reports are associated with EB, and less commonly, with fetus papyraceus. 2,4,8 Our patient's biopsies were not consistent with EB, and his prenatal history was not consistent with fetus papyraceus.…”

Section: Discussionmentioning

confidence: 68%

Multidisciplinary management of a previously unreported presentation of severe aplasia cutis congenita

Davis

Voller

Gonzalez

et al. 2021

Pediatric Dermatology

View full text Add to dashboard Cite

First described by Cordon in 1767, aplasia cutis congenita (ACC) is characterized by the complete or partial absence of skin at birth. 1 ACC has an estimated annual incidence of 1 in 10 000 live births.Affected patients present with translucent, well-demarcated, ulcerated areas devoid of various tissue components, which can include the epidermis, dermis, subcutaneous tissue, and bone. 2 ACC occurs along the scalp vertex in 85% of cases. 2,3 To date, the etiology of ACC is unclear and appears to be multifactorial. Most cases are isolated, but there have been reported associations with multiple genetic syndromes and concomitant anomalies. 2,[4][5][6] Factors such as genetic mutations, teratogen exposure, trauma, neural tube defects, intrauterine infections, fetus papyraceus, and intrauterine vascular compromise have been described as possible etiologies. 2,3 Associated genetic mutations tend to be sporadic, but familial cases with dominant and recessive inheritance patterns have been described, as well as cases of post-zygotic mosaicism. 3 We present the case of a 3-month-old boy who presented at birth with a diagnosis of ACC of unknown etiology involving his face, neck, chest, abdomen, back, and extremities, affecting approximately 80% of his total body surface area (TBSA). This case adds to the literature due to the patient's extensive scalp-sparing ACC, which led to respiratory compromise and required multidisciplinary surgical management.

show abstract

Section: Discussionmentioning

confidence: 68%

Multidisciplinary management of a previously unreported presentation of severe aplasia cutis congenita

Davis

Voller

Gonzalez

et al. 2021

Pediatric Dermatology

View full text Add to dashboard Cite

show abstract

“…Surgical management, on the contrary, includes various procedures. Standard surgical care includes primary wound closure, skin grafting (autologous or allografts),[ 5 26 57 ] local scalp flaps with or without tissue expansion,[ 37 54 ] free flaps,[ 26 ] muscle flaps,[ 52 ] full-thickness or split-thickness skin grafts,[ 1 45 ] and cranial vault reconstruction using bone grafts. [ 16 37 ] Specialized surgical techniques such as utilizing bipedicle opposing local flaps,[ 12 32 ] rotational flaps,[ 24 31 42 50 ] or L-shaped flaps[ 2 ] have been used with satisfying results.…”

Section: Discussionmentioning

confidence: 99%

Aplasia cutis congenita: Two case reports and discussion of the literature

Blionas¹,

Giakoumettis²,

Antoniades³

et al. 2017

Surg Neurol Int

View full text Add to dashboard Cite

Background:Aplasia cutis congenita (ACC) is a part of a heterogeneous group of conditions characterized by the congenital absence of epidermis, dermis, and in some cases, subcutaneous tissues or bone usually involving the scalp vertex. There is an estimated incidence of 3 in 10,000 births resulting in a total number of 500 reported cases to date. The lesions may occur on every body surface although localized scalp lesions form the most frequent pattern (70%). Complete aplasia involving bone defects occurs in approximately 20% of cases. ACC can occur as an isolated defect or can be associated with a number of other congenital anomalies such as limb anomalies or embryologic malformations. In patients with large scalp and skull defects, there is increased risk of infection and bleeding along with increased mortality and therefore prompt and effective management is advised.Case Description:We describe two cases of ACC, involving a 4 × 3 cm defect managed conservatively and a larger 10 × 5 cm defect managed surgically with the use of a temporo-occipital scalp flap. Both cases had an excellent outcome.Conclusions:Multiple treatment regimens exist for ACC, but there is no consensus on treatment strategies. Conservative treatment has been described and advocated, but many authors have emphasized the disadvantages of this treatment modality. Decision between conservative and surgical management must be individualized according to lesion size and location.

show abstract

“…In the pediatric population, this product has been used to reconstruct both acquired and congenital conditions including, but not limited to, congenital melanocytic nevi, burn contractures, aplasia cutis, myelomeningoceles, and vascular malformations. 7 8 9 10 Integra can furthermore obviate the traditional demand for large full-thickness skin grafts and instead permit the use of split-thickness skin grafts thereby reducing donor site morbidity. This can be observed in hand reconstruction, where full-thickness grafts have traditionally been utilized in lieu of split-thickness skin grafts to prevent scarring and secondary contracture.…”

Section: Integramentioning

confidence: 99%

The Use of Biologic Wound Agents in Pediatric Reconstructions

et al. 2022

View full text Add to dashboard Cite

The indications for using biologic wound agents have expanded greatly since first being employed for acute burn management. The majority of the literature details the use of said agents in the adult population; however, there is little representation regarding their uses for reconstructing defects typically observed in the pediatric population. Ironically, children, and to a lesser extent adolescents, greatly benefit from their use given the reduced skin laxity and amount of surrounding tissue available for locoregional tissue transfer when compared with adults. Herein, we detail the use of acellular and cellular biologic wound agents in the pediatric population.

show abstract

Aplasia Cutis Congenita

Abstract: Therapeutic, IV.

Cited by 46 publications

References 25 publications

Multidisciplinary management of a previously unreported presentation of severe aplasia cutis congenita

Multidisciplinary management of a previously unreported presentation of severe aplasia cutis congenita

Aplasia cutis congenita: Two case reports and discussion of the literature

The Use of Biologic Wound Agents in Pediatric Reconstructions

Contact Info

Product

Resources

About