The endoscope has long been an invaluable tool in assessment of the paediatric airway. Recently, its applications for definitive surgery of the airway have greatly increased, due to innovative surgical techniques, development of new instruments, improvements in anaesthesia and availability of new medications for endoscopic use. This review discusses the move towards endoscopic techniques in the management of paediatric airway disorders.

Aims: To report on the use of holmium yttrium aluminium garnet (YAG) laser in the treatment of acquired posterior choanal atresia following radiotherapy in patients with nasopharyngeal carcinoma.

Materials and methods: Five patients with acquired bilateral choanal atresia and two with unilateral choanal atresia in the posterior choanae were identified following treatment of nasopharyngeal carcinoma by external radiotherapy, from July 1998 to April 1999. The mean age was 44.6 years (range, 22–65 years). Two patients had stage two and five had stage three disease according to Ho's classification. All patients received 66 Gy of external irradiation delivered to the nasopharynx and a mean dose of 61 Gy (39.8–72 Gy) to the neck. Five patients received an additional external boost of 20 Gy and two received 14 Gy delivered to the parapharyngeal region. Two other patients also received intracavitatory brachytherapy of 18 Gy delivered to the nasopharynx. One patient had concurrent chemotherapy by cisplatinum during external radiotherapy. The mean onset of nasal symptoms was 17 months (range, 2–38 months) following irradiation. All patients were treated by transnasal endoscopic holmium YAG laser resection. Merocel® epistaxis packing and a modified nasopharyngeal tube were used to stent the nasal airway for two weeks post-operatively.

Results: The mean follow up was 26.8 months (range, 20–32 months) after surgery. All patients were symptom free on follow up. Five patients (71 per cent) retained full patency in the posterior choanae. Two patients (28 per cent) had bilateral mild restenosis in the postnasal space, not requiring revision surgery. The surgical fields were almost bloodless. No adverse post-operative complications occurred, except for delayed nasal septal perforation in one patient and unilateral exposure of the septal cartilage in another; no further treatment was required in either patient.

Conclusion: Acquired posterior choanal atresia is an unusual complication following radiotherapy, and it can occur early after treatment. It can be successfully treated by transnasal endoscopic holmium YAG laser resection of the scar tissue, with minimal bleeding. A two week period of Merocel® nasal packing and nasopharyngeal tube stenting was sufficient to prevent severe restenosis in the posterior choanae; nasal breathing through the tube lumen was possible during this time.

We report here a very rare case of bilateral congenital choanal atresia untreated for the 27 years after birth. A 27-year-old Japanese man complaining of long-term nasal obstruction, snoring and daytime sleepiness visited our hospital. He exhibited the characteristic appearances of long face and bilateral hypoplastic cheeks. Examination by flexible fiberscopy and computed tomography led us to diagnose him with bilateral choanal atresia. Subsequent polysomnography revealed the number of apnoea or hypopnoea episodes per hour (apnoea—hypopnoea index, or AHI) to be over 10. On the basis of these observations, we modified the diagnosis to obstructive sleep apnoea syndrome (OSAS) secondary to congenital choanal atresia.

Choanal atresia is a significant congenital anomaly, which may be associated with other congenital anomalies. Recommendations have been previously proposed for neonatal screening of all newborns to detect this condition. A previously unreported, simple method using the automatic tympanometer to screen newborns for congenital choanal atresia is proposed. A prospective study was performed over a one-month period on all newborns at El-Sahel Teaching Hospital, Cairo, to evaluate the efficacy of this manoeuvre.

The nostrils of 143 newborn infants were evaluated during the study period. The sensitivity of the technique in diagnosing a patent nostril was 100 per cent. The specificity of the technique for detecting distal obstruction was 100 per cent. There were no complications of the described procedures. The study revealed that the novel method of screening for choanal atresia in newborns using the automatic tympanometer is simple, minimally invasive and highly reproducible.

Bilateral choanal atresia is potentially life-threatening. The condition almost always presents in the new born, with alternating phases of respiratory distress and apnoea relieved by crying. We report a rare case of bilateral choanal atresia presenting for the first time at 22 years of age. The patient had no features of cyanosis or apnoea, presenting only with bilateral nasal obstruction, rhinorrhoea and anosmia. His neonatal history was unremarkable. No syndromic association was noted excepting for telecanthus. The presence of bony atresia was confirmed on computed tomography (CT) scan and transnasal endoscopic surgery was used to obtain a patent airway. This case is a rare report of bilateral choanal atresia presenting for the first time in adult life.

Although transnasal techniques to treat choanal atresia have become the standard, risks remain of restenosis of the opened choana requiring dilatations. In addition, a new technique without stenting and requiring less invasive post-operative care would be an improvement. With this in mind, in a non-randomized trial, a modification of the transnasal surgical repair was used in eight patients with unilateral choanal atresia by which no stents were used. While the standard procedure consists of partial resection of the bony septum and the atretic plate, we additionally removed lower parts of the anterior wall and the floor of the sphenoid sinus, creating a new airway canal from the nasal cavity to the sphenoid into the epipharynx. Post-operative care by the patient and the surgeon was noted. The success of choanal patency was followed by nasal endoscopy and rhinomanometry with a mean post-operative follow-up time of 1.9 years. In all patients, both choanae remained patent confirmed by nasal endoscopy and rhinomanometry showing almost symmetric resistance. Post-operative care comprised antibiotic prophylaxis and nasal douching with saline solution by the patient; no further treatment by the surgeon was necessary. According to these long-term results we would recommend this type of surgery for two reasons: the use of stents no longer necessary and post-operative care by the surgeon can be minimized.

The genetic aspects of choanal atresia have not been clearly defined though it is probably a multifactorial trait as in cleft lip and cleft palate. The appearance of the condition in both single and successive generations supports this contention.

Choanal atresia can occur as an isolated anomaly, but is more commonly associated with one or more concomitant congenital anomalies. In this report two sisters both in their teens presented with unilateral choanal atresia as an isolated anomaly. Endoscopic trans-nasal repair of choanal atresia was performed in both of them as this offers excellent visualisation and access.

Five patients with choanal atresia were treated, using a trans-nasal endoscopic Holmium:YAG laser. The age of patients ranged from 15 months to 53 years. The nasal endoscope provides excellent visualization. The advantage of the Holmium:YAG laser is its fibre-optic delivery, allowing the laser to be hand-held, giving greater precision. Good nasal airways were achieved in four out of five cases with a single treatment. The procedure was found to be safe, quick, with minimal morbidity and a shorter in-patient stay.

Craniometaphyseal and craniodiaphyseal dysplasia are rare genetic disorders of bone due to modelling errors of long bones and skull bones. These syndromes present with multiple ENT symptomatology from an early age. The diagnostic distinction can now be made radiologically by serial skeletal survey which is important for prognosis. We review the clinical, radiological, computed tomography (CT) scan, otological, audiological and histopathological findings in two cases with craniodiaphyseal, and two cases with craniometaphyseal dysplasia, and report our experiences of medical and surgical treatment to date.

In the craniodiaphyseal dysplasia, the hearing abnormality progressed from an initial conductive to a mixed loss on serial audiometric follow up. Temporal bone CT scans showed narrowing of the middle ear cavity, internal auditory meatus, and facial nerve canal at the geniculate ganglion. Benefits from choanal stenosis surgery, craniofacial remodelling and dacrocystorhinostomy were shortlived. Calcitriol therapy with a low calcium diet did not alter the clinical course of progression in our cases. The underlying defect, causing net bone formation in these phenotypically similar syndromes, appears to be different when based on the differing biochemical responses to calcitriol and bone biopsy findings. Increased numbers of osteoblasts were found in bone biopsies from both cases with craniodiaphyseal dysplasia.

Early recognition is crucial in these conditions as therapy directed at the underlying bony defect has the best chance of success if initiated in infancy (Cole et al., 1988; Fanconi et al., 1988; Key et al., 1988).

We report two cases of unilateral choanal atresia and one of choanal stenosis treated successfully by endoscopic fenestration, with no recurrence at a mean follow-up of 18 months. Rigid endoscopy and axial CT scanning confirm the clinical diagnosis and this technique avoids the need for stenting or prolonged postoperative stay.

The use of the rigid endoscope in the management of posterior choanal atresia represents a significant advancement in choanal surgery. It provides an extremely sharp image with a magnified overview. It enables the surgeon to see the tips of his instruments, so that the bone is removed safely under direct endoscopic vision. It allows assessment of the size of the opening, in comparison to the normal choana. It ensures greater precision in flap preservation. The technique is short in time and safe, with early recovery and short hospitalization.