Congenital Nasal Pyriform Aperture Stenosis: Diagnosis and Management

2009 ◽  
Vol 46 (3) ◽  
pp. 262-267 ◽  
Author(s):  
Marion Devambez ◽  
Alexis Delattre ◽  
Pierre Fayoux
Keyword(s):  
Surgical Treatment ◽  
Genetic Disorder ◽  
Tertiary Care ◽  
Case Series ◽  
University Hospital ◽  
Treatment Modalities ◽  
Nasal Decongestants ◽  
Sublabial Approach ◽  
Pyriform Aperture

Objectives: To review diagnosis and treatment modalities in congenital nasal pyriform aperture stenosis in a newborn population. Study Design: A 6-year retrospective case series review. Mean follow-up of 21.8 months. Setting: University hospital, tertiary care center. Patients: Twenty-one consecutive patients treated for congenital nasal pyriform aperture stenosis. Diagnosis was suspected on physical examination and confirmed by computed tomography scan. Associated abnormalities were present in 15 patients. Surgical Treatment: Nineteen patients underwent surgical treatment focused on external bony margins of pyriform aperture and the osseous anterior edge of the inferior turbinate, drilling by a sublabial approach and associated with a partial turbinectomy in seven cases. A stent was placed for 7 to 10 days. A conservative treatment based on topical nasal decongestants and gastroesophageal reflux treatment was proposed for two patients. Results: Postoperative follow-up revealed septal ulceration in five cases with septal perforation in one case and development of synechiae in two cases. Follow-up revealed normal nasal breathing and pyriform aperture growth after surgery. Conclusion: Our experience confirmed the link described between congenital nasal pyriform aperture stenosis and holoprosencephaly, but associated extracraniofacial malformations suggest that congenital nasal pyriform aperture stenosis should be integrated in a systemic malformative syndrome or genetic disorder and lead us to propose an exhaustive dysmorphology assessment. Some infants may be treated by conservative management, and severely affected patients may undergo surgery by a sublabial approach.

scholarly journals Exclusive endoscopic transcanal transpromontorial approach: a new perspective for internal auditory canal vestibular schwannoma treatment

2017 ◽  
Vol 126 (1) ◽  
pp. 98-105 ◽  
Author(s):  
Daniele Marchioni ◽  
Matteo Alicandri-Ciufelli ◽  
Alessia Rubini ◽  
Babara Masotto ◽  
Giacomo Pavesi ◽  
...  
Keyword(s):  
Skull Base ◽  
Tertiary Care ◽  
Internal Auditory Canal ◽  
Case Series ◽  
University Hospital ◽  
Cerebrospinal Fluid Leakage ◽  
Complication Rates ◽  
First Case ◽  
The University

OBJECTIVE The aim of this study was to describe the first case series in which an exclusive endoscopic transcanal transpromontorial approach (EETTA) was used to treat small vestibular schwannomas (VSs) and meningiomas of the internal auditory canal (IAC). METHODS The authors performed a retrospective review of patients who had undergone surgery using an EETTA to the IAC at 2 university tertiary care referral centers during the period from November 2011 to January 2015. RESULTS Ten patients underwent surgery via an EETTA for the treatment of VS in the IAC at the University Hospital of Modena or the University Hospital of Verona. The patients had Koos Grade I or II tumors and American Academy of Otolaryngology–Head and Neck Surgery (AAO-HNS) Class D hearing status preoperatively. Gross-total resection was achieved in all patients. No major complications such as cerebrospinal fluid leakage or hemorrhage were reported. In 7 of 10 (70%) patients, facial nerve function was normal immediately after surgery (Rough Grading System [RGS] Grade I). Two patients presented with a transitory facial palsy immediately after surgery (RGS Grade II–III) but experienced complete recovery during the follow-up period. The mean follow-up was 10 months. CONCLUSIONS The EETTA proved to be successful for the removal of VS or meningioma involving the cochlea, fundus, and IAC, with possible lower complication rates and less invasive procedures than those for traditional microscopic approaches. The potential for the extensive and routine use of this approach in lateral and posterior skull base surgery will depend on the development of technology and surgical refinements and on the diffusion of skull base endoscopic skills among the otolaryngological and neurosurgical communities.

Cystic dilation of the ventriculus terminalis

2012 ◽  
Vol 17 (1) ◽  
pp. 86-92 ◽  
Author(s):  
Mario Ganau ◽  
Andrea Talacchi ◽  
Paolo C. Cecchi ◽  
Claudio Ghimenton ◽  
Massimo Gerosa ◽  
...  
Keyword(s):  
Surgical Treatment ◽  
Clinical Status ◽  
Relevant Literature ◽  
University Hospital ◽  
Neurological Deficits ◽  
Herniated Disc ◽  
Treatment Modalities ◽  
Consecutive Series ◽  
Type I

Object The ventriculus terminalis, an embryological remnant consisting of the ependymal-lined space of the conus medullaris, can occasionally become symptomatic after cystic dilation. In the existing literature, consisting of 32 cases, the preferred type of management (conservative vs surgical) is still debated. The object of this study was to report the surgical results in a consecutive series of 10 adult patients with cystic dilation of the ventriculus terminalis (CDVT), to match them with data retrieved from the relevant literature, and specifically to validate a new recent clinical classification. Methods The authors reported 13 new cases of CDVT treated in the Department of Neurosurgery at University Hospital in Verona, Italy. Treatment modalities and clinical and radiological outcomes, both early and at follow-up, were analyzed and compared with a preoperative classification of clinical presentation, as established by de Moura Batista and colleagues (2008). Results Surgical treatment seemed to guarantee the resolution of CDVT. Dorsolumbar laminotomy, myelotomy, and cystic drainage were performed in 10 patients. Patients with Type I symptoms (nonspecific complaints) often presented with comorbidities (herniated disc or facet hypertrophy) confusing their clinical status. The surgical treatment of patients with Type I symptoms promoted good results only if the diagnosis of CDVT was definitive and symptoms had rapidly evolved. In patients with Type II (focal neurological deficits) and III (sphincter disturbances) symptoms, surgical treatment sustained improvement even at the late follow-up. Conclusions While confirming the usefulness of de Moura Batista and colleagues' classification in its impact on prognosis, the authors propose a revision of the classification with subgroups Type Ia (nonspecific symptoms without clear relation to CDVT), which is best treated conservatively, and Type Ib (rapid onset and invalidating unspecific complaints without comorbidities), which may benefit from surgical evacuation.

Midcarpal instability

2015 ◽  
Vol 41 (1) ◽  
pp. 86-93 ◽  
Author(s):  
D. G. Hargreaves
Keyword(s):  
Surgical Treatment ◽  
Treatment Options ◽  
Case Series ◽  
Definite Diagnosis ◽  
Stress Tests ◽  
Functional Instability ◽  
Painful Wrist ◽  
Relative Rarity ◽  
Passive Stress

Midcarpal instability is a collective term for a number of conditions where the instability of the wrist is predominantly between the proximal and distal carpal rows. It has been regarded as relatively rare and infrequently requires surgical treatment. Palmar midcarpal instability is the most commonly found type of midcarpal instability and can be responsible for causing a clunking and painful wrist. The diagnosis is made on clinical grounds using the midcarpal instability provocative tests. Standard imaging and arthroscopic inspection do not usually confirm a definite diagnosis, but are important in excluding other pathologies. The classification and staging has been described using the extent of palmar translocation of the distal carpal row, which is elicited on passive stress tests. As this is a functional instability, it may be that a functional staging description might be better, and a proposed scheme is described. Treatment options including partial wrist fusions, tenodesis stabilizations and arthroscopic capsular shrinkage have been described in small case series with limited follow-up. There are no comparative series or randomized studies because of the relative rarity of this condition.

scholarly journals Clinical and laboratory follow-up after hospitalization for COVID-19 at an Italian tertiary care center

2021 ◽  
Author(s):  
Michele Spinicci ◽  
Iacopo Vellere ◽  
Lucia Graziani ◽  
Marta Tilli ◽  
Beatrice Borchi ◽  
...  
Keyword(s):  
Infectious Diseases ◽  
Care Center ◽  
Tertiary Care ◽  
University Hospital ◽  
Tertiary Care Center ◽  
Persistent Symptom ◽  
Post Discharge

Abstract We evaluated 100 post-acute COVID-19 patients, a median of 60 days (IQR 48-67) after discharge from the Careggi University Hospital, Italy. Eighty-four (84%) had at least one persistent symptom, irrespective of COVID-19 severity. A considerable number of hospital re-admission (10%) and/or infectious diseases (14%) during the post-discharge period was reported.

Follow-Up Care Barriers for Patients with Orofacial Clefts

2021 ◽  
pp. 105566562110421
Author(s):  
Joshua Van Swol ◽  
Bethany J. Wolf ◽  
Julia Toumey ◽  
Phayvanh Pecha ◽  
Krishna G. Patel
Keyword(s):  
Outcome Measure ◽  
Tertiary Care ◽  
University Hospital ◽  
Exclusion Criteria ◽  
Actual Patient ◽  
The Mean ◽  
Return Visits ◽  
Significant Factors ◽  
Patient Attendance

Objective The aim of this study was to evaluate whether a patient with a cleft's age, associated syndrome, cleft phenotype or travel distance affects their follow-up rate. Design This study is a retrospective review of patients with CL/P treated by a craniofacial clinic. Setting The setting was a craniofacial clinic at a tertiary care university hospital. Patients, Participants Candidates were patients seen by the craniofacial clinic between January 2007 and December 2019. An initial pool of 589 patients was then reduced to 440 due to exclusion criteria. Interventions None Main Outcome Measure(s) The outcome measure was actual patient attendance to the craniofacial team compared to the team goal expectation of annual return visits. Results The mean age of participants at the end of the study was 9.0 ±  5.4 years with a mean follow-up period (total possible follow-up period length based on patient age at presentation and study window) of 5.5 ±  3.6 years. There was no association between cleft phenotype, type of syndrome, or distance to the clinic with attendance. Children with syndromes had an 11% decrease in the odds of attending follow-up visits with each 1-year increase in age compared to a 4% decrease in children without syndromes. Conclusions The only significant factors determining patient attendance were the presence of a syndrome and increasing age.

scholarly journals Surgical Correction of Penoscrotal Web: A Case Series With Literature Review

2020 ◽  
Author(s):  
Volkan Sarper Erikçi
Keyword(s):  
Surgical Treatment ◽  
Clinical Characteristics ◽  
Surgical Techniques ◽  
Case Series ◽  
Scrotal Skin ◽  
Penile Skin ◽  
Surgical Experience ◽  
Postoperative Results ◽  
Pediatric Urologist

INTRODUCTION: Penoscrotal webbing (PSW) is an anomaly of penis and it includes penile and scrotal skin aberration. There are various surgical techniques for repairing PSW with different terminologies. Herein we present our surgical experience of Z-plasty procedure in these cases. METHODS: In this retrospective study, 5 patients with an average age of 46 months who were diagnosed and under follow-up for PSW, between June 2017 and May 2019 were included. Along with demographic and clinical characteristics, treatment and follow-up records were collected. RESULTS: Isolated PSW was observed in 4 patients and one patient had an associated megameatus intact prepuce (MMIP) of a hypospadias variant in addition to PSW. Circumcision and ventral prepuce reconstruction of the penis with the aid of "Z-plasty" solved problem and acceptable postoperative results were obtained. DISCUSSION AND CONCLUSION: PSW is a condition that warrants surgical treatment. During the management of these children, in the case of suspicion of penile skin abnormality at the time of circumcision, it should be deferred and should be consulted to a pediatric surgeon or a pediatric urologist. Gentle surgical treatment is recommended for a favourable surgical and psychological result

Oronasal Transfixion Suture to Prevent Uplifted Nasal Floor Deformity in Cleft Lip and Palate Patients: A 5-Year Follow-Up

2018 ◽  
Vol 56 (3) ◽  
pp. 390-394
Author(s):  
Yuta Nakajima ◽  
Shunsuke Yuzuriha ◽  
Fumio Nagai ◽  
Kenya Fujita ◽  
Masahiko Noguchi
Keyword(s):  
Private Practice ◽  
Cleft Lip ◽  
Chart Review ◽  
Outcome Measure ◽  
Cleft Lip And Palate ◽  
Tertiary Care ◽  
University Hospital ◽  
Nasal Floor ◽  
Orbicularis Oris Muscle

Objective: In unilateral cleft lip and palate, the reconstructed nasal floor is sometimes uplifted regardless of the reconstructive method used. We used a 5-0 absorbable anchoring suture, the oronasal transfixion suture (ONT suture), to fasten the reconstructed nasal floor to the orbicularis oris muscle to prevent this deformity. This study was performed to evaluate the effects of the ONT suture. Design: Blind retrospective study of photography and chart review. Setting: Shinshu University Hospital, tertiary care, Nagano, Japan. Private practice. Patients: Ninety-three consecutive patients with unilateral complete cleft lip and palate who had undergone primary nasolabial repair in our department and affiliated hospitals between 1999 and 2011 participated in this study. Finally, 45 patients were included. Interventions: The ONT suture was put in place at the time of primary nasolabial repair. Main Outcome Measure: The height of the nasal floor was evaluated on submental view photographs at 5 years old. Results: The ONT suture was applied in 21 patients. The height of the nasal floor on the cleft side was significantly closer to that on the noncleft side with the ONT suture than without the ONT suture ( P = .008). Conclusions: The ONT suture is effective to prevent uplifted nasal floor deformity on the cleft side// in unilateral complete cleft lip and palate at the time of primary nasolabial repair.

Frequency of Success in Surgery of Vesicovaginal Fistula by Layered Closure with Graft Repair

2021 ◽  
Vol 15 (10) ◽  
pp. 3479-3481
Author(s):  
Anila Mujadid Qureshi ◽  
Azra Parveen Rajpar ◽  
Ishrat Saba Mari ◽  
Khalida Avesi ◽  
Kousar Fatima ◽  
...  
Keyword(s):  
Success Rate ◽  
Vesicovaginal Fistula ◽  
Case Series ◽  
University Hospital ◽  
Blue Dye ◽  
Urogenital Fistula ◽  
Vesicovaginal Fistulae ◽  
University Setting ◽  
Distressing Condition

Introduction: Vesicovaginal fistulae is abnormal communication between bladder and vagina that cause continous dribling of urine. It is physically, mentally and socially distressing condition. There are various approaches for surgeries of these urogenital fistulae with different success-rate that depend upon the experience of surgeon and surgical procedures. This study can help us to estimate the success rate of layered repair with graft in vaginal route to make stragedy to adopted in severe patient. Objective: To determine frequency of success in surgery on vesic-ovaginal fistulae by layered closured with graft repair procedures among patients admitted in Isra University. Setting: Obstetrics & Gynecology department in Isra university hospital Duration: 6 months from 10.2.2014 to 10.8.2014 Study Design: Case series Subject and methods: A total of 100 patients after having surgery for vesicovaginal fistula by layered closure with graft repair was included in this study. History and examination of all subjects were taken. The follow up visit was planned after 3 weeks of surgery. All women was questioned for recurrence of continuous urinary leakage and that without such symptoms proved by absence of leakage on methylene blue dye test was labeled as ‘success’. Results: - Frequency of success in surgery on vesic-ovaginal fistulae by layered closured with graft repair procedures was observed in 88% cases. Conclusion: The success rate of VVF repair by layered closured with graft repair procedures is high. It is concluded that obstetric urogenital fistula is a preventable condition. Keywords: Vesicovaginal fistulae, Layered closured, Graft repair, urogenital fistula

Three Patients with CPT1A Deficiency: Literature Review and Case Series

2020 ◽  
Author(s):  
Naser Ali Mirhosseini ◽  
Mahdieh Saatchi ◽  
Sana Taghiyar
Keyword(s):  
Genetic Disorder ◽  
Case Series ◽  
Autosomal Recessive Inheritance ◽  
Abnormal Liver Function Test ◽  
Medical Procedures ◽  
Main Method ◽  
Appropriate Treatment ◽  
Liver And Kidney ◽  
Renal Tubular

Background: Carnitine palmitoyltransferase 1A deficiency is a rare genetic disorder with autosomal recessive inheritance pattern of fatty acid metabolism secondary to CPT1A mutation. Several dozen infants and children have been described with a deficiency of the liver and kidney CPT1 isoenzyme (CPT1-A). Clinical manifestation includes fasting-induced hypoketotic hypoglycemia, occasionally with extremely abnormal liver function test (LFT) and rarely with renal tubular acidosis. Acyl carnitine analysis has been the main method for the diagnosis of CPT1A deficiency.  Prompt treatment of hypoglycemia includes intravenous fluid containing 10% dextrose. To prevent hypoglycemia, infants should eat frequently during the day and have cornstarch continuously at night. Fasting should not last more than 12 hours during illness, surgery, or medical procedures. Case Presentation: We reported three patients with CPT1A deficiency presented with hypoglycemia and Reye like syndrome in early childhood that with early diagnosis and treatment they are well in follow-up. Conclusion: Prognosis of this genetic disorder will be good with appropriate treatment.

scholarly journals Surgical outcome of isolated congenital supravalvular pulmonary stenosis: a case series

2019 ◽  
Vol 3 (2) ◽  
Author(s):  
Vikas Kumar ◽  
Sachin Mahajan ◽  
Vivek Jaswal ◽  
Shyam Kumar Singh Thingnam
Keyword(s):  
Pulmonary Stenosis ◽  
Main Pulmonary Artery ◽  
Tertiary Care ◽  
Case Series ◽  
Surgical Correction ◽  
Medium Term ◽  
Constriction Band ◽  
Supravalvular Stenosis ◽  
Mid Term Results

Abstract Background Supravalvular stenosis of main pulmonary artery is a rare anomaly characterized by the presence of constriction band just above the pulmonary valve. It is mostly acquired after intervention on the pulmonary trunk or less commonly is congenital in origin associated with complex congenital cardiac malformations and very rarely can present as an isolated native congenital supravalvular pulmonary stenosis (SPS). Case summary We present a series of four cases of isolated congenital SPS who underwent surgical correction at our tertiary care institute over 8 years. Mean age of the patients was 2.25 ± 0.96 years with all of them being males. Mean peak systolic gradient across the stenosis was 82 ± 21.48 mmHg ranging from 60 mmHg to 110 mmHg. There was no early and medium-term mortality with 100% survival at mean follow-up of 31 months (range 7–85 months). Discussion Surgical correction of congenital SPS carries excellent early and mid-term results with almost no mortality and very low risk of re-intervention for restenosis.

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