Ignazio La Mantia

Holoprosencephaly (lobar form) associated with bilateral vocal cord palsy

There have been many causes associated with bilateral vocal cord palsy, both congenital and perinatal. Until now, the main congenital causes of bilateral vocal cord palsy have been associated with meningomyelocele, Arnold-Chiari malformation, and hydrocephalus. We report a patient with bilateral vocal cord palsy associated with the lobar form of holoprosencephaly. In this case, bilateral vocal cord palsy was caused by the disruption of the cortical laryngeal motoneurons. Neonatal stridor must be carefully evaluated by the neonatologist to exclude severe cerebral anomalies. (J Child Neurol 2001;16:932-934).

Effectiveness of Intranasal Sodium Hyaluronate in Mitigating Adverse Effects of Nasal Continuous Positive Airway Pressure Therapy

Background Nasal continuous positive airway pressure (CPAP) in moderate-to-severe cases of obstructive sleep apnea can cause nasal discomfort and other undesirable problems. Objective The aim of our study was to test the effects of sodium hyaluronate on nasal problems that patients experienced in their daily lives, sleepiness, nasal resistance to airflow, nasal mucociliary clearance, changes in inflammatory markers, and compliance to CPAP in three groups of patients with obstructive sleep apnea syndrome on CPAP therapy. Methods A total of 102 patients with a confirmed diagnosis of obstructive sleep apnea syndrome (apnea-hypopnea index [AHI] of ≥10/hour) were randomized into three treatment groups: (1) hyaluronate plus CPAP, (2) saline solution plus CPAP, and (3) CPAP-only groups. Outcome measures were the extent of improvement in the Mini Rhinoconjunctivitis Quality of Life Questionnaire (MiniRQLQ) mean scores, sleepiness, nasal resistance to airflow, nasal mucociliary clearance, changes in inflammatory markers, and compliance to CPAP. Significant improvement in each outcome measure was determined by comparing scores at baseline and after 4 weeks for each treatment group. Results Significant improvement in the MiniRQLQ overall mean score was observed both in the hyaluronate plus CPAP (p <0.00001) and saline solution plus CPAP groups (p <0.01), although the hyaluronate plus CPAP group had better improvement compared with the saline solution plus CPAP group (0.24 versus 0.12, respectively). An increase in nasal inflammatory markers and saccharin transit test score was observed in all three groups, although it was statistically lower in the hyaluronate plus CPAP group (all p <0.001). Conclusion Intranasal hyaluronate showed significant benefits in patients who received CPAP therapy, but future studies over a longer period of time after treatment should be performed to corroborate our findings.

An Unusual Headache: Red Ear Syndrome

A 4-year-old boy presented with a 2-year history of unilateral recurrent ear erythema (generally on the left ear) that was associated with episodic ear swelling, discomfort, and a burning sensation (Figure 1). These episodes occurred up to three times every month; each episode lasted for approximately 1 h and spontaneously resolved. Initially, the episodes were isolated, but during the last 6 months, they began to be associated with a migraine without aura simultaneous to ear redness. In the interval between two episodes, the patient had no problem. His perinatal history and childhood development were reportedly normal. Visual inspection results of the pinna and otoscopic examination results were bilaterally normal. The head and neck examination and allergological assessment were within normal limits as well as laboratory tests and a magnetic resonance imaging (MRI) of the brain. A neurological visit led to a diagnosis of idiopathic red ear syndrome (RES) being made. The patient started treatment with cetirizine for 2 weeks and showed a slight reduction in the frequency of attacks.

Chronic kidney disease in children: Assessment of oral health status

BACKGROUND Oral disease may be more prevalent in people with chronic kidney disease (CKD) due to the underlying pathology and its treatment. In children, it can elicit a wide spectrum of oral manifestations, including saliva changes, mucosal lesions, oral infection, gingivitis, and dental anomalies (mostly hypoplasia). OBJECTIVES The aim of the study was to determine the oral health status in a group of children with CKD and to compare with that from healthy controls. MATERIAL AND METHODS A clinical cross-sectional study was conducted between June 2016 and September 2017 on 126 pediatric patients. Oral findings in a group of 65 children suffering from CKD were compared with a control group consisting of 61 individuals, free of any disease. We obtained an assessment of the oral health status by collecting carious, debris, calculus, gingival conditions, hypoplasia, and salivary flow rate data by using specific indexes. Medical and dental history was obtained for each subject. RESULTS We found that children with CKD have a lower prevalence of caries for both permanent dentition (p = 0.019) and primary dentition (p = 0.008), while the prevalence of calculus, debris, gingivitis and enamel hypoplasia seems to be higher (all p < 0.005). A significantly reduced salivary flow rate was also noted in the CKD children, both in unstimulated (p = 0.037) and stimulated (p = 0.026) conditions. CONCLUSIONS CKD pediatric patients are more likely to present oral and dental changes than healthy children, so proper oral care and preventive measures should be taken to avoid potentially severe dental problems.

Cobblestone Appearance of the Nasopharyngeal Mucosa

A 27-year-old male presented with a 2-year history of paroxysmal sneezing and nasal congestion, which mostly occurred during April to June. Flexible fiberoptic rhinoscopy revealed slight hypertrophy of both inferior turbinates, with a normal pinkish hue and a marked cobblestone appearance of the nasopharyngeal mucosa, particularly at the torus tubarius and superior segment of the fossa of Rosenmuller (Figure 1). With a positive skin prick test result, a diagnosis of allergic rhinitis was made. The patient was underwent sublingual allergen-specific immunotherapy that was combined with the administration of antihistamines and an intranasal steroid at the start of treatment. At follow-up, 6 months after the start of treatment, the patient reported marked relief from his allergic rhinitis symptoms, and on performing flexible fiberoptic rhinoscopy, he did not show signs of cobblestone mucosa.