scholarly journals P.114 Benign tumors of peripheral nerves in children at a tertiary-care pediatric hospital

Author(s):  
A Yaworski ◽  
K Koujok ◽  
K Cheung ◽  
Y Ying ◽  
H McMillan

Background: Tumors affecting peripheral nerves in children are rare. Accurate diagnosis ensures that management is appropriate and timely. Methods: We review the clinical presentation and utility of investigations of children with intrinsic tumors affecting peripheral nerves at the Children’s Hospital of Eastern Ontario (CHEO). Results: From 2009-2019, 14 cases were identified. Mean age of symptom onset was 8.2 years (range 0.3 to 17.3 years). Presenting symptoms included painless muscle wasting (2/14), focal muscle weakness (7/14), contracture (1/14), pain (1/14) or a painless, palpable mass (3/14). MRI was useful at differentiating benign pediatric nerve tumors. Peripheral nerve lipomatosis demonstrated a classic “spaghetti string” appearance. Patients with perineurioma showed evidence of enhancing, nodular lesions while intraneural ganglionic cysts display cystic lesion within the nerve. Neurofibromas appear like a “bag of worms” while schwannomas are more eccentrically positioned around the nerve. Nerve conduction studies (NCS) or electromyography (EMG) were performed in 11/14 patients. Biopsies were performed in 9 patients and surgical management in 4 patients. Conclusions: The rare nature of peripheral nerve tumors in children can pose diagnostic challenges. NCS/EMG are important to assist with localization, and MRI important at distinguishing benign tumors. Key MRI, clinical and NCS features can guide management, potentially avoiding invasive procedures.

Author(s):  
Josee Paradis ◽  
Agnieszka Dzioba ◽  
Hamdy El-Hakim ◽  
Paul Hong ◽  
Frederick K. Kozak ◽  
...  

Abstract Background To evaluate the clinical presentation of choanal atresia (CA) in tertiary centers across Canada. Methods Multi-centre case series involving six tertiary care pediatric hospitals across Canada. Retrospective chart review of patients born between 1980 and 2010 diagnosed with CA at a participating center. Results The health charts of 215 patients (59.6% female) with CA were reviewed and included in this study. The mean age of patients at time of CA presentation was 0.4 months (range 0.1 to 7.2 months) for bilateral CA and 37.8 months (range 0.1 to 164.1 months) for unilateral cases. The most common presenting symptoms for bilateral CA in decreasing order were respiratory distress (96.4%), feeding difficulties (68.2%), and rhinorrhea (65.5%), and for unilateral cases in decreasing order were rhinorrhea (92.0%), feeding difficulties (24.7%), and respiratory distress (18.0%). For the majority of patients (73.2%), the obstruction comprised mixed bony and membranous tissue, with only 10.5% presenting with a purely membranous obstruction. Familial history of CA was confirmed in only 3.3% of cases. One half of patients with CA presented with one or more associated anomalies and 30.6% had a syndrome. Conclusions The present investigation is the first national multi-institutional study evaluating the clinical presentation of CA over three decades. The present cohort of CA patients presented with a breadth of co-morbidities with highly variable presentations, with bilateral cases being more severely affected than unilateral cases. Further investigation into hereditary linkages to CA development is warranted. Graphical abstract


Author(s):  
Alexander Scarborough ◽  
Robert J MacFarlane ◽  
Michail Klontzas ◽  
Rui Zhou ◽  
Mohammad Waseem

The upper limb consists of four major parts: a girdle formed by the clavicle and scapula, the arm, the forearm and the hand. Peripheral nerve lesions of the upper limb are divided into lesions of the brachial plexus or the nerves arising from it. Lesions of the nerves arising from the brachial plexus are further divided into upper (proximal) or lower (distal) lesions based on their location. Peripheral nerves in the forearm can be compressed in various locations and by a wide range of pathologies. A thorough understanding of the anatomy and clinical presentations of these compression neuropathies can lead to prompt diagnosis and management, preventing possible permanent damage. This article discusses the aetiology, anatomy, clinical presentation and surgical management of compressive neuropathies of the upper limb.


Author(s):  
Sanjana S. Malokar ◽  
Saurabh V. Kothari ◽  
Onkar H. Nadgouda

Background: The following study is about the clinical profile and outcome of patients with acute non traumatic paraparesis. It includes the aetiology, clinical presentation and the outcome of various cases of acute non traumatic paraparesis. Paraplegia or paraparesis could be defined as loss of function of both legs as a result of disease or injury of the spinal cord, spinal roots, peripheral nerves or myopathies. Acute non-traumatic paraparesis is a neurological emergency. Reversible causes of acute paraplegia can be treated successfully if diagnosed early.Methods: The observational study was done in the department of general medicine at D. Y. Patil Hospital, Navi Mumbai with sample size of 75 patients over 1 year.Results: With early diagnosis prognosis of acute non traumatic paraparesis can be improved which was evaluated over period of 3 months.Conclusions: Acute non-traumatic paraparesis is a neurological emergency. Reversible causes of acute paraplegia can be treated successfully if diagnosed early. It is important to diagnose and classify all cases into compressive and non-compressive lesions based on presenting symptoms because the management of the two differs.


2019 ◽  
Vol 6 (3) ◽  
pp. 769
Author(s):  
Maragadha Mani ◽  
Anand A.

Background: Varicose veins are common vascular disorder. The study was conducted to analyse the clinical presentation, treatment options and complications of varicose veins.Methods: This observational study was conducted in Thanjavur Medical College Hospital. Adults with clinically diagnosed unilateral or bilateral varicose veins of lower limbs were studied in surgical wards between August 2013 to July 2014. The demographic data and presenting symptoms, signs and previous treatment were collected using a structured proforma. Thorough clinical examination, duplex scan and abdominal and pelvic examination were done in all cases to find out the secondary causes. Peripheral vascular system was examined. All the patients were followed regularly for the period of one month to one year after treatment.Results: A total of 60 cases were included. Most participants belonged to 2nd and 3rd decade of life. 55 (91%) participants were male and5 (9%) participants were females. 45 (75%) participants were agriculturists. 10 (17%) participants had bilateral involvement. Most participants 30 (50%) participants had 1 to 5 years duration of disease, most common clinical presentation of the study participants was varicosity with 70.0%, followed by lipodermo sclerosis, leg pain, hyperpigmentation, pruritus as 63.0%, 56.0%, 15% and 11.6% respectively. 17 (28.33%) patients were present with a venous ulcer. 32 (53%) participants were managed surgically, and 28 (47%) participants were managed conservatively. Most participants were treated with multiple ligation 21.8% followed by trendelenburg operation+ stripping18.75%.Conclusions: Regarding the treatment, surgery is the treatment of choice for primary varicose veins and conservative treatment for secondary varicose veins.


Author(s):  
Ishrat Zuber ◽  
Vaishali Chaurasia

Background: Ectopic pregnancy is a catastrophic and life-threatening condition and one of the commonest acute abdominal emergency in day-to-day practice. Objectives of present study were to know the incidence of Ectopic pregnancy at Tertiary care centre, the age group, parity and risk factor of ectopic pregnancy, the clinical presentation of ectopic pregnancy and the outcome and management of ectopic pregnancy.Methods: It was a retrospective study conducted at Chirayu Medical College and Hospital, Bhopal from 1st June 2009 to 31st May 2014. A total of 42 patients with ectopic pregnancy were analyzed on clinical presentation, clinical findings, investigations, operative findings and outcome.Results: A majority of women (64.29%) were in the age group of 21-30 years and 78.57% were multigravida. Commonest risk factors were previous history of abortion (23.80%) and pelvic inflammatory disease (14.28%) Amenorrhea (92.85%) and pain abdomen (89.22%) were the most common presenting symptoms. Ampulla of fallopian tube was the commonest site (45.23%). Unilateral salpingectomy was performed in 40.47% patients. There were no maternal deaths and majority (80.96%) were discharged within seven days.Conclusions: Ectopic pregnancy still remains one of the major causes of maternal morbidity and mortality. Early diagnosis and referral in hemodynamically stable state along with use of minimal access surgery or medical management can change the scenario of ectopic pregnancy in the developing world.


2020 ◽  
Vol 2020 ◽  
pp. 1-4 ◽  
Author(s):  
Che-Yuan Kuo ◽  
Chieh-Chih Tsai ◽  
Shu-Ching Kao ◽  
Wen-Ming Hsu ◽  
Catherine Jui-Ling Liu

Purpose. To compare the clinical characteristics and treatment outcome between benign and malignant lacrimal sac tumors. Methods. We retrospectively reviewed the medical records of all patients with pathologically confirmed lacrimal sac lesions from 1995 to 2018 in a tertiary medical center. Results. Among 65 eligible cases, 46 (70.8%) were benign lacrimal sac tumors and 19 (29.2%) were malignant lacrimal sac tumors. Secondary malignancy from nasal or paranasal cancer accounted for 47% of malignant lacrimal sac tumors. The patient’s mean age at the time of diagnosis was 60 years in the benign group and 48 years in the malignant group (p=0.03). The most common presenting symptoms were a palpable lump/mass and epiphora in both groups. Palpable mass extending above the medial canthal tendon was noted in 9% of the benign group and in 74% of the malignant group, respectively (p<0.001). Bloody tears were noted in 5% of the benign group and in 20% of the malignant group (p=0.21). In the malignant group, 10 (52.6%) were primary tumors and 9 (47.4%) were secondary tumors. The primary sites of the metastatic tumor to the lacrimal sac area were mostly from neighboring paranasal sinuses and nasal cavity. The recurrence rate was higher in patients with malignant tumors, as compared to the benign tumors (42% and 6%, respectively, p=0.001). The metastatic rate was 47% and the mortality rate was 53% in malignant lacrimal sac tumors. Conclusion. Although benign and malignant lacrimal sac tumors may present similar initial symptoms, timely diagnosis and intervention for malignant lacrimal sac lesions are important because they tend to be infiltrating tumors with a poor outcome.


Neurosurgery ◽  
2007 ◽  
Vol 60 (4) ◽  
pp. E777-E778 ◽  
Author(s):  
Shunsuke Kawamoto ◽  
Hadzki Matsuda ◽  
Keisuke Ueki ◽  
Yoshifumi Okada ◽  
Phyo Kim

Abstract OBJECTIVE Neuromuscular choristomas (NMC) are rare benign tumors of the peripheral nerves. We report an NMC affecting the oculomotor nerve. CLINICAL PRESENTATION An 18-year-old girl presented with long-standing intermittent retro-orbital pain and oculomotor paresis. Magnetic resonance imaging scans demonstrated a small nodular lesion on the left oculomotor nerve, similar to the findings for a schwannoma. INTERVENTION The tumor was resected with the parental oculomotor nerve, which was reconstructed using a peroneal nerve graft. Postoperatively, the patient became pain-free, and her oculomotor function partially recovered. Histologically, the lesion consisted of well-differentiated smooth muscle fibers intermingled with mature nerve elements consistent with the diagnosis of an NMC, although the possibility of leiomyoma in this rare location was not excluded completely. CONCLUSION NMC may need histological confirmation for diagnosis if they occur in the intracranial space. The resection is feasible, and the function of the affected nerve can be at least partially restored with the nerve reconstruction.


2021 ◽  
Vol 11 ◽  
Author(s):  
Hyojung Park ◽  
Min-Sun Kim ◽  
Jiwon Lee ◽  
Jung-Han Kim ◽  
Byong Chang Jeong ◽  
...  

PurposePheochromocytoma (PCC) and paraganglioma (PGL) (PPGL) are rare neuroendocrine tumors, and data on managing these conditions in children and adolescents are lacking. The objective of this study was to demonstrate the clinical presentation and treatment outcomes in children and adolescents with PPGL in a single tertiary care center in Korea.MethodsThis retrospective study included 23 patients diagnosed with PCC (n = 14) and PGL (n = 9) before the age of 21 at Samsung Medical Center (from June 1994 to June 2019). We describe age, gender, family history, clinical characteristics, laboratory findings, pathologic findings, therapeutic approaches, and treatment outcomes.ResultsOf the 23 patients, 14 had PCC and nine had PGL. The median age at diagnosis was 16.8 years (range, 6.8–20.8 years). The common presenting symptoms were hypertension (n = 10), headache (n = 9), palpitation (n = 4), and sweating (n = 4). The plasma or 24-hour urine catecholamine and/or metabolite concentrations were markedly elevated in 22 patients with PPGL, but were normal in one patient with carotid body PGL. All tumors were visualized on computed tomography. Genetic tests were performed in 15 patients, and seven patients showed mutations in RET (n = 3), SDHB (n = 3), and VHL (n = 1). All patients underwent surgery, and complete excision was performed successfully. Three patients with metastasis underwent postoperative adjuvant therapy.ConclusionThis study suggests that pediatric PPGL tends to be extra-adrenal and bilateral and shows a higher potential for genetic mutations. Considering the hereditary predisposition of pediatric PPGL, genetic screening tests are strongly recommended, and lifelong follow-up is needed to detect recurrence and metastasis. Further research with a larger sample size and routine genetic screening is needed to better understand the genetic conditions and long-term prognosis of PPGL.


2012 ◽  
Vol 2012 ◽  
pp. 1-5 ◽  
Author(s):  
Hamad S. Al-Muhaimeed ◽  
Hazem Y. Abdelwahed ◽  
Essam A. Elgamal ◽  
Ghassan M. Alokby ◽  
Ameen M. Binnasser ◽  
...  

Dermoid cysts of the head and neck are rare congenital benign tumors. According to the literature they represent about seven percent of all dermoids and less than one percent of all intracranial neoplasms. Extradural dermoid cysts are very rare. We report a case of intracranial extradural dermoid cyst of mastoid bone. We believe that this is the second documented extradural dermoid cyst, the first case reported in the literature (Ammirati et al., 2007) was in close relation to the petrous apex but ours is in close relation to mastoid antrum. Hearing loss was the only clinical presentation in this case, while neurological symptoms were the main presenting symptoms in the first reported case. We present our management of this rare case with respect to the clinical, radiological, histopathological, and surgical aspects and conclude that dermoid tumors, though rare, need to be included in differential diagnosis of middle ear lesions.


2007 ◽  
Vol 22 (6) ◽  
pp. 1-8 ◽  
Author(s):  
Claude-Edouard Châtillon ◽  
Marie-Christine Guiot ◽  
Line Jacques

Benign peripheral nerve lesions of lipomatous, vascular, and chondromatous origin are very rare. Only one previous case of brachial plexus involvement by such a tumor has been reported. The authors report on their experience with peripheral nerve tumors in three patients and review the available literature on these topics. The three cases discussed include a 44-year-old woman with an intraneural lipoma of the right middle trunk, a 40-year-old woman with an intraneural hemangioma infiltrating the right posterior cord, and a newborn male with a predominantly cartilaginous hamartoma originating from the right C-5 nerve root. The literature review yielded six previous cases of intraneural lipoma, approximately 50 cases of lipofibromatous hamartoma, 13 cases of intraneural hemangioma, and no previous case of cartilaginous hamartoma originating from a nerve. Intraneural lipomas are well encapsulated, and gross-total resection can be achieved. Lipofibromatous hamartomas are diffusely infiltrative; decompressive debulking and neurolysis is often the most appropriate initial approach for patients with symptomatic lesions. Resection of intraneural hemangiomas can be achieved but may require nerve resection and repair in some cases. Debulking has been reported to provide prolonged symptomatic relief in these lesions, and preoperative embolization and postoperative radiotherapy were beneficial in the case presented here. To the authors' knowledge, this is the first reported case of a cartilaginous hamartoma infiltrating a peripheral nerve. Gross-total resection of symptomatic intraneural lipomas is feasible and apparently curative. The optimal treatment for lipofibromatous hamartomas and vascular and chondromatous lesions of the peripheral nerves is uncertain and should be guided by the severity of symptoms.


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