scholarly journals Plexiform Neurofibroma of the Larynx: A Challenging Management Dilemma

2013 ◽  
Vol 3 (2) ◽  
pp. 55-57
Author(s):  
Gauri Kapre

ABSTRACT Introduction Among the many causes responsible for stridor in the pediatric population, tumors of the larynx are relatively rare. Rarer still is the presence of endolaryngeal neurofibromas. Plexiform neurofibromas are associated with type I neurofibromatosis (NF1). Their typical characteristic is that they are diffuse tumors with indistinct margins, which makes complete resection a challenge and the chances of recurrences higher. Objective To document our experience with endolaryngeal neurofibromas and to discuss the treatment options available for this rare condition. Methods We present two cases of plexiform neurofibromas in pediatric patients. Both children presented with large supraglottic masses which interfered with breathing and swallowing. They also fulfilled the criteria for type I neurofibroma. Endolaryngeal approach with microdebrider and laser-assisted surgical excision was performed in both cases. Result Following surgery, both patients had uneventful recovery and no subsequent breathing or swallowing difficulties. Conclusion The dilemma in the management of endolaryngeal neurofibroma is the choice between endolaryngeal laser and aggressive external approach surgery. A short review of the limited existing literature shows that it is wiser to limit the surgery to as complete a resection as is possible endoscopically. How to cite this article Nerurkar NK, Kapre G. Plexiform Neurofibroma of the Larynx: A Challenging Management Dilemma. Int J Phonosurg Laryngol 2013;3(2):55-57.

2019 ◽  
Vol 70 (1) ◽  
pp. 190-184 ◽  
Author(s):  
Ovidiu Gabriel Bratu ◽  
Alexandru Ionut Cherciu ◽  
Adrian Bumbu ◽  
Sorin Lupu ◽  
Dragos Radu Marcu ◽  
...  

Retroperitoneal tumors, once considered uncommon, have been reported in the last years in such numbers that they cannot be considered anymore a rare condition. Tumor recurrence following surgical excision is quite common and a tumor that was considered originally as benign in many cases can recur as a malignant tumor. The purpose of this paper is to underline the difficulties in terms of establishing a correct diagnosis and a proper therapeutic protocol when facing a retroperitoneal mass of unknown origin, as well as to present the available data regarding prognosis, treatment options and tumor recurrence. Retroperitoneal tumors can be classified as benign or malignant; solid, cystic or both; single or multiple, and of varied histological types. A high percentage of patients with retroperitoneal tumors are discovered in advanced stages, usually seeking medical help for symptoms related to nearby organ compression/invasion. This pathology requires a prompt and adequate multidisciplinary management, in order to achieve disease control and to reduce the recurrence rate. Complete surgical resection is the potential curative treatment for retroperitoneal tumors and it is best managed in high-volume centers, by a multidisciplinary team. Complete oncological tumor resection and tumor grade remain the most important predictors for local recurrence and disease-specific survival. Further research is required in order to define the role of radiotherapy, as well as to discover new biological therapies that target various molecular pathways involved in retroperitoneal cancers.


2017 ◽  
Vol 5 ◽  
pp. 2050313X1774051
Author(s):  
Dean J Samaras ◽  
Andrew C Kingsford

Extensive hypertrophic scarring of the halluces secondary to chronic onychocryptosis is a rare condition, which causes significant physical and psychosocial effects. In this case, a 31-year-old male developed large lesions on both great toes after he delayed treatment of chronic hallucal onychocryptosis for over a decade. Current treatment options for hypertrophic and keloid lesions in the foot and ankle vary considerably and differentiation is critical for appropriate treatment planning. In this case, surgical excision with total matrixectomy (modified Zadik–Syme) was considered optimal management. Histopathology testing confirmed the diagnosis of irritated hypertrophic scar secondary to onychocryptosis. The patient was monitored closely and at 3 months post-operatively, the incisional scars exhibited progressive maturation, and there was no recurrence of the lesions and no nail regrowth. Furthermore, the halluces were only marginally shorter providing good function and cosmesis. At the long-term follow-up consultation (5.5 years), the patient indicated complete satisfaction and had returned to regular footwear and social activities. Chronic onychocryptosis can trigger and facilitate proliferation of large benign keloid-like fibrous lesions; excision with total matrixectomy can provide an excellent long-term outcome.


Author(s):  
Mate Naszai ◽  
James Ramsden

Cholesteatoma is a rare condition affecting 9-12.6 adults and 3-15 children per 100,000 per annum [1–4], with a more aggressive presentation in the paediatric population [5]. Intermittent otorrhea (ear discharge) is the presenting complaint in over half of cholesteatoma patients [6, 7]. The peak incidence of cholesteatoma is 5-15 years of age [8] which overlaps significantly with a period of high incidence in otitis media [9] and externa [10], diseases that often present the same way as cholesteatoma. This results in diagnosis that may take several years. Left untreated, cholesteatoma can cause significant lasting damage in the form of deafness, vertigo, facial paralysis, meningitis, and brain abscesses which may prove fatal [11]. Current treatment options are limited to surgical excision with the aim to establish a safe and manageable ear, while maintaining hearing is secondary. Improving surgical instrumentation has allowed a better success rate, however, revision surgeries remain a mainstay of practice. In practical terms, this means that those affected by bilateral disease often undergo surgery 4 or more times [12]. This represents a significant burden for patients. The decision about the exact surgical approach (canal wall up vs canal wall down) is a careful balancing act of safety versus functionality, and the pros and cons must be weighed in light of available evidence and the skill of the surgeon [13].


Author(s):  
Neha Jain ◽  
Shama Shishodia ◽  
Ruchima Dham ◽  
Suparna Roy ◽  
Sachin Goel

<p class="abstract">Schwannomas are rare, solitary, slow growing, smooth surfaced and well encapsulated tumors. Schwannomas of head and neck region account for 25-40% of all the cases. Approximately 1%–12% of schwannomas occur intraorally, the tongue being the most common site. Complete surgical excision is the treatment of choice.  In this article, we describe a case of tongue schwannoma in a child, along with diagnostic and treatment options of tongue lesions. The tongue mass was completely excised via trans-oral approach using coblation method. The patient followed up for 1 year; he had an uneventful recovery and no recurrence.</p>


Diagnostics ◽  
2021 ◽  
Vol 11 (11) ◽  
pp. 2112
Author(s):  
Adrian Constantin ◽  
Florin Achim ◽  
Dan Spinu ◽  
Bogdan Socea ◽  
Dragos Predescu

Introduction: Idiopathic megacolon (IM) is a rare condition with a more or less known etiology, which involves management challenges, especially therapeutic, and both gastroenterology and surgery services. With insufficiently drawn out protocols, but with occasionally formidable complications, the condition management can be difficult for any general surgery team, either as a failure of drug therapy (in the context of a known case, initially managed by a gastroenterologist) or as a surgical emergency (in which the diagnostic surprise leads additional difficulties to the tactical decision), when the speed imposed by the severity of the case can lead to inadequate strategies, with possibly critical consequences. Method: With such a motivation, and having available experience limited by the small number of cases (described by all medical teams concerned with this pathology), the revision of the literature with the update of management landmarks from the surgical perspective of the pathology appears as justified by this article. Results: If the diagnosis of megacolon is made relatively easily by imaging the colorectal dilation (which is associated with initial and/or consecutive clinical aspects), the establishing of the diagnosis of idiopathic megacolon is based in practice almost exclusively on a principle of exclusion, and after evaluating the absence of some known causes that can lead to the occurrence of these anatomic and clinical changes, mimetically, clinically, and paraclinically, with IM (intramural aganglionosis, distal obstructions, intoxications, etc.). If the etiopathogenic theories, based on an increase in the performance of the arsenal of investigations of the disease, have registered a continuous improvement and an increase of objectivity, unfortunately, the curative surgical treatment options still revolve around the same resection techniques. Moreover, the possibility of developing a form of etiopathogenic treatment seems as remote as ever.


2005 ◽  
Vol 57 (suppl_4) ◽  
pp. ONS-E407-ONS-E407 ◽  
Author(s):  
Fortino Salazar ◽  
Andre Machado ◽  
Sudish Murthy ◽  
Nicholas M. Boulis

Abstract OBJECTIVE AND IMPORTANCE: The present article describes a rare presentation of Type I neurofibromatosis (NFI) involving adjoining intercostal plexiform neurofibromas, as well as the novel use of thoracoscopy to guide surgical resection. This presentation highlights the manner in which NFI may affect selective regions of the body disproportionately through genetic mosaicism. CLINICAL PRESENTATION: A 40-year-old man had intractable neuropathic anterior right chest wall pain in the distribution of T2 and T3. Magnetic resonance imaging and computed tomography of the chest revealed masses deep to the second and third ribs in the axilla. Biopsy of a subcutaneous nodule in the right chest wall revealed a small neurofibroma. An extensive workup revealed no masses outside the region of the right chest wall and no stigmata of NFI. INTERVENTION: The patient underwent right-sided thoracoscopy for identification of the intrathoracic neurofibromas and placement of spinal needles to localize the anterior and posterior extent of the masses. These landmarks were used to guide a transaxillary approach to third rib resection and nerve-sparing neurofibroma resection. CONCLUSION: Isolated adjoining intercostal plexiform neurofibroma is a unique presentation of mosaic NFI. Because of its limited penetrance, this variant may present as a regional pain syndrome. Thoracoscopy can be used effectively to guide intercostal nerve sheath tumor resection.


2019 ◽  
pp. 199-206
Author(s):  
О. З. Скакун ◽  
С. В. Федоров ◽  
О. С. Вербовська ◽  
І. З. Твердохліб

Distinctive atrioventricular type I heart block is diagnosed when the PQ interval is 0.30 s. or more. Prolongation of the PQ interval more than 0.50 s. is a very rare condition. Usually it is associated with a pseudo-pacemaker syndrome. The last one manifests itself with dizziness, syncope, general weakness, shortness of breath upon physical exertion, cough, seizures, cold sweat, a feeling of pulsation in the head, neck and abdomen, a headache, paroxysmal nocturnal dyspnea, swelling of the lower extremities, tachypnea and jugular venous pulsation. The P wave appears immediately after the previous QRS complex. Atrial contraction occurs at the moment when the ventricles don’t relax after the previous contraction; due to the fact that pressure in the ventricles at this moment is higher than in the atria, the tricuspid and mitral valves remains closed. During the atrial contraction, most of the blood is ejected not into the ventricles, but backward into the pulmonary veins from the left atrium and into the venae cavae from the right atrium. Also, an atrial kick is absent which results in a less ventricular filling. There is increased pressure in the atria leading to their distension and excessive secretion of the atrial natriuretic peptide. A case report of the distinctive atrioventricular type I heart block associated with the pseudo-pacemaker syndrome is described. The patient suffered from a pre-syncope, short-term dizziness during the previous two days, tinnitus, general weakness, feeling of pulsation in the abdomen, neck, head, which interfered with his sleep. He developed these complaints after an infectious disease, which manifested as a runny nose and sore throat. In this patient, an extremely prolonged PQ interval up to 0.70 s. was observed. Also, episodes of Mobitz I and Mobitz type II atrioventricular block were detected. During the monitoring of patient state, the interval PQ was gradually shortening, and in 1 month it reached the normаl duration. It can be assumed that in the case of distinctive atrioventricular type I heart block, a significant prolongation of the refractory period in the rapid pathways of the AV-node plays a key role in the pathogenesis of this condition. According to the recommendations of the ACC/AHA (1998), for patients with distinctive atrioventricular type I heart block accompanied by the pseudo-pacemaker syndrome and documented alleviation of symptoms with temporary AV pacing, the pacemaker implantation should be considered (IIaB). The implantation of dual chamber pacemaker may reduce symptoms and lead to an improvement in the functional state of patients, in whom shortening of the interval between atrial and ventricular contractions improves hemodynamics. For asymptomatic patients with the PQ interval of ≥ 0.30 s, pacemaker is not recommended. The distinctive atrioventricular type I heart block in patients with pseudo-pacemaker syndrome is a rare condition and often remains undiagnosed. But it may have a benign course with a gradual normalization of the PQ interval. Indications for permanent pacemaker implantation should be reviewed as this block may be completely reversible. A permanent pacemaker may be used in the case of absence of positive dynamics in a shortening of the PQ interval.    


2021 ◽  
pp. 1-3
Author(s):  
Priyanka Prasanna ◽  
Chenni S. Sriram ◽  
Sarah H. Rodriguez ◽  
Utkarsh Kohli

Abstract Sialidosis, a rare autosomal recessive disorder, is caused by a deficiency of NEU1 encoded enzyme alpha-N-acetyl neuraminidase. We report a premature male with neonatal-onset type II sialidosis which was associated with left ventricular dysfunction. The clinical presentation and subsequent progression which culminated in his untimely death at 16 months of age are succinctly described. Early-onset cardiovascular involvement as noted in this patient is not well characterised. The case report is supplemented by a comprehensive review of the determinants, characteristics, and the clinical course of cardiovascular involvement in this rare condition.


2021 ◽  
pp. 1-4
Author(s):  
Baher M. Hanna ◽  
Wesam E. El-Mozy ◽  
Sonia A. El-Saiedi

Abstract Isolated sub-pulmonary membrane is a rare condition, the origin of which has been debatable. Transcatheter treatment of pulmonary valve atresia with intact interventricular septum by radiofrequency perforation and balloon dilatation to restore biventricular circulation is gaining more popularity, with improving results over time. We report in our experience of 79 cases in 10 years the development of a sub-pulmonary membrane in 4 cases: causing significant obstruction requiring surgical excision in one case that revealed a fibrous membrane on pathology; causing mild right ventricular outflow tract obstruction in another and not yet causing obstruction in 2. On cardiac MRI, the right ventricular outflow tract and the right ventricular outflow tract/pulmonary atresia angle showed no morphological abnormalities.


Sign in / Sign up

Export Citation Format

Share Document