Nasal and Paranasal Sinus Surgery in Children with Cystic Fibrosis

1987 ◽  
Vol 96 (4) ◽  
pp. 367-372 ◽  
Author(s):  
Dennis M. Crockett ◽  
Trevor J. McGill ◽  
Gerald B. Healy ◽  
Ellen M. Friedman ◽  
Lesley J. Salkeld
Keyword(s):  
Cystic Fibrosis ◽  
Autosomal Recessive ◽  
Nasal Polyposis ◽  
Autosomal Recessive Disorder ◽  
Sinus Surgery ◽  
Fibrocystic Disease ◽  
Paranasal Sinus Surgery ◽  
Extent Of Surgery ◽  
Sinus Drainage

Cystic fibrosis (mucoviscidosis; fibrocystic disease of the pancreas) is an inherited autosomal recessive disorder that results in generalized dysfunction of exocrine glands. Chronic pansinusitis with nasal polyposis occurs frequently in this disease. Polypectomy and sinus drainage procedures are often required in those cases refractory to medical therapy. Recurrence of nasal polyps following removal is common. Controversy exists regarding the timing and extent of surgery. The management of 40 children with cystic fibrosis who underwent nasal polypectomy, intranasal ethmoidectomy, and Caldwell-Luc procedures to treat extensive sinusitis and nasal polyposis forms the basis for this report. Follow-up data indicate that fewer recurrences and longer symptom-free intervals result when intranasal ethmoidectomy and Caldwell-Luc procedures are combined with polypectomy.

Nasal Polyposis in Children with Cystic Fibrosis: A Long-Term Follow-up Study

2002 ◽  
Vol 111 (12) ◽  
pp. 1081-1086 ◽  
Author(s):  
Matthew W. Yung ◽  
Jim Gould ◽  
Graham J. G. Upton
Keyword(s):  
Cystic Fibrosis ◽  
Nasal Polyposis ◽  
Sinus Surgery ◽  
Computed Tomographic ◽  
Long Term Follow Up ◽  
Grade 3 ◽  
Postoperative Improvement ◽  
Median Interval

The incidence of nasal polyposis among children 5 to 18 years of age with cystic fibrosis (CF) was investigated with a systematic examination of all children on the local CF register. Out of 23 children with CF, 13 had endoscopic evidence of nasal polyposis. Four children had grade 2 polyposis, and 9 children had grade 3 polyposis. Complete opacity of the maxillary sinus was identified on a computed tomographic sinus scan in all but 2 of the children. Only 1 child had a developed frontal sinus. Between 1989 and 2000, 12 children underwent radical endoscopic sinus surgery for their nasal polyposis. There was good postoperative improvement in all of the children; however, 7 eventually required revision surgery because of recurrence of the nasal polyps. The median interval between repeated sinus surgeries was 4 years (range, 18 months to more than 6 years). This information can help in the counseling of parents when sinus surgery is considered for children with CF.

Acute proptosis secondary to bilateral pansinus mucopyocele with orbital compartment syndrome: a rare presentation, its management and outcome

2021 ◽  
Vol 14 (7) ◽  
pp. e241972
Author(s):  
Khushboo Chauhan ◽  
Tanya Sharma ◽  
Bhavana Sharma ◽  
Samendra Karkhur
Keyword(s):  
Compartment Syndrome ◽  
Nasal Polyposis ◽  
Signs And Symptoms ◽  
Sinus Surgery ◽  
Rare Presentation ◽  
Diagnostic Dilemma ◽  
Guided Exploration ◽  
Rapid Relief ◽  
Maxillary Sinuses

A 37-year-old Indian female presented with forward protrusion of left eye for one week, associated with progressive diminution of vision. The patient had undergone sinus surgery for nasal polyposis a year ago. On examination, there was severe proptosis and limitation of extraocular movements in all gazes. CT-scan revealed a heterogeneous expansile mass, arising from left fronto-ethmoidal sinus, infiltrating left orbit. MRI showed T2-hyperintense, peripherally enhancing areas in bilateral nasal cavity (right >left), bilateral ethmoidal sinuses and frontal, sphenoidal and maxillary sinuses. Otorhinolaryngology consultation was sought and in view of vision threatening proptosis — with likely orbital compartment syndrome — emergent endoscopic guided exploration and orbital decompression was undertaken. Rapid relief of signs and symptoms was achieved, with normal vision, extraocular movements and resolution of proptosis. Cytology and microbiology ruled out infectious or malignant aetiology and helped in resolving a diagnostic dilemma. Histopathology confirmed the diagnosis of mucopyocele and follow-up period was uneventful.

scholarly journals Therapeutic guidelines for the treatment of cystic fibrosis

Pharmacia ◽  
2021 ◽  
Vol 68 (1) ◽  
pp. 151-154
Author(s):  
Maria Becheva ◽  
Petar Atanasov
Keyword(s):  
Cystic Fibrosis ◽  
Autosomal Recessive ◽  
Autosomal Recessive Disease ◽  
Clinical Evaluations ◽  
Number Of Patients ◽  
Therapeutic Guidelines ◽  
Therapeutic Measures ◽  
One Year ◽  
Respiratory Rehabilitation

Cystic fibrosis (CF) is a complex, systemic autosomal recessive disease that affects the functions of the respiratory system, the digestive tract and all exocrine glands. The frequency for Europe averages 1: 2500 to 1: 3500 live births. The total number of patients with cystic fibrosis in Bulgaria is about 180. About 10% of the patients are diagnosed at birth. About 60–70% of patients are diagnosed before they reach one year of age. Respiratory symptoms predominate in the clinical picture in patients with cystic fibrosis and determine the prognosis in more than 90% of the patients. The treatment of patients with cystic fibrosis is strictly individualized, pharmacological and non-pharmacological and requires a comprehensive therapeutic approach. The complex therapy also includes bronchodilators, NSAIDs, corticosteroids, respiratory rehabilitation in combination with general body massage. Continued courses of broad-spectrum antibiotics are required to suppress chronic infection. With the progression of the disease, complications such as atelectasis, pneumothorax and pulmonary hemorrhages are observed. The establishment of specialized centers with trained and experienced professionals is essential in order to provide optimal patient care. These include frequent clinical evaluations, follow-up of complications, and early interventions for the treatment of patients with cystic fibrosis. The aim of the article is to familiarize the audience with the therapeutic measures applied in the treatment of patients with cystic fibrosis.

scholarly journals A Roadmap to the Brittle Bones of Cystic Fibrosis

2011 ◽  
Vol 2011 ◽  
pp. 1-10 ◽  
Author(s):  
Ashwini P. Gore ◽  
Soon Ho Kwon ◽  
Antine E. Stenbit
Keyword(s):  
Cystic Fibrosis ◽  
Lung Transplantation ◽  
Bone Disease ◽  
Bone Health ◽  
Autosomal Recessive ◽  
Multidisciplinary Approach ◽  
Early Recognition ◽  
Autosomal Recessive Disorder ◽  
Fatal Disease ◽  
Brittle Bones

Cystic fibrosis (CF) is an autosomal recessive disorder which despite advances in medical care continues to be a life-limiting and often fatal disease. With increase in life expectancy of the CF population, bone disease has emerged as a common complication. Unlike the osteoporosis seen in postmenopausal population, bone disease in CF begins at a young age and is associated with significant morbidity due to fractures, kyphosis, increased pain, and decreased lung function. The maintenance of bone health is essential for the CF population during their lives to prevent pain and fractures but also as they approach lung transplantation since severe bone disease can lead to exclusion from lung transplantation. Early recognition, prevention, and treatment are key to maintaining optimal bone health in CF patients and often require a multidisciplinary approach. This article will review the pathophysiology, current clinical practice guidelines, and potential future therapies for treating CF-related bone disease.

Safety of paranasal sinus surgery in patients with cystic fibrosis

1998 ◽  
Vol 108 (12) ◽  
pp. 1813-1815 ◽  
Author(s):  
Douglas L. Schulte ◽  
Jan L. Kasperbauer
Keyword(s):  
Cystic Fibrosis ◽  
Paranasal Sinus ◽  
Sinus Surgery ◽  
Paranasal Sinus Surgery

Cystic fibrosis

2018 ◽  
Author(s):  
Rowland J. Bright-Thomas ◽  
Andrew M. Jones
Keyword(s):  
Cystic Fibrosis ◽  
Gastrointestinal Tract ◽  
Reproductive System ◽  
Autosomal Recessive ◽  
Pulmonary Infection ◽  
Autosomal Recessive Disorder ◽  
Carrier State ◽  
Male Reproductive System ◽  
Morbidity And Mortality ◽  
Basic Defect

Cystic fibrosis is the most common lethal autosomal recessive disorder in Caucasians. There is no known survival advantage of the heterozygote carrier state. Chronic progressive pulmonary infection and bronchiectasis are the major causes of morbidity and mortality. The disease affects all ductal systems where the basic defect is manifest, including the pancreas, gastrointestinal tract, sinuses, hepatobiliary system, and male reproductive system, and has significant effects on nutrition and growth.

Longitudinal Follow-up of Malignant Osteopetrosis by Skeletal Radiographs and Restriction Fragment Length Polymorphism Analysis After Bone Marrow Transplantation

PEDIATRICS ◽  
1992 ◽  
Vol 90 (6) ◽  
pp. 986-989
Author(s):  
ROBERT E. SCHROEDER ◽  
F. LEONARD JOHNSON ◽  
MICHAEL J. SILBERSTEIN ◽  
WILMA L. NEUMAN ◽  
JEANNE M. HOAG ◽  
...  
Keyword(s):  
Autosomal Recessive ◽  
Fragment Length Polymorphism ◽  
Autosomal Recessive Disorder ◽  
Polymorphism Analysis ◽  
Newborn Period ◽  
Normal Bone ◽  
Malignant Osteopetrosis ◽  
Osteoclast Function ◽  
Infantile Malignant Osteopetrosis

Infantile malignant osteopetrosis is a rare autosomal recessive disorder characterized by presentation in the first months of life with manifestations related to an underlying defect in osteoclast function. Abnormal osteoclast activity paired with normal bone formation by osteoblasts leads to development of densely sclerotic fragile bones. Encroachment on the marrow cavities by hyperostotic bone results in profound anemia and thrombocytopenia, with extramedullary ullary hematopoiesis and hypersplenism. Deficits in immune function can lead to presentation with overwhelming sepsis in the newborn period. Narrowing of the optic and auditory foramina can lead to progressive blindness and hearing loss. Until recently, the prognosis for this disorder had been uniformly dismal with death usually occurring within a few months.l-3

Bilateral Antrochoanal Polyp with Diabetes: A Rare Presentation of Cystic Fibrosis

2010 ◽  
Vol 3 (3) ◽  
pp. 173-176
Author(s):  
Monica Gupta ◽  
Manish Gupta
Keyword(s):  
Cystic Fibrosis ◽  
Endoscopic Sinus Surgery ◽  
English Literature ◽  
Functional Endoscopic Sinus Surgery ◽  
Sinus Surgery ◽  
Adolescent Male ◽  
Antrochoanal Polyp ◽  
Rare Presentation ◽  
First Case

Abstract Bilateral antrochoanal (AC) polyps have been rarely reported in english literature. Here we report the first case of an adolescent male with bilateral AC polyps, cystic fibrosis and diabetes. The case was managed successfully by functional endoscopic sinus surgery (FESS). The patient has been under follow-up for the last six months with no signs of recurrence.

Meticulous endoscopic sinus surgery to prevent recurrence of massive nasal polyposis

2012 ◽  
Vol 126 (8) ◽  
pp. 789-794 ◽  
Author(s):  
S M S Hoseini ◽  
B Saedi ◽  
K Aghazadeh
Keyword(s):  
Endoscopic Surgery ◽  
Endoscopic Sinus Surgery ◽  
Nasal Polyposis ◽  
Demographic Data ◽  
Effective Means ◽  
Sinus Surgery ◽  
Previous Surgery ◽  
Endoscopic Findings ◽  
History Of

AbstractObjective:To evaluate the effect of meticulous endoscopic surgery, including opening of all involved sinus cells, on the subsequent symptoms and endoscopic findings of patients with massive nasal polyposis.Study design and method:One hundred patients with massive nasal polyposis resistant to medical treatment were selected. We documented each patient's demographic data, associated diseases, endoscopic findings, Lund–Mackay score and Sino-Nasal Outcome Test 22 (SNOT22) symptom score. All patients were followed up for at least two years to evaluate any recurrence.Results:Of the 100 patients, 20 per cent had a history of asthma and 27 per cent had undergone previous surgery. All underwent endoscopic sinus surgery. After two years of follow up, 8 per cent had recurrence requiring surgery. Recurrence was significantly associated with a history of asthma (p < 0.001) and the histopathological presence of eosinophilia (p = 0.014).Conclusion:Meticulous endoscopic opening of all involved sinus cells can be a safe and effective means of controlling massive nasal polyposis, with an acceptable recurrence rate.

Allergic Rhinitis and Aspirin-Exacerbated Respiratory Disease as Predictors of the Olfactory Outcome after Endoscopic Sinus Surgery

2009 ◽  
Vol 23 (3) ◽  
pp. 348-353 ◽  
Author(s):  
Michael Katotomichelakis ◽  
Maria Riga ◽  
Spyridon Davris ◽  
Gregorios Tripsianis ◽  
Maria Simopoulou ◽  
...  
Keyword(s):  
Allergic Rhinitis ◽  
Respiratory Disease ◽  
Endoscopic Sinus Surgery ◽  
Nasal Polyposis ◽  
Sinus Surgery ◽  
Olfactory Acuity ◽  
Aspirin Exacerbated Respiratory Disease ◽  
Negative Effect ◽  
Time Frames

Background Unlike the functional outcomes of endoscopic sinus surgery, which have been thoroughly studied, the effect of the surgery on olfactory performance and the relative predictive factors have not been adequately assessed by literature. Allergic rhinitis and aspirin-exacerbated respiratory disease (AERD) are examined as potential confounding factors of the olfactory outcome in patients with extensive nasal polyposis and rhinosinusitis treated with functional endoscopic sinus surgery (FESS). Methods A population of 116 adults with severe nasal polyposis was subjected to FESS after failure of the appropriate medical treatment. The olfactory outcome was quantified by Sniffin’ Sticks at the 1st, 3rd, and 6th postoperative month in relation to the concomitant presence of allergic rhinitis (n = 62) or AERD (n = 18). Results Allergic patients seemed to perform worse than nonallergic patients at all time frames. However, when patients with similar olfactory acuity, age, and medical history are compared, allergic rhinitis does not seem to affect the postoperative improvement of the composite threshold-discrimination-identification scores. The same seems to apply for the likelihood of acquiring normosmia after surgery. On the contrary, AERD significantly limits the recovery of olfactory function at all follow-up examinations and patients with AERD are unlikely to become normosmic. Conclusion The olfactory recovery after FESS for nasal polyposis is significantly affected by the concomitant presence of AERD. Although allergy seems to have a general negative effect on olfactory acuity, it was not found to affect the extent of the olfactory improvement, when patients with comparable preoperative characteristics are addressed.

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