Prenatal diagnosis of a "living" oropharyngeal fetus in fetu: a case report.

BACKGROUD: Fetus in fetu (FIF) is a rare malformation in which a parasitic twin within its more mature twin. Most of the FIF locate in the retroperitoneum and are acardiac and anencephalic. CASE PRESENTATION: Here, we describe a unique case of oropharyngeal fetus in fetu with a rudimentary two-chambered heart detected by prenatal ultrasonography. The parents terminated this pregnancy after counseling. Macroscopic examination found a solid mass between the oral and fetal chest, with a rudimentary two-chambered heart at the lowest part of the mass. Microscopic findings showed amniotic membrane, skin, cartilage, gastrointestinal and neural tissue. CONCLUSIONS: Prenatal ultrasound can identify rudimentary organs suspecting FIF from early pregnancy. Detection of fetal heart beat facilitates differential diagnosis with teratomas, providing essential information for parental consulting and management.

Effectiveness and Safety of Sclerotherapy for Treatment of Low-Flow Vascular Malformations of the Oropharyngeal Region.

PURPOSE: To evaluate the efficacy and safety of sclerotherapy with sodium tetradecyl sulfate (STS) and bleomycin for treatment of venous malformations (VMs) of the oropharyngeal region. MATERIALS AND METHODS: A retrospective study of 33 patients with 46 VMs of the buccal and pharyngolaryngeal cavity associated with impairment of eating, respiration, or elocution was performed. Individual lesions were divided based on their anterior or posterior location, using the base of the tongue as an anatomic landmark. Lesion size was estimated with the use of orthogonal measurements on magnetic resonance or ultrasound images before and after treatment to assess radiologic response. Sclerotherapy sessions were performed under ultrasound, fluoroscopic, and, if needed, endoscopic guidance. Clinical response was assessed with the use of the Manchester Orofacial Pain Disability Scale. Methods for airway management were also compiled. RESULTS: Following sclerotherapy, average VM diameter was reduced by 31.4% (P < .0001) on a per-patient basis and by 30.8% (P < .0001) on a per-lesion basis. The Manchester score improved by an average of 37.0% (P = .013). Four patients reported a worsening of symptoms, and 11 patients experienced symptomatic recurrence. Complications include pneumonia (5 patients) and urgent placement of a post-procedure tracheostomy (4 patients). Patients with posterior malformations experienced more complications (emergency tracheostomies in 4 and pneumonias in 4). CONCLUSIONS: Sclerotherapy using STS is an efficient treatment for venous malformations of the buccal and pharyngolaryngeal cavity but can lead to significant complication for posterior lesions. Careful assessment of the airway is needed before treatment, and prophylactic tracheotomy should be considered in patients with posterior lesions.

Perinatal diagnosis and management of oropharyngeal fetus in fetu: A case report.

Fetus in fetu is an extremely rare congenital anomaly. We describe the perinatal diagnosis and management of a fetus with oropharyngeal and cervical fetus in fetu. High-resolution ultrasonography with 3-dimensional rendering can identify increased risks of airway obstruction in utero. Early identification allows a multidisciplinary team to be assembled for a scheduled ex utero intrapartum treatment procedure.

Cone Beam Computed Tomography Analysis of Oropharyngeal Airway in Preadolescent Nonsyndromic Bilateral and Unilateral Cleft Lip and Palate Patients.

OBJECTIVE: The objective of this study was to assess the volume, area, and dimensions of the oropharyngeal airway (OPA) in a previously repaired nonsyndromic unilateral cleft lip and palate (UCLP) versus bilateral cleft lip and palate (BCLP) patients when compared with noncleft controls using cone beam computed tomography (CBCT). DESIGN: This was a retrospective case-control study. SETTING: The Cleft Care Center and outpatient clinic that are affiliated to our faculty were the settings for the study. PARTICIPANTS: A total of 58 CBCT scans were selected of preadolescent individuals: 14 BCLP, 20 UCLP, and 24 age- and gender-matched noncleft controls. VARIABLES: Variables were volume, cross-sectional area (CSA), midsagittal area (MSA), and dimensions of OPA. STATISTICAL ANALYSIS: One-way analysis of variance and post hoc tests were used to compare variables. Statistical significance was set at P ≤ .05. RESULTS: UCLP showed significantly smaller superior oropharyngeal airway volume than both controls and BCLP ( P ≤ .05). BCLP showed significantly larger CSA at soft palate plane and significantly larger MSA than both UCLP and controls ( P < .05). CONCLUSIONS: UCLP patients at the studied age and stage of previously repaired clefts have significantly less superior oropharyngeal airway volume than both controls and BCLP patients. This confirms that preadolescents with UCLP are at greater risk for superior oropharyngeal airway obstruction when compared with those BCLP and controls. Furthermore, BCLP patients showed significantly larger CSA at soft palate plane and MSA than both controls and UCLP patients. These variations in OPA characteristics of cleft patients can influence function in terms of respiration and vocalization.

Evaluation of morphological changes in pharynx with dynamic CT and MRI in snoring patients.

BACKGROUND: Identifying the craniofacial abnormalities that cause snoring and the narrowest area of the upper airway creating obstructions can help to determine the proper method of treatment. AIM: To identify the factors that can cause snoring and the areas of the airway that are the most likely to collapse with upper airway imaging. MATERIAL AND METHODS: Axial pharynx examinations with CT (computerized tomography) and magnetic resonance imaging (MRI) were performed to 38 patients complaining of snoring and 12 patients who did not complain of snoring. The narrowest areas of nasopharynx, hypophraynx, oropharynx, bilateral para-pharyngeal fat pad and para-pharyngeal muscle thickness were measured. RESULTS: In snoring patients, the narrowest part of the upper airway was the retro-palatal region in the oropharynx, as measured with both imaging methods. When patients with and without snoring were compared, the former that a higher body mass index and neck diameter and a narrower oropharynx area. In dynamic examinations, we determined that as para-pharyngeal muscle thickness increased, medial-lateral airway diameter and the oropharynx area decreased. CONCLUSIONS: The narrowest section of the airway is the retro-palatal region of the oropharynx, measured both with CT and MRI.

Concurrence of the tortuosity of bilateral common and left internal carotid arteries in a case with common origin of the innominate trunk and left common carotid artery.

Anatomical variations of carotid arterial system, which are not infrequently encountered, have great impact on the surgical approaches of the neck. Although few reports on common carotid artery tortuosity have been published, no case of symptomatic concurrent common carotid and internal carotid artery tortuosity has been reported. Herein, we report the first case with concurrent common origin of the innominate trunk and left common carotid artery and common and internal carotid artery tortuosity presenting with an oropharyngeal mass.

Correlating videofluoroscopic swallow study findings with subjective globus location.

OBJECTIVE: Patients with globus, the sensation of something stuck in the throat, are evaluated by otolaryngologists, gastroenterologists, and speech pathologists and often undergo multiple tests and interventions. We hypothesize that a videofluoroscopic swallow study (VFSS) is useful to characterize globus etiology and correlate subjective globus location to atypical VFSS findings. METHOD: Retrospective chart review of all patients undergoing VFSS over a 24-month period with a primary complaint of globus. Globus was characterized by the patient as above the thyroid notch, between the thyroid notch and sternum, or substernal. VFSS findings were categorized as oropharyngeal, pharyngoesophageal, or esophageal based on nine VFSS abnormalities and then further broken out for subgroup analyses. RESULTS: Of 216 patients meeting study criteria, 109 patients localized globus above the thyroid notch, 74 between the thyroid notch and sternum, and 33 substernal. One hundred ninety-five patients (90.3%) had at least one finding on VFSS that could account for symptoms, and the majority had multiple. In fact, 21 patients (9.7%) with dysphagia localized above the thyroid notch had evidence of distal esophageal abnormalities, and 15 (6.9%) with dysphagia localized substernal had oropharyngeal abnormalities. CONCLUSION: Whereas VFSS was likely to identify abnormalities, these areas relate poorly overall with the patient's subjective globus location, and the clinical utility of the study is questionable. LEVEL OF EVIDENCE: 4 Laryngoscope, 129:335-338, 2019.

A case of maxillary duplication with a soft palate reconstruction using a forearm flap.

Maxillary duplication is a rare congenital anomaly that occurs in the jaw/mouth area. It is generally regarded as sporadic in nature. Total or subtotal soft palate reconstruction for oropharyngeal defects, which include post-surgical and congenital defects, presents a difficult surgical challenge. A maxillary duplication in which the soft palate is reconstructed using a vascularized forearm flap is described. The velopharyngeal insufficiency in the present case is caused by the almost complete deficiency of the soft palate, suggesting that a conventional pharyngeal flap operation with localized mucosal myocutaneous flaps would not produce favorable results in terms of postoperative contractions in the pharyngeal flaps. In such cases, the reconstruction of the soft palate using vascularized free forearm flaps, guided by flexibility regarding the size and adequate thickness of the flaps, may be useful.

A unique anomaly of the ear: Oculo-auriculo-vertebral spectrum or an isolated disruption?

We present a rare case of a neonate with an isolated congenital condition of his right ear involving the outer ear, middle ear, eustachian tube, and the facial nerve, with an external opening into the skull that connects to the oropharynx. Taking this bizarre aspect of the exterior lesion and the oropharyngeal communication into account, we consider the condition presented here, which to our knowledge is the first of its kind to have resulted from a vascular disruption. Laryngoscope, 1927-1931, 2018.

Aspiration and Dysphagia in the Neonatal Patient.

Dysphagia and aspiration are commonly encountered problems in the neonatal population. It is often multifactorial in nature and management should be tailored to the individual patient. Multiple causes should be considered, including anatomic abnormalities, neurologic/developmental delay, cardiopulmonary disease/infection, and gastroesophageal reflux disease, in addition to those cases where a definitive reason may not be identified. Management should be multidisciplinary in nature and surgical intervention may be indicated in certain populations of patients. Here, we discuss the presentation, workup, and management of the neonatal patient with dysphagia and aspiration.

Oropharyngeal atresia in a preterm infant: a case report and review of the literature.

Oropharyngeal atresia is a rare and often fatal condition that presents soon after birth with severe respiratory distress. We present a case of a premature infant who initially was suspected to have tracheo-esophageal atresia due to prenatal ultrasound findings of polyhydramnios and absent stomach bubble, but was found instead to have oropharyngeal atresia and a complete persistent buccopharyngeal membrane. This case is the first described in which the patient was successfully intubated through a small slit in the persistent membrane.

Direct intralesional ethanol sclerotherapy of extensive venous malformations with oropharyngeal involvement after a temporary tracheotomy in the head and neck: Initial results.

BACKGROUND: The purpose of this study was to evaluate the safety and efficacy of direct intralesional ethanol sclerotherapy for venous malformations (VMs) with oropharyngeal involvement after a temporary tracheotomy. METHODS: A retrospective assessment was carried out to evaluate the efficacy of direct intralesional ethanol sclerotherapy on 21 consecutive patients presenting with extensive VMs involving the oropharynx in the head and neck and who had undergone tracheotomy. RESULTS: Of the 21 patients, 4 were treated once and 17 were treated from 2 to 5 times. The duration of follow-up was, on average, 9.1 months. Of the 21 patients, 7 (33.3%) had complete palliation, whereas the rest (66.7%) achieved partial palliation. Minor complications occurred in 12 of the 21 patients. CONCLUSION: Direct intralesional ethanol sclerotherapy after a temporary tracheotomy is a safe and effective treatment for extensive VMs involving oropharyngeal areas of the head and neck. © 2016 Wiley Periodicals, Inc. Head Neck 39: 288-296, 2017.

Epignathus with oropharynx destruction

Epignathus, is a rare oropharyngeal teratoma arising from the head and neck region. Sporadic cases have been described with associated intracerebral teratoma. Even more infrequent and extraordinary is the circumstance of a teratoma with oropharynx destruction. We describe the case of a fetus with pharyngeal mass that completely destroyed the oral cavity. The histological examination revealed an immature teratoma (G3); only one other G3 case has been described.

Persistent buccopharyngeal membrane in an adult.

Persistence of the buccopharyngeal membrane (BPM) also called the oropharyngeal membrane is a rare congenital oropharyngeal anomaly. We report a case of an adult aboriginal male patient with a membrane that closed his oropharyngeal isthmus except for a 2 cm diameter central perforation. The patient had no symptoms related to this membrane and no other congenital anomalies were found. This finding has not previously been reported in an adult. The embryology and management of this rare condition is discussed.

Systematic Evaluation of the Upper Airway in a Sample Population: Factors Associated with Obstructive Sleep Apnea Syndrome.

OBJECTIVES: To investigate the anatomy of the upper airway (UA) of a representative sample of the adult population of São Paulo city, Brazil, and to identify factors associated with the presence of obstructive sleep apnea syndrome (OSAS), as confirmed using full-night polysomnography (PSG). STUDY DESIGN: Cross-sectional study. SETTING: Population-based sample. METHODS: A 3-stage sampling procedure was used to proportionally recruit adult residents of São Paulo city according to gender, age, and socioeconomic status. A complete evaluation was performed, including a systematic evaluation of the UA prior to conducting PSG. RESULTS: Nine-hundred ninety-three (90.2%) of the participants were seen by an ear, nose, and throat (ENT) specialist. Individuals who were diagnosed with OSAS (32.9%) presented a higher frequency of nasal symptoms and structural abnormalities (both nasal and oropharyngeal) compared with those without OSAS. No anatomical differences were observed in the facial skeleton. An abnormal nasal structure visible via anterior rhinoscopy was the only UA factor predicting OSAS after adjustments for the other common OSAS risk factors (male sex, aging, obesity, and increased neck circumference). CONCLUSION: This is the first study in which a systematic evaluation of the UA was followed by a sleep study in a population-based sample. In a sample of the general population that had not previously been screened for OSAS, having an abnormal nasal structure was found to be a risk factor for OSAS, in conjunction with other well-established clinical and demographic factors, such as male gender, increased age, increased neck circumference, and body mass index.

Obstructive sleep apnea syndrome in a family with Crouzon's syndrome.

Craniofacial anomalies are recognized causes of obstructive sleep apnea syndrome (OSAS) in children. Current literature is limited due to rarity of cases. Furthermore, the mechanism of upper airway obstruction is not clearly understood. We would like to report a family (father and 2 sons) who are suffering from Crouzon's syndrome. The two brothers (ages 1 and 3) were found to have significant obstructive sleep apnea syndrome (OSAS) with failure to thrive. Nasal continuous positive airway pressure (CPAP) markedly improved their OSAS and resulted in accelerated weight gain. The nasoendoscopy and magnetic resonance imaging (MRI) scan taken during natural sleep showed that choanal stenosis, maxillary hypoplasia, posteriorly displaced tongue, lengthened soft palate and adenoid tissues were important in the pathogenesis of upper airways obstruction in Crouzon's syndrome. Nasal CPAP improved airway obstruction by opening a narrow slit as demonstrated by MRI. Our results suggest that OSAS occurred in children with Crouzon's syndrome and that nasal CPAP was a useful treatment modality.

Syndrome of imperforate oropharynx with costovertebral and auricular anomalies.

This is the second report of a syndromic form of imperforate oropharynx associated with costovertebral and auricular anomalies.

Laron syndrome abnormalities: spinal stenosis, os odontoideum, degenerative changes of the atlanto-odontoid joint, and small oropharynx.

BACKGROUND AND PURPOSE: Patients with Laron syndrome have an inborn growth hormone resistance. We investigated abnormalities in the upper airways and cervical spine in patients with Laron syndrome. METHODS: We prospectively examined 11 patients (one child aged 9 years and 10 adults aged 36-68 years), 10 of whom underwent MR imaging of the spine or head; nine, radiography of the cervical spine; and four, CT of C1-C2. The width of the spinal canal was evaluated visually and quantitatively and compared with reference values. The smallest diameter of the oropharynx and the thickness of the palate were measured and compared with reference values. Nine age-matched female patients referred for MR imaging for unrelated reasons served as control subjects. RESULTS: Cervical spinal stenosis was present in seven of the adult patients, within a confidence interval of 95%. Anomaly of the dens compatible with os odontoideum was present in three patients, causing focal myelomalacia in two. The atlanto-odontoid joint showed osteoarthritic changes in six of the adult patients. The mediolateral diameter of the oropharynx was significantly smaller in the patients with Laron syndrome than in the control subjects (P <.005). There was no difference in the thickness of the soft palate. CONCLUSION: Patients with Laron syndrome develop significant narrowing of the cervical spinal canal and early degenerative changes of the atlanto-odontoid joint. Laron syndrome is associated with os odontoideum causing myelomalacia. The dimensions of the oropharynx are small. Patients may be prone to neurologic morbidity and sleep disturbances. Routine MR imaging of the cervical spine is recommended in these patients.

Elective oro-tracheal intubation to diagnose sleep apnea syndrome in children with Down's syndrome and ventricular septal defect.

Three children with Down's syndrome, ventricular septal defect, pulmonary hypertension and clinical evidence of upper airway obstruction had the trachea electively intubated while pulmonary artery pressures were monitored. In two, the pulmonary pressures immediately decreased and systemic arterial oxygen saturations increased. Both of these children showed dramatic clinical improvement after tonsillectomy. These cases indicate that elective tracheal intubation is a valuable diagnostic procedure to demonstrate the role of upper airway obstruction in the maintenance of reversible pulmonary hypertension in children with Down's syndrome and right to left shunts.

Giant hamartoma of the oropharynx.

Giant polypoidal hamartomas of the pharynx and oesophagus are rare benign tumours of unknown origin, exceptionally arising from the oropharynx. We report the case of a 74-year-old man who developed sudden nausea and a foreign body sensation. Shortly afterwards he regurgitated a 25 x 3 x 1.5 cm pedunculated fleshy mass, still attached to the inside of his thorat. The patient was anaesthetised, the mass traced to the right tonsillar fossa and adjacent oropharyngeal wall. The pedicle was clamped and the lesion excised. Histology was consistent with a giant oropharyngeal hamartoma. We discuss the pathogenesis and potential complications of this condition. The literature is reviewed.