Split Rib Cranioplasty for Aplasia Cutis Congenita and Traumatic Skull Defects

Abstract

Aplasia cutis is a rare skin defect usually presenting over the vertex of the skull. An underlying bone defect is found in approximately 20% of patients. Most skull defects close spontaneously. However, when there are no signs of ossification, closure is mandatory. We present our experience in three patients. Our first patient had an aplasia cutis with a skull defect. The split rib graft procedure was used without complications, and a good cosmetic and functional result was achieved. The second patient was operated on for cerebral bleeding after an arteriovenous aneurysm, and a bony defect could not be closed after that procedure. At a later stage, the defect was filled with split rib grafts, and sufficient protection was achieved and documented after more than 30 years. The third patient was born with an aplasia cutis congenita with a skull defect. The bony defect was filled with split rib grafts without complications at an age of 5 years. Follow-up shows a functional result with a firm skull. Patients with aplasia cutis may have skull defects that will not close by themselves. We present three patients with a bony defect who were reconstructed with split rib grafts. After a long period of follow-up, there remains good cosmetic and functional results. Defects of the skull in children can be reconstructed with split rib grafts that will accommodate the growing skeleton and give good protection of the brain from an early age on.

Cranioplasty in Children with Split Rib Graft

“…For the developing paediatric cranial vault, the use of synthetic materials is not ideal because they lack growth potential hence fail to adapt with the developing neurocranium. Infection, dislodgement, migration, thermal sensitivity and implant exposure also have been reported [4,5]. Autogenous bone is better suited for grafting in paediatric patients because it meets the goals of cranioplasty while potentially allowing ongoing growth [6].…”

A case of extensive Aplasia Cutis Congenita with underlying skull defect and central nervous system malformation: discussion of large skin defects, complications, treatment and outcome

“…11 Of the cases without identifiable causes or other associated problems, none of the patients had a solitary area of scalp ACC that was as extensive as the patient we present. [12][13][14][15][16][17][18][19] It is reported that only 15 to 20% of cases of scalp ACC are associated with an underlying bony defect. 2,20 However, the frequency of an underlying osseous defect is higher in larger areas of ACC.…”

“…5,12,18 There was one death, which was attributed to respiratory failure. 11 The treatment for ACC has long been controversial.…”

“…Management of a large skin defect in ACC can be conservative, with dressings as described above, or surgical options. Surgical options include skin grafts, 5,6,8 local flaps 7,12,13,16,18 or free flaps. 22 Potential post-surgical problems encountered include sagittal sinus hemorrhage upon scar debridement, 18 flap necrosis 12,13 and graft loss.…”

“…Surgical options include skin grafts, 5,6,8 local flaps 7,12,13,16,18 or free flaps. 22 Potential post-surgical problems encountered include sagittal sinus hemorrhage upon scar debridement, 18 flap necrosis 12,13 and graft loss. 5 Eight of the 16 reviewed cases of large scalp ACC described the surgical treatment of the skin defect: three split-thickness skin grafts, 5,6,8 three rotation flaps 7,12,13 and two advancement flaps.…”