PubMed · 11449928
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J Laurence. 2001. Facial wasting.. https://pubmed.ncbi.nlm.nih.gov/11449928/
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BACKGROUND: The prognosis for Pierre Robin sequence is often closely correlated with direct postnatal asphyxia (ARDS) and permanent cerebral damage. Unfortunately, all of the well-known treatments entail many problems, which often lead to medical care of the patient for many years. Distraction osteogenesis was recently introduced as a very promising procedure. We revised this concept for newborn children aged up to 3 months. MATERIALS AND METHODS: The first patient was operated with an extraoral distractor at an age of approx. 3 months. The second patient was operated at an age of approx. 2 months. A new distractor was used, conceived specifically by our department for newborn children. RESULTS: Distraction osteogenesis was successful in both cases. After 20 days the first patient was able to breathe without the tracheotomy cannula. In the second case, we removed the nasopharyngeal tube successfully on the 7th postoperative day. The bilateral length gain amounted to 20 mm in the first case and 15 mm in the second. DISCUSSION: The usual jaw distraction osteogenesis has many disadvantages in babies: scar formation in the places depressed by pins and early pin loss. For the above-mentioned reasons, we developed a new type of extraoral distractor, which solves these problems. Less required space and the absence of pins resulting in fewer disfiguring scars are the clinical advantages of this new distractor.
Infantile neuroaxonal dystrophy (INAD) is an autosomal recessive, neurodegenerative disease with onset in the first or second year of life. It has been reported that INAD shows numerous phenotype characteristics including problems associated with vision, hearing and physical coordination. It has however been very rare to see facial dysmorphism in these children. The study analyzes a girl and boy of a first cousin marriage with infantile neuroaxonal dystrophy affected at birth. At infancy, the children were examined in the Cerrahpaşa Medical Faculty Genetic Research Center, Istanbul. They had typical INAD features such as the lack of head control, vision, speech, sitting, and walking which are also seen in children with other congenital abnormalities. These children showed remarkable dysmorphism in the face which included prominent forehead, strabismus, small nose, fish mouth (boy), micrognathia, and large and low-settled ears. The presence of these facial features makes the patients appear unique and diagnosis more accurate. While these features are commonly seen diagnosis may be difficult at its onset. Until now this appearence has not been reported in INAD patients. In conclusion, in the first few months of life without any clinical or neurological signs, the physician should also consider diagnosing the disease of the infant as INAD.
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