ABSTRACT
Pierre Robin Sequence (PRS) is defined by a constellation of characteristics including micrognathia, glossoptosis and airway obstruction. PRS can occur in isolation or can be associated with syndromes and another anomalies. Airway obstruction and feeding difficulties are the major presenting issues, and the severity of the condition ranges from mild, with minimal to no symptoms, to severe, with overt obstruction resulting in apnoeas, severe respiratory distress and cyanosis. The presence of airway obstruction can result in obstructive sleep apnoea and abnormalities in gas exchange, as well as exacerbation of already present feeding difficulties and failure to thrive, secondary to mismatch of caloric intake to energy usage associated with increased effort of breathing. Management of airway obstruction for infants with PRS varies between centres. This paper explores the surgical and non-surgical management options available, their effectiveness and pitfalls in children with PRS. Despite the pros and cons of each management option, it is evident that resource availability and multidisciplinary clinical support are key factors to successful management.
Subject(s)
Airway Obstruction , Osteogenesis, Distraction , Pierre Robin Syndrome , Sleep Apnea, Obstructive , Infant , Child , Humans , Pierre Robin Syndrome/complications , Pierre Robin Syndrome/diagnosis , Pierre Robin Syndrome/surgery , Osteogenesis, Distraction/methods , Sleep Apnea, Obstructive/complications , Dyspnea , Treatment OutcomeABSTRACT
Cheyne-Stokes respiration (CSA-CSR) is a form of central sleep apnea characterized by alternating periods of hyperventilation and central apneas or hypopneas. CSA-CSR develops following a cardiac insult resulting in a compensatory increase in sympathetic activity, which in susceptible patients causes hyperventilation and destabilizes respiratory control. The physiological changes that occur in CSA-CSR include hyperventilation, a reduced blood gas buffering capacity, and circulatory delay. In adults, 25% to 50% of patients with heart failure are reported to have CSA-CSR. The development of CSA-CSR in this group of patients is considered a poor prognostic sign. The prevalence, progression, and treatment outcomes of CSA-CSR in children remain unclear with only 11 children being described in the literature. The lack of data is possibly not due to the paucity of children with severe heart failure and CSA-CSR but because they may be under-recognized, compounded by the absence of routine polysomnographic assessment of children with moderate to severe heart failure. Building on much broader experience in the diagnosis and management of CSA-CSR in adult sleep medicine and our limited experience in a pediatric quaternary center, this paper will discuss the prevalence of CSA-CSR, its' treatment options, outcomes in children, and the potential future direction for research in this understudied area of pediatric sleep medicine.
Subject(s)
Heart Failure , Sleep Apnea, Central , Adult , Humans , Child , Cheyne-Stokes Respiration/therapy , Cheyne-Stokes Respiration/diagnosis , Cheyne-Stokes Respiration/etiology , Hyperventilation/complications , Sleep Apnea, Central/complications , Sleep Apnea, Central/therapy , Heart Failure/complications , Heart Failure/therapy , SleepABSTRACT
OBJECTIVE: To analyse the dose-dependent effect of body mass index (BMI) categories for common pregnancy outcomes. METHODS: A retrospective cohort study of all deliveries that occurred between 1 January 2005 and 31 December 2009 in a tertiary maternity centre, in Sydney Australia. Common pregnancy outcomes were analysed against World Health Organization (WHO) BMI categories using multiple logistic regression analysis. RESULTS: From a total of 18,304 pregnancies, 9087 singleton pregnancies with complete data-sets were identified. Of these pregnancies, 4000 (44%) had a normal BMI, 470 (5.2%) were underweight, 2293 (25.2%) were overweight, 1316 (14.5%) were obese class I, 630 (6.9%) were obese class II and 378 (4.2%) were obese class III. Using the normal BMI category as the reference, there was a clear dose effect of BMI categories for hypertension (P < 0.001), pre-eclampsia (P < 0.001), caesarean section (P < 0.001), macrosomia (P < 0.001), large for gestational age (P < 0.001), small for gestational age (P < 0.001) and neonatal respiratory distress (P = 0.039). In contrast, despite a significant association with BMI (P < 0.001), a dose-dependent effect was not found for gestational diabetes. CONCLUSION: The results of our study have important clinical significance as the data, using WHO BMI categories, more accurately help stratify risk assessment in a clinically relevant dose-dependent relationship.