ABSTRACT
Adams-Oliver syndrome is a rare disorder with varying degrees of scalp and cranial bone defects as well as limb anomalies, which can range from mild to more pronounced manifestations. In mild cases, closure of these defects can be achieved with a conservative approach. However, surgical closure is recommended in cases where the defect is extensive and includes cranial involvement. Several complicated cases of Adams-Oliver syndrome have been reported, in which flap failures were encountered and other alternatives had to be used to close critical scalp defects. Here, the case of a 4-year-old child with Adams-Oliver syndrome and a complex cranial defect with exposed titanium mesh is described. The patient was successfully treated with epidermal growth factor (EGF) infused foam dressings and subsequent split-thickness skin grafting. The EGF has been highlighted for its essential role in dermal wound repair through the stimulation of the proliferation and migration of keratinocytes, and showed accelerated wound healing when used in partial or full-thickness skin wounds.
Subject(s)
Bandages , Ectodermal Dysplasia/therapy , Epidermal Growth Factor/therapeutic use , Limb Deformities, Congenital/therapy , Scalp Dermatoses/congenital , Scalp/surgery , Skull/surgery , Surgical Flaps , Child, Preschool , Humans , Imaging, Three-Dimensional , Scalp/abnormalities , Scalp Dermatoses/therapy , Skull/abnormalities , Surgical Mesh , Titanium , Tomography, X-Ray ComputedSubject(s)
Anemia, Megaloblastic , Malabsorption Syndromes , Vitamin B 12 Deficiency , Humans , Infant , Male , SyndromeABSTRACT
We present premature female twin fetuses with concordant extremely shortened ribs, short limbs, macrocephaly, median cleft upper lip and facial dysmorphism. Based on radiological criteria and the pattern of associated abnormalities, a lethal short rib-polydactyly syndrome (Beemer-Langer type) was diagnosed. The differential diagnosis of this entity is discussed.
