On arrival in the operating room, pulse oximetry, electrocardiography, and non-invasive blood pressure monitoring were established. Premedication was not performed before general anesthesia.
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In addition, we prepared masks of various sizes for manual ventilation. For the prevention of difficulty to ventilate during the induction of anesthesia, we stood by with a difficult airway management cart and a bronchial fiberscope.
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A 12-lead electrocardiogram, chest radiogragh, and blood tests were carried out as preoperative examinations, and no abnormality was indicated.įull size image Management of general anesthesiaĭifficulty inserting of the intravenous catheter was expected because of the skin folds on the limbs therefore, a 24-G line had been inserted in the right forearm by a pediatrician on the day before the operation. 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. EtiologyĪutosomal dominant and autosomal recessive transmission, as well as sporadic cases have been reported.Barber-Say syndrome (BSS) is a very rare congenital disorder characterized by macrostomia, cutis laxa, and other features. Other less frequent findings include cleft palate, hearing loss, mild psychomotor delay and genital abnormalities. Dental abnormalities described include taurodontism, shovel-shaped incisors, delayed eruption of deciduous dentition and premature apical closure. Teeth are present, but with overgrown gingiva. Clinical descriptionīBS presents with congenital generalized hypertrichosis, facial dysmorphism (typically with bilateral ectropion, absent or sparse eyebrows and lashes, hypertelorism/telecanthus, broad nasal bridge, bulbous nose, anteverted nostrils, macrostomia, thin lips and misshapen ears), hyperlaxity and redundancy of the skin with deep folds, nipple hypoplasia and absence of mammary glands. BBS is a rare entity described in eleven patients to date.
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