scholarly journals Lingual Frenectomy in Joubert Syndrome

2017 ◽  
Vol 18 (8) ◽  
pp. 728-731 ◽  
Author(s):  
Vivek Gurjar ◽  
Minal Gurjar ◽  
Channaveer Pattanshetti ◽  
Banashree Sankeshwari
Keyword(s):  
General Anesthesia ◽  
Surgical Intervention ◽  
Joubert Syndrome ◽  
Rare Disorder ◽  
Anesthetic Care ◽  
Long Term Follow Up ◽  
Utmost Care ◽  
Distinguishing Features

ABSTRACT Aim Our aim is to present a case of a rare disorder, such as Joubert syndrome (JS) which required oral surgical intervention under general anesthesia at a very young age to help in achieving normal developmental milestones. Background Ciliopathies are an emerging class of diseases of which JS is a significant member. There have been very few cases of JS with its distinguishing features which have been reported in recent literature. Case report We herewith present a case of JS who reported to our unit with a complaint of speech abnormality. Intraoral examination revealed a tongue-tie which was planned for surgical release. As any drugs that interfere with respiratory depression have to be used with utmost care, adequate precautions were taken in this case and the patient was treated under general anesthesia. The tongue-tie was released and associated hamartomatous outgrowths from the floor of the mouth were also excised. Appropriate postoperative care was taken and the patient was discharged. Conclusion The patient treated by us did not reveal any significant intraoperative or postoperative complications. Treatment of cases of JS required a team effort of multiple dental and medical specialists. Long-term follow-up of such patients is required so that they achieve normal chronological development. Clinical significance Joubert syndrome being a rare disorder requires special anesthetic care during any surgical procedure, especially that involving the oral cavity. The need and timing of surgical intervention in such cases should be carefully assessed and appropriate precautions should be taken. How to cite this article Gurjar V, Gurjar M, Pattanshetti C, Sankeshwari B. Lingual Frenectomy in Joubert Syndrome. J Contemp Dent Pract 2017;18(8):728-731.

Joubert syndrome: long-term follow-up

2004 ◽  
Vol 46 (10) ◽  
Author(s):  
Peter R Hodgkins ◽  
Christopher M Harris ◽  
Fatima S Shawkat ◽  
Dorothy A Thompson ◽  
Kling Chong ◽  
...  
Keyword(s):  
Joubert Syndrome ◽  
Long Term Follow Up

scholarly journals The Long-Term Follow-Up of Patients with Cystine Stones: A Single-Center Experience for 13 Years

2021 ◽  
Vol 10 (7) ◽  
pp. 1336
Author(s):  
Toshifumi Takahashi ◽  
Shinya Somiya ◽  
Katsuhiro Ito ◽  
Toru Kanno ◽  
Yoshihito Higashi ◽  
...  
Keyword(s):  
Surgical Intervention ◽  
Median Number ◽  
Small Sample ◽  
Age At Diagnosis ◽  
Surgical Interventions ◽  
Significant Difference ◽  
Long Term Follow Up ◽  
Cystine Stones

Introduction: Cystine stone development is relatively uncommon among patients with urolithiasis, and most studies have reported only on small sample sizes and short follow-up periods. We evaluated clinical courses and treatment outcomes of patients with cystine stones with long-term follow-up at our center. Methods: We retrospectively analyzed 22 patients diagnosed with cystine stones between January 1989 and May 2019. Results: The median follow-up was 160 (range 6–340) months, and the median patient age at diagnosis was 46 (range 12–82) years. All patients underwent surgical interventions at the first visit (4 extracorporeal shockwave lithotripsy, 5 ureteroscopy, and 13 percutaneous nephrolithotripsy). The median number of stone events and surgical interventions per year was 0.45 (range 0–2.6) and 0.19 (range 0–1.3) after initial surgical intervention. The median time to stone events and surgical intervention was 2 years and 3.25 years, respectively. There was a significant difference in time to stone events and second surgical intervention when patients were divided at 50 years of age at diagnosis (p = 0.02, 0.04, respectively). Conclusions: Only age at a diagnosis under 50 was significantly associated with recurrent stone events and intervention. Adequate follow-up and treatment are needed to manage patients with cystine stones safely.

Chronic Plantar Heel Pain: Treatment with a Short Leg Walking Cast

1996 ◽  
Vol 17 (1) ◽  
pp. 41-42 ◽  
Author(s):  
Christopher L. Tisdel ◽  
Marion C. Harper
Keyword(s):  
Complete Resolution ◽  
Surgical Intervention ◽  
Pain Treatment ◽  
Heel Pain ◽  
Treatment Modalities ◽  
Plantar Heel Pain ◽  
Long Term Follow Up

The efficacy of a short leg walking cast in the treatment of chronic plantar heel pain was assessed for 32 patients with 37 involved extremities treated over a 2-year period. All patients had failed numerous other treatment modalities and had been symptomatic for an average of 1 year. Long-term follow-up for 24 patients with 28 involved extremities revealed complete resolution of pain for 7 extremities (25%), improvement for 17 (61%), and no improvement for 4 (14%). Ten (42%) patients were completely satisfied with cast treatment, 3 (12%) were satisfied with reservations, and 11 (46%) were dissatisfied. Casting appears to be a reasonable option for patients with recalcitrant heel pain and should be offered before surgical intervention.

scholarly journals LGG-40. NATURAL COURSE AND MANAGEMENT OF SMALL ASYMPTOMATIC LESION SUSPECTED OF LOW-GRADE GLIOMA IN CHILDREN

2020 ◽  
Vol 22 (Supplement_3) ◽  
pp. iii374-iii374
Author(s):  
Ai Muroi ◽  
Takao Tsurubuchi ◽  
Hidehiro Kohzuki ◽  
Ryoko Suzuki ◽  
Hiroko Fukushima ◽  
...  
Keyword(s):  
Surgical Intervention ◽  
Natural Course ◽  
Low Grade ◽  
Diffuse Astrocytoma ◽  
Mr Images ◽  
Initial Examination ◽  
Long Term Follow Up ◽  
Intracranial Lesions

Abstract OBJECTIVE The natural course of incidentally discovered small intracranial lesions has not been well discussed. Surgical intervention, including resection and biopsy, could be achieved if the lesion is growing. We present 13 cases with incidentally found, small non-enhancing lesions without related symptoms. METHODS We retrospectively reviewed a series of 13 children with T1 hypointense and T2 hyperintense intracranial lesions less than 20 mm in diameter without enhancement. We excluded the patients with NF-1 or Tuberous sclerosis. RESULTS Most patients underwent MRI for headache unrelated to the lesions. All cases were located supratentorially. The median age of the patients at the initial examination was 8.9 years (range, 2.2–14.6). Of these children, 2 patients (15.3%) underwent surgery because of progression on follow-up MR images. The pathological diagnosis was compatible with diffuse astrocytoma. Patients were followed for a median of 55 months (range, 11–87) and the overall survival rate was 100%. No patient experienced increase in size after 3 years of follow-up. CONCLUSIONS In most patients with small intracranial lesions, the lesions remained stable and conservative management was appropriate. However, in a few cases, the lesions changed in size or quality and surgical intervention was necessary. Long-term follow-up at least 3 years is mandatory.

Early and late outcomes of surgical repair of double-chambered right ventricle: a single-centre experience

2020 ◽  
Vol 30 (3) ◽  
pp. 409-412
Author(s):  
Murat Surucu ◽  
İlkay Erdoğan ◽  
Birgül Varan ◽  
Murat Özkan ◽  
N. Kürşad Tokel ◽  
...  
Keyword(s):  
Ventricular Septal Defect ◽  
Right Ventricle ◽  
Septal Defect ◽  
Surgical Intervention ◽  
Systolic Pressure ◽  
Presenting Symptoms ◽  
Long Term Follow Up ◽  
The Right

AbstractObjective:Double-chambered right ventricle is characterised by division of the outlet portion of the right ventricle by hypertrophy of the septoparietal trabeculations into two parts. We aim to report our experiences regarding the presenting symptoms of double-chambered right ventricle, long-term prognosis, including the recurrence rate and incidence of arrhythmias after surgery.Methods:We retrospectively investigated 89 consecutive patients who were diagnosed to have double-chambered right ventricle and underwent a surgical intervention from 1995 to 2016. The data obtained by echocardiography, cardiac catheterisation, and surgical findings as well as post-operative follow-up, surgical approaches, post-operative morbidity, mortality, and cardiac events were evaluated.Results:Median age at the time of diagnosis was 2 months and mean age at the time of operation was 5.3 years. Concomitant cardiac anomalies were as follows: perimembranous ventricular septal defect (78 patients), atrial septal defect (9 patients), discrete subaortic membrane (32 patients), right aortic arch (3 patients), aortic valve prolapse and/or mild aortic regurgitation (14 patients), and left superior caval vein (2 patients). The mean follow-up period was 4.86 ± 4.6 years. In these patients, mean systolic pressure gradient in the right ventricle by echocardiography before, immediately, and long-term after surgical intervention was 66.3, 11.8, and 10.4 mmHg, respectively. There were no deaths during the long-term follow-up period. Surgical reinterventions were performed for residual ventricular septal defect (2), residual pulmonary stenosis (1), and severe tricuspid insufficiency (1).Conclusion:The surgical outcomes and prognosis of double-chambered right ventricle are favourable, recurrence and fatal arrhythmias are unlikely in long-term follow-up.

Long-Term Follow-Up for Children Treated With Surgical Intervention for Chronic Rhinosinusitis

2006 ◽  
Vol 116 (12) ◽  
pp. 2099-2107 ◽  
Author(s):  
Rodney P. Lusk ◽  
Marcella R. Bothwell ◽  
Jay Piccirillo
Keyword(s):  
Chronic Rhinosinusitis ◽  
Surgical Intervention ◽  
Long Term Follow Up

Surgical management of uterine torsion through ventral celiotomy in eleven mares

2019 ◽  
Author(s):  
Jasmeet Singh Khosa ◽  
Arun Anand ◽  
Vandana Sangwan ◽  
Shashi Kant Mahajan ◽  
Jitendra Mohindroo ◽  
...  
Keyword(s):  
Cesarean Section ◽  
Surgical Management ◽  
Surgical Intervention ◽  
Normal Position ◽  
Inhalation Anesthesia ◽  
Uterine Torsion ◽  
Long Term Follow Up ◽  
Veterinary Hospital

This article describes the surgical management of uterine torsion by midline celiotomy for cesarean section in 11 mares presented with signs of colic at a teaching veterinary hospital. The mares were either in full term of gestation (n = 6) or in advanced stage of pregnancy (n = 5). Majority of the mares (44.5%) were in fourth parity. 78% cases of uterine torsion were post cervical and clockwise and (22%) cases were post cervical and anticlockwise. For surgical intervention, mares were anesthetized using a combination of xylazine (1.1 mg/kg) and ketamine (2.2 mg/kg), intravenously and maintained on isoflurane inhalation anesthesia. Midline celiotomy was performed, and foals were delivered by cesarean section. Before closing the abdominal wound, the uterus was detorted manually and confirmed per-vaginally for its normal position. After long-term follow-up, the study reported 90.0% (10/11) survival rate out of which, seven were bred successfully.

scholarly journals A Rare but Fascinating Disorder: Case Collection of Patients with Schnitzler Syndrome

2018 ◽  
Vol 2018 ◽  
pp. 1-4 ◽  
Author(s):  
Maaman Bashir ◽  
Brittany Bettendorf ◽  
Richard Hariman
Keyword(s):  
Interleukin 1 ◽  
Response To Therapy ◽  
Rare Disorder ◽  
First Line ◽  
First Line Therapy ◽  
Schnitzler Syndrome ◽  
Long Term Follow Up ◽  
Case Collection

Background. Schnitzler syndrome is a rare disorder characterized by a chronic urticarial rash and monoclonal gammopathy (IgM in more than 90% of the cases). It is difficult to distinguish from other neutrophilic urticarial dermatoses, and diagnosis is based on the Strasbourg criteria. Interleukin-1 is considered the key mediator, and interleukin-1 inhibitors are considered first line treatment. Here, we present two cases of Schnitzler syndrome, both successfully treated with anakinra. Objectives. To increase awareness regarding clinical presentation, diagnosis, and treatment of this rare disorder. Cases. We describe the clinical features and disease course of two patients with Schnitzler syndrome, diagnosed using the Strasbourg criteria. Both were treated with anakinra with remarkable response to therapy. Conclusion. Schnitzler syndrome is a rare and underdiagnosed disorder. High suspicion should be maintained in patients with chronic urticaria-like dermatoses, intermittent fevers, and arthralgias. A serum protein electrophoresis and immunofixation should be performed in these patients. The diagnosis is important to recognize as Schnitzler syndrome is associated with malignancy. A lymphoproliferative disorder develops in about 20% of patients at an average of 7.6 years after onset of symptoms. Thus, patients warrant long-term follow-up. IL-1 inhibitors are extremely effective in relieving symptoms and are considered first line therapy.

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