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.

Localized immunoglobulin light chain amyloidosis: Novel insights including prognostic factors for local progression.

In localized light chain amyloidosis (locAL), amyloidogenic light chains (aLC) are produced and deposited locally by a B-cell clone. We present 293 patients with immunohistochemically confirmed locAL. Lung (nodular pulmonary) with 63 patients was the most involved organ. The aLC was λ in 217 cases (κ:λ ratio 1:3). A local B-cell clone was identified in 30% of cases. Sixty-one (21%) had a concomitant autoimmune disorder (cAD). A monoclonal component (MC) were present in 101 (34%) patients and were more frequent in subjects with cAD (51% vs 34%; P = .03). Cigarette smoking was more prevalent in lung locAL (54% vs 37%; P = .018). After a median follow-up of 44 months, 16 patients died and 5- and 10-years locAL progression-free survival (PFS) were 62% and 44%. Interestingly, locAL-PFS was shorter among patients with an identified clonal infiltrate at amyloid deposition site (40 vs 109 months; P = .02) and multinuclear giant cells and/or an inflammatory infiltrate resulted in longer locAL-PFS in lung involvement (65 vs 42 months; P = .01). However, no differences in locAL PFS were observed in patients with cAD, a MC and involved organ site. Treatment was administered in 163 (54%) patients and was surgical in 135 (46%). Median locAL-PFS after first treatment was 56 months. Responders had longer locAL-PFS (78 vs 17 months; P < .001). Three patients with lung locAL and a MC were diagnosed as systemic AL amyloidosis at follow-up. In summary, locAL pathogenesis seems to be heterogeneous and the clonal infiltrate leads local progression.

Factors influencing time-dependent decannulation after pediatric tracheostomy according to the Kaplan-Meier method.

PURPOSE: The aim of this study was to determine whether there were differences in decannulation rates and time to decannulation in children depending on the indication for tracheostomy, age, and maturity at birth. STUDY DESIGN: Retrospective chart review and prospective interview by questionnaire. METHODS: The medical records of 106 pediatric patients (age 0-18 years) tracheostomized between January 1 1999 and January 1 2019 were reviewed. Patients were divided into three different groups depending on the indication for tracheostomy: unsafe airway (37.7%), long-term respiratory dependence (50.9%), or bronchopulmonary toilet for aspirations (11.3%). RESULTS: 40 patients were successfully decannulated. The time-dependent decannulation rate after 2 and 5 years was 28.3% and 40.5% for patients with an unsafe airway, 42.4% and 66.8% for patients with long-term respiratory dependence, and 41.7% and 70.8% for patients needing bronchopulmonary toilet, respectively. After 2 and 5 years, patients aged 0-12 months at the time of tracheostomy were decannulated in 13.1% and 50.2% of cases, 1-5-year-olds in 35.3% and 48.2% of cases, 6-10-year-olds in 70% and 70% of cases, and 11-18-year-olds in 66.6% and 66.6% of cases, respectively. However, in a multivariate analysis, prematurity was found to be the only significant unfavorable variable (p = 0.013). Maturely born patients had an odds ratio of 3.87 (95% CI 1.32-11.33) for successful decannulation. This effect was present only in the first 5 years of life. CONCLUSION: Factors indicating problems with decannulation are an unsafe airway, a young age at the time of tracheostomy, and prematurity at birth.

Transnasal flexible esophagoscopy (TNE): an evaluation of the patient's experience and time management.

The aim of this study was to evaluate patient's experience when performing transnasal flexible endoscopy using EndoSheath Technology without sedation in an ENT outpatient department. Patients were seen at the laryngological clinic of the Department of Otorhinolaryngology, Head and Neck Surgery, University Hospital Heidelberg, presenting with complaints of reflux like throat cleaning, persistent cough, globus sensation, heartburn, or voice problems. First, we performed stroboscopy. In cases where physical examination findings revealed the presence of LPR, we performed a transnasal flexible esophagoscopy (TNE) using sterile EndoSheath Technology under local anesthesia. 55 patients were investigated and completed a questionnaire on subjective discomfort that they felt during the procedure. The different steps of the examination were assessed separately. Complications were noted down by the surgeon. All patients underwent a complete examination of the upper aerodigestive tract. The time needed for preparation, examination and cleaning measures was recorded as well. The average preparation time for each examination was 24 min. No complications were observed during the procedure. The procedure was well tolerated by all patients and was classified on average as "low-grade unpleasant". In summary, TNE is a safe, quick and well-tolerated procedure that can be performed in a regular examination room under local anesthesia without sedation.

Immunosuppressive effect of tryptophan metabolites in composite tissue allotransplantation.

BACKGROUND: Hand transplantations have intensified immunological research into composite tissue allotransplantation to induce tolerance. Pregnancy is a successful, natural model of immunological tolerance. The enzyme indoleamine 2,3-deoxygenase plays an important role by catabolizing the amino acid tryptophan. The resulting metabolites have been shown to be immunosuppressive. The effect of tryptophan metabolites has not been investigated in vascularized organ transplantation before. In this study, the authors applied to composite tissue allotransplantation what nature has developed for pregnancy, and examined the immunosuppressive effect of tryptophan metabolites in a model of hind limb transplantation. METHODS: Thirty-three allogeneic hind limb transplantations in the rat (Lewis --> Brown-Norway) were performed in three groups. Group A (n = 12) received no immunosuppression, group B (n = 13) received tryptophan metabolites (kynurenine and 3-hydroxyanthranilic acid) locally and systemically, and group C (n = 8) served as a control group receiving FK506. The timing of rejection was assessed by clinical observation. RESULTS: Rejection of the allogeneic hind limb occurred on average 6.58 days after transplantation in group A (no immunosuppression) and after 8.15 days in group B (tryptophan metabolites). Rejection was significantly delayed (log-rank test, p < 0.01). No rejection was seen with application of FK506 during the follow-up period of 21 days. CONCLUSIONS: For the first time, tryptophan metabolites have been applied in vascularized composite tissue allotransplantation and showed a significant immunosuppressive effect. These promising first results need further dose-effect and toxicological studies to increase the still limited immunosuppressive effect and define the clinical role these metabolites may play in the future.