A Graceful EXIT impeded by obstetrical complications.

We report an ex utero intrapartum therapy-to-airway procedure in which obstetric factors dramatically influenced the sequence of events necessary to complete the procedure.

Epiglottic aplasia in an infant with Joubert syndrome.

Congenital aplasia of the epiglottis is a rare condition with variable presentation ranging from respiratory distress requiring surgical airway to an asymptomatic finding. Epiglottic aplasia is presumed to be caused by arrest of development of laryngeal structures and is most commonly associated with syndromic conditions, though isolated episodes of aplasia of the epiglottis do exist. In this report, we present a term infant with multiple congenital anomalies who was noted to have a hoarse cry prompting laryngoscopy. This showed complete absence of the epiglottis. Subsequent genetic testing showed mutations in the CPLANE1 gene that is associated with Joubert syndrome. Our patient was able to be discharged home on a thickened formula diet and is eating and gaining weight appropriately. Here, we present a review of the currently available literature of other cases of congenital epiglottic aplasia or hypoplasia discussing the presentation, management and outcomes in these cases.

[EXIT: Experiences from Karolinska University Hopsital]. / EXIT ­ säkraste förlossningsmetod för foster med livshotande luftvägshinder - Samlade erfarenheter från Karolinska universitetssjukhuset.

The main, but not sole, indication for an Ex-utero Intrapartum Treatment (EXIT) delivery is an airway obstruction due to either laryngeal atresia or tumors in the head and neck region. Here we present our Institution's experience with eleven cases: three teratomas, four lymphatic malformations, two laryngeal atresias and two dermoid cysts. The EXIT procedure was used to secure the fetal airway while maintaining uteroplacental gas exchange and fetal hemodynamic stability through the umbilical circulation. Five fetuses required tracheostomy. Only one fetal death occurred due to extensive growth of a teratoma preventing us from establishing an airway. No other fetal or major maternal complication occurred. The EXIT procedure is a complex procedure and these rare cases should be referred to a center with a dedicated and experienced multidisciplinary team.

The Effect of Fibroblast Growth Factors in Grafted Fascia into the Vocal Fold of Rabbits

Abstract Introduction The human larynx is a very important organ for communication. Many conditions lead to scarring of the vocal folds, decreasing voice quality. Objective We aimed to determine whether fibroblast growth factors (FGFs) may influence tissue integration of grafted fascia into the vocal folds of an animal model. Methods This is an experimental animal study with 12 adult rabbits that were submitted to a grafting fragment obtained from superficial cervical fascia into the vocal fold lamina propria, bilaterally. The right vocal fold was injected with FGFs. The animals were sacrificed after 1 month or 12 months, depending on the group they were assigned to, and a histological analysis of their vocal folds was performed.We analyzed the histological changes (such as the presence of fibrosis and neovascularization) induced by the acute or chronic inflammatory reactions. Results The FGFs induced acute inflammatory changes in all animals after 1 month of the initial experiment. The presence of FGFs triggered more fibrosis than the expected due to the surgical procedure itself when compared with the control side of all animals after 12 months of the initial experiment. Conclusions Fibroblast growth factors alone do not represent a good therapeutic option in phonosurgery, since we observed higher levels of fibrosis in the vocal fold lamina propria. Further studies combining more substances may be necessary to elucidate the best option to be used in this kind of surgery. (AU)

Vallecular cyst in the pediatric population: Evaluation and management.

OBJECTIVE: To review the presentation of pediatric vallecular cysts and outline an approach for evaluation and management. METHODS: Medical records of patients diagnosed with vallecular cyst between 2005 and 2017 were reviewed. Data on demographics, clinical characteristics, diagnostic methods, surgical procedures, and outcomes were collected and analyzed. A comprehensive literature search for pediatric cases of vallecular cyst was conducted for comparative analysis. RESULTS: Twenty patients underwent surgery for congenital vallecular cysts during the study period. Age at diagnosis ranged from birth to 8 years (median age = 1.1 years). The most common preoperative symptom was inspiratory stridor (45%) followed by feeding difficulties (40%). Eight patients (40%) initially presented with laryngomalacia and 7 (35%) with feeding difficulties. Imaging was obtained in 16 patients and consisted of plain films, ultrasound, computed tomography (CT), or magnetic resonance imaging (MRI). Marsupialization of the cyst was performed in all 20 patients. Three patients (15%) presented with recurrence. CONCLUSION: Vallecular cysts can cause feeding difficulties due to upper airway obstruction and pressure at the laryngeal inlet. Diagnostic work-up for vallecular cysts should include a detailed medical history, complete head and neck examination including endoscopic examination, and appropriate imaging, as each of these components complements the histopathologic findings. Our findings indicate that performing marsupialization is associated with low rate of recurrence or complication.

[Clinical anatomic study on the segment and adjacent of tract of congenital pyriform sinus fistula].

Objective: To investigate the anatomic tract of congenital pyriform sinus fistula (CPSF). Methods: A total of 90 patients with CPSF undergoing open surgery between August, 2007 and March, 2017 at the Department of Guangdong General Hospital were retrospectively analyzed. Results: The tracts of all the fistulas actually walked far different from those of theoretical ones. A whole fistula may be divided into 4 segments according to adjacent anatomy of CPSF. The posterior inner segment to the thyroid cartilage was initial part of the fistula. It originated from the apex of pyriform sinus, then piercing out of the inferior constrictor of pharynx inferiorly near the inferior cornu of the thyroid cartilage (ICTC), and descended between the lateral branch of the superior laryngeal nerve and the recurrent laryngeal nerve. The ICTC segment was the second part of the fistula, firstly piercing out of the inferior constrictor of pharynx and/or cricothyroid muscle, and then entering into the upper pole of thyroid. The relationship between fistula and ICTC could be divided into three types: type A (medial inferior to ICTC) accounting for 42.2% (38/90); type B (penetrate ICTC) for 3.3% (3/90); and type C (lateral inferior to ICTC) for 54.5% (49/90). The internal segment in thyroid gland was the third part of fistula, walking into the thyroid gland and terminating at its upper pole (92.2%, 83/90) or deep cervical fascia near the upper pole of thyroid (7.8%, 7/90). The lateral inferior segment to thyroid gland was the last part of the fisula, most of which are iatrogenic pseudo fistula, and started from the lateral margin of thyroid gland. Conclusions: CPSF has a complicated pathway. Recognition of the tract and adjacent anatomy of CPSF will facilitate the dissection and resection of CPSF in open surgery.

Dificultad respiratoria y estridor en un lactante de 1 mes por un quiste sacular laríngeo: no todo es laringomalacia / Congenital laryngeal saccular cyst as aetiology of stridor and respiratory distress in an infant. Not everything is laryngomalacia

Introducción: La causa de estridor congénito más frecuente es la laringomalacia, pero también puede originarse a partir de quistes en la vallécula, laringoceles y quistes saculares congénitos, hemangiomas y otras anomalías congénitas de la laringe. Caso clínico: Niña lactante de 41 días de vida, llevada al servicio de urgencias del hospital por presentar un estridor inspiratorio progresivo, con un componente postural de empeoramiento significativo, apneas y dificultad para la alimentación. En la interconsulta urgente con ORL, se realiza una nasofibroscopia, en la que se detecta una tumoración posiblemente quística en el repliegue aritenoepiglótico izquierdo. Se deriva a la paciente al hospital de referencia, donde se confirma la lesión mediante una nueva nasofibrolaringoscopia más tomografía computarizada cervical preoperatoria. Se opera con incisión y marsupialización. El estudio anatomopatológico descartó la presencia de malignidad y confirmó la sospecha diagnóstica. La evolución postoperatoria fue buena. Conclusión: El alto índice de sospecha en las anomalías congénitas de la laringe reviste una gran importancia para realizar un diagnóstico precoz y disminuir la morbimortalidad. El quiste sacular congénito es una lesión inusual, que hay que considerar en el diagnóstico diferencial en los casos de estridor y dificultad respiratoria progresiva en neonatos y lactantes

Introduction: Laryngomalacia is the most common congenital anomaly of the larynx and is also called congenital laryngeal stridor. A less common aetiology of stridor in newborns and infants are laryngocele, congenital laryngeal saccular cyst, hemangiomas, and others congenital anomalies of the larynx. Case report: A 41-days old female presented with progressive stridor, history of postural component of significant worsening, apneas, and feeding difficulty. In the urgent consultation with ENT, flexible fiberoptic laryngoscopy showed a swelling of the left arytenoepiglotic fold. The diagnosis was confirmed at tertiary referral hospital by a cervical CT scan and direct laryngoscopy. The surgical procedure consisted of an incision in the cyst and marsupialization. The histopathological study ruled out malignancy and confirmed the suspected diagnosis. She presented a good postoperative evolution. Conclusion: The high index of suspicion in the congenital anomalies of the larynx is of great importance to make an early diagnosis and decrease morbidity and mortality. The saccular congenital cyst is an unusual lesion. Although rare, it should be included in the differential diagnosis of stridor and progressive obstruction of the airway in neonates and infants

Laryngoscopy and Bronchoscopy in an Infant With a Congenital Laryngeal Web Complicated by Bilateral Pneumothoraces: A Case Report.

Neonates with laryngeal webs pose unique challenges to the anesthesiologist. We present a 2-day-old neonate with aphonia and stridor who underwent microdirect laryngoscopy. Intraoperatively, a Cohen type 4 laryngeal web was diagnosed not immediately amenable to resection. Therefore, the decision was made for endotracheal intubation and subsequent tracheostomy. After endotracheal intubation, there was acute respiratory compromise and oxygen desaturation that improved moderately after urgent tracheostomy. A chest radiograph revealed a large pneumothorax. Our experience suggests that in the presence of high-grade laryngeal webs, the possibility of intraoperative development of pneumothorax should be considered if respiratory difficulties are encountered.

Congenital Vallecular Cyst: ARare and Potentially Lethal Condition.

Congenital vallecular cyst is a rare laryngeal lesion, accounting for 10-20% of all laryngeal cysts, with a potential to cause severe upper airway obstruction, which can be fatal. It can cause stridor, apnea, cyanosis, respiratory distress, and feeding difficulties. Diagnosis requires a high level of clinical suspicion and helps in timely intervention. Direct laryngoscopy is gold standard for definitive diagnosis. Treatment options include aspiration, marsupialization, and surgical excision. Here, we report a case of 7-week-old infant with complain of recurrent episodes of cyanosis when agitated, since the age of 3 weeks, admitted with impression of apparent life-threatening events (ALTEs). Extensive investigations were non-conclusive, which were done in local hospital. Direct laryngoscopy was performed in our hospital and showed presence of a vallecular cyst. Thus complete excision of cyst was done in the same setting with dramatic relief of symptoms.

[A case of congenital cyst of epiglottis caused by severe upper respiratory tract obstruction].

The main clinical manifestations were respiratory distress，perioral cyanosis and Upper respiratory obstruction symptoms.The epiglottis opened by laryngoscope,can see a gray white semi elliptical sacs.Clinical diagnosis:congenital cyst of epiglottis.

Airway tissue engineering for congenital laryngotracheal disease.

Regenerative medicine offers hope of a sustainable solution for severe airway disease by the creation of functional, immunocompatible organ replacements. When considering fetuses and newborns, there is a specific spectrum of airway pathologies that could benefit from cell therapy and tissue engineering applications. While hypoplastic lungs associated with congenital diaphragmatic hernia (CDH) could benefit from cellular based treatments aimed at ameliorating lung function, patients with upper airway obstruction could take advantage from a de novo tissue engineering approach. Moreover, the international acceptance of the EXIT procedure as a means of securing the precarious neonatal airway, together with the advent of fetal surgery as a method of heading off postnatal co-morbidities, offers the revolutionary possibility of extending the clinical indication for tissue-engineered airway transplantation to infants affected by diverse severe congenital laryngotracheal malformations. This article outlines the necessary basic components for regenerative medicine solutions in this potential clinical niche.

Familial Sulcus Vergeture: Further Evidence for Congenital Origin of Type 2 Sulcus.

A 29-year-old otherwise healthy woman presented with a lifetime history of hoarseness because it had begun to interfere with her career. Examination of both the woman and her 60-year-old father revealed bilateral sulcus vergeture, without inflammation or lesions attributable to phonotrauma. The woman responded well to injection augmentation; the father declined treatment. Combined with existing descriptions of other family groupings, all with sulcus vergeture without signs of inflammation, clinical progression, and little or no apparent behavioral component, this report further suggests that sulcus vergeture (Ford type 2) and sulcus vocalis (Ford type 3) are entirely different entities, despite architectural similarity.

The clinical characteristics of congenital laryngeal saccular cysts.

CONCLUSION: The first step when treating newborns and infants with hoarseness and dyspnea is to consider the possibility of CLSCs. The appropriate operation can then be chosen based on the cyst sub-type. Complete resection of the cyst wall is the key to preventing recurrence. BACKGROUND: A laryngeal saccular cyst is an unusual congenital lesion that appears clinically during the neonatal period or early infancy. Because few reports on congenital laryngeal saccular cysts (CLSCs) exist, this study investigated the clinical features of patients with a history of CLSCs to determine the clinical characteristics of this disease. METHODS: The data from 28 CLSC patients admitted to Beijing Tongren Hospital from July 2004 to September 2014 were reviewed. Gender, age at onset of symptoms, number of surgical procedures performed, effect of the operation, and CLSC classification were analyzed. RESULTS: CLSCs accounted for 0.79% of laryngeal operations during the study period. Of the cases examined, 35.7% (10/28) had first been treated as laryngomalacia. With respect to cyst type, 17.9% (5/28) were anterior laryngeal saccular cysts, and 82.1% (23/28) were lateral saccular cysts. The patients underwent a total of 53 surgeries, including 21 procedures performed at other hospitals. The time to recurrence of the cysts following needle aspiration ranged from 5-10 days. The time to recurrence after the roof of the cyst was excised ranged from 1-10 months. There were no recurrences after the complete resection of the cyst wall using a CO2 laser and microsuturing of the wound surfaces via an endoscopic procedure, which is much better than the 41% of endoscopic de-roofing cases which recurred reported by Mitchell et al.

Primer caso de tratamiento ex utero intraparto realizado por vía vaginal en un feto con síndrome de obstrucción congénita de la vía aérea superior / The first case of exutero intrapartum treatment performed using the vaginal route in a fetus with congenital high airway obstruction syndrome

El síndrome de obstrucción congénita de la vía aérea superior (CHAOS), es una condición infrecuente que causa asfixia o muerte perinatal inmediata, de no mediar una estrategia terapéutica que permita permeabilizar la vía aérea del paciente durante el nacimiento. El diagnóstico prenatal, es fundamental para delinear estrategias de tratamiento perinatal con el fin de minimizar la morbimortalidad de niños con anomalías congénitas. El tratamiento ex-útero intraparto (EXIT) es el procedimiento de elección. Clásicamente se realiza mediante una cesárea programada, manteniendo el soporte fetal a través de la circulación útero-placentaria. Se requiere un equipo altamente calificado y un trabajo coordinado para concretar el procedimiento en estas condiciones. Objetivo: El objetivo es reportar un caso de Síndrome de CHAOS, en el que se realizó un procedimiento EXIT en un niño nacido por parto vaginal, con la participación de un equipo multidisciplinario de profesionales de dos Instituciones Públicas de la Ciudad de Buenos Aires, en el marco de un Programa Conjunto de Diagnóstico y Tratamiento Fetal (AU)

Congenital high airway obstruction syndrome (CHAOS) is a rare entity causing perinatal asphyxia or immediate death if no therapeutic strategy is undertaken to correct airway patency at birth. Prenatal diagnosis is essential to plan perinatal strategies to decrease morbidity and mortality in children with congenital anomalies. The exutero intrapartum treatment (EXIT) is the procedure of choice. Classically, a programmed cesarean section is performed while the fetus is maintained on uteroplacental circulation. A highly trained team is required in the coordinated effort to perform the procedure. Aim: The aim of this study was to report on a case of CHAOS managed with an EXIT procedure in a child born through vaginal delivery performed by a multidisciplinary team of professionals belonging to two public institutions of the city of Buenos Aires in the framework of the Joint Program of Fetal Diagnosis and Treatment (AU)