Late detection of cleft palate.

Cleft palate only (CPO) is a common congenital malformation, and most patients are diagnosed within the first weeks after birth. Late diagnosis of the cleft palate (CP) could initially result in feeding and growth impairment, and subsequently speech and hearing problems later in life. The purpose of this study is to retrospectively investigate (1) at which age CPO is diagnosed and (2) how the presence of syndromes and other factors relate to the age at diagnosis. The mean age of all children at our centre with CPO included between 1997 and 2014 at diagnosis (n = 271) was 1 year and 4 months. In all, 24.8% (n = 67) was older than 12 months when diagnosed, and 37.3% (n = 101) of all children had been diagnosed >30 days. These findings remain valid when a cut-off point of 14 days is used (44.3% late). Moreover, the grade of the cleft was a determining factor for successful diagnosis; submucous clefts were detected much later on average (89.3% > 30 days; p = .000). Similar results were found using Kaplan-Meier survival analyses. CONCLUSION: CPO is often diagnosed late. Patients diagnosed ≤30 days after birth more often presented with an associated disorder. Early diagnoses became more frequent as the severity of the cleft increased (grades 1-4). Professionals should perform more thorough intra-oral investigations, including manual palpations and visual inspections of the palate; they should be made more aware of the frequent accompanying symptoms. WHAT IS KNOWN: The presence of cleft palate only (CPO) is known to negatively affect feeding, hearing, speech and (social) development. Submucous clefts are often underdiagnosed due to their difficulty to detect. As far as we know the literature shows that symptomatic submucous CPs are often diagnosed at an average age of 4.9 years. WHAT IS NEW: 37.3% respectively of all children with CPO were diagnosed relatively late (>30 days after birth), 24.8% was older than 12 months when diagnosed. Mean age of all children with CPO was 1 year and 4 months. We conclude that midwives and pediatricians should perform more through intra-oral investigations of all new-borns, including both a manual palpation, als well a visual inspection of the palate.

Awake Flexible Fiberoptic Laryngoscopy to diagnose glossoptosis in Robin Sequence patients.

BACKGROUND: Robin Sequence (RS) is usually defined as the combination of micrognathia, glossoptosis and upper airway obstruction. No objective criteria to diagnose RS exist. To compare management strategy results, a single RS definition using objective criteria is needed. The most frequently used primary diagnostic tool for glossoptosis is awake Flexible Fiberoptic Laryngoscopy (aFFL). OBJECTIVES: To determine the reliability of the aFFL videos as an independent diagnostic tool itself, rather than on the complete evaluation of a patient. DESIGN, SETTING, PARTICIPANTS: All RS individuals from an existing cohort with an available aFFL video were included retrospectively. Thirty age-matched patients without pathologic findings on aFFL were used as controls. aFFL videos were scored by six otolaryngologists as: a. Marked glossoptosis, b. Mild glossoptosis, c. Severity unknown, d. No glossoptosis, e. Insufficient video quality. Videos were anonymised and rated twice, in altered sequences, after a washout period of minimally 2 weeks. MAIN OUTCOME MEASURES: Inter-rater and intrarater agreement. RESULTS: Twenty-six videos of 16 RS patients and 30 videos of controls were included. Inter-rater agreement was fair in the whole group (κ: 0.320) and RS group (κ: 0.226), and fair to moderate in determining presence of glossoptosis (total group κ: 0.430; RS κ: 0.302; controls κ: 0.212). The intrarater agreement for the presence of glossoptosis in RS was moderate (κ: 0.541). CONCLUSIONS: aFFL offers fair to moderate inter-rater agreement, with moderate intrarater agreement, in evaluating glossoptosis in RS. Using aFFL as the single tool in choosing management strategies in RS seems insufficient. There is need for a more reliable, patient friendly diagnostic tool or an internationally accepted aFFL scoring system, to diagnose glossoptosis in RS.

Improved depiction of pterygopalatine fossa anatomy using ultrahigh-resolution magnetic resonance imaging at 7 tesla.

PURPOSE: To study the anatomy of the pterygopalatine fossa (PPF) using ultrahigh-resolution magnetic resonance imaging. METHODS: A human cadaveric tissue block containing the pterygopalatine fossa was examined on a clinical 7-Tesla magnetic resonance imaging system. Subsequently, cryosections of the tissue block were created in a coronal plane. The cryosections were photographed and collected on adhesive tape. The on-tape sections were stained for Mallory-Cason, in order to detail the anatomic structures within the fossa. Magnetic resonance images were compared with surface photos of the tissue block and on-tape sections. RESULTS: High-resolution magnetic resonance images demonstrated the common macroscopic structures in the PPF. Smaller structures, best viewed at the level of the operation microscope, which have previously been obscured on magnetic resonance imaging, could be depicted. Some of the orbital pterygopalatine ganglion branches and the pharyngeal nerve were clearly viewed. CONCLUSIONS: In our experience with one human cadaver specimen, magnetic resonance imaging at 7 Tesla seems effective in depicting pterygopalatine fossa anatomy and provides previously unseen details through its demonstration of the pharyngeal nerve and the orbital pterygopalatine ganglion branches. The true viability of depicting the pterygopalatine fossa with ultrahigh-resolution MR will depend on confirmation of our results in larger studies.

Sluder's neuralgia: a trigeminal autonomic cephalalgia?

The objective was to formulate distinctive criteria to substantiate our opinion that Sluder's neuralgia and cluster headache are two different clinical entities. A systematic review was carried out of all available, original literature on Sluder's neuralgia. Pain characteristics, periodicity and associated signs and symptoms were studied and listed according to frequency of appearance. Eleven articles on Sluder's neuralgia were evaluated. Several differences between Sluder's neuralgia and cluster headache became evident. Based on described symptoms, new criteria for Sluder's neuralgia could be formulated. Sluder's neuralgia and cluster headache could possibly be regarded as two different headache syndromes, and Sluder's neuralgia could be a trigeminal autonomic cephalalgia.

Hearing sensitivity in adults with a unilateral cleft lip and palate after two-stage palatoplasty.

OBJECTIVE: To evaluate long-term hearing and middle ear status in patients treated for a unilateral complete cleft lip and palate (UCLP) by two-stage palatoplasty. METHODS: Forty-nine UCLP patients aged 17 years and older were included in this retrospective study. Patients were invited for a multidisciplinary long-term follow-up of their treatment at a tertiary center for craniofacial surgery in the Netherlands. ENT assessment included tympanometry and pure-tone audiometry. Medical files were searched for medical and surgical history. RESULTS: In total, 19.4% of the patients had significant long-term hearing loss (PTA > 20 dB), comprising conductive hearing loss in 21.5% of the patients. In the majority (70%), this hearing loss was more pronounced at higher frequencies. In 25% the high fletcher index showed hearing thresholds above 20 dB. Ventilation tubes were placed at least once in 78.7% of the patients. The frequency of tube insertion was positively correlated with the incidence of reduced tympanic compliance (tympanogram type B) and the need for a pharyngoplasty. CONCLUSION: The present study reports long-term hearing outcomes in UCLP patients with hard palate closure at 3 years of age. Persistent hearing loss was observed in 19.4% of our patients (PTA > 20 dB), mainly at the higher frequencies. Ventilation tube placement was associated with reduced tympanic compliance and higher risk on hearing loss (PTA air conduction). Our high incidence of velopharyngeal incompetence during early childhood, which is likely associated with Eustachian tube dysfunction, might have contributed to these results.