BRONCHOSCOPY

1944 Menten ML, Middleton TO. Cystic fibrosis of the pancreas: report of 18 proved cases. Am J Dis Child 1944; 67:355-359.
Another paper based on a search through previous paediatric autopsies revealed 18 cases of CF from The Children’s Hospital, Pittsburgh; all had diffuse definite dilatation of the pancreatic acini and associated pneumonia. The authors state that radiological examination of the lungs showed a remarkable similarity in the unusual enlargement of the hilar shadows and a bilateral shadow decreasing to the periphery and suggest this as a diagnostic feature. Three had bronchoscopy during life- in one showing a normal trachea and in two reddened thickened mucous membrane containing thick mucopurulent material. This is the first mention of the bronchoscopic appearances seen in cystic fibrosis.

1948 Atkins JP. Bronchoscopic observations on the pulmonary aspects of fibrocystic disease of the pancreas. Ann Otol (St Louis) 1948; 57:791-801. [PubMed]
An early report of bronchoscopy in children with CF using the rigid bronchoscope – the only type available at that time. Atkins stated the expiratory intrusion of the posterior wall into the lumen produced crescenteric cross section of the bronchi not differing from that seen in bronchial asthma. Prior to this Menton & Middleton (1944 above), reporting 18 children with CF from Pittsburgh in 1944, incidentally described the bronchoscopic appearances in three patients who were so examined. Later Robert Wood in the USA pioneered the use of flexible bronchoscopy in children from 1975 onwards (See Wood et al, 1973 & 1975 below).

1975 Wood RE, Wanner A, Hirsch J, Farrell PM. Tracheal mucociliary transport in patients with cystic fibrosis and its stimulation by terbutaline. Am Rev Respir Dis 1975; 111:733-8. [PubMed]
Robert Wood observed the movement of Teflon discs passing up the trachea through a bronchoscope in 14 adults with cystic fibrosis. The discs moved at 2.6 mm/min in CF and 20.1 mm/min in controls; terbutaline increased the speed of movement in CF to 5.5mm/min. The authors speculated, correctly as it has now become apparent, that this “may play a role in the pathogenesis of pulmonary disease”.
Robert Wood pioneered fibreoptic bronchoscopy in children with CF. The 3.5mm paediatric version of the bronchoscope he used in 1975 had no suction port which he circumvented by attaching a fine Teflon tube to the side allowing its use down to children aged 18 months; it was developed by the Olympus Corporation into the first flexible pediatric bronchoscope in 1978 at Dr Wood’s request.

1980 Wood RE, Sherman JM. Pediatric flexible bronchoscopy. Ann Otol Rhinol Laryngol 1980; 89:414-416. [PubMed]
The first report of the use of the paediatric flexible fibreoptic bronchoscope in children by Robert Wood who pioneered the technique. He described a prototype flexible paediatric bronchoscope that he had used to perform both diagnostic and therapeutic procedures on pediatric patients ranging from infants of 840 g to children aged 14 years. Flexible bronchoscopy, with appropriate instrumentation and careful attention to physiological requirements of the patient, was found to be safe and effective in young patients. Wood correctly forecast that with this instrument, the indications for bronchoscopy in children would be considerably expanded. (Also there is an excellent review of flexible bronchoscopy by Wood RE. J Pediatr 1988; 112:1-6 [PubMed] ; also Wood RE. “Pediatric flexible bronchoscopy: The inside story”. In: Doershuk CF, (Ed.). Cystic Fibrosis in the Twentieth Century. Cleveland: AM Publishing Ltd, 2001:112-119.With permission). Expansion continues and it has even been suggested that every infant identified by newborn screening should be bonchoscoped to obtain cultures of the lower airways (Hilliard TN et al. Arch Dis Child 2007; 92:898-899 ). [PubMed]

2007 Hilliard TN, Sukhani S, Francis J, Madden N, Rosenthal M Balfour-Lynn I, Bush A, Davies JC. Bronchoscopy following diagnosis with cystic fibrosis. Arch Dis Child 2007; 92:898-899. [PubMed]
The authors recently changed their practice and performed bronchoscopy following a diagnosis of cystic fibrosis. On a retrospective review of 25 children, Pseudomonas aeruginosa was detected in bronchoalveolar lavage for the first time in five children (20%) and Staphylococcus aureus in four (16%). Lavage culture was positive in eight of the 18 children without respiratory symptoms. The authors suggest that these findings highlight the potential of bronchoscopy following diagnosis, even in asymptomatic children.

Whether to recommend bronchoscopy in an asymptomatic screened infant with CF is dependent on many factors, not least, where the infant was born and the facilities and skill available for paediatric respiratory investigation. Also, the treatment policy of the unit responsible for the care of the infants. Also there is the potential danger of infecting an, as yet uninfected, infant with the instrument if sterilisation has been faulty; also hospitalisation does present a definite infection risk to infants with CF. If the infants were started on prophylactic flucloxacillin from diagnosis (as is recommended by the UK CF Trust's expert Antibiotic Group 2009), it is very unlikely that S. aureus would have been cultured. Also units where screening has been routine for many years, such as Leeds, have managed to achieve very low levels of chronic Pseudomonas infection using only frequent throat cultures, cough swabs and serum antibody levels to recognise and treat early P. aeruginosa infection. If an infant with CF had repeatedly negative upper respiratory tract cultures and negative Pseudomonas antibody levels it would be very unlikely there would be positive bronchial cultures. So bronchoscopy for all at diagnosis, although it may be decided is desirable, is definitely a policy that needs careful discussion before being applied generally.

The recent multicentre study completed in 2009 comparing regular bronchoscopy with routine care led by Claire Wainwright of Brisbane does not show a significant advantage for those who have regular bronchoscopies.

2008 Wainwright CE, Grimwood K, Carlin JB, Vidmar S, Cooper PJ, Francis PW, Byrnes CA, Whitehead BF, Martin AJ, Robertson IF, Cooper DM, Dakin CJ, Masters IB, Massie RJ, Robinson PJ, Ranganathan S, Armstrong DS, Patterson LK, Robertson CF. Safety of bronchoalveolar lavage in young children with cystic fibrosis. Pediatr Pulmonol 2008; 43:965-972. [PubMed]
As part of Dr Claire Wainwright’s study of bronchoalveolar lavage (BAL) directed therapy, 333 BALs were carried out on 107 children median age 23.5 months (1.6 - 67.5 months); 170 (51%) were for exacerbations. 8.7% were followed by fever and 3% clinically significant episodes. 52% had minor adverse events.
The authors concluded that although adverse events were common they were usually transient and well tolerated. Parents should be warned that infants with respiratory infections had an increased risk of post-BAL fever. This is an important ongoing study further progress of which was reported by Claire Wainwright at the 2009 NACFC in Minneapolis. The study concluded in 2009 and did not establish a case of managing the respiratory infections using regular bronchoscopies but did provide a vast amount of useful information - undoubtedly one of the studies of the decade!