Obstructive sleep apnoea in Treacher Collins syndrome: prevalence, severity and cause.

This cohort study in 35 patients (13 children) evaluates the prevalence, severity and anatomical cause of obstructive sleep apnoea syndrome (OSAS) in patients with Treacher Collins syndrome. Ambulatory polysomnography was performed cross-sectionally to determine OSAS prevalence and severity. All upper airway related surgical interventions were evaluated retrospectively. In 11 patients, sleep endoscopy, and flexible and rigid endoscopy were applied to determine the level of anatomical obstruction of the upper airway. The overall prevalence of OSAS in Treacher Collins patients was 46% (54% in children; 41% in adults). Thirty-eight upper airway related surgical interventions were performed in 17 patients. Examination of the upper airway revealed various anatomical levels of obstruction, from the nasal septum to the trachea. Most significant obstruction was found at the level of the oro/hypopharynx. OSAS in Treacher Collins patients is an important problem so all patients should be screened for OSAS by polysomnography. Endoscopy of the upper airways was helpful in determining the level of obstruction. Surgical treatment at one level will not resolve OSAS in most patients because OSAS in Treacher Collins has a multilevel origin. Non-invasive ventilation (continuous positive airway pressure or bilevel positive airway pressure) or tracheotomy should be considered as a treatment modality.

Obstructive sleep apnea in children with syndromic craniosynostosis: long-term respiratory outcome of midface advancement.

Almost 50% of patients with Apert, Crouzon or Pfeiffer syndrome develop obstructive sleep apnea (OSA), mainly due to midface hypoplasia. Midface advancement is often the treatment of choice, but the few papers on long-term outcome report mixed results. This paper aimed to assess the long-term respiratory outcome of midface advancement in syndromic craniosynostosis with OSA and to determine factors contributing to its efficacy. A retrospective study was performed on 11 patients with moderate or severe OSA, requiring oxygen, continuous positive airway pressure (CPAP), or tracheostomy. Clinical symptoms, results of polysomnography, endoscopy and digital volume measurement of the upper airways on CT scan before and after midface advancement were reviewed. Midface advancement had a good respiratory outcome in the short term in 6 patients and was ineffective in 5. In all patients without respiratory effect or with relapse, endoscopy showed obstruction of the rhino- or hypopharynx. The volume measurements supported the clinical and endoscopic outcome. Despite midface advancement, long-term dependence on, or indication for, CPAP or tracheostomy was maintained in 5 of 11 patients. Pharyngeal collapse appeared to play a role in OSA. Endoscopy before midface advancement is recommended to identify airway obstruction that may interfere with respiratory improvement after midface advancement.

Sleep-related breathing disorders in prepubertal children with Prader-Willi syndrome and effects of growth hormone treatment.

CONTEXT: Recently, several cases of sudden death in GH-treated and non-GH-treated, mainly young Prader-Willi syndrome (PWS), patients were reported. GH treatment in PWS results in a remarkable growth response and an improvement of body composition and muscle strength. Data concerning effects on respiratory parameters, are however, limited. OBJECTIVE: The objective of the study was to evaluate effects of GH on respiratory parameters in prepubertal PWS children. DESIGN: Polysomnography was performed before GH in 53 children and repeated after 6 months of GH treatment in 35 of them. PATIENTS: Fifty-three prepubertal PWS children (30 boys), with median (interquartile range) age of 5.4 (2.1-7.2) yr and body mass index of +1.0 sd score (-0.1-1.7). INTERVENTION: Intervention included treatment with GH 1 mg/m2.d. RESULTS: Apnea hypopnea index (AHI) was 5.1 per hour (2.8-8.7) (normal 0-1 per hour). Of these, 2.8 per hour (1.5-5.4) were central apneas and the rest mainly hypopneas. Duration of apneas was 15.0 sec (13.0-28.0). AHI did not correlate with age and body mass index, but central apneas decreased with age (r = -0.34, P = 0.01). During 6 months of GH treatment, AHI did not significantly change from 4.8 (2.6-7.9) at baseline to 4.0 (2.7-6.2; P = 0.36). One patient died unexpectedly during a mild upper respiratory tract infection, although he had a nearly normal polysomnography. CONCLUSIONS: PWS children have a high AHI, mainly due to central apneas. Six months of GH treatment does not aggravate the sleep-related breathing disorders in young PWS children. Our study also shows that monitoring during upper respiratory tract infection in PWS children should be considered.

Management of stridor in neonates and infants.

Stridor is the sound caused by abnormal air passage into the lungs and can exist in different degrees and be caused by obstruction located anywhere in the extra-thoracic (nose, pharynx, larynx, trachea) or intra-thoracic airway (tracheobronchial tree). Stridor may be congenital or acquired, acute, intermittent or chronic. Laryngotracheal inflammation (croup) is the most common cause of acute stridor. Laryngotracheomalacia is the most common cause of congenital, chronic stridor. Stridor is a clinical sign and not a diagnosis. The golden standard in the workup of stridor is an upper and lower airway endoscopy under general anaesthesia. Endoscopic examination under general anaesthesia requires a multidisciplinary approach and close cooperation between anaesthesiologist, paediatrician, ENT surgeon and nursing staff. Following this procedure, a place in the intensive care unit should be available for those cases presenting with stridor in which a definite diagnosis could not yet be established. Although important, pre-endoscopy assessment including history, physical examination and radiological examination, is only a guide to the type and degree of pathology found during endoscopy. About 1 out of 10 infants are found to have lesions in more than one anatomical site of the upper aerodigestive tract.

Management of obstructive sleep apnea syndrome in children with craniofacial malformation.

Children with craniofacial syndromes, especially those with midfacial hypoplasia, micrognathia, or deformation of the cranial base, are frequently suffering from obstructive sleep apnea syndrome (OSAS). It is important to recognize this condition. Diagnostic methods and therapeutic developments are discussed. Experience with 31 patients in the Sophia Children's Hospital is presented. The majority of these infants suffered from moderate or severe OSAS. Treatment varied from symptomatic (e.g. continuous positive airway pressure) to curative. These therapies could often prevent a tracheotomy. Still more curative treatment options are needed.

Management of subglottic haemangioma.

Two contrasting methods of treatment for paediatric subglottic haemangioma are presented. Dr Hoeve describes his results using intralesional steroids and short-term intubation, and Dr Froehlich discusses his outcomes with open surgical excision. Mr Bailey then reviews the currently available therapeutic options with special reference to each of the above techniques, and suggests an optimum plan of management given our current state of knowledge. The conclusion is that very small haemangiomas may not require treatment, or may be amenable to CO2 laser vaporization. Medium-sized lesions seem suitable for intra-lesional steroids and intubation, but large ones are probably best managed by primary submucous resection. Very large haemangiomas, and especially those which are circumferential or in which magnetic resonance imaging (MRI) shows extension down into the trachea and/or through the tracheal wall into the surrounding tissues, may be more safely dealt with by performing a tracheostomy and awaiting spontaneous involution.

Malformation and stenosis of the cricoid cartilage in association with Larsen's syndrome.

Three patients with the typical features of Larsen's syndrome are described. All three developed severe respiratory symptoms caused by a congenital subglottic stenosis. Tracheotomy and treatment of the stenosis by means of laryngotracheoplasty resulted in complete collapse of the cricoid cartilage and the proximal tracheal skeleton. Lack of rigidity of the laryngeal and tracheal cartilages in patients with Larsen's syndrome could well be responsible for this failure. Surgical treatment consisted of resection of the stenotic and collapsed areas and end-to-end anastomosis. This therapy was eventually successful in all three patients.

"Tonight something special is happening in this neighbourhood." Report on experimental distribution of condoms to clients of prostitutes.

PIP: The incidence of sexually transmitted diseases (STDs) among prostitutes and their clients is relatively high in the Netherlands, although condoms have been known for a long time in prostitution. As part of the 1987 national condom campaign the STD Foundation developed public education activities targeting prostitutes, their partners, clients, and procurers trying to convince them about safer sexual contact to prevent STDs. Members of the working group Client and Prostitution (KLEP) offered their cooperation. A total of 18 condom distributions were carried out between January-November 1988. About 5000 clients of prostitutes were approached in the redlight districts of 9 towns and cities after members of the KLEP personally had informed prostitutes and procurers by means of leaflets in 5 languages about the purpose of the campaign and guidelines had been drawn up about distribution. Positive remarks were often heard, however, there were also those who ignored the distributors. Some of the positive results included the prostitutes' wish for safer sex and that the procurers frequently handed out condoms to clients in clubs and private houses. A follow-up project in preparation envisions reaching clients after contact with the prostitute, organization of local discussion evenings after condom distribution, and the provision of telephone numbers and addresses. The distribution of condoms frequently prompted prostitutes to inquire about STD/AIDS prevention, thus local and regional organizations might consider launching prevention activities. In November 1989 a report containing these recommendations was to be presented to representatives of local and regional organizations in localities where condom distribution had taken place.^ieng

Dietary zinc deficiency has no effect on auditory brainstem responses in the rat.

Zinc has been shown to effect--in vitro--a number of processes associated with neurotransmission. We have tested whether the rate of impulse conduction--in vivo--as measured from the latencies of auditory brainstem responses (ABR), is influenced by dietary zinc deficiency in the rat. Dietary zinc deficiency for up to 26 wk had no effect on the wave I-IV interval compared to zinc-adequate fed animals. The results are discussed in relation to the observed constancy of brain overall and extracellular fluid zinc concentrations under conditions of dietary zinc deficiency.

Sinusitis: hidden source of sepsis in postoperative pediatric intensive care patients.

Paranasal sinusitis is reported as a complication of prolonged nasal intubation and the source of sepsis in adult intensive care patients. In surgical neonates with congenital malformations, prolonged intubation with a nasotracheal (NT) or NG tube is often necessary, but sinusitis with complicating sepsis is seldom reported. Sinus x-rays may confirm the diagnosis; in infancy, prolonged nasal intubation delays the pneumatization of the sinuses and the mastoids, resulting in additional diagnostic problems. In a 1-yr period, we saw three patients with multiple septic episodes in which the source of sepsis was undetectable. Despite the absence of clinical symptoms and radiologic evidence of sinusitis or mastoiditis, surgical drainage revealed pus and led to the disappearance of septic episodes and ear, nose, and throat problems. There is an association between prolonged NT and NG intubation, and sinusitis or mastoiditis as an unrecognized source of sepsis in young infants. Absence of radiologic evidence of sinusitis or mastoiditis causes pitfalls in diagnosis and is related to delayed pneumatization of the sinuses and the mastoid in prolonged nasal intubation in young infants.