Conservative Management of Extensive Type V Aplasia Cutis Congenita in a Term Neonate Following Fetus Papyraceus: A Case Report and Guide for Outpatient Pediatric Management.

BACKGROUND

Type V aplasia cutis congenita (ACC) associated with fetus papyraceus presents management challenges for general pediatricians. While surgical intervention is often considered for extensive defects, conservative management may be effective with appropriate patient selection and protocols. To our knowledge, this is one of the most extensive cases of Type V ACC managed conservatively in the outpatient literature, and it provides a reproducible wound care protocol where none currently exists.

CASE: We report a female infant born at 37 weeks gestation with extensive Type V ACC involving circumferential areas of the torso, hips, posterior thighs, and posterior scalp (∼37-40% BSA) following intrauterine demise of a monochorionic co-twin. Conservative management using moist wound healing principles with Aquaphor, nonadherent dressings, and multidisciplinary care resulted in complete re-epithelialization within 6 weeks without surgical intervention. Outpatient management was successfully implemented with comprehensive family education and home health support.

CONCLUSION

Even extensive, circumferential Type V ACC can be managed successfully without surgery in hemodynamically stable infants who receive structured conservative wound care and family education. General pediatricians should consider this approach as a reasonable first-line strategy before surgical referral, reserving surgery for cases with failed conservative management, infection unresponsive to therapy, or significant functional impairment.