Surgical Treatment of Velopharyngeal Insufficiency

Velopharyngeal insufficiency (VPI) is a condition where the soft palate and posterior oropharynx fail to close adequately, leading to complications such as abnormal speech, nasal regurgitation and nasal emission. Although there exist many approaches to treating VPI depending on the shape and severity of the insufficiency, this chapter describes the three most frequently used and well-researched techniques: the Furlow Palatoplasty (double-opposing Z-palatoplasty), the creation and placement of a pharyngeal flap, and a sphincter pharyngoplasty. This chapter contains an introduction to VPI causes and treatment, a description of patient assessment methods, step-by-step instructions for the different operative procedures, and the recovery process.

Acute-onset isolated bilateral palatal palsy in an adolescent

A 16-year-old patient presented with sudden-onset difficulty in swallowing food especially for liquids with nasal regurgitation and rhinolalia with no history of fever and limb weakness. Examination revealed bilateral palatal palsy with absence of gag reflex. Other neurological examinations were normal. Investigations were done to rule out any known pathology leading to such a presentation. The symptoms were attributed to an idiopathic acute-onset-acquired bilateral palatal palsy, in the absence of any identifiable cause. This is a rare presentation in adolescent age with no case reported in the literature so far in this age group. Medical management was started and patient showed complete improvement within 2 weeks of his symptoms. Early diagnosis and aggressive management of this condition lead to a favourable prognosis.

Velopharyngeal dysfunction

This chapter explores velopharyngeal dysfunction (VPD), its impact, aetiology, assessment, and management. VPD may cause hypernasal, weak, and unintelligible speech, facial grimacing, nasal turbulence and nasal regurgitation, middle ear problems, and sinusitis. Such manifestations may be distressing for the patient, be associated with poor academic outcomes, be negatively perceived by others, lead to bullying and withdrawal, and result in relationship and employment difficulties. Like the effect of a visible difference on psychosocial confidence and self-esteem, the manifestations of VPD on emotional well-being and social interaction do not necessarily correlate with the amount of speech, language, or communication dysfunction. Normal anatomy, neuromotor function, and speech learning are prerequisites for effective velopharyngeal function. A problem with any of these components may result in a velopharyngeal mechanism that fails to efficiently and fully close throughout oral speech sound formation leading to VPD and an ensuing speech disorder. Perceptual speech evaluation is used to diagnose VPD. Lateral videofluoroscopy and nasoendoscopy are commonly deployed to assess velopharyngeal function and determine management strategies. Surgical interventions include pharyngoplasty, pharyngeal flaps, and velopharyngeal augmentation. Non-surgical interventions include speech prostheses and nasal obturators for speech improvement.

Free flap for soft palate reconstruction: long-term functional evaluation of a new technique

Purpose The soft palate (SP) has a complex anatomy and physiology. Reconstruction after tumour resection is a challenge, and procedures that only restore bulk don’t give good results. We aim to present a new technique for the in-setting and the functional outcomes. Methods We retrospectively included in a monocentric retrospective cohort study every patient with a first diagnosis of a soft palate squamous cell carcinoma (SPSCC), who underwent a tumoral resection with a free flap reconstruction, from February 2013 to July 2017. For the in-setting, a special care is given for the flap in-setting: we suture the flap more caudally than usual under the tongue base, creating a neo-posterior pilar. The primary outcome was the deglutition function, assessed by the M. D Anderson Dysphagia Inventory (MDADI). We also analyzed the patient’s quality of life with the FOSS score and the occurrence of nasal regurgitation or larynx aspiration. Results We included twenty patients, with a median follow-up of 26.5 months. The median MDADI score was 89, and the mode was 93. A Fisher test shows a significant improvement of MDADI scores for unilateral vs bilateral reconstructions (p = 0.03). The median FOSS score was 2, and the mode was 2. Seven (35%) patients complained of nasal regurgitation, three (15%) reported episodic laryngeal aspiration.

Surgical management in submucous cleft palate patients

Objectives The submucous cleft palate (SMCP) is considered to be the most subtle type of cleft palate. Early detection is important to allow on time intervention by speech therapy and/or surgical repair before the children already develop compensatory speech mechanisms. The purpose of this study was to investigate at what time children with a SMCP present, to determine when children are operated, and to analyze the postoperative outcomes for in SMCP children. Patient and methods Medical records from 766 individuals registered in the cleft registry in the Wilhelmina’s Children’s’ Hospital, Utrecht, were retrospectively reviewed. Inclusion criteria were children diagnosed with SMCP. The following data were collected: age at diagnosis, physical examination, age at surgery, surgical technique, speech therapy pre- and post-surgery, otitis media, secondary cleft surgery, family history, syndromes, and other anomalies. Results In total, 56 SMCP children were identified. The mean age of diagnosis was 44.0 months (range 0–150, SD = 37.0). In 48 children (85.7%), surgical intervention was performed (Furlow plasty, intravelar veloplasty, pharyngoplasty, or Furlow combined with buccal flap). Conclusion This retrospective study reconfirms that SMCP often presents late, even in a country with a modern healthcare system and adequate follow-up of all newborns by the so-called youth doctors in “children’s healthcare centers” up to the age of 4 years old. Almost 86% of patients ultimately needed palate surgery when SMCP was suspected. Clinical relevance Any child presenting with repeated episodes of otitis media, nasal regurgitation, or speech difficulties should have prompt consideration for SMCP as diagnosis.

Fulminant Guillain Barre syndrome with recurrent lung collapse

Fulminant Guillain-Barré syndrome (GBS) is a rapidly progressive form of polyneuropathy in which patients demonstrate eventual flaccid quadriplegia and an absence of brainstem function. Most patients present initially with a mild upper respiratory or gastrointestinal illness and have non-diagnostic cerebral imaging studies. Here we report the case of child aged 7 who was admitted initially with complaints of weakness of lower limbs lasting for 4-5 hours. He had difficulty in standing and walking, associated with pain in both lower limbs. Flaccid weakness rapidly progressed over 12 hours to involve both upper limbs along with difficulty in swallowing and nasal regurgitation of feeds. He was intubated and mechanically ventilated for respiratory failure. Over the period of time, after confirmation with all the test reports and symptoms, other differential diagnoses were ruled out and fulminant GBS was considered and hence plasmapheresis was started. Over the period of 3 months his muscle power slowly improved. During discharge, he was able to lift limbs against minimal resistance, turn sideways on bed, sit up with minimal support and likewise stand with support.

Surgical Management of Velopharyngeal Insufficiency Due to Unilateral Oropharyngeal Agenesis in a Patient With Stickler Syndrome

Velopharyngeal insufficiency (VPI) results from defects interfering with closure of the velopharyngeal port. It can lead to many issues ranging from nasal regurgitation to severe speech abnormalities. Treatment is tailored to patient-specific etiology and severity, often involving surgical correction. A rare, and therefore seldom, described cause of VPI is isolated unilateral agenesis of the soft palate. We describe the case of a 2-year-old patient with Stickler syndrome possessing a unique anatomic presentation of this pathology, managed successfully with a unilateral pharyngeal flap.

Oral mucosal lesions in children with and without cleft lip and palate: a case control study

Orofacial clefts are a major health problem affecting individuals worldwide. Affected children would be having functional and aesthetic problems, such as breastfeeding difficulties due to improper oral seal and nasal regurgitation. Hearing and speech difficulties are also associated with the aperture. Oral mucosal lesions are commonly missed due to higher concern over the cleft than the minority of these conditions. The purpose of the study was to assess the presence or absence of oral mucosal lesions in children with and without cleft lip and palate. A total of 89000 cases were reviewed between June 2019 to March 2020 for the incidence of oral mucosal lesions in individuals with and without cleft lip and palate only. The present study consists of 30 children divided into two groups: children with cleft lip and palate and children without cleft lip and palate. In both groups, the presence of oral mucosal lesions was noted. Absence of oral mucosal lesions in both children with cleft lip and palate and children without cleft lip and palate. In this study, there is no evidence regarding oral mucosal lesions in children with and without cleft lip and palate.

A RARE CASE OF CONGENITAL PALATAL FISTULA WITH NORMAL SOFT PALATE AND UVULA

Cleft lip and palate are commonly observed congenital anomaly. But congenital palatal stula is very uncommon. Only few cases have been reported in literature globally. Irrespective of the doubts in etiology and pathogenesis, due to its rarity it is important to report these rare cases in the literature as and when it comes to the notice of the clinicians. Herein,we present a case of two-year-old child presented to us with isolated congenital palatal stula. Apart from nasal regurgitation on feeding and URTI, all other things were normal. Midline, rectangular stula of 2 cm x 1.5 cm extending from incisive foramen and involving posterior edge of hard palate was seen. Patient responded well to the surgery and postoperative period was uneventful.