Surgical Management of Choanal Atresia: Two Classic Cases and Review of the Literature.

Choanal atresia is a rare congenital airway malformation that presents a unique surgical challenge for pediatric otolaryngologists. Here we report two classic cases of choanal atresia and examine the surgical approaches to this entity. The first case was a four-day-old female with a history of CHARGE syndrome and bilateral mixed membranous and bony choanal atresia confirmed by a CT scan. After undergoing transnasal endoscopic repair, choanal stents were placed for four weeks, and the patient was seen three months postoperatively and found to be doing well with no respiratory concerns. The second case involved a healthy three-year-old female presenting with unilateral combined membranous and bony atresia. Following successful endoscopic repair, she was seen at a three-month follow-up with no signs of restenosis. Additionally, a literature review was performed to evaluate updates since the 2012 Cochrane Review on surgical treatment of congenital choanal atresia.

Bilateral Cervical Pilomatricoma: A Diagnostic Dilemma for the Pediatric Otolaryngologist.

Pilomatricomas are benign skin tumors often encountered by otolaryngologists but frequently misdiagnosed. Although they can occur at any age, they commonly present in children as a discolored superficial lesion adhered to the overlying skin. Accurate preoperative diagnosis is crucial for appropriate management, which is surgical in most cases. Here, we present bilateral pilomatricomas mimicking features of several other diagnoses in a pediatric patient. The patient was successfully treated with surgical excision. This case presented a unique diagnostic challenge, as the lesions exhibited features of several common diagnoses. In general, surgical management of pilomatricoma is curative, and recurrence is rare.

Apnea-hypopnea index severity as an independent predictor of post-tonsillectomy respiratory complications in pediatric patients: A retrospective study.

INTRODUCTION: Despite the presence of clinical practice guidelines for overnight admission of pediatric patients following adenotonsillectomy, variance in practice patterns exists between pediatric otolaryngologists. The purpose of this study is to examine severity of apnea-hypopnea index (AHI) as an independent predictor of postoperative respiratory complications in children undergoing adenotonsillectomy. METHODS: Retrospective chart review of all children undergoing adenotonsillectomy at a large tertiary referral center between January 2015 and December 2019 who underwent preoperative polysomnography and were admitted for overnight observation. Charts were reviewed for total adverse events and respiratory events occurring during admission. RESULTS: Overall, respiratory events were seen in 50.6% of patients with AHI ≥10 and in 39.6% of patients with AHI <10. The overall mean AHI was 19.2, with a mean of 28.1 in the AHI ≥10 subgroup vs 4.6 in the AHI <10 subgroup. There was no statistical correlation or increased risk between an AHI ≥10 and having a pure respiratory event, with a relative risk of 1.19 (.77-1.83, P = .43). There was a statistically significant difference between the mean AHI of those with any adverse event and those without (21.6 vs 13.4, P = .008). There is additionally an increased risk of any event with an AHI over 10, with a relative risk of 1.51 (1.22-1.88, P < .0001). CONCLUSION: Preoperative AHI of 10 events per hour was not a predictor of postoperative respiratory complications. However, there was a trend for those with a higher AHI requiring additional supportive measures or a prolonged stay. Practitioners should always use their best judgment in deciding whether a child warrants postoperative admission following adenotonsillectomy.

The role of CDHR3 in susceptibility to otitis media.

Otitis media (OM) is common in young children and can cause hearing loss and speech, language, and developmental delays. OM has high heritability; however, little is known about OM-related molecular and genetic processes. CDHR3 was previously identified as a locus for OM susceptibility, but to date, studies have focused on how the CDHR3 p.Cys529Tyr variant increases epithelial binding of rhinovirus-C and risk for lung or sinus pathology. In order to further delineate a role for CDHR3 in OM, we performed the following: exome sequencing using DNA samples from OM-affected individuals from 257 multi-ethnic families; Sanger sequencing, logistic regression and transmission disequilibrium tests for 407 US trios or probands with OM; 16S rRNA sequencing and analysis for middle ear and nasopharyngeal samples; and single-cell RNA sequencing and differential expression analyses for mouse middle ear. From exome sequence data, we identified a novel pathogenic CDHR3 splice variant that co-segregates with OM in US and Finnish families. Additionally, a frameshift and six missense rare or low-frequency variants were identified in Finnish probands. In US probands, the CDHR3 p.Cys529Tyr variant was associated with the absence of middle ear fluid at surgery and also with increased relative abundance of Lysobacter in the nasopharynx and Streptomyces in the middle ear. Consistent with published data on airway epithelial cells and our RNA-sequence data from human middle ear tissues, Cdhr3 expression is restricted to ciliated epithelial cells of the middle ear and is downregulated after acute OM. Overall, these findings suggest a critical role for CDHR3 in OM susceptibility. KEY MESSAGES: • Novel rare or low-frequency CDHR3 variants putatively confer risk for otitis media. • Pathogenic variant CDHR3 c.1653 + 3G > A was found in nine families with otitis media. • CDHR3 p.Cys529Tyr was associated with lack of effusion and bacterial otopathogens. • Cdhr3 expression was limited to ciliated epithelial cells in mouse middle ear. • Cdhr3 was downregulated 3 h after infection of mouse middle ear.

Otitis media susceptibility and shifts in the head and neck microbiome due to SPINK5 variants.

BACKGROUND: Otitis media (OM) susceptibility has significant heritability; however, the role of rare variants in OM is mostly unknown. Our goal is to identify novel rare variants that confer OM susceptibility. METHODS: We performed exome and Sanger sequencing of >1000 DNA samples from 551 multiethnic families with OM and unrelated individuals, RNA-sequencing and microbiome sequencing and analyses of swabs from the outer ear, middle ear, nasopharynx and oral cavity. We also examined protein localisation and gene expression in infected and healthy middle ear tissues. RESULTS: A large, intermarried pedigree that includes 81 OM-affected and 53 unaffected individuals cosegregates two known rare A2ML1 variants, a common FUT2 variant and a rare, novel pathogenic variant c.1682A>G (p.Glu561Gly) within SPINK5 (LOD=4.09). Carriage of the SPINK5 missense variant resulted in increased relative abundance of Microbacteriaceae in the middle ear, along with occurrence of Microbacteriaceae in the outer ear and oral cavity but not the nasopharynx. Eight additional novel SPINK5 variants were identified in 12 families and individuals with OM. A role for SPINK5 in OM susceptibility is further supported by lower RNA counts in variant carriers, strong SPINK5 localisation in outer ear skin, faint localisation to middle ear mucosa and eardrum and increased SPINK5 expression in human cholesteatoma. CONCLUSION: SPINK5 variants confer susceptibility to non-syndromic OM. These variants potentially contribute to middle ear pathology through breakdown of mucosal and epithelial barriers, immunodeficiency such as poor vaccination response, alteration of head and neck microbiota and facilitation of entry of opportunistic pathogens into the middle ear.

Thyroglossal duct cyst anterior to the sternum: a novel presentation of a common lesion.

Thyroglossal duct cysts (TDCs) arise in roughly 7% of the general population and are typically diagnosed in childhood within the first decade of life. Typically, patients present with a painless, midline neck mass in close proximity to the hyoid bone which classically elevates with deglutition and tongue protrusion. We present a case of TDC found anterior to the sternum, a major deviation from the classical understanding of this lesion. The patient was treated successfully with modified Sistrunk procedure. This case underscores the need for clinicians to maintain a wide differential while working up paediatric patients presenting with neck masses. Furthermore, we emphasise that TDC must always be considered in cases of midline paediatric neck masses, even when found in unusual locations such as presented here.

Preventing unnecessary tympanostomy tube placement in children.

OBJECTIVE: In 2013 the American Academy of Otolaryngology published tympanostomy tube guidelines for children; Action Statement 6 recommends against tube placement without middle ear effusion (MEE) at time of assessment. To date, little research has directly evaluated this recommendation in reducing the need for ear tubes. We evaluated the effectiveness of this recommendation and potential risk factors that influence the success of watchful waiting. METHODS: Retrospective chart review collecting demographics, daycare status, smoking exposure, and time of year of visit. Children aged 6 months to 12 years without MEE on presentation, but with 3 or more episodes of acute otitis media (AOM) in 6 months or 4 or more episodes in 12 months, were assigned to watchful waiting (WW) treatment. These patients were followed every 4 months or returned sooner with additional infections. Any continued AOM, or MEE on follow up leading to tube placement, defined WW failure. RESULTS: 123 patients met criteria, with 81 still in WW to date (66% success rate). 42 children failed WW and received tympanostomy tubes (34% failure rate). There were no statistically significant associations between age, race, gender, smoking exposure, daycare, or month of presentation between children who failed WW compared to children receiving tubes. CONCLUSIONS: Tympanostomy tube guidelines mitigate unnecessary tube placement in a majority of children with recurrent AOM without MEE. To our knowledge, this is the first study supporting the 2013 recommendations, with a 66% success rate. Additionally, no significant associations between modifying risk factors in those who failed watchful waiting were identified.

Utility of preoperative hematologic screening for pediatric adenotonsillectomy.

We conducted a 3-year retrospective study to examine the results of preoperative hematologic screening, the incidence of postoperative bleeding, and the possible relationship between the two factors in patients who had undergone tonsillectomy with or without adenoidectomy. Our study population was made up of 875 patients--441 boys and 434 girls, aged 2 to 18 years (mean: 7.52 ± 4.25)--who had been treated at our institution from January 2004 through December 2006. In addition to demographic data, we compiled information on each patient's medical and surgical history, personal and family history of abnormal bleeding, indication for tonsillectomy, and preoperative hematologic screening results. The latter included determinations of the prothrombin time, activated partial thromboplastin time, international normalized ratio (INR), and platelet count. A total of 748 patients (85.5%) had normal findings on preoperative hematologic screening, and 127 (14.5%) had at least one abnormality. Postoperatively, hemorrhagic complications occurred in 31 children (3.5%)--in 22 of the 748 patients with normal screening results (2.9%) and in 9 of the 127 with a screening abnormality (7.1%); the difference between the two groups was statistically significant (p = 0.041). The abnormalities in the latter group consisted of an elevated INR but no otherwise identifiable coagulopathy. Another 14 patients with an abnormal screening result (11.0%) were found to have at least one coagulopathy that was newly diagnosed during our preoperative evaluation; they were treated perioperatively, and none bled postoperatively. Of 21 patients who had a personal or family history of abnormal bleeding, 5 (23.8%) were found to have a coagulopathy, but none bled following surgery. In conclusion, we found that preoperative hematologic screening identified patients with undiagnosed coagulopathies, and with appropriate treatment our surgeons were able to prevent some bleeding events in these patients. Our finding that patients with a mildly elevated INR had a higher incidence of postoperative hemorrhage warrants further study.

Intracapsular vs. extracapsular tonsillectomy: a comparison of pain.

OBJECTIVE: To measure differences in postoperative pain in children undergoing intracapsular tonsillectomy vs. extracapsular tonsillectomy. STUDY DESIGN: Prospective clinical trial carried out at a tertiary children's hospital over 1 year. METHODS: The study included 43 patients age 5 to 19 years with adenotonsillar hypertrophy, 27 undergoing extracapsular tonsillectomy and 16 undergoing intracapsular tonsillectomy. Tonsillectomy was performed in either an intracapsular or extracapsular fashion using bipolar electrosurgical scissors and monopolar suction cautery for hemostasis. Residents under faculty supervision performed all operations. Postoperative pain was assessed using the Wong-Baker Faces Pain Scale through postoperative day 10. The patients recorded in a standardized diary pain scores and analgesic use. The main outcome measure was postoperative pain as measured by the Wong-Baker Faces Pain Scale. The quantity of postoperative pain medicine consumed was a secondary outcome measure. RESULTS: Average postoperative pain for extracapsular tonsillectomy was 5.21 on a 10-point scale versus 2.75 for intracapsular tonsillectomy (P < .0001). CONCLUSIONS: Intracapsular tonsillectomy with bipolar scissors results in less postoperative pain than extracapsular tonsillectomy with bipolar scissors in children age 5 to 19 years.

Congenital nasal malformations.

The nose is a prominent feature of the human face. Congenital malformations of the nose, whether functional or anatomic, affect the physiologic and psychologic wellness of children who have these anomalies. Congenital nasal abnormalities may be overt or subtle and can occasionally cause life-threatening emergencies at birth. A discussion of nasal embryology and development provides the basis for the discussion of some of the important congenital abnormalities seen in clinical practice. The final portion of the article is devoted to several of the more common syndromes in which nasal abnormalities are encountered.

Effectiveness of adenotonsillectomy in the resolution of nocturnal enuresis secondary to obstructive sleep apnea.

OBJECTIVES: To investigate the relationship between obstructive sleep apnea (OSA) syndrome and nocturnal enuresis (NE) in patients who required tonsillectomy or adenoidectomy. STUDY DESIGN: Retrospective chart review with prospective collection of data. METHODS: All charts of patients ages 2 to 18 years that had tonsillectomy or adenoidectomy over a 44 month period were reviewed for presence of NE and indication for surgery. Those patients with a positive history of both NE and OSA were surveyed to determine whether there was no change in enuresis, decreased enuresis, or no enuresis postoperatively. RESULTS: Three hundred twenty-six children who had undergone tonsillectomy or adenotonsillectomy had data regarding enuresis available in their charts. One hundred seven of these 326 (32.8%) children had a positive history of enuresis. Of the 107 children with a positive history, 44 (41.1%) were female, and 63 (58.9%) were male. All 107 children with enuresis underwent adenotonsillectomy for OSA. None of the children who had a history of recurrent adenotonsillitis or chronic tonsillitis reported enuresis as a presenting symptom. Of the 107 children with a positive preoperative history of NE, 57 (53.3%) agreed to participate in the second phase of the study. Postoperatively, 61.4% (35) of the children were free of enuresis, 22.8% (13) had a decrease in enuresis, and 15.8% (9) had no change in enuresis. A chi-square test showed a statistically significant difference among the groups (P < .0001). CONCLUSIONS: NE is a relatively common finding in children with OSA symptoms. NE resolves or markedly improves in the vast majority of these patients postoperatively.

Oxymetazoline is equivalent to ciprofloxacin in preventing postoperative otorrhea or tympanostomy tube obstruction.

OBJECTIVE: To compare the effectiveness of ciprofloxacin and oxymetazoline solutions instilled after tympanostomy tube placement in the prevention of postoperative otorrhea and tube occlusion. STUDY DESIGN: Prospective cross-sectional series. METHODS: We reviewed all bilateral myringotomy and tube placement operations performed by two full-time attending pediatric otolaryngologists during a 9 month period. Data from 488 patients who underwent surgery for otitis media were collected. Demographic and clinical variables including age, sex, number of tube insertions in the past, previous adenoidectomy, type of effusion present at surgery, and type of drop prescribed postoperatively were recorded. All patients were evaluated in the office 2 to 4 weeks postoperatively. Multivariate logistic regression analysis was used to estimate the relationship of these variables with the occurrence of otorrhea and tube patency. Odds ratios were calculated. RESULTS: No significant differences in postoperative otorrhea or tube patency were found between ciprofloxacin (Ciloxan) and oxymetazoline solutions (Afrin, Visine LR). CONCLUSION: Oxymetazoline and ciprofloxacin solutions are equivalent in the prevention of postoperative otorrhea and tube occlusion after tympanostomy tube placement. The implications for medication cost and potential adverse reactions are discussed.

Contraleteral deafness following ear surgery

Rare cases of contralateral hearing loss following surgery have been reported, usually associated with acoustic neuroma resection. We present one case of contralateral sensorineural hearing loss following middle ear reconstruction for congenital malformation, and one case following resection of a glomus jugulare tumor. Review of the literature confirms the rarity of contralateral hearing loss following ear surgery, and an autoimmune mechanisms seems most likely. Aggressive evaluation and treatment are indicated