Endoscopic cricopharyngeal myotomy in infants.

Cricopharyngeal achalasia is a rare cause of dysphagia in young children. Conservative treatments involve medical therapy and serial dilations or injections. Definitive cure can be achieved with cricopharyngeal myotomy. We report two cases of young children, each less than one year of age, who underwent endoscopic laser-assisted cricopharyngeal myotomy. Following surgery, both infants were able to advance to age appropriate diets prior to discharge. Post-operative modified barium swallow demonstrated complete resolution of achalasia in both patients. To the best of our knowledge, this is the first case series of infants undergoing successful endoscopic cricopharyngeal myotomy.

Endoscopic cricopharyngeal myotomy for management of cricopharyngeal achalasia (CA) in an 18-month-old child.

A 6-month-old patient presented with dysphagia and failure to thrive. Video fluoroscopic swallow study (VFSS), esophagogastroduodenoscopy, and manometry were diagnostic for CA. A gastrostomy tube was placed at 8 months. Botulinum toxin injection improved symptoms, but within 10 weeks symptoms returned. At 18 months, an uncomplicated endoscopic CPM was performed. A postoperative VFSS demonstrated cricopharyngeal bar resolution. Within 3 months, patient was feeding orally without a G tube. Pediatric CPA treatment options consist of dilation, botox, and transcervical CPM. To our knowledge, this is the youngest patient treated with endoscopic CPM. Intraoperative video and photographs are presented.

Urgencias en trastornos del movimiento que cursan con rigidez / Movement disorder emergencies accompanied by rigidity

Introducción. En el año 2002 se definieron las urgencias en trastornos del movimiento como cualquier trastorno neurológico, agudo o subagudo, en el que la presentación clínica está dominada por un trastorno del movimiento primario, y donde un fallo en el diagnóstico precoz puede resultar en una morbimortalidad importante. En este trabajo se revisarán aquellas urgencias en trastornos del movimiento que cursan con rigidez. En primer lugar las distonías agudas, siguiendo con el Síndrome Neuroléptico Maligno y por último una miscelánea de patologías. Objetivo. Revisar los avances más notorios publicados en la literatura científica en los últimos años en aquellas patologías que cursan con rigidez. Desarrollo. Se ha revisado la literatura de los últimos años y se presentan los avances más significativos en la patogenia, diagnóstico, y tratamiento, así como las principales perspectivas futuras en dichos campos (AU)

Introduction. In 2002 movement disorder emergencies were defined as any neurological disorder, either acute or subacute, in which the clinical presentation is dominated by a primary movement disorder, and where misdiagnosis in the early stages can result in important morbidity and mortality rates. This work reviews those movement disorder emergencies that are accompanied by rigidity. These are considered in the following order: first, acute dystonias, followed by neuroleptic malignant syndrome and, lastly, an assortment of other pathologies. Aims. The purpose of this study is to review the most significant advances in pathologies accompanied by rigidity that have recently been reported in the scientific literature. Development. The literature from the last few years was reviewed and we present the most significant advances in pathogenesis, diagnosis and treatment, as well as the main future perspectives in those fields (AU)

The use of botulinum toxin for pediatric cricopharyngeal achalasia.

OBJECTIVES: Cricopharyngeal achalasia is an uncommon cause of feeding difficulties in the pediatric population, and is especially rare in infants. Traditional management options include dilation or open cricopharyngeal myotomy. The use of botulinum toxin has been preliminarily reported for cricopharyngeal achalasia in children as a modality for diagnosis and management. This study describes the use of botulinum toxin as a definitive treatment for pediatric cricopharyngeal achalasia. METHODS: A retrospective analysis was performed of three patients who were diagnosed with cricopharyngeal achalasia and underwent botulinum toxin injections to the cricopharyngeus muscle. The charts were reviewed for etiology, botulinum toxin dosage delivered, length of follow-up, postoperative need for nasogastric tube placement, and swallow studies. RESULTS: A total of 7 botulinum toxin injections into the cricopharyngeus muscle were performed in three infants with primary cricopharyngeal achalasia between April 2006 and February 2011. Mean dosage was 23.4 units per session (range: 10-44 units), or 3.1 U/kg (range: 1.4-5.3 U/kg). Mean interval period between injections was 3.3 months (range: 2.7-4.0 months). Mean follow-up period was 22.1 months (range: 3.4-44.5 months). One patient required hospital readmission after injection for presumed aspiration but recovered without need for surgical intervention. No long-term complications were noted post-operatively. All patients improved clinically and ultimately had their nasogastric feeding tubes removed. CONCLUSIONS: Botulinum toxin appears to be a safe and effective option in the management of primary cricopharyngeal achalasia in children, and may prevent the need for myotomy.

Lateral congenital anomalies of the pharyngeal apparatus: part III. cadaveric representation of the course of second and third cleft and pouch fistulas.

"Stepladder" surgery for fistula from second or third pharyngeal cleft and pouch is "blind." Neither intraoperative methylene blue injection and probing nor preoperative imaging (fistulogram ultrasound, computed tomography, magnetic resonance imaging) reveal three-dimensional anatomic relations of fistulas. This article describes the most common second and third fistula courses and demonstrates representation of their tracts with wires in human cadavers. A second cleft and pouch fistula, at its external opening, pierces superficial cervical fascia (and platysma), then investing cervical fascia, and travels under the sternocleidomastoid muscle, superficial to the sternohyoid and anterior belly of omohyoid. It ascends along the carotid sheath, and at the upper border of the thyroid cartilage it pierces the pretracheal fascia. Characteristically, it courses between the carotid bifurcation and over the hypoglossal nerve. After passing beneath the posterior belly of the digastric muscle and the stylohyoid, it hooks around both glossopharyngeal nerve and stylopharyngeus muscle. The fistula reaches the pharynx below the superior constrictor muscle. The course of a third cleft and pouch fistula is similar until it has pierced pretracheal fascia; then it passes over the hypoglossal nerve and behind the internal carotid, finally descending parallel to the superior laryngeal nerve, reaching the thyrohyoid membrane cranial to the nerve.

Cricopharyngeal myotomy for primary cricopharyngeal dysfunction caused by a structural abnormality localized in the cricopharyngeus muscle: report of a case.

Primary cricopharyngeal dysfunction (PCD) is a rare idiopathic disorder of the upper esophageal sphincter (UES), characterized by oropharyngeal dysphagia, frequent aspiration, and narrowing at the level of the UES. Cricopharyngeal myotomy (CPM) has been used to treat oropharyngeal dysphagia of different causes including anatomic, neuromuscular, iatrogenic, inflammatory, neoplastic, and idiopathic; however, the indications for CPM and predictors of its outcome are not clearly defined. We report a case of PCD with hypertonic UES caused by a structural abnormality localized in the cricopharyngeus muscle, visualized as a cricopharyngeal bar, which we treated successfully by CPM, achieving long-term relief.

Congenital cricopharyngeal achalasia in a 4.5-year-old managed by cervical myotomy: a case report.

INTRODUCTION: Congenital cricopharyngeal achalasia (CCA) is a rare disorder in children characterized by inappropriate contraction of the cricopharyngeus muscle, resulting in the inability to relax the upper esophageal sphincter during deglutition. We report the diagnostic process and management of a relatively older patient who underwent cricopharyngeal myotomy at the age of 4.5 years. METHODS: A retrospective review of the case and clinical follow-up was performed. RESULTS: This young patient had a long history of dysphagia, choking, nasal reflux and recurrent pneumonia and croup since birth and was diagnosed with CCA at 22 months of age. She underwent balloon dilation of the cricopharyngeus muscle shortly thereafter with only transient relief of her symptoms of feeding difficulty (choking and aspiration). The parents were reluctant for her to undergo further interventions until 2 years later when they consented to cricopharyngeal myotomy. She underwent transcervical myotomy at age 4.5 years and had complete relief of her symptoms. She had no post-operative complications and has done well for nearly 12 months following myotomy. DISCUSSION: Our patient is one of the oldest children reported to have undergone myotomy, recovered quickly, and had no difficulty swallowing at any time following surgery. We suggest transcervical cricopharyngeal myotomy as the preferred treatment due to its lasting effects and repeated success in relieving dysphagia in young patients with CCA.

Obstructive sleep apnea in an infant with prominent glossopharyngeal muscles: therapy and outcome.

We present a case of prominent glossopharyngeal muscles causing severe obstructive symptoms in a 3-month-old child. Preoperative sleep studies and dynamic microdirect laryngoscopy were used in evaluation of the patient. Postoperative sleep studies confirmed a substantial reduction in apneic events after release of the muscle folds. The literature addresses the variant anatomy, and treatment options are discussed. To our knowledge, this is the first case report of prominent glossopharyngeal muscles leading to apneic events. We propose that in patients with prominent glossopharyngeal folds, surgical release may significantly improve symptoms of sleep apnea and in severe cases prevent tracheostomy.

[Nasendoscopic comparison for cleft palate repair after velopharyngeal muscle reconstruction].

OBJECTIVE: To find out the nasendoscopic changes of velopharyngeal configuration and movement after palatoplasty with or without velopharyngeal muscle reconstruction. METHODS: The nasendoscopy was taken in forty-one patients with palatoplasty, 22 repaired by velopharyngeal muscle reconstruction and 19 with modified von Langenbeck's procedure (non-reconstructive group). RESULTS: In patients with velopharyngeal muscle reconstruction, the velopharyngeal ports are smooth and full with a definite reduction in size than patients without velopharyngeal muscle reconstruction. During phonation, the complete and marginal velopharyngeal competence rate in reconstructive group (90.91%) is higher than the group of non-reconstruction (37.31%) The major velopharyngeal closure is circular movement in reconstructive group, otherwise coronal closure in nonconstructive group. CONCLUSIONS: Based the observation of nasendoscopy, the velopharyngeal muscle reconstruction in palatoplasty has more definite improvement to velopharyngeal closure than non-reconstructive procedure. Palatoplasty with velopharyngeal muscle reconstruction could reduce the size of velopharyngeal port and make the complete velopharyngeal closure easier.

[Variations of the constrictor pharyngeal muscles in humans].

Fifteen halves of ten nonrandomized human cadavers were dissected under a stereoscopic microscope in order to examine the existence of variations in the constrictor pharyngeal muscles. Obvious variations of the constrictor muscles were found in three cases. The results are summarized as follows: 1. In the first case of variations, the superior constrictors arose from the pterygomandibular raphe and merged into the outer surfaces of the middle and inferior constrictors. This muscle was supplied by the upper part of the pharyngeal branches of the vagus. 2. In the second case, the muscle bundle of inferior constrictors originated from the thyroid cartilage and passed upward, joining the muscular fibers of the outer surface of the superior constrictors arising from the pterygomandibular raphe. Some of these muscular fibers extended to the surface of the posterior part of the pharyngobasilar fascia. This muscle was innervated by the lower part of the pharyngeal branches of the vagus. 3. In the third case, the lower part of the superior constrictors passed laterally between the stylopharyngeus and the glossopharyngeal nerve, running between the external and internal carotids in the retrostyloid space and reaching the cervical fascia around the submandibular triangle. This anomalous muscular bundle of superior constrictors was innervated by the carotid branch of the glossopharyngeal nerve and the middle part of the pharyngeal branches of the vagus.