Dental Management and Orofacial Manifestations of a Patient With Robinow Syndrome: Case Report

Abstract

Robinow syndrome (RS) is an extremely rare condition. Characteristic craniofacial findings of RS include a fetal facial appearance, ear abnormalities and oral findings. The aim of this case report was to evaluate the oral findings of a 26-year-old man with RS and to describe the dental treatments performed. The patient had short stature, vertebral anomalies, short and broad fingers, a fetal facial appearance, gingival hyperplasia, fissured tongue, caries and multiple impacted teeth. Periodontal and restorative dental treatments were performed under aseptic conditions with due precautions. No surgical treatment was performed to the impacted teeth because of the lack of symptoms.

Oral findings in Robinow Syndrome: a case report in pediatric patient

“…4 Hypertension, heart and kidney disease are frequent findings associated to RS and may represent complicating factors for surgeries. 9,11 Our patient did not report any systemic problem during anamnesis, despite that, medical authorization were requested before the extraction of supernumerary and retained primary teeth. Cardiac, renal disease, hypertension and vertebral anomalies have been reported in older patients, 9,11,13 it seems that such problems may arise in childhood or later.…”

“…[5][6] Recognize facial and oral findings is important to planning dental treatment and some common findings in a Robnow syndrome patient were also observed in our clinical case as short stature, mesomelic limb shortening, short and broad fingers, macrocephaly and a dysmorphic face with prominent eyes, flat nasal bridge, frontal bossing, hypertelorism, short noses and dental anomalies. 6,8,9 According to Mazzeu et al, 7 over 79% of patients with RS present these characteristic facial features. Other associated malformations include cleft lip and palate, syndactyly and heart defects 6,7 that were not observed in the patient.…”

“…In relation to orofacial manifestations of the Robinow syndrome, Basman et al 9 described a case with a boy that presented tongue tip absence, ankyloglossia, arched palate and delayed tooth eruption. In our case, these findings were also present, except the arched palate.…”

“…Other articles cited the need for gingival and orthodontic corrections in patients with RS, successfully performing common treatment. 9,13 Only one case has been found reporting severe hyperplastic gingival tissue impeding both normal tooth eruption as well as orthodontic movement, probably caused by the use of growth hormone. 8 This case describes uncommon oral findings and some important aspects to avoid complications associated with the Robinow syndrome.…”

“…[6][7][8] Usual oral findings comprise dental anomalies and dental crowding, gingival hyperplasia and tongue abnormalities. 6,9 The aim of this article is to present a case of a child with autosomal recessive Robinow syndrome and describe the facial and dental abnormalities.…”