Palatoplasty in a patient with Seckel syndrome
An 8-year-old male child was presented to our outpatient department with complaints of a wide cleft palate causing poor feeding, repeated upper respiratory infections, and inability to verbalize. The parents of the child expressed their concerns for an early surgical correction.\nOn detailed history, the parents revealed that he was conceived by Intra Uterine Insemination (IUI, donor husband) of nonconsanguineous parents. The father has had infertility from severe oligospermia and the mother had hyperprolactinemia for which they had undergone treatment 1½ years earlier. Mother also gave history of hypertension, diabetes mellitus and severe oligohydramnios, during the gestational period. Antenatal Intra Uterine Growth Retardation (IUGR) has been noticed in all trimesters. Born at 34 weeks of gestation by normal vaginal delivery with birth weight of 930 g, he cried immediately after birth. He was kept on ventilator for almost 1 month for lung hypoplasia. His developmental milestones were slightly delayed. A history of uneventful dental restoration under sedation or GA in another tertiary care hospital was present. General physical and systemic examination showed evidence of characteristic features of Seckel syndrome, i.e., microcephaly, retroganthia, bird-like face [], large eyes, small low set ears, dental caries, long neck, clinodactly, bilateral elbow contracture, left undescended testis, and urinary and fecal incontinence because of small spina bifida, high arch palate with incomplete cleft of palate []. His weight was 9.5 kg and height was 100 cm [].\nDetailed investigations showed normal hematological profiles with no abnormality on echocardiography. Chest x-ray showed a tubular cardiac shadow with downward slanting rib cage and normal lung fields. His other two female siblings, also conceived by IUI, showed normal development with no evidence of Seckel syndrome. The patient was scheduled for a pushback palatoplasty under GA and hence an informed parental consent was taken both by the anesthesiologists and surgical team.\nGA was administered using inhalational induction with sevoflurane in titrated doses while preserving spontaneous respiration in view of an anticipated difficult airway. A fully equipped difficult airway cart was kept ready. After achieving IV cannulation using 24G cannula, and ascertaining chest rise with bag mask ventilation, endotracheal intubation was facilitated using a short-acting neuromuscular blocking agent (NMB) with un-cuffed RAE tube size 4.5 mm, after initial failed attempt with tube size 6.0 mm due to narrowing of subglottic region. Anesthesia was maintained with oxygen and air along with titrated doses of sevoflurane and intermittent doses of atracurium. Monitoring in the form of ECG, heart rate, NIBP, EtCO2, and temperature was instituted.\nPushback palatoplasty was done for incomplete cleft palate []. The mucoperiosteal flaps were thin and hypoplastic. The lateral alveolar grooves were packed with collagen sheet for postoperative hemostasis. The intraoperative period was uneventful. The total operative time was 75 minutes. Postoperatively, patient was kept in intensive care for monitoring of any apneic spells, respiratory failure, or oral bleed.