Mark C. Fletcher, DMD, MD

• Division of Oral and Maxillofacial Surgery
• University of Connecticut School of Dental Medicine
• Avon Oral and Maxillofacial Surgery
• Avon, Connecticut

Limited self-feeding skills pulse pressure definition medical generic triamterene 75mg on line, limited oral motor development beyond bottlefeeding prehypertension and chronic kidney disease buy generic triamterene pills, and delayed cognitive development all affect participation in compensatory feeding strategies that may be recommended fetal arrhythmia 34 weeks proven triamterene 75 mg. Understanding regarding behavior problems may be gained from this assessment blood pressure time of day cheap triamterene, thereby allowing for structuring of behavioral interventions blood pressure medication overdose death buy cheap triamterene on-line. In the presence of decreased orofacial tone blood pressure 8959 order triamterene 75 mg with amex, food materials may tend to collect in the anterior or lateral sulci. Improper tongue control may propel food material out of the mouth rather than posteriorly for swallowing. A child with a weak suck or poor coordination of the sucking process tends to present with multiple sucks per swallow to compensate for the lack of efficiency. Nasopharyngeal regurgitation may occur with poor timing of velopharyngeal closure during the swallow or may be caused by muscular weakness or paralysis preventing adequate closure. It also may be suggestive of cricopharyngeal achalasia, as the bolus does not pass into the esophagus with contraction of the pharyngeal muscles. Rather, the bolus remains under pressure in the hypopharynx and may be expelled into the nasopharynx when the velum descends. Attention to articulation and voice quality also may provide useful information, as the same structures used for the oropharyngeal phases of feeding are used for speech production. Options include applying 2% topical viscous lidocaine to the endoscope, administering a 1:1 mixture of oxymetazoline (Afrin) and 2% tetracaine to the nasal passages by a hand-activated atomizer, or employing both of the preceding options. When applied to the distal end of the endoscope, 2% viscous lidocaine anesthetizes the nasal mucosa at points where the endoscope comes into contact with it, thus benefiting all patients. In children who are younger than 1 year of age and those with complex medical or neurologic conditions such as congenital heart disease or bronchopulmonary dysplasia, lidocaine applied to the endoscope is used as the sole anesthetic agent. We also prefer this approach in children without a tracheotomy who have a potentially tenuous airway. In children older than age 1 who are medically stable, we use a 1:1 mixture of oxymetazoline and 2% tetracaine and administer a dose of 0. Oxymetazoline is a nasal decongestant that causes the nasal mucosa to vasoconstrict, thereby increasing the size of the nasal passage. To provide flexibility of sites for passage of the endoscope, both nasal passages are anesthetized. Although the degree of absorption is unknown, we have encountered no toxic complications with this protocol. To prevent the risk of anesthetizing the hypopharynx, the child should be upright with the head in a neutral position; the parent often assists in holding the head stationary. Materials such as milk, vanilla pudding, and goldfish crackers facilitate this by providing adequate color contrast; clear liquids are particularly difficult to see. Visualization is also hindered in childen who accept only small volumes of food or drink. A single drop of this coloring is added to a food or liquid sample and then offered to the child. Individuals at risk for methemoglobinemia include those with increased gut permeabiltity (eg, premature infants and infants with celiac disease or irritable bowel disease). Although it has a strong odor and alters the flavor of foods, it is often readily accepted by children. Determining and Preparing Food Items the swallowing assessment is performed both with thin liquids and with liquids that have varying degrees of viscosity. A variety of pureed, smooth, and solid textures are also used, as developmentally appropriate. As in all dysphagia evaluation and treatment, attention should be paid to possible allergies, preferences, and restrictions based on religious dietary laws. The parent is often given the opportunity to bottle feed the infant during the study. Given that nasal obstruction can interfere with swallowing function (particularly in infants), nasal patency should be documented as the endoscope is advanced through the nose. The function of the velopharyngeal sphincter is evaluated while the scope is in the nasopharynx. Incomplete or inefficient velopharyngeal closure also may interfere with the generation of tongue-base driving pressure required for bolus propagation into the hypopharynx. The hypopharynx is evaluated by passing the endoscope beyond the free margin on the soft palate. As the larynx descends, there is less separation of the respiratory and digestive tracts. As a result, food materials, liquids, and secretions can pass more readily over the tip of the epiglottis, leading to an increased chance of aspiration. The pyriform sinuses are evaluated for masses and asymmetry that may interfere with the swallowing process. Special attention is paid to the possible presence of laryngeal anomalies, which can interfere with proficient swallowing and airway protection. The degree of pooled secretions at the beginning of the examination should be noted. Also, the symmetry of pharyngeal contraction during swallowing should be documented. This may occur for a variety of reasons, including an underlying sensory deficit, poor oral motor control, incoordination of swallowing timing, poor pharyngeal clearance, or inadequate relaxation of the cricopharyngeal sphincter. Excessive pooling and poor management of secretions are immediately apparent when the hypopharynx is viewed endoscopically. Oral secretions will mix with the coloring, thus improving visualization of the secretions. Gustatory stimulation (eg, drops of carbonation or citrus flavor) or thermal stimulation (eg, using a Popsicle or crushed ice) may improve secretion management and stimulate spontaneous swallowing. Patients with excessive secretions that are not cleared or that increase during the initial part of the examination are at risk for aspiration. In patients with normal hypopharyngeal sensation and pharyngeal clearance mechanisms, secretions do not accumulate in the hypopharynx. Premature spillage is the escape of material over the tongue base in the absence of purposeful oral transfer before the initiation of swallowing. The degree of spillage needs to be evaluated with respect to the ability of the patient to initiate a clearing swallow. Mild delay in the initiation of swallowing can be evident by material moving across the tongue base into the vallecular space and into the pyriform sinus region before initiation of the swallow. Premature spillage of a volume sufficient to start filling the pyriform sinuses is of concern, as it may increase the risk of aspiration. If swallowing is not initiated, continued overflow of the bolus into the laryngeal inlet may occur. It is important to note, however, that premature spillage is not always indicative of a pathologic swallow and may reflect a normal developmental pattern of swallowing. Infants often trigger the swallow from the level of the vallecula with a rhythmical fill and contract pattern, without increasing the risk of aspiration. Thin materials have a greater likelihood of flowing through the glottis than do thick materials, which flow more slowly. Frequent laryngeal penetration events of significant volume have the same clinical implication as frank aspiration. Aspiration is defined as the passage of solid or liquid material below the level of the true vocal folds. The potential for this to occur is greatest during the pharyngeal phase of swallowing. Aspiration that occurs during this period can be deduced by visualizing the events that occur immediately after the swallow, when material is expelled from the airway by a cough or by identification of stained glottic or subglottic structures. Aspiration of small volumes of material may be cleared from the airway by normal mucociliary clearance or a cough. Large volumes of aspirated material may, however, reach the distal airway and be only partially cleared, leading to the possibility of developing aspiration pneumonia. Because oral secretions are laden with high concentrations of bacteria from the oral cavity, aspiration of these secretions may lead to chronic lung damage over time. Mild amount of residue coating part of the posterior and lateral pharyngeal walls and the vallecula. Glottic closure in response to mechanical or chemical stimulation of the laryngeal mucosa is the primary 378 Pediatric dysPhagia: etiologies, diagnosis, and ManageMent means of laryngeal protection from aspiration. Within the brainstem, the nucleus tractus solitarius stimulates the nucleus ambiguus, which sends efferent nerve impulses through the recurrent laryngeal nerve to the laryngeal musculature. This reflex arc results in glottic protection through closure of the glottis, a cough, or a swallow. Historically, a specialized air compressor was coupled with a specially designed endoscope that contained an air channel to allow delivery of a calibrated stimulus to the larynx. The compressor could be adjusted to deliver a single pulse or a continuous stream of air to a target structure. The pressures could be regulated between a range of 0 to 10 mm Hg (millimeters of mercury). Also, the appearance of pooled secretions noted in the hypopharynx was closely associated with abnormal sensory thresholds in the hypopharynx. In light of this association, pooled secretions can be used as a surrogate measure of elevated sensory thresholds. The tapping should induce reflexive glottic closure, a cough, or a swallow response. In this scenario of hypopharyngeal hypotonia, additional swallowing may clear the endoscope. If visualization cannot be reestablished, the tip of the endoscope will need to be cleaned by dragging it against the pharyngeal mucosa. During the swallow, the pharyngeal walls constrict against the endoscope, obscuring visualization of events occurring during bolus transfer ("white-out"). Secretions are organized into a bolus, the crying abates, a swallow is performed to clear the oral cavity, and the hypopharynx remains dry. When this does not occur, the amount of pooled secretions correlates with the degree of sensory awareness. Food or liquid material courses over the tongue base prior to the initiation of the swallow. The time taken to subsequently initiate a swallow determines how deep into the hypopharynx the spillage event will reach. The proceduralist sees rhythmic sucking action and spillage into the vallecula, where the swallow is then initiated. Observing multiple swallows that demonstrate triggering of the swallow from the vallecula (with no spillage beyond) indicates clear protection of the airway. In contrast, premature spillage beyond the newborn period should prompt the proceduralist to further explore why the trigger for the swallow is delayed. Although some children with this finding do not require intervention, others may be predisposed to the loss of control of the bolus, thus increasing the risk of laryngeal penetration and aspiration. The closer the bolus comes to the glottic inlet, the greater the risk of aspiration. Whereas food material in the vallecula is far removed from the endolarynx and presents a low risk of aspiration, material reaching the pyriform sinus is far more likely to gain entry into the larynx. The material may accumulate in the pyriform sinus and gain entry into the endolarynx by overflowing the aryepiglottic folds or spilling through the interarytenoid notch. The consistency with which a patient triggers the swallow from a given location (eg, tongue base, vallecula, pyriform sinus) influences treatment recommendations. Spillage to the pyriform sinus that always triggers a brisk swallow response represents a situation associated with less risk of aspiration than a situation in which mate- rial occasionally resides in the hypopharynx for long periods, slowly accumulating over time. Laryngeal Penetration Penetration of food or liquids into the larynx always raises the suspicion of aspiration. When significant laryngeal penetration is identified, it is likely that a patient will aspirate food material over time. Both the degree of penetration and the frequency of penetration events identified during the exam will influence treatment recommendations. Similarly, deep penetration to the level of the vocal folds is more concerning than shallow penetration into the larynx. Small amounts of material on the laryngeal surface of the tip of the epiglottis are not overly concerning; however, larger collections of material within the larynx are often treated the same as frank aspiration events. Events prior to the swallow and after the swallow are clearly visualized, but the loss of view or "white-out" during the actual swallow prevents detection of aspiration that may occur. When the event occurs prior to or immediately following a swallow, the material can be seen falling below the level of the vocal folds. A cough response will often ensue, which allows visualization of the food material to be seen as it is expelled from the airway. Chain swallowing and the associated loss of view during white-out can create a situation in which substantial volumes of material may be aspirated with little to no clinical sign of the problem. Regurgitation of food materials back into the hypopharynx presents a condition in which aspiration may occur. Poor esophageal clearance may lead to an accumulation 380 Pediatric dysPhagia: etiologies, diagnosis, and ManageMent of material in the esophagus that may be expelled superiorly when a patient strains or coughs. Frank gastroesophageal reflux events may also lead to material entering the hypopharynx. The larynx may not be protected during a reflux or regurgitation event, creating the potential for aspiration.

Functional microsurgical partial callosotomy in patients with secondary generalized epilepsies blood pressure normal numbers purchase triamterene cheap. Radiosurgical posterior corpus callosotomy in a child with Lennox-Gastaut syndrome arrhythmia with normal heart rate buy triamterene overnight. Gamma knife radiosurgery for callosotomy in children with drug-resistant epilepsy heart attack headache generic triamterene 75 mg with visa. Surgical treatment of children with medically intractable epilepsy-outcome of various surgical procedures arteria doo buy 75 mg triamterene with mastercard. This would require the use of at least two antiepileptic medications properly dosed for a sufficient duration to assess effect or failure blood pressure chart table purchase generic triamterene on line. This is required pulse pressure 88 generic 75 mg triamterene amex, despite the fact that hypothalamic hamartomas are notoriously resistant to medical management. In addition, secondary epilepsy manifested by complex partial seizures, or other nongelastic semiologies, should not be targeted separately until it is reasonably certain that the hamartoma is no longer driving the epileptic network. However, cognitively normal patients with small lesions may have greater preservation of cognition with laser ablation as opposed to open or endoscopic resection strategies. Patients with epileptic encephalopathy in need of instant therapeutic effect are also more likely to benefit from laser ablation as opposed to delayedeffect therapies like stereotactic radiosurgery. Preoperative workup and patient selection should involve a multidisciplinary epilepsy team. Technical considerations including choice of equipment, trajectory planning, safety mechanisms, and image-guided monitoring are discussed. Although not all patients are ideal candidates for laser ablation, the improved complication profile of laser ablation makes this approach the ideal first surgery for most hypothalamic hamartomas. Here, we present our findings on the impact of this advance in the treatment of this disease. Preoperative Workup the preoperative workup of a patient with hypothalamic hamartoma starts with the recognition of gelastic seizures, which is usually delayed, and subsequent neuroimaging to confirm the diagnosis. Beyond neuroimaging, other diagnostic evaluations have limited utility in the evaluation of hypothalamic hamartoma. The Visualase system has some advantages in treating hypothalamic hamartomas such as a relatively shorter ablation time and the use of low-limit markers that contribute to automatic laser cutoff. However, it does have limitations in the size of the ablated field, which is especially significant when treating giant hamartomas. The Neuroblate system has the advantage of larger ablation field, but it does not have automatic cutoff systems and uses a larger probe to deliver heat to the target. The goal of the surgical planning is twofold: disconnection and volumetric destruction. The first trajectory targets the most inferior, posterior aspect of the hamartoma, and the entry point within the hamartoma is equidistant between the fornix and the mammillothalamic tract on the most anatomically connected side. The software is then used to create an oblique trajectory view, and the trajectory is extrapolated to the surface of the skull to identify the surgical entry site. Avoidance of ependymal surfaces of the ventricular lining is a relative caution, though it is sometimes selected if the optimal trajectory requires traversing the ventricle. When possible, the trajectory diameter should be expanded to 14 mm, which is the largest diameter possible using the Visualase laser fiber with a 3-mm diffuser tip. This widened trajectory should also allow for ablation up to the junction of the hamartoma and the hypothalamus. If part of the hamartoma remains unincorporated in the planned ablation corridor despite this widened trajectory, another trajectory is created to cover that region. The presence of heat sinks within the ablation field should also be considered when planning the trajectories. Significant heat sinks include the pulsatility of the suprasellar cistern, the third ventricle, and the foramen of Monro. Accordingly, we do not routinely target the center of the hamartoma, but rather we adjust the trajectory to traverse the point between the medial one-third and the lateral two-thirds of the diameter of the lesion. Lastly, the "probes-eye" view of the software should be engaged on a T1, gadolinium-enhanced scan with a 3-mm diameter to assure that the planned trajectory is avascular. Implantation of the laser fiber or probe can utilize any stereotactic system that the surgeon is comfortable with. No matter what system is used to acquire the stereotactic trajectory, the implantation begins with a 3. The guide rod is advanced to target after cauterizing through the dura and then removed. The Visualase cooling cannula with a stiffening rod inside is then advanced to target, secured, and the stiffening rod replaced with the Visualase laser fiber. Note the central planning of the trajectory in the superior portion of the hamartoma and the eccentric planning of the trajectory in the inferior portion of the hamartoma to compensate for the surmised heat sink inferiorly. Typically, the 3-mm diffuser tip is better suited to ablating hypothalamic hamartomas due to minimal laser dispersion and thermal delivery past the target along the trajectory which may not be visible on the selected planes of the thermogram. Diffusion imaging, or higher resolution diffusion tensor imaging, is used to assess the immediate edema related to thermal injury. Lastly, the cannula is removed from the brain, the anchor bolt is unscrewed from the bone, and a single stitch is placed to close the wound. Background images, ideally T2 weighted, are then selected, one in the oblique coronal plane and the other in the selected axial plane that optimally visualizes the structures around the hamartoma that need to avoid thermal injury. These include the bilateral fornices, the bilateral mammillothalamic tracts, the optic chiasm and tracts, and the junction between the hypothalamic hamartoma and the hypothalamus. Unused high-limit markers are then used to monitor the temperature of other structures to be spared ablation, but without the automatic laser shut-off capacity. The irrigation to the cooling cannula is turned on and flow is confirmed by acknowledging return of fluid from the irrigation circuit. The laser is then set to a 10-watt limit and tested at sublethal temperatures, about 8 to 10% power, to confirm the ideal placement of the heat source. Once adjustments are made along the trajectory to optimize the heat source placement and the high-limit marker is adjusted accordingly, the laser power is increased to ablating levels. An irreversible damage map, based on the Arrhenius equation, demonstrates destroyed tissue and is represented by change in pixel color to orange. If the lesion extends beyond one ablation height in the coronal plane, then the laser fiber can be pulled out of the cooling cannula by a measured distance and another ablation performed more proximal in the trajectory. However, special care must be taken to preempt and treat postablation edema, which is typically more intense than in open surgery. Preoperative use of high-dose steroids for 1 week prior to ablation followed by 10 days of postoperative steroids minimizes the risk of rapid onset of edema in the hypothalamus. In addition, young children, especially those with giant hamartomas, can have fluctuation of their serum sodium levels, although typically not as severe as with open surgery. Lastly, lesions close to the pituitary stalk should be monitored carefully for postoperative diabetes insipidus. Nineteen of the patients in this series had secondary nongelastic seizures that were improved after ablation and more easily controlled with medications. Thirteen of the patients (22%) required two ablations, and two patients required three ablations. Most patients experienced a near instant improvement in their overall neurological condition; 12% of patients were seizure free and off all antiepileptic medicines. There were no patients with new or worsened hypothalamic obesity following ablation. Technological improvements such as unlimited low-limit markers and heat sink prediction technology will no doubt improve these preliminary outcomes. These sequences reveal the extent of the ablation and confirm the completeness of the targeted ablation. Endoscopic resection of hypothalamic hamartomas for refractory symptomatic epilepsy. Transcallosal resection of hypothalamic hamartomas in patients with intractable epilepsy. Predictors of inpatient complications and outcomes following surgical resection of hypothalamic hamartomas. The laser probe is then positioned and delivered to the target using frameless, robotic, or traditional frame-based stereotaxis. Ablation is an option as initial, subsequent, or repeated treatment, as an adjuvant to other therapies, given that it has no dose limitations. Nevertheless, the adult publications are encouraging to adopt this technology and consider it as the first option in drug-resistant epilepsy in patients with mesial temporal sclerosis, hypothalamic hamartomas, cortical dysplasias (including insular lesions), corpus callosotomy, tuberous sclerosis complex, periventricular heterotopias, and tumors. The thermal mapping is updated every 3, 6, and 8 seconds for single, biplanar and triplanar viewing, respectively. The software calculates the irreversible damage zone based on the Arrhenius rate model of thermal tissue destruction. This applicator produces a cylindrical to ellipsoid light distribution in the tissue along the axis of the 1-cm diffusing element. The laser probe connects to a peristaltic roller pump that circulates sterile saline solution at room temperature to cool both the laser fiber and the adjacent tissue, preventing excessive tissue heating. The procedure can be done using frameless, robotic, or traditional frame-based stereotaxis. A cranial anchor is fitted over a steel rod and is screwed into the drilled hole at the calculated angle, forming a firm anchor point for insertion of the laser applicator. The technology creates a predictable injury that conforms to the shape and size of the targeted lesion(s). More recently, Kang et al28 published their series of 20 patients, both children and adults, and reported seizure freedom rates after 6, 12, and 24 months were 8/15, 4/11, and 3/5, respectively. Those patients who were not seizure free displayed no improvement concerning their seizure status, except for one patient who had seizure frequency decreasing over time after thermotherapy. Four patients had complications represented by catheter misplacement (n = 2), brain edema (n = 1), and catheter damage for failure to the cooling system (n = 1). Thermal ablation therapy for focal malignancy: a unified approach to underlying principles, techniques, and diagnostic imaging guidance. Optical properties of selected native and coagulated human brain tissues in vitro in the visible and near infrared spectral range. Magnetic resonance thermometry-guided laser-induced thermal therapy for intracranial neoplasms: initial experience. Laser interstitial thermal therapy in treatment of brain tumors-the NeuroBlate System. Real-time magnetic resonance-guided laser thermal therapy for focal metastatic brain tumors. Magnetic resonance-guided laser interstitial thermal therapy: report of a series of pediatric brain tumors. Laser interstitial thermal therapy for medically intractable mesial temporal lobe epilepsy. Real-time magnetic resonance-guided stereotactic laser amygdalohippocampotomy for mesial temporal lobe epilepsy. The role of stereotactic laser amygdalohippocampotomy in mesial temporal lobe epilepsy. Magnetic resonance thermometry-guided stereotactic laser ablation of cavernous malformations in drug-resistant epilepsy: imaging and clinical results. Staged laser interstitial thermal therapy and topectomy for complete obliteration of complex focal cortical dysplasias. Magnetic resonance imaging-guided laser interstitial thermal therapy for previously treated hypothalamic hamartomas. Journal Club: Real-time magnetic resonance-guided stereotactic laser amygdalohippocampotomy for mesial temporal lobe epilepsy. Bilateral occipital dysplasia, seizure identification, and ablation: a novel surgical technique. Disabling amnestic syndrome following stereotactic laser ablation of a hypothalamic hamartoma in a patient with a prior temporal lobectomy. The effects of anatomic variations on stereotactic laser amygdalohippocampectomy and a proposed protocol for trajectory planning. Robot-assisted stereotactic laser ablation in medically intractable epilepsy: operative technique. Magnetic resonance imaging-guided focused laser interstitial thermal therapy for subinsular metastatic adenocarcinoma: technical case report. Blount this article defines the nature of surgical failure in the treatment of pediatric epilepsy, and reviews the approach to reoperation in this population. The past decade has seen a paradigm shift in the approach to surgical failure and reoperation in children. Evolving technologies allow more precise localization in reoperation, and increasing use of less-invasive techniques for both localization and lesioning has led to a stepwise approach in the use of progressively invasive interventions.

Long-term seizure outcomes following epilepsy surgery: a systematic review and metaanalysis blood pressure medication effect on running cheap 75mg triamterene overnight delivery. Utility of magnetoencephalography in the evaluation of recurrent seizures after epilepsy surgery heart attack pulse purchase genuine triamterene on line. Neuromagnetic evidence of pre- and post-central cortical sources of somatosensory evoked responses heart attack what to do buy generic triamterene 75 mg on-line. Somatosensory evoked potentials and magnetic fields elicited by tactile stimulation of the hand during active and quiet sleep in newborns hypertension knowledge test purchase triamterene 75mg on line. Magnetoencephalography in children: routine clinical protocol for intractable epilepsy at the Hospital for Sick Children arrhythmia knowledge a qualitative study purchase triamterene 75mg. Somatosensory-evoked fields on magnetoencephalography for epilepsy infants younger than 4 years with total intravenous anesthesia blood pressure log chart pdf purchase discount triamterene. Cortical reorganization in malformations of cortical development: a magnetoencephalographic study. Neuromagnetic evaluation of cortical auditory function in patients with temporal lobe tumors. Somatosensory, auditory, and visual evoked magnetic fields in patients with brain diseases. Reorganization of languagespecific cortex in patients with lesions or mesial temporal epilepsy. Atypical language representation in patients with chronic seizure disorder and achievement deficits with magnetoencephalography. Reliability of language mapping with magnetic source imaging in epilepsy surgery candidates. Such a lesion may be obvious, but it may commonly be quite subtle, and for that reason, the best possible quality of imaging is mandatory: multiplanar, multisequence high-definition imaging, as much as possible with a "high field" magnet (3+ teslas) and a multiple phased-array coil. The best indications for epilepsy surgery are the lesions that are typically associated with severe, drug-resistant epilepsy. Obviously, like in adults but much less commonly, mesial temporal sclerosis is an important cause of refractory epilepsy in children, and therefore a surgical indication. Even in the event of a first seizure, a distinction should be made in children, between a febrile seizure, for which the diagnostic yield of imaging is very low,1 and an a-febrile seizure, in which the diagnostic yield becomes significant. This includes lesional focal epilepsy, specific epileptic syndromes pointing to structural brain abnormalities such as Ohtahara or West syndromes, new status epilepticus, and the so-called catastrophic epilepsies that are associated with a progressive deterioration. Even in patients with "normal" images, a-posteriori review of the images, or repeat imaging, especially with advances in techniques, can help previously undetected structural abnormalities be identified. These need to be acquired in at least two orthogonal planes covering the whole brain, using the minimum slice thickness. In those with temporal lobe epilepsy, the coronal plane should be perpendicular to the long axis of the hippocampus to optimize visualization of the mesial temporal structures. A three-dimensional T1 volume sequence with slice thickness of 1 mm provides excellent gray/white matter contrast, can be reformatted into any orthogonal or nonorthogonal planes and may be subjected to additional post-processing without the penalty of additional imaging time. A systematic approach should be used in order to optimize detection of subtle lesions and double pathologies. This means that during the maturation process, there is a period of months during which this contrast is attenuated, and the diagnosis of subtle cortical dysplasia becomes difficult. On the other hand, repeated focal seizures induce an early myelination which can be used to identify the location of the epileptogenic zone in some cases. However, the changes may reflect the structural abnormalities as well as ipsi- and contralateral metabolic alterations related to the seizures, making the interpretation of the results difficult. Although it is not currently used for diagnosis, it may help in understanding the abnormalities of white matter associated with cortical malformations. Structural image analyses describe various computer-assisted methods devised to improve the rate of detection of subtle brain abnormalities in epilepsy. Unfortunately, these methods are not fully automated and are time consuming and difficult to use in clinical practice. Studies of the functional connectivity are useful to understand how epilepsy might impact the brain function but are not yet part of the presurgical assessment. In contrast, hippocampal sclerosis is less common in pediatric patients compared to adults. They are slow growing tumors with well-defined margin, typically without associated edema or necrosis. Complete removal results in good seizure control or renders the patient seizure free. They are macroscopically observed to be up to eight times larger in children than in adults. The neuronal component does not expand; the glial component rarely can be malignant. Gangliogliomas may present as a solid mass in 43%, a cyst in 5%, and a mixed lesion in 52%. They involve the cortex, usually broaden the gyri and may cause remodeling of the adjacent bone. They usually form discrete, well-demarcated masses in the neocortex, but they are more infiltrative mass in the mesial temporal region. Some may demonstrate intrinsic high T1 signal, due to calcification that may be seen in about 30 to 50% of cases. Enhancement following gadolinium administration is common, seen in up to 60% of cases. Desmoplastic infantile ganglioglioma is a rare, likely congenital tumor that develops in infants. Usually huge, more often suprasylvian in location, the tumor is partly cystic and partly solid. The solid portion incorporates the cortex and is diffusely attached to the dura; it is strongly desmoplastic and may be calcified. In about half the cases, the infant presents with macrocephaly, neurological deficits, and seizures. The tumor usually is stable over the years but a significant increase in size has been documented in a few cases. In contrast to the deep oligodendroglioma, this peripheral epilepsy-associated tumor has a good prognosis. It is located in the cortex and extends across the parenchyma, supratentorial in 98% of cases, mostly temporal (49%). It contains predominantly glial component, but may also contain neuronal elements. Post gadolinium, the nodular solid component as well as the adjacent meninges enhances (dural "tail"). Angiocentric glioma form a homogeneous but ill demarcated cortical-based mass with infiltration of the gyral white matter, sometimes with a transcerebral extension ("stalk") toward the ventricular wall. The gyrus is bulky with effacement of the adjacent sulci but without edema or significant mass effect. The dysmorphic neurons may either be pyramidal or interneuronal, but the balloon cells are of glial origin rather. In any case, the abnormal or misplaced cells may not be correctly connected, and the gyration, which depends on the connectivity, may therefore be abnormal. The lesion is characterized by the bright signal of the cortex and still more, of the white matter, with the cortex being recognizable. The characteristic feature is the transmantle dysplasia: the signal abnormality of the white matter tapers from the dysplastic cortex to the ventricular wall. Radial cellular columns appear early in the cortex and reflect the radial glia-guided migration of the neurons. The superior frontal sulcus (anterior portion, parasagittal) is deeper on the left than on the right. The dysplastic pattern may be acquired postnatally as the disruption of the connectivity would favor the development of giant, ill oriented neurons with an abnormal circuitry. Such findings have been observed in infants following severe perinatal or early postnatal injuries. The main features would be a poor development of a brain lobe and an attenuation of the normal contrast of the subjacent white matter, assumed to reflect a gliosis and demyelination secondary to the repeated seizure activity, rather than to represent the dysplasia itself. It is not known whether, of hippocampal sclerosis and dysplasia, one causes the other or whether they develop together. The normally dark signal of the right temporal white matter is lost as compared with the left. The temporal lobe, and to some degree, the right hemispheres are also smaller on the right than on the left. Experimental study in the mouse has shown that repeated neuronal electrical activity such as occurs in seizures induces myelination. Clinically, it may present early with intractable epilepsy, hemiparesis, hemianopia, and mental retardation. On imaging, one hemisphere is large with an expanded calvarium, and in many but not all, an enlarged lateral ventricle of the affected side. In infants, the white matter of the affected hemisphere may show high T1/low T2 signal, suggesting early myelination,61 likely due to seizure activity. Due to the intractable, poorly controlled seizures and progressive deterioration, functional or anatomical hemispherectomy may be required to control the seizures. Gray matter heterotopia is masses of apparently normal gray matter located in abnormal places. Their epileptogenicity is assumed to result from the abnormal connections they develop. The overlying cortex also is somewhat dysplastic, typically in proportion to the size of the heterotopia. Nodular heterotopia are designated as periventricular (isolated, multiple, or diffuse; never on basal ganglia or thalamus or corpus callosum) and subcortical (often huge, transcerebral, mixed with white matter). Band heterotopia is usually subcortical and corresponds to the lesser end of the agyria/pachygyria spectrum. In about 50% of cases, the patients present with neurological deficits and epilepsy. Usually centered about the sylvian fissure, the malformation may extend variably over the hemispheric convexities, with a disorganized sulcal pattern; it may be uni- or bilateral, usually not symmetrical. Typically, the abnormal cortex is still functional, and the surrounding normal-appearing cortex is epileptogenic, probably because of an abnormal connectivity. The medial cortex on the right side is thick and dark (diffuse microcalcification), while the underlying white matter is bright. In this patient with severe gelastic seizures, a small hypothalamic mass is seen attached to the ventricular side of the mammillary body; the T2 signal is slightly brighter than that of the hemispheric cortex. Classic appearance of the hippocampal sclerosis: bright T2 signal of the left hippocampal head, loss of the internal structure. Hippocampal Sclerosis While hippocampal sclerosis is the most common epileptogenic substrate seen in adult surgical epilepsy series, it is less common in children. Hippocampal volume reduction correlates with the severity of the neuronal cell loss. Surgical indications for schizencephaly-associated epilepsy are uncommon but there are reports of successful surgery of closed lip schizencephaly. A distinction is made between the more anterior parahypothalamic pedunculated hamartomas clinically associated with a central precocious puberty; and the more posterior intrahypothalamic (sessile) hamartomas connected to the mammillary bodies, clinically associated with an early onset severe, typically gelastic epilepsy (which over time results in cognitive deterioration and behavioral problems). There is no enhancement or calcification; a hypothalamic hamartoma may uncommonly be cystic. Uni- or bilateral, intra- or extraventricular, symmetric or not, the mass is of variable size, and grows in proportion to the brain only. It may extend anteriorly to the pituitary stalk, and posteriorly may splay the cerebral peduncles apart and displace the basilar artery. Epilepsy-associated hypothalamic hamartoma can be treated with surgical disconnection or radiosurgery. Clinically, the seizures begin abruptly in a previously normal child and include partial seizures and epilepsia partialis continua. With disease progression, the patients in months develop hemiparesis or hemiplegia and marked cognitive decline. Three years later (b), prominent parenchymal volume loss with extension of the cortical necrosis. The lateral ventricle is larger than before on the right side but also on the left side. Medical treatments are not really efficient, and surgical hemispherectomy may be the best option to stop the progression of the disease. If the medical treatment fails, hemispherectomy may be required on the condition that the other hemisphere is not involved. Using fat saturation, it may show associated abnormalities of the ocular choroid and in the calvarium as well. Acute ischemia with focal edema, bleed or diffuse hemispheric swelling from prolonged seizure activity may be demonstrated. Hemispheric atrophy may result from the seizure activity and/or from the perfusion defect. In infants, the white matter may present with the low T2 signal of a seizure-induced early myelination.

Syndromes

• Death (if there is galactose in the diet)
• Is it getting better, worse, or staying the same?
• The surface of the eye is numbed with eye drops. A fine strip of paper stained with orange dye is touched to the side of the eye. The dye stains the front of the eye to help with the examination.
• Lethargy, sleepiness, not smiling
• Difficulty swallowing
• Repair a torn labrum (a tear in the cartilage that is attached to the rim of your hip socket bone)
• Vision changes
• Age 0-6 months: 1.7* milligrams per day (mg/day)
• Tastier food

For example prehypertension young adults purchase discount triamterene on line, if a child likes the taste of ranch dressing arrhythmia frequently asked questions safe 75 mg triamterene, it may be used as a dip on new items in order to encourage acceptance blood pressure 7550 quality 75 mg triamterene. As stressed by Fraker and Walbert blood pressure medication lip buy triamterene 75 mg online, small changes are likely to be met with more success than large or sudden changes blood pressure journal purchase triamterene pills in toronto. Staying within the range of the different types of foods that children accept to "bridge" toward new foods is generally recommended hypertension before pregnancy triamterene 75mg on line. Repeated exposures to the new foods is also recommended, as it is well recognized that multiple exposures to new foods and flavors are often necessary before acceptance occurs. Children who are accustomed to drinking fluid and eating smooth puree become habituated to using an anterior-posterior tongue pattern to transfer boluses during oral intake. They may have strong sensory reactions if an increased texture of a food is presented and may not yet have acquired the oral motor skills needed to effectively break down and transfer the food for swallowing. These children may respond by "freezing" and allowing the food to stay in the center of the tongue without initiating active tongue movements to prepare and transfer for swallowing. If a texture is presented that requires mastication, they may be unable to initiate the necessary lateral tongue movements and diagonal jaw movements to break down and manipulate the food bolus. If they lose oral control and the food is inadvertently transferred over the tongue base, gagging and choking may ensue. Episodes of gagging, choking, and/or vomiting, coupled with the discomfort of the tactile input of the solid in the oral cavity, may cause these children to develop and maintain a steadfast resistance and adamant refusal of certain textures. In cases where a child lacks the oral motor skills for textured solids due to aversion or lack of experience, starting with treatment strategies that focus on introducing safe non-food stimulation may be indicated. Such techniques provide intraoral tactile input to stimulate the biting and lateral tongue movements that are essential for effective chewing. As the child gains comfort, the next step is to hold the food intraorally and initiate repeated bites to begin to thoroughly break down the food. Placement of the food on the biting surfaces, including the lateral molar surfaces, stimulates the lateral tongue movements that are needed to 572 Pediatric dysPhagia: etiologies, diagnosis, and ManageMent transfer the food intraorally for bolus manipulation prior to transfer for swallowing. Hiding solid lumps or large cracker crumbs within a familiar pureed food is generally not recommended for stimulation of chewing. Children will likely respond with their usual anterior-posterior tongue movement pattern in response to the familiar smooth pureed texture rather than using a different pattern of oral motor movements to manipulate the added texture. Children with sensory overresponsiveness are likely to immediately detect the change in the bolus property and display gagging and/or vomiting. Children with sensory underresponsiveness may not detect the hidden change in texture, which will also result in the transfer of the bolus for swallowing without any change in oral motor pattern, possibly inducing gagging or vomiting. Alternatively, the introduction of an easily dissolvable solid separate from the puree context provides the sensory input of biting and stimulates the lateral tongue movements necessary for the development of an effective chewing pattern. The easily dissolvable property of the solid removes the risk of inadvertent transfer for swallowing and choking. The selection of the easily dissolvable solid type may be most effective when matched to individual taste preferences. Examples of easily dissolvable solids include infant puffs, graham crackers, and buttery crackers. Encouraging the intake of sips of fluid between bites of a new food bolus trial is a strategy that can be used to assist with any persisting oral residual left after the transfer for swallow. As children are learning the oral motor patterns necessary to manage textured foods, the strength of their tongue movements may not be sufficient enough to clear the bolus completely from the oral cavity. Alternating sips of liquid with the solid boluses may help with the transition toward acceptance of the flavor and texture changes. Such restrictions may result in the deficiency of important nutritional components in their diet. Identification of alternative foods or food preparation strategies to match currently accepted sensory characteristics is essential. For example, if a child shows a strong preference for only smooth, pureed foods, intake of a higher calorie smooth food such as Greek yogurt or blended table foods is beneficial. If the child can tolerate some slight texture within a puree, presentation of thicker mashed foods such as hummus, guacamole, and smooth tuna salad may prove to be feasible higher-calorie options. If the child prefers drinking over eating solids, recommendations may include the transition toward intake of higher-calorie smoothie shakes with nutritious ingredients. Specific recommendations by a registered dietitian can be made on an individual basis to target specific nutritional deficiencies within diets that include only a narrow range of preferred and accepted food items. His medical history was negative, with the exception of intermittent gastroesophageal reflux. During the pre-assessment interview with his parents, it became clear that Brandon had demonstrated a strong preference for bottle feeding as an infant, despite best efforts by the parents to offer a range of solid foods. At age 18 months, his oral input consisted of a specific brand and flavor of smooth yogurt, water via a specific cup and straw, a specific type of crunchy oatmeal cereal bar, dry Fruit Loops cereal, and one particular brand of cheddar goldfish crackers. He was unable to tolerate any oral care or tooth brushing, and this was not part of his daily routine. The parents reported that he demonstrated an aversive reaction to the smell of foods, particularly cooking smells associated with dinner items. For example, if a stir-fry meal was being prepared in the kitchen when he was in close proximity, he often became agitated, with intermittent gagging. If presented with a non-preferred food item at a family meal, his typical response was immediate gagging. If he consented to accepting a small taste intraorally, he demonstrated gagging that most often led to vomiting. At the time of the initial interdisciplinary feeding team visit, his intake had dwindled to one specific type of yogurt, and milk. Brandon did not have a regular schedule for meals and snacks and tended to graze on milk from his bottle and his preferred yogurt throughout the day. A multidisciplinary assessment was completed by an interdisciplinary team (physician, occupational therapist, speech pathologist, dietitian, and social worker). During the assessment, a typical meal was simulated, using preferred and non-preferred foods supplied by the family. Brandon sat willingly in a small chair positioned at a child-sized table, and was amenable to presentation of his typical preferred yogurt. When presented with a non-preferred food, his behavior quickly escalated and he abruptly stood by the table, refusing to return to his chair. The parents immediately responded by removing the food, and re-presenting his favorite yogurt and a bottle. He was unable to calm down and his behavior continued to escalate, with coughing, choking, and eventual vomiting. Findings following the team assessment included age-appropriate weight and height, severely limited range of oral intake, intact oral structures and function, adequate oral motor skills for intake of liquid per bottle, and purees by spoon. His sensory overresponsiveness to food smells, the visual presentation of non-preferred foods, and oral care were documented. Recommendations included an upper endoscopy to rule out any underlying physiologic factor. The team recommended a multidisciplinary approach to feeding treatment, with focus initially on the provision of individual sensory integration therapy by the occupational therapist. Brandon responded positively to the sensory strategies and began to show a preference for riding a tricycle during his sensory-based treatment sessions. He was motivated to engage and complete the therapeutic play activities in order to "earn" the tricycle activity. Social stories to introduce the daily therapy activities were used prior to each of the treatment sessions. The parents were coached in the use of therapeutic strategies (use of mealtime routines, presentations of food, strategies for praise, implementation of consequences) and then transitioned into the primary feeding role as the sessions progressed. Brandon was successful in expanding his intake first to a new variety of yogurt flavor, then to a range of smooth table food items. Goals were then shifted to increasing his intake of a range of crunchy and easily dissolvable solids, using the strategy of biting non-food items, to biting through crunchy but immediately dissolvable solids. He made steady and satisfactory progress over the course of weekly multidisciplinary therapy within a 3-month period. Treatment was then decreased to a biweekly cycle for 2 months and then to a monthly follow-up interval for 3 months, with subsequent discharge secondary to satisfactory progress. Sensory integration therapies for children with developmental and behavioral disorders. Supporting children to participate successfully in everyday life by using sensory processing knowledge. Occupational therapy using a sensory integrative approach: a case study of effectiveness. Occupational therapy using a sensory integrative approach for children with developmental disabilities. Systematic review of the research evidence examining the effectiveness of interventions using a sensory integrative approach for children. Effectiveness of cognitive and occupation-based interventions for children with challenges in sensory processing and integration. A systematic review of sensory processing interventions for children with autism spectrum disorders. Caregiver stress and outcomes of children with pediatric feeding disorders treated in an intensive interdisciplinary program. Quality of food-frequency questionnaire validation studies in the dietary assessment of children aged 12 to 36 months: a systematic literature review. Tube feeding in infancy: implications for the development of normal eating and drinking skills. Effect of food texture on the development of chewing of children between six months and two years of age. I also need to go to the dentist, a special doctor who helps me keep my teeth healthy. The dentist has a special office with special chairs that help the dentist and his hygienist get a really good look in my mouth. The toothpaste might taste a little different, and I will keep it in my mouth until the hygienist helps me spit it out with her special tube. The hygienist will also use floss between my teeth to make sure nothing is hiding there! After my teeth are cleaned and checked by the dentist, I will get to do a special activity. Adequate nutritional intake supports optimal growth and development of the child, and successful achievement of infant feeding milestones and appropriate physical growth often serve as an affirmation of parental or caretaker competence. Problems with the developmental feeding process may occur secondary to medical and physiological factors or to environmental factors that shape and maintain problematic feeding behaviors. Although the developmental process of acquiring feeding skills seems instinctual, this process can present a significant challenge and can be a significant source of stress. The overall process of feeding and swallowing is biobehavioral, as it requires intact aerodigestive structures and function, appropriate neurologic control, and a supportive feeding environment with routines and strategies that support continued refinement of infant feeding skills and socioemotional development. Early feeding experiences serve as a catalyst for infant bonding with the caregiver. As the child matures, mealtimes provide a context for building interactional skills as well as opportunities for socialization, celebration, and the development of bonds beyond the immediate family. Feeding practices and dietary customs are passed down from generation to generation, and the achievement of specific milestones such as transitioning to solids or drinking independently from a cup, signal appropriate developmental progress. The stages of behavioral feeding skill development comprise a sequential pattern that consists of three phases - homeostasis, attachment, and separation/individuation. The feeder follows the infant cues, feeding promptly at appropriate intervals and discontinuing the feeding when the infant demonstrates signals of satiety. During the attachment phase, the infant develops reciprocal interactional patterns with caregivers during feeding interactions. During the separation/individuation phase, the infant develops increased awareness of actions and reactions and progresses toward autonomous feeding as cognitive maturation occurs. The infant demonstrates increasing control as independent feeding skills develop, determining the rate of intake and making choices as to which foods to eat. The feeder provides support by providing developmentally suitable foods, regular intervals for meals and snack times, appropriate positioning dur- ing feedings, opportunities for the child to communicate choices between foods, and opportunities for the child to practice and develop independent self-feeding skills. Maladaptive feeding behaviors may occur at any point during the period in which the child is achieving increased autonomy. These behaviors include, but are not limited to , persistent lack of interest in feeding, fears related to feeding, extreme food selectivity, tantrums or disruptive behavior during mealtime, gagging, vomiting, or rumination on foods, and ultimately, inadequate volume of oral intake to support growth and development. The chapters in this section provide an overview of the biobehavioral nature of feeding, environmental factors that influence the feeding process, and the behavioral interventions that are used to treat persistent behavior-based feeding issues. The information presented assumes that oral sensorimotor skills are intact for consumption and that adequate management of medical components of the presenting feeding problem (eg, gastroesophageal reflux disease) is ongoing. In other cases, feeding issues may persist or escalate, thereby presenting serious threats to appropriate growth and development and negatively affecting psychosocial functioning. Furthermore, families of children with feeding problems are at risk for stress and mental health issues. This causes lack of weight gain or the inability to maintain enough weight to grow naturally. In contrast, eating disorders are psychological disorders defined by abnormal eating habits such as bingeing, purging, or fasting. Treatment of inappropriate mealtime behavior is based on behavior modification interventions. Persistent Food refusal Food refusal behaviors include crying, head turning, and batting at the spoon to avoid eating specific foods during mealtimes. Rapid oral intake is often characterized by the intake of inappropriately large-sized bites, with a short and often incomplete oral masticatory phase. If food boluses are transferred for swallowing that contain large chunks of poorly chewed food, there is a risk of choking.

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