This document summarizes a presentation on complex decision making in pediatric dysphagia. It discusses two specific cases - vincristine toxicity in pediatric oncology patients, and diagnosis and management of Type 1 laryngeal clefts. For vincristine toxicity, swallow studies found silent aspiration in patients on chemotherapy. Management involved diet modifications and potentially discontinuing treatment. For laryngeal clefts, a multidisciplinary team was involved in differential diagnosis and surgical repair improved swallow outcomes. The role of speech language pathologists in evaluating and treating both conditions was highlighted.
1. Complex Decision Making in Pediatric Dysphagia Alana Lowry, MS, CCC-SLP Fletcher Allen Health Care Kara Fletcher Larson, MS, CCC-SLP Jennifer Miller, MS, CCC-SLP Children’s Hospital Boston ASHA November 17, 2006 Miami, Florida
44. Laryngeal Cleft Endoscopic repair Lowry, Fletcher, Miller ASHA 2006
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Editor's Notes
Period of drug administration followed by a resting period after which the cycle starts over again. Chemo dose is calculated based on patient’s height and body weight. Chemo is not only toxic to tumor cells adverse reactions usually affect parts of the body that have a rapid turnover such as blood cells and cells of the GI tract.
Define neuropathy: any disease of the nerves Peripheral neuropathy: any syndrome in which muscle weakness, paresthesias, impaired reflexes and autonomic symptoms in the hands and feet common. Autonomic: self controlling, functioning independantly. Relating to the autonomic nervous system: The part of the nervous system that controls involuntary bodily functions. The ANS consists of motor nerves to visceral effectors: smooth muscle, cardiac muscle, glands such as salivary glands, gastric, sweat glands.
Parents want child to complete chemo. Cycle and get concerned regarding interruption of chemo regimen and how that affects their treatment. Prolong their chemo cycle. Also parental concerns regarding the need to reduce the strength of the dose and if that too may affect the outcome of the cancer treatment.
Range of time it took for swallow function to return to pre- vincristine status was weeks to months. Some patients returned for numerous follow up VFSS in the range of 2- >8 at scheduled intervals to assess for improvement. YES some patients did experience a re-currence of symptoms and documented aspiration once the Vincristine was re-started and we took them through the cycle again of altering diet, MD changed strength/dose of V.
Jimmy Fund Clinic at Dana Farber Cancer Institute
7 months into treatments, diagnosed with pna.
Discussion held with oncology team and decision made to withhold vincristine 3 weeks later although Vinc. d/c pt. Continues to exhibit signs of neurotoxicity./2 months later (vinc. Withheld)—shows normal swallow fx
Repeat VFSS was 5 months after last documentation of aspiration in June Patient now healthy
Cotton & Prescott
Tracheoesophageal septum separate these 2 structures at 35th day. Incomplete formation of tracheoesphageal septum results in TEF or LC. 3 1/2 weeks gestation. Cotton & Prescott.
Benjamin-Inglis Classification System 1989. Type 4 extends below the level of the thoracic inlet.
Type 1 supragottic, above the level of the TVF. Type 2 extend below the level of TVF. Type 3 with or without extension into Tracheoesphageal wall. Type 4 extends through the majority of the TE wall.
Type 1-4 from left to right. Slide courtesy of Dr. Reza Rahbar. 4 below the level of the thoracic inlet.
Photo of type 1 LC during DL. LC defect is in the lower right quadrant. Photo courtesy of Dr. Reza Rahbar.
These are S&S of an infant. S&S of older child: suspect LC if normal child with aspiration. These s&s mirror pharyngeal dysphagia. These s&s can be non-specific and must include a high suspicion for LC to prompt further investigation.
How to differentiate from other neurogenic swallow dysfx. LLL pneumonia, atelecticis, pulmonary findings. DLB, DL plus bronchoscopy, to see the changes in the lungs. X-ray showing non-specific infiltrates DLB must be done with “bimanual interaryntenoid palpation.” What about FEES?? WE DO NOT USE FEES AT OUR CENTER TO DX SWALLOWING DYSFX IN PEDS AT ALL AT THIS TIME. DLB IS GOLD STANDARD BUT THERE IS SOME THOUGHT THAT THE DX MAY BE SUBJECTIVE.
This suspicion of T1 LC arose when the incidence of isolated swallow dysfx increased in our pt population. The question then arose why are these pts aspirating? In collaboration with ORL, etc. the dx of LC became an option to consider.
Incidence of isolated LC regardless of type. Cotton, R.T. & Prescott, C.A.J. 1998. Congenital anomalies of the larynx. In Cotton, R.T. & Myer, C.M. (eds). Prescribed paediatric otolaryngology: 497-513. Philadelphia: Lippincott-Raven.
Our incidence rate is not currently available, but our numbers are in line with the most recent research and suggest and increased incidence rate compared to 0.1%. The 9 unrepaired patients are on an altered diet and closely monitored by PCP and ORL MD
The incidence at CHB is also higher than what has been reported in the past and is more comparable to MEEI than Cotton & Prescott. Chien et al 2006. Watters & Russell 2003 and Parsons et al 1998 (6.2% and 7.1 % incidence). Also large center studies.
PH syndrome: include summary. None of our patients have PH or G syndrome. Include other associated anomalies. It is the role of the MD to determine if LC is a marker for a syndrome. Often LC is with pts who have other medical issues. Of TEF pts 6% have LC. (Cotton & Prescott). The LC in these pts often goes undetected until persistent aspiration. PH: autosomal dominant, LC polydactayly, hyperpitutarism. G: dysphagia, hypospadias, hypertelorism, cleft L&P., tendency towards midline defects.
This must contribute to the higher incidence.
ID of this problem led to the development of CAD. PERHAPS THIS IS WHY WE HAVE INCREASED INCIDENCE DUE TO COLLABORATION AMONG CLINICIANS. All tertiary care medical centers need an airway center to provide the best care to these pediatric patients.
We see a number of patients who are normally developing, but have isolated swallow dysfunction with aspiration. When a pt has no underlying etiology for aspiration, one must consider LC as the differential diagnosis. VFSS does not diagnose LC. Must undergo direct laryngoscopy for definitive diagnosis (gold standard).
Robotic procedure eliminate the need for an incision. Intubation for 10 days post-op. This protocol for intubation 10 days post op is also being challenged. Least aggressive tx tried 1st.
Photos courtesy of Dr. Reza Rahbar. This is an example of an endoscopic repair of Type 1 LC provided by our surgeon. It demonstrates the suturing of the cleft and the less invasive method of repair compared to the open surgery.
1-2 VFSS before the referral is made to ORL. When we see plateau and pattern then refer to ORL. Full recovery: on regular diet. Repeating VFSS too soon after surgery (1-2 weeks post-op) may continue to reveal aspiration which may likely be due to the surgery itself (swelling, inflammation).
Pt. accepted honey-thick liquids and nutrition determined well-hydrated