Airway Challenges in Sanjad-Sakati Syndrome (SSS): A Case Report and Review of Literature

Mohamed A Khalil,Talal M Alghamdi,Najeebah A Al Elk,Alaa M Khidr

doi:10.31487/j.scr.2021.07.08

Abstract

Sanjad-Sakati Syndrome (SSS) is a rare syndrome, discovered in Saudi Arabia in 1988. This syndrome is characterized by congenital hypoparathyroidism, severe growth retardation, dysmorphic features, mild to severe mental retardation as well as recurrent chest infections. We presented an eight-year-girl with SSS, with severe growth retardation, mental retardation, resolution of recent chest infection together with a history of bronchial asthma, dysmorphic features, limited mouth opening, micrognathia as well as high susceptibility to difficult intubation. The objective of this case report is to present the successful anaesthetic management of a rare genetic endocrinal syndrome.

Highlights

Sanjad-Sakati Syndrome (SSS) was first reported in Saudi Arabia in 1988, known as hypoparathyroidism–retardation-dysmorphism syndrome [1]
We presented an eight-year-girl with SSS, with severe growth retardation, mental retardation, resolution of recent chest infection together with a history of bronchial asthma, dysmorphic features, limited mouth opening, micrognathia as well as high susceptibility to difficult intubation
An eight-year-old Saudi girl, presented to our hospital for full oral rehabilitation and dental extractions. This girl complains from SSS since birth with congenital hypoparathyroidism, hypocalcemia, hyperphosphatemia, mental retardation, delayed developmental milestones (Figure 1), bronchial asthma and recurrent chest infections

Summary

Introduction

Sanjad-Sakati Syndrome (SSS) was first reported in Saudi Arabia in 1988, known as hypoparathyroidism–retardation-dysmorphism syndrome [1]. The main presentations would be hypocalcemia, hyperphosphatemia, mild to severe mental and growth retardation, seizures, dysmorphic features (micrognathia, abnormal dentition, and dentofacial anomalies), and recurrent chest infections [3]. Anaesthetic management of these patients poses great challenges owing to the susceptibility to difficult intubation, recurrent chest infections as well as great sensitivity to muscle relaxants [4]. An eight-year-old Saudi girl, presented to our hospital for full oral rehabilitation and dental extractions This girl complains from SSS since birth with congenital hypoparathyroidism, hypocalcemia, hyperphosphatemia, mental retardation, delayed developmental milestones (weight 8.6 Kg, height 77 cm) (Figure 1), bronchial asthma and recurrent chest infections. Airway was secured by insertion of nasotracheal re-inforced 4.5 mm internal diameter cuffed tube

Discussion

Conclusion