[Transnasal endoscopic surgery of choanal atresia after radiotherapy for nasopharyngeal carcinoma].

Objective: To discuss the effect of endoscopic dilatation and plasty for choanal atresia after radiotherapy for nasopharyngeal carcinoma. Methods: Nineteen patients with choanal atresia who were admitted to the Department of Otorhinolaryngology Head and Neck Surgery of the First Affiliated Hospital of Nanchang University from Jan. 2011 to Dec. 2018 were reviewed, with 12 males and 7 females aging from 33 to 59 years old. All of the patients had a history of radiotherapy for nasopharyngeal carcinoma and were confirmed by electronic nasopharyngoscope and nasopharyngeal imaging. Among 19 patients, there were 3 cases of unilateral occlusion and 16 cases with bilateral atresia, and all of them were membranous atresia. All patients received the transnasal endoscopic surgery of resecting partial vomer bone while trying to keep normal mucosa tissues and using the septonasal mucoperiosteal flap to repair under general anesthesia. One week after operation, the patients were told to do physiological saline nasal irrigation and received regular clean and observation under endoscope. Descriptive statistical method was used to analyze the outcome. Results: The patients were followed up for 1 year postoperatively by electronic nasopharyngoscopic examination. There was no failure case in all the 19 patients including 16 patients with a wide choana and 3 patients had narrowing of the choana (<50%), with adequate and satisfactory airway. Conclusions: Transnasal endoscopic surgery was an effective treatment for choanal atresia after radiotherapy for nasopharyngeal carcinoma. Application of septonasal mucoperiosteal flap for repairing nasal and nasopharyngeal mucosa would avoid recurrence.

Use of a novel surgical approach for treatment of complete bilateral membranous choanal atresia in an alpaca cria.

CASE DESCRIPTION A 4-hour-old 6.3-kg (13.9-lb) female alpaca cria was evaluated because of severe respiratory distress and difficulty nursing since birth. CLINICAL FINDINGS The cria had open-mouth breathing and cyanotic membranes, with no airflow evident from either nostril. Supplemental oxygen was delivered, and the patient was anesthetized and intubated orotracheally; a CT evaluation of the head confirmed bilateral membranous obstruction of the nasal cavities, consistent with complete bilateral choanal atresia. TREATMENT AND OUTCOME Choanal atresia was treated with an endoscopically assisted balloon-dilation technique, and temporary tracheostomy was performed. Stenosis recurred, requiring revision of the repair and intranasal stent placement 3 days after the first surgery. The tracheostomy tube was removed the next day. Complications during hospitalization included mucoid obstruction of the tracheostomy tube, granulation tissue development in the trachea near the tracheostomy site, mucoid stent obstruction, aspiration pneumonia, and presumed partial failure of passive transfer of immunity. The stents were removed 2 weeks after admission, and the cria was discharged 3 days later. The owner was advised that the animal should not be bred. At last follow-up 3 years later, the alpaca was doing well. CLINICAL RELEVANCE Surgical treatment with a balloon-dilation technique and placement of nasal stents with endoscopic guidance were curative in this neonatal alpaca with bilateral membranous choanal atresia. Computed tomography was useful to determine the nature of the atresia and aid surgical planning. Because a genetic component is likely, owners should be advised to prevent affected animals from breeding.

[Malignant infantile osteopetrosis revealed by choanal atresia: A case report]. / Ostéopétrose maligne infantile révélée par une atrésie des choanes : à propos d'un cas.

Malignant infantile osteopetrosis is a rare genetic disease characterized by increased bone density due to osteoclastic dysfunction. We report on the case of a 3-month-old girl who was referred to our hospital by the ENT department for severe anemia in the context of bilateral choanal atresia. Clinical examination showed failure to thrive, anemia, respiratory distress, bilateral choanal atresia, and chest deformation. The abdomen was soft with large hepatosplenomegaly. We noted a lack of eye tracking, no optical-visual reflexes, and left nerve facial paralysis. The blood count showed normocytic normochromic anemia with severe thrombocytopenia. The infectious work-up and blood smears were negative. The skeleton X-ray showed diffuse bone densification of the skull, long bones, pelvis, vertebrae, and ribs. The facial bone CT confirmed membranous choanal atresia. The molecular biology search for the TCIRG1 gene mutation was not available. The patient had supportive treatment (transfusion, oral steroid, vitamin D, oxygen, nutrition). Bone marrow transplantation was indicated but not available. She died at 6 months in a context of severe anemia and bleeding. Malignant infantile osteopetrosis is rare and symptoms are nonspecific. Diagnosis should be considered in young infants presenting refractory anemia, particularly in the context of choanal atresia. Bone marrow transplantation remains the only curative treatment.

[Transnasal endoscopic choanal plasty for repairing congenital choanal atresia].

OBJECTIVE: To observe the effect of transnasal endoscopic choanal plasty for repairing congenital choanal atresia. METHOD: A retrospective analysis of 15 patients with choanal atresia in Beijing Children's Hospital from January 2006 to September 2012 was made. All the 15 patients accepted electronic nasopharyngoscope examination and CT scanning of the paranasl sinus. The age was between 4 years old and 6 years old. Seven of them were male and 8 were female. Six cases were unilateral atresia, 9 were bilateral atresia. Ten cases (16 sides) were membranous atresia, 2 cases (4 sides) were mixed type atresia and 3 cases (4 sides) were bony atresia. All the patients accepted transnasal endoscopic choanal plasty. A tube was placed in order to support the enlarged nostrils. The nasal tube should be retained for 3 months and kept patency by routine care. Three months after operation, the tube could be removed. The patients were told to do physiological saline nasal irrigation and receive regular review by transnasal endoscopy. RESULT: The patients were followed up for 1 year postoperatively by electronic nasopharyngoscope examination. There was no failure case in all the 15 patients. The total effective rate was 100%. CONCLUSION: Transnasal endoscopic choanal plasty had better efficacy and safety in the treatment of congenital choanal atresia.

Transnasal endoscopic choanal plasty for repairing congenital choanal atresia / 中华耳鼻咽喉头颈外科杂志

<p><b>OBJECTIVE</b>To observe the effect of transnasal endoscopic choanal plasty for repairing congenital choanal atresia.</p><p><b>METHOD</b>A retrospective analysis of 15 patients with choanal atresia in Beijing Children's Hospital from January 2006 to September 2012 was made. All the 15 patients accepted electronic nasopharyngoscope examination and CT scanning of the paranasl sinus. The age was between 4 years old and 6 years old. Seven of them were male and 8 were female. Six cases were unilateral atresia, 9 were bilateral atresia. Ten cases (16 sides) were membranous atresia, 2 cases (4 sides) were mixed type atresia and 3 cases (4 sides) were bony atresia. All the patients accepted transnasal endoscopic choanal plasty. A tube was placed in order to support the enlarged nostrils. The nasal tube should be retained for 3 months and kept patency by routine care. Three months after operation, the tube could be removed. The patients were told to do physiological saline nasal irrigation and receive regular review by transnasal endoscopy.</p><p><b>RESULT</b>The patients were followed up for 1 year postoperatively by electronic nasopharyngoscope examination. There was no failure case in all the 15 patients. The total effective rate was 100%.</p><p><b>CONCLUSION</b>Transnasal endoscopic choanal plasty had better efficacy and safety in the treatment of congenital choanal atresia.</p>

Comentarios al artículos "Uso de guía metálica para intubación traqueal mediante fibroscopia en un neonato con Síndrome de Treacher Collons y atresia de coanas" / No disponible

No disponible

Protein tyrosine phosphatase PTPN14 is a regulator of lymphatic function and choanal development in humans.

The lymphatic vasculature is essential for the recirculation of extracellular fluid, fat absorption, and immune function and as a route of tumor metastasis. The dissection of molecular mechanisms underlying lymphangiogenesis has been accelerated by the identification of tissue-specific lymphatic endothelial markers and the study of congenital lymphedema syndromes. We report the results of genetic analyses of a kindred inheriting a unique autosomal-recessive lymphedema-choanal atresia syndrome. These studies establish linkage of the trait to chromosome 1q32-q41 and identify a loss-of-function mutation in PTPN14, which encodes a nonreceptor tyrosine phosphatase. The causal role of PTPN14 deficiency was confirmed by the generation of a murine Ptpn14 gene trap model that manifested lymphatic hyperplasia with lymphedema. Biochemical studies revealed a potential interaction between PTPN14 and the vascular endothelial growth factor receptor 3 (VEGFR3), a receptor tyrosine kinase essential for lymphangiogenesis. These results suggest a unique and conserved role for PTPN14 in the regulation of lymphatic development in mammals and a nonconserved role in choanal development in humans.

Choanal atresia: current concepts and controversies.

PURPOSE OF REVIEW: Choanal atresia is a common and widely recognized craniofacial disorder characterized by obliteration of the posterior nasal aperture. Given the long time since its original description, controversy persists regarding pathogenesis and optimal surgical techniques. This review addresses current literature on choanal atresia and identifies areas of debate and future opportunities in research. RECENT FINDINGS: Recent molecular mechanisms in retinoic acid receptor development have been described in the pathogenesis of choanal atresia. Whereas surgical treatment is generally believed to be effective in alleviating respiratory symptoms, consistent data confirming efficacy are scarce regarding best surgical approach with and without endoscopic sinus techniques, adjuvant use of stents, use of antiproliferative agents and laser-assisted surgery. Recent studies regarding each technique are discussed. SUMMARY: Despite vigorous research, the pathogenesis remains elusive and unproven. Many surgical techniques have been advocated; however, there is no dominant approach. Trends in treatment are directed towards the use of highly advanced endoscopic approaches with the use of microdebriders, small drill bits and telescopes to minimize traumatic injury that leads to postoperative scarring and restenosis.

Choanal atresia: an unusual serious complication of complementary and alternative medical treatment.

The use of complementary and alternative medicine (CAM) is common among patients with chronic, long-standing nasal disease. Complications resulting from inappropriate CAM practices are sometimes reported, but serious complications such as choanal atresia and stenosis are rare. We report a case of choanal atresia and stenosis due to herbal drug (ie, chemical) cauterization of the nasal cavity in a 39-year-old man. We reviewed the literature on why patients previously given conventional treatment seek alternative therapies. The patient underwent surgical intervention to relieve choanal atresia and stenosis. The postoperative course was unremarkable, and follow-up for 1 year revealed no evidence of restenosis. Choanal atresia and stenosis resulting from CAM treatment are rare. Otolaryngologists should be aware of possible complications occurring in patients receiving such treatments.

Unilateral rhinorrhea and nasal obstruction in an adult.

BACKGROUND: choanal atresia is an uncommon malformation, consisting of congenital blockage of one of both choanae. Symptomatology varies according to the type of atresia and the age of the patient. The bilateral, or complete, form presents in newborns as respiratory distress while unilateral, or incomplete, forms present in older children or adults. CASE REPORT: a 16-year-old girl presented with a lifelong history of right-sided nasal blockage and unilateral watery rhinorrhea. The rhinorrhea increased when the patient bent her head forward. Treatment with antihistamines and topical nasal corticosteroid sprays produced little improvement. The patient's previous medical history was unremarkable except for the expulsion of a coagulated mass through the right nostril a few days after birth, and event that provoked her symptoms. Physical examination was normal except for excoriation of the right external nasal margin. Anterior rhinoscopy revealed watery rhinorrhea, erythematous mucosa in the right nasal cavity and deviated nasal septum. No nasal masses or polyps were found. Physical examination confirmed the absence of ventilation in the right nasal airway and nasal endoscopy indicated right choanal obstruction. DIAGNOSTIC TEST: skin prick tests with aeroallergens were positive for grass pollen and negative for all other allergens tested (mites, moulds, latex, and epithelia). Total IgE was normal (9 IU/ml). No eosinophilia was detected in the nasal secretion specimen. The results of laboratory tests including complete blood count and differential, erythrocyte sedimentation rate, and biochemical parameters were normal. The glucose level in nasal secretion was 4.6 mg/dl. X-rays of the paranasal sinuses and cavum showed no anomalies. Axial fine-cut computed tomography scans (3 3 mm) revealed choanal obstruction with bony and membranous components. CONCLUSIONS: a diagnosis of congenital choanal atresia should be considered in cases of unilateral blockage and rhinorrhea. Nasal endoscopy and computed tomography scans are essential to establish diagnosis.

Unilateral rhinorrhea and nasal obstruction in an adult

Background: choanal atresia is an uncommon malformation, consisting of congenital blockage of one of both choanae. Symptomatology varies according to the type of atresia and the age of the patient. The bilateral, or complete, form presents in newborns as respiratory distress while unilateral, or incomplete, forms present in older children or adults. Case report: a 16-year-old girl presented with a lifelong history of right-sided nasal blockage and unilateral watery rhinorrhea. The rhinorrhea increased when the patient bent her head forward. Treatment with antihistamines and topical nasal corticosteroid sprays produced little improvement. The patient's previous medical history was unremarkable except for the expulsion of a coagulated mass through the right nostril a few days after birth, and event that provoked her symptoms. Physical examination was normal except for excoriation of the right external nasal margin. Anterior rhinoscopy revealed watery rhinorrhea, erythematous mucosa in the right nasal cavity and deviated nasal septum. No nasal masses or polyps were found. Physical examination confirmed the absense of ventilation in the right nasal airway and nasal endoscopy indicated right choanal obstruction. Diagnostic test: skin prick tests with aeroallergens were positive for grass pollen and negative for all other allergens tested (mites, moulds, latex, and epithelia). Total IgE was normal (9 IU/ml). No eosinophilia was detected in the nasal secretion specimen. The results of laboratory tests including complete blood count and differential, erythrocyte sedimentation rate, and biochemical parameters were normal. The glucose level in nasal secretion was 4.6 mg/dl. X-rays of the paranasal sinuses and cavum showed no anomalies. Axial fine-cut computed tomography scans (3 ¥ 3 mm) revealed choanal obstruction with bony and membranous components. Conclusions: a diagnosis of congenital choanal atresia should be considered in cases of unilateral blockage and rhinorrhea. Nasal endoscopy and computed tomography scans are essential to establish diagnosis (AU)

Introducción: la atresia de coanas es una malformación infrecuente que consiste en una obstrucción congénita de una o ambas coanas. La sintomatología varía según el tipo de atresia y la edad del paciente. La forma bilateral completa se manifiesta en el recién nacido con un distrés respiratorio, mientras que las formas unilaterales o incompletas pueden diagnosticarse en niños más mayores o adultos.Caso clínico: mujer de 16 años de edad, enviada para estudio por presentar una historia de larga evolución de obstrucción nasal derecha y rinorrea acuosa unilateral. La rinorrea aumentaba al inclinar la cabeza hacia delante. Poca mejoría a pesar de tratamiento con antihistamínicos y corticoides tópicos. No refería antecedentes personales de interés salvo la expulsión de un coágulo a través de la fosa nasal derecha pocos días después del nacimiento a raíz del cual comenzaron los síntomas. La exploración clínica era normal salvo excoriación del vestíbulo nasal derecho.La rinoscopia anterior mostraba rinorrea acuosa, mucosa nasal eritematosa en la fosa nasal derecha y desviación del tabique nasal. No se observaron masas ni pólipos. El examen clínico permitió confirmar la usencia de ventilación en la fosa nasal derecha y la exploración mediante endoscopia nasal reveló la obstrucción de la coana derecha.Pruebas diagnósticas: los tests cutáneos con neumoalergenos inhalantes fueron positivos para pólenes de gramíneas y negativos para el resto de los alergenos testados (ácaros, hongos, epitelios y látex). IgE total normal (9 UI/ml). Eosinofilia nasal negativa. Hemograma, VSG y parámetros bioquímicos normales. Cifras de glucosa en la secreción nasal 4,6 mg/dl (normal). Rx de senos paranasales y cavum sin hallazgos. TAC de senos paranasales de cortes finos (3 3) demuestra la obstrucción de la coana derecha que presenta un componente óseo y membranoso.Conclusiones: el diagnóstico de la atresia congénita de coanas unilateral debe ser considerado en los casos de obstrucción nasal y rinorrea unilaterales. La endoscopia nasal y la TAC de senos paranasales son fundamentales para el diagnóstico (AU)

Magnesium deficiency as a cause of hypocalcemia in the CHARGE association.

OBJECTIVE: To discover the mechanism of hypocalcemia in a patient with the CHARGE (coloboma, heart disease, atresia choanae, retarded growth and development, genital hypoplasia, and ear anomalies) association. RESEARCH DESIGN: Chemical and metabolic studies of serum, urine, stool, and muscle specimens. SETTING: A university hospital affiliated with a municipal hospital. PARTICIPANT: One patient with the CHARGE association and refractory hypocalcemia. MEASUREMENTS AND RESULTS: Serum calcium level was 0.91 mmol/L (reference range, 2.20 to 2.58 mmol/L) and serum magnesium level was 0.34 mmol/L (reference range, 0.80 to 1.20 mmol/L) prior to any therapy. After parenteral calcium and magnesium therapy, hypocalcemia persisted (1.46 mmol/L), while the serum magnesium level was 0.84 mmol/L. A needle biopsy of skeletal muscle tissue for the magnesium content confirmed a total magnesium deficiency despite normomagnesemia (muscle magnesium content, 517 micrograms/g [reference range, 800 to 1100 micrograms/g]). Magnesium deficiency was secondary to secretion of magnesium into the gastrointestinal tract (with a daily magnesium intake of 190 mg, a 24-hour nondiarrheal fecal excretion of magnesium was 2019 mg/kg [reference range, < 1000 mg/kg of stool weight]). INTERVENTIONS: Hypocalcemia was corrected only after 7 weeks of continual parenteral magnesium supplements to replenish the tissue magnesium deficiency. CONCLUSIONS: Patients with the CHARGE association often have hypocalcemia. Magnesium deficiency (with or without hypomagnesemia) is a cause of the hypocalcemia in at least one patient and may prove of significance in others.

Cocaine toxicity in an infant following intranasal instillation of a four percent cocaine solution.

An 11-week-old infant experienced toxicity following the intranasal instillation of a four percent cocaine solution in preparation for an examination to rule out choanal stenosis. The child recovered fully but required the use of anticonvulsants and admission to the hospital. To our knowledge, this is the first case report where cocaine toxicity was experienced following this routine practice. We present our case and a review of the literature involving other instances where there have been complications with the use of topical cocaine. This report should alert the medical community to the potential complications of using topical cocaine for its anesthetic properties.