Regional differences in admissions and surgical management of pediatric nontuberculous mycobacterial cervicofacial lymphadenitis.

OBJECTIVES: This study aims to determine the overall incidence of medical and surgical admissions related to non-tuberculous mycobacterial cervicofacial lymphadenitis (NTMCL) and determine if rates vary by geographic region in the US. It also aims to assess if the relative frequency of varying treatment modalities for NTMCL differ among geographic regions. STUDY DESIGN: Population-based inpatient registry analysis. SETTING: Academic medical center. METHODS: The Kids' Inpatient Database (2016 and 2019) was used to determine NTMCL-related admissions and common head and neck procedures performed during these admissions were identified. Analysis was performed on regional differences in demographic factors and procedures performed during NTMCL-related admissions. RESULTS: There were 159 weighted admissions (1.31 per 100,000) for NTMCL in 2016 and 2019 in the US, with the Midwest having the highest proportion of NTML-related admissions (1.59:100,000). NTMCL-related admissions were 2.21 times as likely to be elective rather than non-elective in the Midwest when compared to all other geographic regions (p = 0.038). The Midwest was 2.83 times as likely to treat with surgery (p = 0.011), while the Northeast was negatively associated with performing procedures (OR 0.38; p = 0.026). In the Midwest, significantly more excisional surgeries were preformed when compared to other regions, with an OR of 2.98 (p = 0.003). CONCLUSION: The Midwest had the highest incidence of pediatric NTMCL-related admissions and was more likely to perform excisional surgery as primary NTMCL treatment. Regions that rarely see pediatric NTMCL have a more inconsistent approach to management.

Tonsillotomy for Periodic Fever Syndrome: A Randomized and Controlled Trial.

OBJECTIVE: Tonsillectomy is an effective treatment for periodic fever, aphthous stomatitis, pharyngitis, and cervical adenitis (PFAPA) syndrome. Tonsillotomy has a milder operative risk profile and postoperative morbidity in children than tonsillectomy. We aimed to compare the efficacy of tonsillotomy to observation-only in children with PFAPA syndrome at a 3-month follow-up. METHODS: This was a randomized multicenter trial with sequential design. Participants were randomized into a tonsillotomy group and a control group that was only observed. The trial started in 1/2017 and was accomplished in 12/2021 with 16 patients (10 boys, six girls, the mean age 4.2 years). The symptoms were monitored with daily symptom diaries. RESULTS: After the 3-month follow-up, 7/8 patients (87.5%) in the tonsillotomy group and 2/8 (25%) patients in the control group were free from PFAPA symptoms (95% CI 13% to 87%; p = 0.0021). The mean number of days with fever was 2.6 (SD 3.7) in the tonsillotomy group and 8.0 (SD 6.5) days in the control group (n = 8) (p = 0.06). Mean number of fever days compatible with PFAPA syndrome was 0.8 (SD 1.4) in the tonsillotomy group and 6.5 (SD 6.0) in the control group (95%CI -10% to -1%; p = 0.007). Rescue tonsillectomy was needed for all patients in the control group and none of the patients in the tonsillotomy group. CONCLUSIONS: Tonsillotomy might be an effective treatment option for children with PFAPA syndrome. Further studies are needed to clarify the long-term efficacy of tonsillotomy for treating PFAPA. LEVEL OF EVIDENCE: 2 Laryngoscope, 134:968-972, 2024.

A comparison of ultrasound-guided aspiration with open surgical drainage in the management of pediatric acute suppurative cervical lymphadenitis.

OBJECTIVES: Image-guided aspiration has been proposed as an alternative to the traditional open drainage in acute suppurative cervical lymphadenitis, although little evidence comparing these approaches exists. The aim of this study was to compare ultrasound-guided aspiration and open surgical drainage in the management of pediatric acute suppurative cervical lymphadenitis. METHODS: Patients treated for acute suppurative cervical lymphadenitis at a tertiary referral pediatric hospital over a three-year period were retrospectively analyzed. RESULTS: Of eighteen patients included, 9 (50 %) underwent aspiration and 9 (50 %) underwent open surgical drainage. None required repeat intervention or readmission over a median follow up of 10.5 weeks. Those undergoing aspiration had a shorter post-operative length of stay (1.67 vs 4.33 days, p = 0.001) compared with open surgical drainage. CONCLUSION: Ultrasound-guided aspiration of pediatric acute suppurative cervical lymphadenitis is a safe and effective alternative to open drainage.

Management of Periodic Fever, Aphthous Stomatitis, Pharyngitis, Adenitis with Intracapsular Tonsillectomy.

OBJECTIVE: This study aimed to present 2 children clinically diagnosed with periodic fever, aphthous stomatitis, pharyngitis, and adenitis (PFAPA) syndrome and treated with intracapsular tonsillectomy with adenoidectomy (ITA). METHODS: We conducted a retrospective analysis of 2 children who were referred for an otolaryngology consultation between 2019 and 2022 for surgical treatment of PFAPA syndrome. Both patients had symptoms strongly suggestive of PFAPA and were at risk for total tonsillectomy (TT) complications. ITA was performed using a microdebrider. Both patients were followed up postoperatively to assess for symptomatic resolution and complications. RESULTS: Two children exhibited recurrent febrile episodes prior to ITA. The procedure was efficacious in both patients, with neither experiencing postoperative complications or recurring PFAPA symptoms for over 1 year after surgery. CONCLUSION: Our study reported on the use of ITA as a surgical treatment option for PFAPA. We showed that ITA eliminated febrile attacks and was safely performed without postoperative complications in 2 pediatric patients after 1-year follow-up. Future studies involving larger cohorts of PFAPA patients and lengthier follow-ups will need to be conducted to further evaluate ITA as a surgical option. Laryngoscope, 134:1967-1969, 2024.

Current management of cervicofacial nontuberculous mycobacterial infections in the pediatric population.

PURPOSE OF REVIEW: The purpose of this review is to analyze and consolidate recently published literature to provide updated guidelines on the diagnosis and management of nontuberculous mycobacterial lymphadenitis (NTM LAD) in the pediatric population and to suggest areas of further research. RECENT FINDINGS: Diagnosis of NTM LAD relies on a detailed clinical history, physical examination, laboratory tests, and imaging techniques. Treatment strategies vary widely, with a shift towards complete surgical excision being observed due to its higher cure rate, improved aesthetic outcomes, and lower recurrence rates. However, patient-specific factors must be considered. The role of genetic factors, such as Mendelian susceptibility to mycobacterial disease (MSMD), is being increasingly recognized and could lead to targeted therapies. SUMMARY: Despite strides in the understanding and management of NTM LAD, substantial gaps remain in key areas such as the role of diagnostic imaging, optimal treatment parameters, postoperative care, and surveillance strategies. In this article, we explain our approach to NTM using the most relevant evidence-based medicine while offering directions for future work.

Toxoplasmic Lymphadenitis of the Head and Neck Region.

OBJECTIVE: We analyzed the clinical characteristics and treatment results in patients with a final diagnosis of toxoplasmic lymphadenitis after surgery. METHODS: A total of 23 patients with a final diagnosis of toxoplasmic lymphadenitis of the head and neck region after surgery from January 2010 to August 2022 were enrolled. RESULTS: All patients with toxoplasmic lymphadenitis presented with a neck mass and a mean age of over 40. The most common location of toxoplasma lymphadenitis in the head and neck was neck level II in 9 patients, followed by level I, level V, level III, the parotid gland, and level IV. Three patients had masses in multiple regions of the neck. Preoperative diagnosis (based on imaging tests, physical examination, and fine-needle aspiration cytology results) was benign lymph node enlargement in 11 cases, malignant lymphoma in 8 cases, metastatic carcinoma in 2 cases, and parotid tumors in 2 cases. All patients underwent surgical resection and were diagnosed with toxoplasma lymphadenitis based on the final biopsy. There were no major complications after surgery. A total of 10 patients (43.5%) received additional antibiotics after surgery. There was no recurrence of toxoplasmic lymphadenitis during the follow-up period. CONCLUSIONS: It is challenging to assess the diagnostic accuracy of preoperative examination in toxoplasma lymphadenitis; hence, surgical resection is necessary to differentiate it from other diseases.

Successful treatment with laparoscopic extended posterior sectionectomy for inflammatory pseudotumor in the liver with para-aortic lymphadenitis.

Inflammatory pseudotumor (IPT) is a rare disease that requires a differential diagnosis from malignancies. We describe a case of hepatic IPT with para-aortic lymphadenopathy, treated with a stepwise strategy of laparoscopic surgery. A 61-year-old woman was referred with a liver lesion. Computed tomography revealed a 13 cm well-defined lesion in segments VII-VI. The patient also had bead-like enlarged lymph nodes from the perihilar to the para-aortic regions. Although percutaneous lymph node biopsy showed no evidence of malignancy, 18 F-fluorodeoxyglucose positron emission tomography revealed accumulation in the lesion and lymph nodes. Lymph nodes were harvested laparoscopically for intraoperative pathological examination. With no evidence of malignancy, laparoscopic liver resection was continuously performed as a diagnostic treatment. The patient was given a pathological diagnosis of IPT and was discharged on the 16th day and is well 2 years after surgery. The minimally invasive laparoscopic approach to diagnostic treatment could be useful with secure advantages.

Tonsillectomy reduces the family impact of periodic fever, aphthous stomatitis, pharyngitis and cervical adenitis (PFAPA) syndrome and improves health-related quality of life in affected children.

BACKGROUND: Periodic fever, aphthous stomatitis, pharyngitis and cervical adenitis (PFAPA) syndrome is an autoinflammatory disorder that primarily affects young children, and typically gives rise to fever episodes that recur monthly for several years. This study investigated the impact of PFAPA syndrome on the families of affected children, the health-related quality of life (HRQOL) of children with the syndrome, and how these factors were influenced by tonsillectomy. METHODS: This prospective cohort study included 24 children with typical PFAPA syndrome that were referred for tonsillectomy, of whom 20 underwent the procedure. The control group consisted of randomly selected children from the general population. Family impact and HRQOL were measured using the standardized, validated questionnaires Pediatric Quality of Life Inventory™ (PedsQL™) Family Impact Module (FIM) and PedsQL™ 4.0 Generic Core Scales (GCS). Parents to children with PFAPA completed the questionnaires before and 6 months after their child underwent tonsillectomy, and HRQOL was measured both between and during PFAPA episodes. The Wilcoxon signed-rank test was used to compare data before and after tonsillectomy in the patient group, while the Mann-Whitney test was used for comparison of the patient and control groups. RESULTS: Before tonsillectomy, children with PFAPA had significantly lower scores than the control group on the PedsQL™ FIM and the PedsQL™ 4.0 GCS during fever episodes. After tonsillectomy, all patients improved with diminished febrile episodes, which resulted in significantly higher scores regarding both family impact and HRQOL at the time of follow-up. HRQOL of in children with PFAPA improved after tonsillectomy even when compared to afebrile intervals before the procedure. The differences between PFAPA patients and controls were eliminated after tonsillectomy. CONCLUSION: PFAPA syndrome has a profound negative impact on the families of affected children. Tonsillectomy that leads to cessation or reduction of fever episodes eases the impact of the disease on the family. HRQOL in children with PFAPA is low during febrile episodes and similar to healthy controls in between episodes. The improvement of HRQOL in patients with PFAPA after tonsillectomy compared to the afebrile intervals before tonsillectomy highlights that the constantly recurring fevers may affect the children's well-being even between fever episodes.

Long-Term Esthetic Outcome of Different Treatment Modalities for Nontuberculous Mycobacterial Cervicofacial Lymphadenitis.

BACKGROUND: Nontuberculous mycobacterial (NTM) cervicofacial lymphadenitis is a rare infection which almost exclusively occurs in children, most commonly children 0-5 years old. It can leave scars in highly visible areas. The present study aimed to evaluate the long-term esthetic outcome of different treatment modalities for NTM cervicofacial lymphadenitis. METHODS: This retrospective cohort study included 92 participants with a history of bacteriologically proven NTM cervicofacial lymphadenitis. All patients were diagnosed at least 10 years prior and were aged >12 years upon enrollment. Based on standardized photographs, the scars were assessed by subjects with the Patient Scar Assessment Scale, and by five independent observers with the revised and weighted Observer Scar Assessment Scale. RESULTS: The mean age at initial presentation was 3,9 years and the mean follow-up time was 15.24 years. Initial treatments included surgical treatment (n = 53), antibiotic treatment (n = 29) and watchful waiting (n = 10). Subsequent surgery was performed in two patients, due to a recurrence after initial surgical treatment, and in 10 patients initially treated with antibiotic treatment or watchful waiting. Esthetic outcomes were statistically significantly better with initial surgery, compared to initial non-surgical treatment, based on patient scores of scar thickness, and based on observer scores of scar thickness, surface appearance, general appearance and the revised and weighted sum score of all assessment items. CONCLUSIONS: The long-term esthetic outcome of surgical treatment was superior to non-surgical treatment. These findings could facilitate the process of shared decision making. LEVEL OF EVIDENCE: Level III.

Microbiology Culture Results and Antibiotic Prescribing in Pediatric Patients With Lymphadenitis.

BACKGROUND: Bacterial lymphadenitis is a common reason for antibiotic treatment and hospitalization in children. The literature available addressing the bacterial etiology of lymphadenitis recommends the use of narrow-spectrum agents to cover common pathogens. We suspect that patients at our institution receive unnecessarily broad-spectrum antimicrobial agents. The primary objective of this study was to characterize the microbiology and antibiotic use in lymphadenitis patients. METHODS: Retrospective review of children admitted over a 10-year period with an International Classification of Diseases Ninth or Tenth Edition code for lymphadenitis. Patients were included if they were <18 years old, admitted to the inpatient ward, and had intraoperative lymph node cultures collected. RESULTS: A total of 131 patients admitted with lymphadenitis had lymph node cultures collected and were included. Seventy-two (72/131; 55%) patients had positive lymph node culture results with pathogenic bacteria. The predominant pathogens were Staphylococcus aureus (56/72; 77.8%) and Streptococcus pyogenes (10/72; 13.9%). The most common inpatient empirical regimen was ampicillin-sulbactam. Of the 72 patients with typical pathogens identified, 80.6% were sensitive to a first-generation cephalosporin, whereas 86.1% were sensitive to a ß-lactam/ß-lactamase inhibitor. CONCLUSION: Patients presenting to our institution with acute bacterial lymphadenitis were predominantly found to have methicillin-susceptible S. aureus lymphadenitis that could be empirically treated with cefazolin. At our institution, there is little advantage to the most commonly used broad-spectrum agent, ampicillin-sulbactam.

Long-term efficacy of tonsillectomy/adenotonsillectomy in patients with periodic fever aphthous stomatitis pharyngitis adenitis syndrome with special emphasis on co-existence of familial Mediterranean fever.

Periodic fever, aphthous stomatitis, pharyngitis, adenitis (PFAPA) syndrome is one of the most common autoinflammatory fever disorders in the childhood which may co-exists with familial Mediterranean fever (FMF) causing treatment complexity. As the role of surgery in PFAPA syndrome is still controversial, in this paper, our aim is to present our results of tonsillectomy/adenotonsillectomy in the treatment of PFAPA syndrome. Archives of a tertiary care hospital were investigated for patients who underwent tonsillectomy or adenotonsillectomy due to PFAPA Syndrome between 2010 and 2020. 344 patients were found but only 281 of them were accessible. Through phone call interview and chart review methods, preoperative and postoperative the number and severity of the attacks and general satisfaction after the operation were recorded and analyzed. Also, patients with concomitant FMF were analyzed separately. A total of 281 patients were included in the study. There was no improvement in 10 (3.55%) patients. Eight (2.84%) patients showed mild improvement, 29 (10.32%) patients had moderate improvement and 234 (83.27%) patients had full recovery after tonsillectomy. There were 266 PFAPA patients without FMF. No improvement, mild improvement, moderate improvement, and full recovery in this patient group were 5 (1.9%), 6 (2.3%), 25 (9.4%) and 230 (86.5%), respectively. FMF was present in 5.33% (15/281) of the patients. In PFAPA + FMF group 5 patients had no improvement (33.3%), 2 had mild improvement (13.3%), 4 had moderate improvement (26.7%) and 4 had full recovery (26.7%). Benefit of tonsillectomy was significantly lower in the patients with concomitant FMF when compared to the patients who did not have FMF (p < 0.001). Age of diagnosis, age of operation, severity of the disease, type of operation, and gender were found to have no significant relationship with the benefit from surgery (p < 0.05). According to the findings of this study, tonsillectomy is an effective long-term treatment for PFAPA syndrome with success rate of 83.27%. Also, preoperatively FMF should be considered in these patients, which dramatically reduces surgical efficacy.

Clinical characterization, long-term follow-up, and response to treatment of patients with syndrome of undifferentiated recurrent fever (SURF).

OBJECTIVES: To describe a homogeneous group of patients with undifferentiated recurrent fevers followed-up in a tertiary referral center for systemic autoinflammatory diseases (SAIDs). METHODS: Patients with undifferentiated recurrent fevers seen at our Center from 2008 to 2021 and followed-up for at least one year were included in a retrospective study. Monogenic recurrent fevers, patients carrying variants of unknown origin and PFAPA (Periodic Fever, Aphthous Stomatitis, Pharyngitis, Adenitis) syndrome were excluded. RESULTS: Fifty patients (34 male, 16 female) were included in the study. The median age at onset was 3 years, and the median follow-up was 3.3 years. At baseline, arthralgia (70%) and abdominal pain (65%) were the most frequent manifestations. NSAIDs or steroids on demand had a variable and transient effect. Tonsillectomy was ineffective in the 10 patients (20%) that underwent surgery. Forty-eight patients (96%) were treated with colchicine. A complete response (absence of fever) was achieved in 31 patients (64.6%). Nine patients (18%) showed a partial response, with a median reduction of fever episodes per year of 72%. Nine patients (16.7%) were considered resistant to colchicine. The presence of generalized lymphadenopathy and, to a lesser extent, exudative tonsillitis was associated with a lack of response to colchicine. CONCLUSIONS: We describe the largest series of patients with syndrome of undifferentiated recurrent fever (SURF) reported in the literature so far. SURF should be considered as a distinct clinical entity in the context of multifactorial autoinflammatory diseases.

Long-Term Outcome of Surgical Treatments for Nontuberculous Mycobacterial Cervicofacial Lymphadenitis in Children.

PURPOSE: Information on long-term treatment outcome for nontuberculous mycobacterial (NTM) cervicofacial lymphadenitis in children is scarce. The purpose of this study is to evaluate long-term outcome for surgical treatment, which is the mainstay treatment modality. METHODS: This case series describes recurrence rates of surgically treated NTM cervicofacial lymphadenitis patients with a follow-up of at least 10 years. The current study data were partially collected from a randomized, prospective, multicenter, multidisciplinary trial (CHIMED study), which was conducted between 2000 and 2006 to determine the optimal treatment for NTM cervicofacial lymphadenitis in children. After the CHIMED trial inclusion ended, our institute continued to serve as a referral center. This enabled us to enlarge the surgical CHIMED cohort by adding patients who were treated during 2007 to 2010 in our center and collect the rest of the current study data. RESULTS: About 427 children with chronic cervicofacial lymphadenopathy were analyzed. Among these, 290 had microbiologically confirmed cervicofacial mycobacterial infections (n = 3 Mycobacterium tuberculosis, n = 1 Mycobacterium bovis, n = 286 NTM). Of these 286 children with NTM cervicofacial lymphadenitis, 189 were treated surgically (median age: 41 months, range: 9-144, 46.0% males). The affected lymph nodes were excised in 151 children (79.9%), and curettage was performed in 38 children (20.1%). One patient (0.07%) experienced a reactivation/recurrence 2 years after surgical excision and required another surgical excision. Three children (7.9%) experienced infection reactivation/recurrences after curettage, confirmed by redness or a draining fistula, within the first year after healing. Two of these 3 patients were treated with additional surgical excisions. CONCLUSION: The long-term outcome of surgical excision for NTM cervicofacial lymphadenitis is favorable with a low recurrence rate. Curettage or a conservative wait-and-see approach can be considered an alternative in advanced and surgically challenging cases. However, healing will take longer, and late recurrences are possible.

Surgical management of parotid non-tuberculous mycobacteria lymphadenitis in children: A pediatric tertiary-care hospital's experience.

OBJECTIVE: Non-tuberculous mycobacteria (NTM) represents an important etiology of cervicofacial lymphadenitis (CFL) and skin/soft tissue infections in children. It can also affect the salivary glands, including the parotid gland, which is unique due to the presence of intra-salivary lymph nodes. There are no established guidelines for treatment of NTM CFL. NTM lymphadenitis was historically surgically treated; recently the literature supports initial medical treatment. Treatment decisions have been dependent on the extent of disease, preference of providers, and risk of surgical complications. The goal is to report our experience in surgical outcomes of NTM CFL with involvement of the parotid gland after pre-operative medical management. METHODS: A retrospective case series of patients with NTM affecting the parotid gland at a tertiary care pediatric hospital between 2004 and 2020. RESULTS: Seventy-two patients were referred for surgical evaluation of possible parotid NTM. Thirty-three patients underwent surgical excision. Fifteen patients were identified with presumed NTM infection involving the parotid gland. There were twelve females and three males with a mean age of 2.0 years (SD 1.55; range 1-6 days) at the time of surgery. All underwent surgical excision with parotidectomy. The most common pre-operative antimycobacterial therapy used was a combination of clarithromycin and rifampin. All 15 patients had pathological findings consistent with NTM infection (granulomatous lymphadenitis). Forty percent (n = 6) of patients had positive stains with acid-fast bacilli (AFB), with Mycobacterium avium as the most common species (n = 5). The majority of patients, 86.67% (n = 13), had complete resolution of infection after surgery. Clarithromycin and rifampin were the most common post-operative antimycobacterial treatment (mean 81.5 days, SD 110.14, range 2-411 days). The most common complication experienced was acute (<3 months) lower facial nerve paresis (40%, n = 6), but no patient had permanent facial paralysis. CONCLUSION AND RELEVANCE: Parotidectomy is a safe and efficacious treatment in patients with NTM CFL affecting the parotid gland after incomplete resolution with antimycobacterial therapy. Further investigation to optimize duration of antimycobacterial treatment is necessary. We highlight the experience of a high-volume tertiary care pediatric hospital with surgical management of this disease.

Periodic Fever, Aphthous Stomatitis, Pharyngitis, and Cervical Adenitis Syndrome: Relapse and Tonsillar Regrowth After Childhood Tonsillectomy.

OBJECTIVES/HYPOTHESIS: Tonsillectomy is an effective treatment for periodic fever, aphthous stomatitis, pharyngitis, and cervical adenitis (PFAPA) syndrome, but the role of adenoidectomy, as well as later tonsillar regrowth, is unclear. To find out if the volume of lymphoid tissue is pivotal to the efficacy, we analyzed the association between the relapse of the symptoms of PFAPA syndrome and regrowth of tonsillar tissue after tonsillectomy or adenotonsillectomy. STUDY DESIGN: Prospective cohort study of operated PFAPA pateints. METHODS: We invited all patients that had undergone tonsillectomy or adenotonsillectomy due to PFAPA syndrome at the Oulu University Hospital, Oulu, Finland, between the years 1990 and 2007, at the age of ≤12 years, to a follow-up visit, after an average period of 9.8 years after their diagnoses. Out of the 132 invited, 94 (71%) participated in the follow-up study. RESULTS: At the follow-up study visit, 5 (5%) of the 94 PFAPA syndrome cases experienced recurrent fevers. The regrowth of palatine tonsillar tissue was seen in four of them (80%) as compared to 19/89 (21%) of symptom-free patients (P = .006). Two of the patients with clear PFAPA relapse at the time of the study visit were reoperated with clear effect on the symptoms. At the time of the study visit, 59/63 (94%) of the patients who had undergone adenotonsillectomy and 30/31 of the patients (97%) who had undergone tonsillectomy earlier were free of fever flares (P = .99). CONCLUSION: Palatine tonsil regrowth was associated with PFAPA syndrome relapse after tonsillectomy. Reoperation might be a treatment option in these patients. LEVEL OF EVIDENCE: 4 Laryngoscope, 131:E2149-E2152, 2021.

Lingual Tonsillectomy: Successful Treatment of Periodic Fever, Aphthous Stomatitis, Pharyngitis, and Cervical Adenitis Syndrome.

BACKGROUND: PFAPA (periodic fever, aphthous stomatitis, pharyngitis, and cervical adenitis) syndrome is diagnosed clinically. Adult-onset PFAPA syndrome is rare and often has a more diverse clinical presentation that its childhood counterpart. This is the first reported case of adult-onset PFAPA syndrome with complete response to lingual tonsillectomy. CASE SUMMARY: A 41-year-old man was evaluated for periodic fevers associated with uvulitis, cervical lymphadenitis, pharyngitis, and lower extremity rash. He had a variable response to steroids and was intolerant of colchicine. Laboratory workup revealed intermittent elevation of erythrocyte sedimentation rate and C-reactive protein level. Computed tomography neck and laryngoscopy confirmed adenoidal and lingual tonsillar hypertrophy. He underwent adenoidectomy and lingual tonsillectomy with resolution of symptoms. CONCLUSIONS: Hypertrophy of the remaining lymphoid structures within Waldeyer's ring may be associated with remote recurrence of PFAPA syndrome after tonsillectomy. Lingual tonsillectomy may be an alternative treatment strategy in select patients with PFAPA, prominent lingual hypertrophy, and incomplete response to steroids.

Pre- and postoperative quality of life and emotional/behavioural problems in children with PFAPA.

OBJECTIVE: This study aimed to evaluate the effects of surgical intervention on quality of life, emotional/behavioural problems and school absenteeism in children with periodic fever, aphthous stomatitis, pharyngitis and cervical adenitis (PFAPA.) METHOD: A total of 56 children aged between 4 and 12 years, diagnosed with PFAPA and undergoing tonsillectomy with/without adenoidectomy at a tertiary hospital were included in the study. The Questionnaire for Quality of Life Assessment for Children and Adolescents Parent Form (Kid-KINDL) and the Strengths and Difficulties Questionnaires (SDQ) were used to evaluate quality of life and emotional/behavioural problems, respectively. The cases were assessed before and three months after surgery. RESULTS: The mean (SD) age of the children was 6.64 (3.03) years, and 58.9% (n = 33) were boys. The numbers of pre- and post-operative periodic fever episodes were 3.0 (1.7) and 0.6 (0.9) (p < 0.001), those for school absenteeism were 10.28 (5.53) and 2.85 (2.95) days (p < 0.001) and those for hospital presentations were 3.8 (2.5) and 1.1 (1.1) (p < 0.001), respectively, all of which were significantly lower in children with PFAPA at three months after the operation. The surgical procedures were effective in improving quality of life and emotional/behavioural problems, as evidenced by the significant differences between the pre- and post-treatment scores in outcome measures, including SDQ and Kid-KINDL (p < 0.001). CONCLUSIONS: This study demonstrated that the surgical approach exhibited positive effects in terms of improving quality of life and reducing emotional/behavioural problems and school absenteeism in children with PFAPA.

Cervicofacial non-tuberculous mycobacterial lymphadenitis: clinical determinants of incomplete surgical removal.

In patients with non-tuberculous mycobacterial cervicofacial lymphadenitis, incomplete surgical removal of infected lymph nodes leads to delayed healing and a higher recurrence rate, with eventual spontaneous drainage through the skin. However, complete surgical removal is not always achievable due to the extent of the infected tissue and proximity to vulnerable structures, such as the facial or accessory nerve. The aim of this study was to identify the clinical determinants of the (in)ability to perform complete surgical removal. The electronic health records of patients aged 0-15 years with bacteriologically proven non-tuberculous mycobacterial cervicofacial lymphadenitis, who underwent surgical treatment and preoperative sonographic imaging, were analysed. This was a case-control study. A total of 103 patients met the inclusion criteria. Most of the infections were unilateral, submandibular, and caused by Mycobacterium avium. Multiple logistic regression analysis revealed that higher age (odds ratio 1.24, 95% confidence interval 1.04-1.47) and fistulization (odds ratio 3.15, 95% confidence interval 1.13-8.75) were significantly associated with a limited ability to surgically remove all infected tissue. However, a larger sonographic lymph node size was not significantly associated. These findings could aid clinicians when informing the parent(s)/guardian(s) of the patient preoperatively and in properly estimating the intraoperative and postoperative course.

Submandibular lymphadenitis due to Mycobacterium malmoense: first reported case from Greece.

An 8-year-old girl with subacute submandibular lymphadenitis and no other complaints is described. After failure of parenteral antistaphylococcal therapy, she underwent incision and drainage of the involved lymph node. The responsible pathogen was identified as Mycobacterium malmoense by GenoType CM assay and sequencing of the 16S ribosomalRNA (rRNA) gene. The patient remains healthy, 11 months after surgery, even though it took approximately 4 months for the surgical incision to heal completely. While M. malmoense is a relatively common cause of non-tuberculous mycobacteria (NTM) lymphadenitis in Northern Europe, this is the first reported case from Greece. We conclude that in a young child with lymphadenitis without systemic symptoms, the microbiology laboratory should be notified in advance in order to extend the duration of mycobacterial cultures. Application of molecular methods will increase the number of reported cases of rare NTM in the future.