Extensive Aplasia Cutis Congenita and the Risk of Sagittal Sinus Thrombosis

Kantor, Jonathan; Yan, Albert C.; Hivnor, Chad; Honig, Paul J.; Kirschner, Richard E.

doi:10.1001/archderm.141.5.554

Cited by 30 publications

(19 citation statements)

References 12 publications

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“…However, most of these cases have either identifiable potential causes [4][5][6][7][8] or other associated anomalies, such as limb abnormalities consistent with Adams-Oliver Syndrome, 9,10 or other areas of ACC. 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.…”

Section: Discussionmentioning

confidence: 99%

“…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.…”

Section: Discussionmentioning

confidence: 99%

“…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. 16,18 All patients were healing well at follow-up, except for some partial necrosis of one of the skin grafts and two of the rotation flaps.…”

Section: Discussionmentioning

confidence: 99%

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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

2009

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Aplasia Cutis Congenita (ACC) is a rare condition characterized by the absence of a portion of skin at birth. Skin defects are usually small (0.5 to 3 cm) and located on the scalp. Although there can be other physical or genetic abnormalities, ACC is most often a benign isolated condition. Rarely is an underlying bony defect present, and this association increases the rate of complications. We report a case of a newborn male with ACC of the entire crown and vertex scalp, non-ossified parietal skull and dysplastic corpus callosum. The patient's skull and skin defects were treated non-surgically, and he recovered well.

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Section: Discussionmentioning

confidence: 99%

Section: Discussionmentioning

confidence: 99%

Section: Discussionmentioning

confidence: 99%

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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

2009

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“…Other threats to survival include thrombosis of the superior sagittal sinus [26], meningitis [25], sepsis [27], local infection [2,18,19,24,28], CSF leak [25,29,30], and hyponatremia [13,17]. Defects in the dura may also allow herniation or prolapse of the brain with mechanical injury to the parenchyma [3,25,29,30,31,32] and cerebral necrosis [17].…”

Section: Risksmentioning

confidence: 99%

Aplasia Cutis Congenita of the Scalp, Composite Type: The Criticality and Inseparability of Neurosurgical and Plastic Surgical Management

Winston

Ketch²

2016

Pediatr Neurosurg

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Background/Aims: The object of this report is to review the management of patients having the composite type of aplasia cutis congenita in the context of the relevant literature on this condition. Methods: Clinical records, neuroimaging and photographic documentation of identified cases of composite type aplasia cutis congenita, with a comprehensive review of the literature, are the material basis of this report. Results: Two neonates with composite type aplasia cutis congenita are described as examples of this disorder, and their management, including complications, is discussed. Both had satisfactory outcomes. Conclusions: Early aggressive surgical management with scalp rotation flaps has a low rate of complications, provides satisfactory functional and esthetic outcome, minimizes hospital stays, and should provide cost-effective care by reduction of the time to secure wound closure. Most bony defects, even large ones, commonly ossify completely. Optimal case management requires a synchronized neurosurgical and plastic surgical team. Intuitive nonsurgical and surgical approaches by the inexperienced can be hazardous.

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“…In diesem Bereich können auch knöcherne Defekte sowie ein Fehlen der Dura vorliegen. Bei größeren Defekten, besonders über einer Fontanelle, besteht die Gefahr der Blutung aus dem Sinus sagittalis mit Mortalitäts-raten zwischen 20-30% [7,9]. Als weitere Komplikation wird auch eine Ruptur der Dura mit der Folge einer bakteriellen Meningitis beschrieben [9].…”

Section: Epidemiologie Systematik Pathogeneseunclassified