A case of solitary median maxillary central incisor (SMMCI) syndrome with bilateral pyriform aperture stenosis and choanal atresia

Solitary median maxillary central incisor syndrome is a rare disorder involving midline abnormalities. It may present with life threatening respiratory distress in the neonate secondary to nasal malformations. These include pyriform aperture stenosis and choanal atresia. We present the first reported case of simultaneous choanal atresia and pyriform aperture stenosis in a neonate with solitary median maxillary central incisor syndrome. The clinical presentation and the management of congenital pyriform aperture stenosis are discussed.     

Congenital pyriform aperture stenosis: surgery and evaluation with three-dimensional computed tomography

OBJECTIVE: We described the surgical efficacy of congenital pyriform aperture stenosis by measuring the width of the nasal pyriform aperture with three-dimensional computed tomography. METHODS: Six patients with congenital pyriform aperture stenosis accepted surgical intervention. Three-dimensional computed tomography was performed for preoperative and postoperative evaluation of nasal pyriform aperture width. We marked the midpoint between the tip of nasal bone and the anterior nasal spine. The distances between the bilateral nasal processes of the maxilla and between the lateral rims of the pyriform aperture were measured by crossing the midpoint horizontally. RESULTS: Among the six patients (three male and three female patients), the mean age was 76.2+/-23.9 days. Silicon stents were removed 6 to 7 days after operation. During 9 months of follow-up, there were no cases of restenosis, respiratory failure, or cyanosis. The mean preoperative and postoperative interprocess distances were 4.5+/-0.84 and 8.7+/-1.37 mm, respectively, and data were statistically significant at a confidence level of P <.05. The mean preoperative and postoperative interwall distances were 9.8+/-0.75 and 10.8+/-1.5 mm, respectively. CONCLUSIONS: Congenital pyriform aperture stenosis should be suspected whenever there is both severe nasal obstruction and difficulty in passing a small catheter or nasogastric tube through the anterior nasal valve. Operation is the most extreme treatment, but it is effective for congenital pyriform aperture stenosis. Nasal stenting for 7 days seemed to be adequate. The use of three-dimensional computed tomography to evaluate preoperative and postoperative nasal pyriform aperture is effective and reliable.     

Congenital nasal pyriform aperture stenosis: a report of 10 cases and literature review

Introduction

Congenital nasal pyriform aperture stenosis (CNPAS) is a rare cause of neonatal upper airway obstruction. This study is to review the presentation and management of this uncommon condition in a tertiary paediatric ENT centre in Scotland, United Kingdom.

Methods

Retrospective case note review between 2003 and 2011. A literature search was performed using MEDLINE and EMBASE between April and June 2011 using search terms ‘pyriform aperture’ in combination with diagnosis and treatment. Suitable references were also selected and reviewed.

Results

A total of 10 cases were identified and 50% of cases were managed surgically. Surgical widening of the pyriform aperture was performed at an average of 14 days at birth. Associated malformations include holoprosencephaly, hypopituitarism, cardiac and urogenital malformations.

Conclusion

CNPAS is a treatable cause of upper airway obstruction. Early recognition is vital to appropriate management. Treatment is either conservative or surgical and depends on the severity of symptoms.     

Congenital nasal pyriform aperture stenosis: Feeding evaluation and management

Purpose

We identified four patients with congenital nasal pyriform aperture stenosis (CNPAS) with feeding and respiratory difficulties. While feeding difficulties have been described in infants with other causes of nasal obstruction (e.g., choanal atresia), little attention has been focused on feeding in infants with CNPAS.

Findings

All four patients were treated with nasal steroids and nasal saline and two underwent surgical repair of the CNPAS at 2 months of age. Except for gastroesophageal reflux in one patient, upper gastrointestinal tract (UGI) contrast studies were normal in all patients. Three patients underwent bedside swallow evaluations, two of whom participated in videoflouroscopic swallow studies (VFSS) because of limited oral intake or respiratory problems during feeding. These three patients presented with intact oral-motor coordination at rest and during non-nutritive sucking, and nasal whistling, nasal congestion, or breathing difficulties/desaturations with oral feeding. On VFSS, one infant had trace aspiration with thin and thick liquids. Three patients required supplemental feeding during the first year of life. All three patients with normal brain MRIs were successful oral feeders by 16 months of age, the fourth is undergoing feeding therapy at 24 months of age.

Summary

Feeding problems are common in infants with CNPAS and supplemental feedings may be needed even after management of airway difficulties. Infants with CNPAS should undergo comprehensive feeding/swallowing evaluations which may include bedside swallow evaluations and VFSSs. The prognosis for transitioning to total oral feedings is good and related to the extent of the airway obstruction and presence of other comorbidities.     

Nasal pyriform aperture stenosis in adults

BACKGROUND: Congenital nasal pyriform aperture stenosis (CNPAS) leading to respiratory insufficiency in infants has been well documented. Nevertheless, the nasal pyriform aperture stenosis (NPAS) entity in adulthood has not been discussed at all. The normative data of the width of the pyriform aperture, which has not been revealed before, is necessary for evaluation of the adult subjects with nasal bony inlet stenosis. The aim of this study was to define the normative data about the pyriform aperture width measured in 80 adult subjects with paranasal sinus symptoms without nasal obstruction and, additionally, to report the surgical results of two representative cases with NPAS. METHODS: The nasal bony inlet width measurements have been achieved in axial computerized tomographic sections to establish normative data. The maximal distance between the nasal processes of the opposite maxillary bones was measured. Two adult males complaining of nasal obstruction with overt pyriform aperture stenosis were treated surgically via a sublabial approach. RESULTS: The mean widest dimension of the nasal pyriform aperture was 21.6 +/- 2.2 mm (range, 17-27 mm) in 80 adult subjects. This width was 21.9 +/- 2.1 mm (range 18-27 mm) in men and 21 +/- 2.2 mm (range, 17-26 mm) in women. Stenotic pyriform apertures in two patients were widened surgically by drilling via a sublabial approach from 12 and 10 mm to 24 and 21 mm, respectively. CONCLUSION: NPAS should be included in the list of differential diagnosis for nasal obstruction. Surgical enlargement using a sublabial approach was found an effective and adequate treatment in pyriform aperture stenosis.     

Congenital nasal pyriform aperture stenosis: Diagnosis,management and technical considerations

BackgroundCongenital nasal pyriform aperture stenosis (CNPAS) is a rare cause of nasal obstruction in neonates with respiratory distress manifestations. Diagnosis is made with craniofacial computed tomography, prompt and precise treatment creates good outcomes in these patients.AimTo present our experience in diagnosis and management considerations with this rare pathology and a case series of our surgically managed patients.MethodsA retrospective, analytical study of CNPAS patients surgically managed over a period of seven years. Evaluation and follow up was reviewed.ResultsThirteen patients were evaluated; pyriform aperture mean width was 5.5 mm. Of these patients, 31% also had Congenital Midnasal Stenosis. Medical treatment failed for all the patients and they required surgical enlargement of the pyriform aperture. No complications were seen and all patients improved in symptoms and development.ConclusionsCongenital nasal obstruction can be fatal in new-borns, CNPAS is a rare differential diagnosis that must be completely evaluated, properly treated with conservative management and if it fails, with well-planned and executed surgery. Follow-up shows high rates of success.     

Evaluation of endoscopic coblation treatment for obliteration of congenital pyriform sinus fistula

Background: Congenital pyriform sinus fistula is a very rare branchial apparatus malformation. Conventional open surgery for fistulectomy might fail to excise the lesion completely, leading to continual recurrence. The aim of this study was to provide an innovative endoscopic coblation technique for patients with pyriform sinus fistula and evaluate its intermediate-term effectiveness.

Methods: Retrospective case series with 112 patients (age range 3–36 years) between 2013 and 2016 and underwent endoscopic coblation of the sinus fistula. Data collected including patient demographics, presenting symptoms, diagnostic methods, prior and subsequent treatments, length of hospital stay, and recurrence were analyzed.

Results: Of the 112 cases, there were no postoperative complications observed except temporary reddish swelling in three patients. Four cases were lost to follow-up. Of 108 patients, 106 experienced no recurrence after their first endoscopic coblation of the sinus tract. The remaining two patients with recurrence in the follow-up were ultimately treated with recoblation without complications or further recurrences. Also, Endoscopic coblation can be used to treat seven patients with recurrence who had experienced open resection. In our series, median follow-up period was 1.5 years.

Conclusion: Endoscopic coblation is an effective approach for most patients. We advocate using this minimally invasive technique as first line of treatment for pyriform sinus fistula.     

The measurement of congenital nasal pyriform aperture stenosis in infant

OBJECTIVE: In the study we use the image of three dimensional computed tomography (3D-CT) to assess the different widths of the bony framework of congenital nasal pyriform aperture stenosis (CNPAS). METHODS: We select 17 infants under 4 months old diagnosed as CNPAS for this study. There were four categories of distance measurement. The distance between the caudal end of the nasal bone (nasal tip) and the anterior nasal spine (NPAH). The distance between bilateral bony inner wall of the nasal pyriform aperture at the midpoint of the aperture height (MIVD). The interprocess distance at the level of lower 1/4 of the pyriform aperture height (IPD). The narrowest distance between the bilateral nasal processes (NIPD). RESULTS: There were 17 infants, nine males and eight females. At the time of performing 3D-CT, the mean age was 49.5+/-35.9 days. The results of measurement were: NPAH: 10.9+/-1.23mm, MIVD: 8.2+/-0.89mm, IPD: 4.9+/-0.93mm, and NIPD: 4.4+/-0.73mm. The MIVD is significantly wider than both IPD and NIPD (p<0.01). There is high correlation between IPD and NIPD. There is no significant difference on age between gender in this study. However, the IPD (4.4+/-0.74) and NIPD (3.9+/-0.47) of male patients are broader, respectively, than those of female patients with significant difference (p<0.05). CONCLUSION: The measurement of CNPAS by means of 3D-CT may provide useful data for evaluation of the width in different parts of nasal pyriform aperture. These may be used for evaluation of pre- and postoperative status and future investigation in CNPAS patients.     

声反射鼻测量计对婴幼儿进行鼻腔测量

目的 :研究正常婴幼儿鼻腔最小横截面积 (MCA)及其距前鼻孔的距离 (DCA)、双侧鼻腔容积 (NV)、双侧鼻咽部容积 (NPV)的正常值范围。方法 :应用声反射鼻测量计测量正常婴幼儿 175例 ,同时测量急性上呼吸道感染婴幼儿 10 8例作为对照。结果 :正常婴幼儿双侧MCA范围为 0 .2 0～ 0 .5 0cm2 ,DCA范围 1.93～ 2 .47cm ,NV范围 2 .6 9～ 4.75cm3 ,NPV范围 3.83～ 8.92cm3 ,与对照组比较 ,MCA、DCA、NV差异均有显著性意义 (P <0 .0 5 )。结论 :声反射鼻测量计可以客观地评价婴幼儿鼻气道 ,本检测可为婴幼儿鼻腔生理、病理研究 ,鼻腔疾病的辅助诊断及疗效判定提供参考依据。     

内镜下等离子射频消融术治疗儿童先天性梨状窝瘘232例

目的 探讨儿童先天性梨状窝瘘的诊断、手术适应证及内镜下低温消融术的疗效。方法 回顾分析郑州大学第一附属医院内镜下低温消融术治疗的232例儿童先天性梨状窝瘘的临床资料。结果 217例(93.5%)为左侧梨状窝瘘,14例(6.0%)为右侧梨状窝瘘,1例(0.4%)双侧梨状窝瘘。男女比约1.1∶1.0。232例均行内镜下梨状窝瘘口低温消融术。术后复发17例(7.3%)、声音嘶哑12例(5.2%),均无呛咳、食管损伤或咽瘘。结论 内镜下梨状窝瘘口低温消融术治疗先天性梨状窝瘘具有微创、并发症较少、复发率低等优点,是治疗先天性梨状窝瘘的首选手术方案之一。     

Treatment of congenital nasal cavity stenosis by balloon dilatation in a newborn: a case report

Neonates are obligate nasal breathers, and any form of neonatal nasal obstruction may have serious consequences. Prompt diagnosis and appropriate treatment are essential to avoid severe hypoxia. Congenital bony nasal stenosis (CBNS) is an extremely rare cause of neonatal nasal airway obstruction and can easily be confused with choanal atresia or stenosis. This is a paper to describe a balloon dilatation technique that can be an effective alternative to surgery for the treatment of congenital nasal cavity stenosis, with minimal stress to the patient.     

显微支撑喉镜下低温等离子微创治疗儿童先天性梨状窝瘘

目的探讨显微支撑喉镜下低温等离子微创治疗儿童急性感染期（AIS）与炎症静止期（IQS）先天性梨状窝瘘（CPSF）的临床疗效。方法回顾性分析2017年12月—2019年12月上海交通大学附属儿童医院收治并行低温等离子微创治疗的51例CPSF患者临床资料,其中AIS 22例,IQS 29例,2组患儿均在气管插管全麻支撑喉镜下行低温等离子射频消融封闭梨状窝内瘘口,而AIS的患儿同期行颈部脓肿切开引流术,留置纱条,术后每日颈部换药。全部患儿术后留置胃管２周,应用抗生素治疗7～10 d。观察其疗效、术后复发率及并发症。结果3例处于IQS的患儿分别于术后第5、6、7天因颈部脓肿形成行切开排脓手术,换药4 d后痊愈。2例AIS的患儿术后出现一过性声嘶,于术后1个月内恢复正常。术后随访2～26个月,1例IQS患儿术后半年复发,2次全麻内镜下低温等离子内瘘口烧灼术,其余50例CPSF内瘘口1次封闭,未发生咽瘘、喉返神经损伤、大出血等并发症,无复发,疗效显著。结论推荐显微支撑喉镜下低温等离子微创根治术作为CPSF的首选疗法,对于IQS的CPSF宜实施单纯内瘘口烧灼术,但术前充分告知家属,术后可能因颈部局部脓肿形成,需要切开引流、换药,对于AIS的CPSF,不必等待炎症控制以后,直接同期行颈部脓肿切开引流术。     

Bilateral nasal vestibular stenosis: a case of rhinoscleroma and review of surgical techniques

Vestibular stenosis is an uncommon but debilitating cause of nasal obstruction. It is caused by disruption of the nasal vestibular lining with secondary proliferation of granulation and fibrous tissue. A number of techniques have been described for repair of the stenotic segment. Correction can be difficult because of the tendency of wound contracture and recurrence. We present a surgical technique that employs the concept of excision of the stenotic segment, enlargement of the vestibule and placement of a full thickness skin flap. Its ability to resist contracture obviates the need for post-operative stenting and gives good result.