Recurrent laryngeal lymphangiomatosis in a patient with Proteus syndrome: A case report.

Proteus syndrome is a rare genetic disease characterized by an asymmetrical growth of individual parts of the body and has only been described in single cases. This patient presented with recurrent manifestations of a laryngeal and hypopharyngeal lymphangioma, which were treated with laser surgery, systemic therapy, and sclerotherapy. The reported data depict the diagnosis and treatment in the department of otorhinolaryngology, head and neck surgery of the university hospital Heidelberg from 2019 until May 2023. The recurrent endoscopy of the upper airway was performed using a flexible HD-endoscope and the Visera Elite video tower from Olympus, Hamburg. The 29-year old female patient initially presented in February 2019 with stridor and exertional dyspnea due to a lymphatic malformation of the left larynx and hypopharynx. In April 2019 there was no improvement by sclerotherapy with Picibanil, so that systemic therapy with the PIK3CA inhibitor alpelisib was initiated (03-07/2020) and discontinued due to a high side effect profile. In the course of 2021-2023, three microlaryngoscopies with laser surgical resection and renewed sclerotherapy of the lymphangioma with Picibanil were carried out due to fluctuating findings. After these interventions a stable disease could be established until May 2023. Laser surgical therapy is currently described as the therapy of choice in lymphangiomas in the head and neck region and also showed the highest effectiveness in our patient. In case of airway obstruction in particular, it can bring rapid symptom relief. Alternatively, and with a lower surgical risk, local improvements have been reported by sclerotherapy, which was less effective in the presented case. Rare syndromic diseases require multidisciplinary collaboration. In the case of laryngeal lymphangiomatosis, other treatment options should be considered in addition to surgical excision, especially in the case of recurrence.

[The complex pediatric middle ear hemangioma]. / Das komplizierte kindliche Mittelohrhämangiom.

Hemangiomas in the pediatric middle ear are a rare occurrence. We present the case of a 3-year-old boy with unilateral hearing impairment and recurrent otitis media with effusion due to a hemangioma in the middle ear. This caused a venous sinus thrombosis, osteomyelitis, and mastoiditis. Interdisciplinary treatment was performed. This case shows that even benign lesions in the middle ear can cause severe, life-threatening complications due to the close proximity of important anatomic structures in the middle ear. Every case of asymmetric hearing loss should thus be examined in detail.

Auditory processing in children with language-based learning problems: a magnetencephalography study.

We examined basic auditory temporal processing in children with language-based learning problems (LPs) applying magnetencephalography. Auditory-evoked fields of 43 children (27 LP, 16 controls) were recorded while passively listening to 100-ms white noise bursts with temporal gaps of 3, 6, 10 and 30 ms inserted after 5 or 50 ms. The P1m was evaluated by spatio-temporal source analysis. Psychophysical gap-detection thresholds were obtained for the same participants. Thirty-two percent of the LP children were not able to perform the early gap psychoacoustic task. In addition, LP children displayed a significant delay of the P1m during the early gap task. These findings provide evidence for a diminished neuronal representation of short auditory stimuli in the primary auditory cortex of LP children.