Outcomes and morbidities of patients who survive haemoglobin Bart's hydrops fetalis syndrome: 20-year retrospective review.
INTRODUCTION
Haemoglobin Bart's hydrops fetalis syndrome was once considered a fatal condition. However, advances over the past two decades have enabled survival of affected patients. Data relating to their morbidities and outcomes will help medical specialists formulate a management plan and parental counselling.


METHODS
All babies with the syndrome who survived beyond the neonatal period and were subsequently managed long-term in eight public hospitals in Hong Kong from 1 January 1996 to 31 December 2015 were included. Patient and parent characteristics, antenatal care, reasons for continuation of pregnancy, intrauterine interventions, perinatal course, presence of congenital malformations, stem-cell transplantation details, and long-term neurodevelopmental outcomes were reviewed.


RESULTS
A total of nine patients were identified, of whom five were female and four male. The median follow-up duration was 7 years. All were Chinese and were homozygous for the Southeast Asian α-thalassaemia deletion. Five of the nine mothers received antenatal care at a public hospital and opted to continue the pregnancy after antenatal diagnosis and counselling. Despite intrauterine transfusions, all babies were born with respiratory depression and required intubation and mechanical ventilation during the neonatal period. Hypospadias was identified in all four male infants. Growth retardation, global developmental delay, and residual neurological deficits were noted in two-thirds of the patients. Haematopoietic stem-cell transplantation was performed in two patients, who became transfusion-independent.


CONCLUSIONS
Survival of patients with Bart's hydrops fetalis syndrome is possible but not without short- and long-term complications; local epidemiology is comparable to that documented for an international registry. Detailed antenatal counselling of parents with a non-judgemental attitude and cautious optimism are imperative.
['Chan|W Yk|WY|', 'Leung|A Wk|AW|', 'Luk|C W|CW|', 'Li|R Ch|RC|', 'Ling|A Sc|AS|', 'Ha|S Y|SY|']
D005260:Female D006455:Hemoglobins, Abnormal D006801:Humans D015160:Hydrops Fetalis D007231:Infant, Newborn D008297:Male D009017:Morbidity D011247:Pregnancy D011296:Prenatal Diagnosis D012189:Retrospective Studies D017085:alpha-Thalassemia
Morbidity Hydrops fetalis Hong Kong Alpha-thalassemia Thalassemia