![]() Furthermore, he had no episode of a seizure before the surgery. 1), but another physical abnormality was not observed, including neurologic abnormality. On physical examination, the previously observed findings of macrostomia and redundant skin on the limbs were recognized (Fig. The patient was 1 year and 6 months old, his height was 75 cm, and his weight was 9.9 kg just before the operation. Plastic surgery (repair of the corners of the mouth, shortening of the lingual frenulum, and shortening of the upper lip frenulum) for macrostomia was planned. ![]() From these malformations, he was clinically diagnosed with BSS by pediatricians based on searching both the Pub-Med database ( ), and the University of Ryukyu Database for Malformation Syndromes ( ). Characteristic forms of abnormality were observed such as macrostomia, gingival dysplasia, ocular telecanthus, hypertrichosis, dark brown and dry skin with redundant folds on the limbs, and hypospadias. Mask ventilation and the insertion of an intravenous catheter may be difficult in the general anesthesia of patients with BSS, and anesthetic management requires caution.Ī Japanese male was born with a length of 50.4 cm and a weight of 3390 g by vacuum extraction, and his Apgar scores were 9 and 9 at 1 and 5 min after birth, respectively. ![]() Stiffening of both arms occurred for several seconds one hour after the operation ended, but the patient did not develop other complications. The operation was completed without incident. There was no laryngeal anatomical abnormality, and tracheal intubation was readily possible. After rocuronium was administered, the larynx was spread with a Macintosh laryngoscope. A mask for adults provided a superior fit and was effective in preventing air leakage from the corners of the mouth. The patient wore a mask and was ventilated manually after loss of consciousness with intravenous anesthesia. As insertion of the peripheral intravenous catheter was difficult, it was inserted before the surgery by a pediatrician. Case presentationĪ bilateral repair of the corners of the mouth under general anesthesia was planned for an 18-month-old male with macrostomia the child was 75 cm in height and weighed 9.9 kg. We report our experience of performing general anesthesia on a Japanese child with BSS. The presentĪtion, however, varies depending on the severity of the case.Barber-Say syndrome (BSS) is a very rare congenital disorder characterized by macrostomia, cutis laxa, and other features. There are only 20 cases in medical literature. Barber Say syndromeīarber Say syndrome is a rare condition which presents with symptoms of excessive hair growth and atrophic skin. Further examination did not reveal any significant findings. Skin over the patient’s chest was atrophic with atrophic nipples. Hirsutism of the forehead, neck and back were also evident. Examination showed prognatism, macrostomia with thin lips, bulbous nose with anteverted nares, telecanthus, hypertelorism, sparse eyebrows and eyelashes, ectropion, ablepharon and an aged appearance. In addition, her external auditory canals were narrow and tortuous, her ears were low set small with a prominent antihelix. Further examination showed a redundant frontal skin with dark thick hair. Her anthropometric measurement were recorded as height, 147.5 cm (10th percentile) weight, 37.4 kg (15th percentile), and head circumference, 52.2 cm (40th percentile). ![]() Although, there were no signs of any visceral abnormalities during the neonatal period.ĭoctors evaluated the patient at the age of 13. The patient’s physical examination was remarkable of severe hirsutism of the back, redundant thick skin, helix agenesis of both ears, macrostomia, telecanthus, hypertelorism and ablepharon. The pregnancy and delivery were both normal and the child was born at a healthy weight of 3400 g and length 50 cm. She was was the eighth child of nonconsanguineous parents her mother was 41, whereas father 42 years old when the child was born. Her medical history revealed that she was the last of eight otherwise healthy siblings. This article describes the case of a 14 year-old diagnosed with Barber Say syndrome. Case of a 14-year-old with a multiple congenital anomaly pattern.
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