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1.
BMC Urol ; 21(1): 152, 2021 Nov 08.
Article in English | MEDLINE | ID: mdl-34749695

ABSTRACT

BACKGROUND: Midureteral stenosis is very rare in children and can cause congenital hydronephrosis. We report our experience treating children with congenital midureteral stenosis at our center, focusing on the differences in preoperative diagnosis and treatment compared with other congenital obstructive uropathies. METHODS: We retrospectively reviewed the medical records of 26 children diagnosed with congenital midureteral stenosis at our center between January 2007 and December 2020, such as preoperative examination methods, intraoperative conditions, and postoperative follow-up results. RESULTS: Of the 1625 children treated surgically for ureteral narrowing, only 26 (1.6%) were diagnosed with midureteral stenosis, including 15 infants and 11 children. Eighteen (69.2%) were boys, 13 (50%) were affected on the left side, and 23 (88.5%) had isolated ureteral stenosis. Overall, 13 (50%) of the children presented with prenatal hydronephrosis, and 13 (50%) presented with abdominal pain or a mass. All the children had undergone urinary ultrasound and intravenous urography preoperatively; the diagnostic rate of ultrasound was 92.3%. Only 7 (26.9%) children had undergone pyelography. All the children had undergone surgery. The ureteral stenotic segment was less than 1 cm long in 25 (96.2)% of the children. The mean follow-up duration was 22 months (range: 6-50 months). One child developed anastomotic strictures. Urinary tract obstruction was relieved in the other children without long-term complications. CONCLUSIONS: Congenital midureteral stenosis is rare, accounting for 1.6% of all ureteral obstructions, and its diagnosis is crucial. Urinary ultrasound has a high diagnostic rate and should be the first choice for midureteral stenosis. Retrograde pyelography can be used when the diagnosis is difficult, but routine retrograde pyelography is not recommended. Congenital ureteral stenosis has a relatively short lesion range, largely within 1 cm. The treatment is mainly resection of the stenotic segment and end-to-end ureteral anastomosis, with a good prognosis.


Subject(s)
Constriction, Pathologic/congenital , Constriction, Pathologic/diagnostic imaging , Ureteral Diseases/congenital , Ureteral Diseases/diagnostic imaging , Child , Child, Preschool , Constriction, Pathologic/complications , Constriction, Pathologic/surgery , Female , Humans , Hydronephrosis/etiology , Male , Retrospective Studies , Ultrasonography , Ureteral Diseases/complications , Ureteral Diseases/surgery , Ureteral Obstruction/etiology , Urography
2.
Rev Med Suisse ; 15(665): 1765-1768, 2019 Oct 02.
Article in French | MEDLINE | ID: mdl-31580021

ABSTRACT

Congenital tracheal stenosis is a rare malformation of the fibrino-cartilaginous tracheal skeleton, frequently associated with cardiac malformations. The symptoms and the age of presentation varie according to the severity of the stenosis. There is a risk of airway obstruction. The diagnosis is based on endoscopy and surgery is usually required.


La sténose trachéale congénitale est une malformation rare du squelette fibro-cartilagineux de la trachée, fréquemment associée à des malformations cardiaques. Le tableau clinique et l'âge de présentation varient en fonction de la sévérité de la sténose. Il existe un risque d'obstruction des voies aériennes. Le diagnostic repose sur l'endoscopie et le traitement est chirurgical.


Subject(s)
Constriction, Pathologic/congenital , Constriction, Pathologic/surgery , Trachea/abnormalities , Airway Obstruction , Constriction, Pathologic/diagnosis , Humans , Trachea/surgery , Tracheal Stenosis/congenital , Tracheal Stenosis/diagnosis , Tracheal Stenosis/surgery
3.
Harefuah ; 156(10): 619-622, 2017 Oct.
Article in Hebrew | MEDLINE | ID: mdl-29072377

ABSTRACT

INTRODUCTION: Congenital stenosis (partial obstruction) of the small intestine is uncommon in comparison to atresia (complete obstruction). The clinical manifestations of stenosis could be delayed up to several weeks post-delivery. We present a case wherein a baby with a prenatal ultrasound examination showed a suspicious picture of small bowel obstruction. However, the clinical manifestations after delivery and imaging studies were misleading and non-classic; therefore, the resection of the stenotic portion of the ileum was delayed until the baby was one month of age. After the operation the baby stopped vomiting but diarrhea continued. The infant's formula was changed to formula containing medium chain triglyceride (MCT) instead of long chain triglyceride which proved effective in stopping the diarrhea. We emphasis the importance of including the differential diagnosis of congenital obstruction in any baby with vomiting (especially bile stain vomiting) despite the fact that the baby had diarrhea and not constipation and the imaging studies were not obvious.


Subject(s)
Constriction, Pathologic/congenital , Intestinal Obstruction/congenital , Constriction, Pathologic/diagnostic imaging , Humans , Ileum , Infant , Infant, Newborn , Intestinal Obstruction/diagnostic imaging , Ultrasonography , Vomiting/etiology , Vomiting/prevention & control
4.
Eur Arch Otorhinolaryngol ; 273(3): 587-91, 2016 Mar.
Article in English | MEDLINE | ID: mdl-25762353

ABSTRACT

To investigate whether differences existing in the distance between facial nerve (FN) and round window niche opening among congenital aural atresia (CAA), congenital aural stenosis (CAS) and a normal control group and to assess its effect on the round window implantation of vibrant soundbridge, CT images of 10 normal subjects (20 ears), 27 CAS patients (30 ears) and 25 CAA patients (30 ears) were analyzed. The distances from the central point of round window niche opening to the terminal point of the horizontal segment, the salient point of pyramidal segment, the beginning point of the vertical segment, and the vertical segment of the facial nerve (abbreviate as OA, OB, OC, OE, respectively) were calculated based on three-dimensional reconstruction using mimics software. The results suggested that the pyramidal segment of the FN was positioned more closely to round window niche opening in patients with both CAA and CAS groups than that in control group, whereas there was no significant difference between CAA and CAS group (P < 0.05). The vertical portion of the FN was positioned more closely to round window niche opening in the CAA group than those in both the CAS and control groups with statistical significance (P < 0.05). Furthermore, the vertical portion of the FN was positioned more closely to round window niche opening in the CAS group than that in control group (P < 0.05). In conclusion, the dislocation between facial nerve and round window niche in patients with congenital auditory canal malformations could have significant effects on the round window implantation of vibrant soundbridge. Moreover, three-dimensional measurements and assessments before surgery might be helpful for a safer surgical approach and implantation of vibrant soundbridge.


Subject(s)
Congenital Abnormalities , Ear Diseases/congenital , Ear/abnormalities , Facial Nerve/diagnostic imaging , Round Window, Ear/diagnostic imaging , Adolescent , Adult , Case-Control Studies , Child , Child, Preschool , Constriction, Pathologic/congenital , Female , Humans , Image Processing, Computer-Assisted , Imaging, Three-Dimensional , Male , Retrospective Studies , Tomography, X-Ray Computed , Young Adult
5.
Ann Otol Rhinol Laryngol ; 133(7): 639-646, 2024 Jul.
Article in English | MEDLINE | ID: mdl-38545892

ABSTRACT

INTRODUCTION: Congenital nasal pyriform aperture stenosis (CNPAS) is a rare condition that results in neonatal respiratory difficulty. The purpose of this systematic review was to compare surgical outcomes of drilling versus dilation techniques in the treatment of CNPAS. METHODS: Pubmed, Embase, and Cochrane Clinical Trials databases were searched for terms "congenital nasal pyriform aperture stenosis" or "pyriform aperture stenosis" from 2010 to 2021. Twenty-five studies were included that evaluated pediatric patients treated surgically for CNPAS with available outcomes data including complications, revisions, and length of stay. RESULTS: A total of 51 patients with CNPAS were pooled from included studies. The median age was 29 days, 56.9% were female, and 54.9% were born full-term. The median pyriform aperture width before surgery was 5.00 mm (IQR = 4.10, 6.45). Forty (78.4%) patients underwent sublabial drilling, while 6 had a dilation procedure performed with hegar cervical dilators, 2 had a balloon dilation, and 3 were dilated with either an acrylic device, endotracheal tube, or bougie. There were no post-operative complications for 76.5% of patients, while a second surgery was required in 9 (17.6%) patients. The median length of stay was 11 days (IQR = 4, 26). No statistically significant difference was observed between sublabial drilling and surgical dilation techniques with respect to complications, need for revision surgery, or length of stay. CONCLUSION: Current literature is insufficient to determine if drilling or dilation is more effective in the treatment of CNPAS.


Subject(s)
Nasal Obstruction , Humans , Nasal Obstruction/surgery , Nasal Obstruction/congenital , Constriction, Pathologic/surgery , Constriction, Pathologic/congenital , Infant, Newborn , Dilatation/methods , Nasal Cavity/abnormalities , Nasal Cavity/surgery , Postoperative Complications/epidemiology
6.
Int J Med Sci ; 10(1): 50-9, 2013.
Article in English | MEDLINE | ID: mdl-23289005

ABSTRACT

BACKGROUND: Chronic persistent low back and lower extremity pain secondary to central spinal stenosis is common and disabling. Lumbar surgical interventions with decompression or fusion are most commonly performed to manage severe spinal stenosis. However, epidural injections are also frequently performed in managing central spinal stenosis. After failure of epidural steroid injections, the next sequential step is percutaneous adhesiolysis and hypertonic saline neurolysis with a targeted delivery. The literature on the effectiveness of percutaneous adhesiolysis in managing central spinal stenosis after failure of epidural injections has not been widely studied. STUDY DESIGN: A prospective evaluation. SETTING: An interventional pain management practice, a specialty referral center, a private practice setting in the United States. OBJECTIVE: To evaluate the effectiveness of percutaneous epidural adhesiolysis in patients with chronic low back and lower extremity pain with lumbar central spinal stenosis. METHODS: Seventy patients were recruited. The initial phase of the study was randomized, double-blind with a comparison of percutaneous adhesiolysis with caudal epidural injections. The 25 patients from the adhesiolysis group continued with follow-up, along with 45 additional patients, leading to a total of 70 patients. All patients received percutaneous adhesiolysis and appropriate placement of the Racz catheter, followed by an injection of 5 mL of 2% preservative-free lidocaine with subsequent monitoring in the recovery room. In the recovery room, each patient also received 6 mL of 10% hypertonic sodium chloride solution, and 6 mg of non-particulate betamethasone, followed by an injection of 1 mL of sodium chloride solution and removal of the catheter. OUTCOMES ASSESSMENT: Multiple outcome measures were utilized including the Numeric Rating Scale (NRS), the Oswestry Disability Index 2.0 (ODI), employment status, and opioid intake with assessment at 3, 6, and 12, 18 and 24 months post treatment. The primary outcome measure was 50% or more improvement in pain scores and ODI scores. RESULTS: Overall, a primary outcome or significant pain relief and functional status improvement of 50% or more was seen in 71% of patients at the end of 2 years. The overall number of procedures over a period of 2 years were 5.7 ± 2.73. LIMITATIONS: The lack of a control group and a prospective design. CONCLUSIONS: Significant relief and functional status improvement as seen in 71% of the 70 patients with percutaneous adhesiolysis utilizing local anesthetic steroids and hypertonic sodium chloride solution may be an effective management strategy in patients with chronic function limiting low back and lower extremity pain with central spinal stenosis after failure of conservatie management and fluoroscopically directed epidural injections.


Subject(s)
Constriction, Pathologic/congenital , Low Back Pain/drug therapy , Skin Absorption , Spinal Stenosis , Adult , Aged , Anesthetics, Local , Constriction, Pathologic/complications , Constriction, Pathologic/drug therapy , Constriction, Pathologic/pathology , Double-Blind Method , Female , Humans , Injections, Epidural , Lidocaine/administration & dosage , Low Back Pain/complications , Lumbar Vertebrae/abnormalities , Lumbar Vertebrae/pathology , Male , Middle Aged , Nerve Block , Pain Management , Prospective Studies , Saline Solution, Hypertonic/administration & dosage , Spinal Stenosis/complications , Spinal Stenosis/drug therapy , Steroids
7.
J Orthop Surg Res ; 17(1): 100, 2022 Feb 16.
Article in English | MEDLINE | ID: mdl-35172868

ABSTRACT

OBJECTIVE: Surgical treatment of lumbar spinal stenosis (LSS) in octogenarians (patients aged ≥ 80 years) has been a challenge. Inter-spinal distraction fusion (ISDF)-a minimally invasive procedure-was used for treating LSS in octogenarians. This retrospective cohort study aimed to investigate the clinical efficacy and safety of a minimally invasive ISDF technique for LSS in octogenarian patients. METHODS: From April 2015 to April 2019, octogenarian patients who underwent lumbar fusion surgery due to single-segment LSS were included. The patients were grouped into the ISDF group and posterior lumbar interbody fusion (PLIF) group based on the type of surgery. Clinical outcomes were evaluated using scores of the visual analog pain scale (VAS), Oswestry Disability Index (ODI), and Japanese Orthopedics Association (JOA) scale. Radiographs were assessed for the intervertebral angle (IA), lumbar lordosis (LL), and posterior disc height (PDH). After 2 years postoperatively, all patients underwent computed tomography (CT) to evaluate the fusion condition. Perioperative data and related complications were recorded. RESULTS: Sixty-two patients were included (mean age: 82.22 ± 1.95 years). The ISDF and the PLIF groups had 34 and 28 patients, respectively. The average follow-up time was 2.1 ± 0.25 years. There was no significant difference in VAS, ODI, JOA, and PDH scores between both groups preoperatively and at each postoperative time-point. The IA and LL showed significant differences between both groups after surgery (p < 0.05). The postoperative IA in the ISDF group were significantly lower than the preoperative values, while that in the PLIF group were markedly increased. The PLIF group had an increased LL compared with that preoperatively (p < 0.05), while the LL in the ISDF did not significantly change. The operative time, blood loss, hospital stay time, and the rate of perioperative complications of the ISDF group were significantly lower than those of the PLIF group (p < 0.05). There was no significant difference in the fusion rates between both groups. CONCLUSION: ISDF surgery is a viable method for octogenarian patients with LSS that provides a similar clinical efficacy, shorter operative time, less blood loss, shorter hospital stay time, and fewer complications, compared to the PLIF surgery.


Subject(s)
Lumbar Vertebrae/surgery , Minimally Invasive Surgical Procedures/methods , Spinal Fusion/methods , Spinal Stenosis/surgery , Aged, 80 and over , Constriction, Pathologic/congenital , Female , Humans , Lumbar Vertebrae/abnormalities , Lumbar Vertebrae/diagnostic imaging , Male , Octogenarians , Retrospective Studies , Spinal Fusion/adverse effects , Spinal Stenosis/diagnostic imaging , Treatment Outcome
8.
Ear Nose Throat J ; 101(2): NP58-NP61, 2022 Feb.
Article in English | MEDLINE | ID: mdl-32697108

ABSTRACT

Congenital nasal pyriform aperture stenosis (CNPAS) is a rare anomaly causing respiratory distress in newborns. While the primary surgical technique is well established, the timing of the removal of the stents and the management of restenosis remain a matter of debate. We report a case of a female newborn affected by CNPAS with the recurrence of respiratory distress after primary surgery due to the early removal of nasal stents, causing an overgrowth of granulation tissue. This report notes that restenosis was successfully managed by repeating the procedure over a 14-day period, with soft polyvinyl chloride uncuffed tracheal tubes acting as nasal stents.


Subject(s)
Nasal Cavity/surgery , Nasal Obstruction/congenital , Nasal Obstruction/surgery , Stents , Constriction, Pathologic/congenital , Constriction, Pathologic/diagnostic imaging , Constriction, Pathologic/pathology , Constriction, Pathologic/surgery , Endoscopy , Female , Humans , Imaging, Three-Dimensional , Infant, Newborn , Nasal Cavity/diagnostic imaging , Nasal Cavity/pathology , Nasal Obstruction/diagnostic imaging , Nasal Obstruction/pathology , Recurrence , Respiratory Distress Syndrome, Newborn/etiology , Tomography, X-Ray Computed
9.
Can Vet J ; 52(10): 1115-8, 2011 Oct.
Article in English | MEDLINE | ID: mdl-22467968

ABSTRACT

A diagnosis of an aberrant right subclavian artery was made in a 3-month-old Boston terrier. Surgical correction was performed after confirming adequate collateral circulation. Reports of surgical correction and evaluation of the perioperative thoracic limb blood pressure are rare in dogs.


Subject(s)
Dogs/abnormalities , Dogs/surgery , Esophageal Stenosis/veterinary , Subclavian Artery/abnormalities , Subclavian Artery/surgery , Animals , Constriction, Pathologic/congenital , Constriction, Pathologic/surgery , Constriction, Pathologic/veterinary , Esophageal Stenosis/congenital , Esophageal Stenosis/surgery , Female , Treatment Outcome
10.
Ital J Pediatr ; 47(1): 189, 2021 Sep 16.
Article in English | MEDLINE | ID: mdl-34530869

ABSTRACT

BACKGROUND: Congenital nasal pyriform aperture stenosis (CNPAS) is a rare condition that may occur alone or as part of a multi-formative syndrome. Management remains difficult. There is no specific treatment protocol. Traditional surgery would be anachronistic; a non-invasive or minimally invasive therapeutic option is required. However, the rarity of the disease and the infantile context render randomised clinical trials difficult. CASE PRESENTATION: We present the case of a one-month-old Caucasian boy with CNPAS. He presented to the Emergency Department of the Bambino Gesù Pediatric Hospital with nasal obstruction, noisy breathing, feeding difficulties, and suspected sleep apnoea. During hospitalisation, he underwent overnight pulse oximetry, airway endoscopy, and maxillofacial computed tomography (CT); the final diagnosis was CNPAS with moderate obstructive sleep apnoea syndrome. We successfully treated the patient using an innovative strategy that involved collaboration between ear-nose-and-throat surgeons and orthodontists. CONCLUSIONS: A combination of minimally invasive balloon surgery and placement of a palatal device may successfully treat CNPAS; it may also treat other types of nasal bone stenosis. Future studies may allow the development of practice consensus treatment strategies.


Subject(s)
Constriction, Pathologic/therapy , Dilatation , Nasal Cavity/abnormalities , Nasal Obstruction/therapy , Palatal Expansion Technique , Constriction, Pathologic/congenital , Humans , Infant, Newborn , Male , Nasal Obstruction/congenital
11.
Ear Nose Throat J ; 100(3_suppl): 343S-346S, 2021 Jun.
Article in English | MEDLINE | ID: mdl-33683976

ABSTRACT

The bony cochlear nerve canal transmits the cochlear nerve as it passes from the fundus of the internal auditory canal to the cochlea. Stenosis of the cochlear nerve canal, defined as a diameter less than 1.0 mm in transverse diameter, is associated with inner ear anomalies and severe to profound congenital hearing loss. We describe an 11-month-old infant with nonsyndromic congenital sensorineural hearing loss with cochlear nerve canal stenosis. Next-generation sequencing revealed heterozygous mutations in MYH9 and MYH14, encoding for the inner ear proteins myosin heavy chain IIA and IIC. The patient's hearing was rehabilitated with bilateral cochlear implantation.


Subject(s)
Cochlear Nerve/abnormalities , Hearing Loss, Sensorineural/congenital , Myosin Heavy Chains/genetics , Myosin Type II/genetics , Vestibulocochlear Nerve Diseases/congenital , Constriction, Pathologic/congenital , Female , Humans , Infant , Medical Illustration
12.
Rev Stomatol Chir Maxillofac ; 111(3): 155-7, 2010 Jun.
Article in French | MEDLINE | ID: mdl-20167343

ABSTRACT

INTRODUCTION: Congenital nasal pyriform aperture stenosis (CNPAS) is a rare and an unusual cause of airway obstruction in newborns. OBSERVATION: We report the case of a female neonate delivered by C-section at 39 weeks of amenorrhea for hydramnios and macrosomia. She presented with mandibular retrognathia, nasal saddling, submucous cleft palate, and loud mouth respiration. She presented with cyanosis every time feeding was attempted. CT revealed permeable choans with a single central incisor and nasal pyriform aperture stenosis. Nasal respiration returned to normal progressively after 56 days of hospitalization. The status was unchanged at 5 months. DISCUSSION: CNPAS is a rare cause of congenital nasal obstruction. It is sometimes associated to a median incisor syndrome. The diagnosis should be made as early as possible for an optimal management. Cyanosis and swallowing disorders may be lethal consequences. Associated abnormalities should be screened for with TDM or MRI. The treatment depends on the severity and may be surgical for a severe stenosis. The prognosis is good if no severe malformation or mental retardation is associated.


Subject(s)
Nasal Cavity/abnormalities , Nasal Obstruction/congenital , Adult , Cleft Palate/pathology , Constriction, Pathologic/congenital , Female , Follow-Up Studies , Humans , Infant, Newborn , Mouth Breathing/congenital , Retrognathia/pathology
13.
Int J Pediatr Otorhinolaryngol ; 134: 110053, 2020 Jul.
Article in English | MEDLINE | ID: mdl-32344234

ABSTRACT

Congenital external auditory canal stenosis (EACS) is a spectrum of abnormalities affecting the external and middle ear. We report a 6 year-old patient with EACS affecting the lateral fibrocartilaginous canal that was successfully repaired. This patient highlights a variant of EACS characterized by lateral soft tissue narrowing with normal osseous development. Most previous studies of CAA have described severe forms associated with complete atresia, bony stenosis, and middle ear malformations. Stenosis affecting only the fibrocartilaginous canal is a milder form resulting from premature arrest of the canalization process during embryologic development, and may predispose to cholesteatoma formation.


Subject(s)
Cholesteatoma/complications , Ear Canal/abnormalities , Fibrocartilage/pathology , Child , Cholesteatoma/surgery , Constriction, Pathologic/congenital , Constriction, Pathologic/surgery , Ear Canal/pathology , Ear Canal/surgery , Female , Humans
14.
Article in English, Spanish | MEDLINE | ID: mdl-31495444

ABSTRACT

BACKGROUND: Congenital nasal pyriform aperture stenosis (CNPAS) is a rare cause of nasal obstruction in neonates with respiratory distress manifestations. Diagnosis is made with craniofacial computed tomography, prompt and precise treatment creates good outcomes in these patients. AIM: To present our experience in diagnosis and management considerations with this rare pathology and a case series of our surgically managed patients. METHODS: A retrospective, analytical study of CNPAS patients surgically managed over a period of seven years. Evaluation and follow up was reviewed. RESULTS: Thirteen patients were evaluated; pyriform aperture mean width was 5.5mm. Of these patients, 31% also had Congenital Midnasal Stenosis. Medical treatment failed for all the patients and they required surgical enlargement of the pyriform aperture. No complications were seen and all patients improved in symptoms and development. CONCLUSIONS: Congenital nasal obstruction can be fatal in new-borns, CNPAS is a rare differential diagnosis that must be completely evaluated, properly treated with conservative management and if it fails, with well-planned and executed surgery. Follow-up shows high rates of success.


Subject(s)
Nasal Obstruction/diagnosis , Nasal Obstruction/surgery , Constriction, Pathologic/congenital , Constriction, Pathologic/diagnosis , Constriction, Pathologic/surgery , Female , Humans , Infant , Infant, Newborn , Male , Nasal Obstruction/congenital , Retrospective Studies
15.
PLoS One ; 15(5): e0233593, 2020.
Article in English | MEDLINE | ID: mdl-32470052

ABSTRACT

BACKGROUND: As lumbar spinal stenosis commonly occurs between the L2 and L5 segments, hip abductors are easily affected. However, studies regarding the gait pattern in these patients from the coronal plane have not yet been conducted. PURPOSE: To determine the effects of lumbar spinal stenosis on the gait pattern (stride width and femorotibial angle) and hip abductor surface electromyography in varied stride widths compared with healthy individuals. STUDY DESIGN: Prospective case-control study. METHODS: Seventeen patients and 20 healthy individuals were enrolled. Each participant completed three gait assessments in their normal gait, adducted gait and abducted gait. The femorotibial angle and surface electromyography signals were measured. Pain scores was used to quantify the degree of discomfort in the gluteal area and medial side of the knee. RESULTS: When the hip abductors' surface electromyography signals were normalized by quadriceps femoris, patients group showed significantly higher activation ratios throughout all gait patterns. Generally, surface electromyography signals and ratios were significantly higher during abducted gait compared with a normal gait. Femorotibial angle became significantly closer to the varus in healthy individuals during abducted gait. When femorotibial angle during normal gait was compared between the two groups, patients group exhibited slightly wider stride width and FTA significantly closer to the varus. Pain scores were significantly higher in the patient group and during abducted gait. CONCLUSION: Wider stride widths indicated increased relative activation of the hip abductors, closer proximity between femorotibial angle and varus, and increased pain scores for discomfort. The same tendency was observed in patients group when compared with healthy individuals. Widening of stride width in patients group despite abductor weakness suggests that additional muscle recruitment may be needed to maintain balance. Furthermore, such a distinctive gait pattern exerts increased loading on the medial knee, relating to the escalated risk of degenerative knee osteoarthritis.


Subject(s)
Constriction, Pathologic/congenital , Gait , Knee Joint/physiopathology , Lumbar Vertebrae/abnormalities , Aged , Biomechanical Phenomena , Case-Control Studies , Constriction, Pathologic/physiopathology , Electromyography , Female , Humans , Knee Joint/physiology , Lumbar Vertebrae/physiopathology , Male , Middle Aged , Prospective Studies
16.
Expert Rev Gastroenterol Hepatol ; 14(6): 445-452, 2020 Jun.
Article in English | MEDLINE | ID: mdl-32380885

ABSTRACT

INTRODUCTION: Benign gastrointestinal strictures are common, and can be congenital or acquired (anastomotic, corrosive, induced by Crohn's disease or endoscopic treatments, etc.). Patients usually present with stricture-related symptoms such as vomiting, dysphagia, dyschezia, abdominal pain, which impair their quality of life. Endoscopic balloon dilation (EBD) is the first-line treatment for most of the benign strictures; however, long-term efficacy is suboptimal, and the recurrence rate can be up to 38%. Endoscopic incision (EI) was firstly reported for treatment of congenital membranous stricture, and then applied to other benign gastrointestinal strictures. AREA COVERED: In the present review, we provided a comprehensive review of EI for the treatment of benign gastrointestinal strictures, mainly focus on the technical details, indication, safety, and efficacy of EI. The present review is expected to provide tips for operators who are going to perform EI. EXPERT OPINION: EI can serve as an alternative method for treatment of gastrointestinal strictures, the best indications are congenital membranous stricture and short-segmental (<1 cm) anastomotic strictures refractory to EBD. EI may also be attempted for strictures induced by other reasons. Combination with other endoscopic methods such as EBD, local steroid injection, stent placement, may improve the efficacy of EI.


Subject(s)
Constriction, Pathologic/surgery , Endoscopy, Gastrointestinal/methods , Gastrointestinal Diseases/surgery , Constriction, Pathologic/congenital , Constriction, Pathologic/diagnosis , Constriction, Pathologic/etiology , Dilatation , Endoscopy, Gastrointestinal/adverse effects , Gastrointestinal Diseases/congenital , Gastrointestinal Diseases/diagnosis , Gastrointestinal Diseases/etiology , Humans , Prosthesis Implantation , Recurrence , Secondary Prevention , Stents
17.
World J Gastroenterol ; 26(4): 448-455, 2020 Jan 28.
Article in English | MEDLINE | ID: mdl-32063693

ABSTRACT

BACKGROUND: Hepatic artery stenosis is a complication of orthotopic liver transplant occurring in 3.1%-7.4% of patients that can result in graft failure and need for re-transplantation. Endovascular therapy with angioplasty and stenting has been used with a high degree of technical success and good clinical outcomes, but tortuous hepatic arteries present a unique challenge for intervention. Suitable stents for this application should be maneuverable and conformable while also exerting adequate radial force to maintain a patent lumen. CASE SUMMARY: Herein we report our experience with a neurovascular Wingspan stent system in a challenging case of recurrent hepatic artery stenosis and discuss the literature of stenting in tortuous transplant hepatic arteries. CONCLUSION: Wingspan neurovascular stent is self-expanding, has good conformability, and adequate radial resistance and as such it could be added to the armamentarium of interventionalists in the setting of a tortuous and stenotic transplant hepatic artery.


Subject(s)
Angioplasty/methods , Blood Vessel Prosthesis Implantation/methods , Cardiovascular Abnormalities/surgery , Hepatic Artery/abnormalities , Stents , Blood Vessel Prosthesis Implantation/instrumentation , Cardiovascular Abnormalities/pathology , Constriction, Pathologic/congenital , Constriction, Pathologic/surgery , Female , Hepatic Artery/surgery , Humans , Middle Aged , Treatment Outcome
18.
Int J Pediatr Otorhinolaryngol ; 132: 109939, 2020 May.
Article in English | MEDLINE | ID: mdl-32065878

ABSTRACT

INTRODUCTION: Congenital midnasal stenosis (MNS) is an extremely rare disease which may be life threatening, and shows difficulty in diagnosis and management. This case series summarizes superiority of using intranasal mometasone furoate spray (IMS) and continuous positive airway pressure (CPAP) to treat nasal obstruction in neonates with MNS. METHODS: This study reviewed six consecutive cases of MNS. RESULTS: Three patients were treated with IMS and CPAP. Two patients were treated with endoscopic balloon dilatation without stenting, followed by IMS and CPAP due to persisting nasal obstruction after the operation. One patient was treated with endoscopic balloon dilatation without stenting alone. CONCLUSION: This study is the first to review the use of CPAP and IMS as an effective therapy for some patients with MNS. It suggests that conservative management with CPAP and IMS may be an alternative therapeutic option to surgery.


Subject(s)
Anti-Inflammatory Agents/therapeutic use , Continuous Positive Airway Pressure , Dilatation , Mometasone Furoate/therapeutic use , Nasal Obstruction/therapy , Respiratory System Abnormalities/therapy , Administration, Intranasal , Anti-Inflammatory Agents/administration & dosage , Conservative Treatment , Constriction, Pathologic/congenital , Constriction, Pathologic/etiology , Constriction, Pathologic/therapy , Endoscopy , Female , Humans , Infant , Infant, Newborn , Male , Mometasone Furoate/administration & dosage , Nasal Cavity/abnormalities , Nasal Cavity/pathology , Nasal Obstruction/etiology , Nasal Sprays , Respiratory System Abnormalities/complications
19.
Int J Pediatr Otorhinolaryngol ; 128: 109705, 2020 Jan.
Article in English | MEDLINE | ID: mdl-31606685

ABSTRACT

BACKGROUND: Congenital nasal pyriform aperture stenosis (CNPAS) is a rare cause of upper airway obstruction in neonates. It can occur either associated with a solitary median maxillary central incisor (SMMCI) in 40-75% of cases or as an isolated morphogenic variant. Brain MRI is routinely performed in patients with CNPAS with a SCMMI due to the concomitant risks of intracranial midline defects of the hypothalamic-pituitary axis (HPA), holoprosencephaly, or associated endocrine dysfunction. However, the role of routine brain imaging and endocrinologic evaluation in patients with isolated CNPAS is not frequently studied given the assumption that isolated CNPAS is unlikely to be associated with any intracranial findings. OBJECTIVES: To evaluate intracranial findings on dual brain MRI imaging in isolated CNPAS patients and to determine if any radiologic, endocrinologic, or genetic abnormalities existed in these patients. METHODS: We performed a single-institution retrospective review and case series of patients with isolated CNPAS from 2006 to 2019. Findings from dual brain MRI imaging, cytogenetic and karyotype testing, and formal endocrinologic testing were analyzed for each patient and compared to reported findings in isolated CNPAS patients within the literature. RESULTS: From 2006 to 2019, 16/29 patients were identified at our institution with isolated CNPAS. Inpatient otolaryngologic consultation, CT, and brain MRI imaging was obtained after a mean postnatal age of 3.21 days, 13.5 days, and 35.91 days, respectively. Dual brain MRI imaging was obtained in 12/16 (75%) patients. In our patients, abnormal MRI findings included hypothalamic hamartoma, pars intermedius cyst, and grades 1 and 4 germinal matrix hemorrhages. No holoprosencephaly was found in any patient. Genetic testing was performed on 10/16 (62.5%) patients. Karyotyping was normal in all patients and microarray testing was abnormal in 2/10 patients that represented a 2p16.3 deletion in one patient and a 7q36 deletion causing an unbalanced translocation mutation of the Sonic Hedgehog gene in the second patient. In the latter patient, a hypothalamic hamartoma and panhypopituitarism was found. Endocrine evaluation was performed in 5 patients and was normal in 3/5 patients. CONCLUSIONS: Our case series of isolated CNPAS patients identified multiple abnormalities on radiologic, genetic, and endocrine testing; one of which that involved a defect of the HPA with panhypopituitarism resulting from a hypothalamic hamartoma. Based upon our findings, dedicated brain MRI imaging, endocrinologic, and genetic testing, should likely be considered for any newly diagnosed, isolated CNPAS patient given the uncommon but potential risk for associated intracranial abnormalities.


Subject(s)
Constriction, Pathologic/congenital , Nasal Cavity/abnormalities , Brain/diagnostic imaging , Chromosome Deletion , Female , Hamartoma/diagnosis , Hedgehog Proteins/genetics , Humans , Hypopituitarism/diagnosis , Hypothalamic Diseases/diagnosis , Infant, Newborn , Magnetic Resonance Imaging , Male , Nasal Obstruction/etiology , Retrospective Studies , Tomography, X-Ray Computed , Translocation, Genetic
20.
Int J Pediatr Otorhinolaryngol ; 132: 109918, 2020 May.
Article in English | MEDLINE | ID: mdl-32035350

ABSTRACT

OBJECTIVES: To illustrate the clinical and radiological presentation of a rare etiology of nasal obstruction in neonates, midnasal stenosis (MNS), including a comparison of nasal dimensions with those of normal infants. METHODS: We retrospectively reviewed medical charts and computerized tomography (CT) imaging of neonates with nasal obstruction diagnosed as stenosis in the midnasal area in a tertiary pediatric medical center. MNS was defined clinically by inability to visualize the middle turbinate with an endoscope despite the absence of stenosis of the anterior aperture or any gross septal deviation. CT measurements of the midnasal width were taken by an experienced neuroradiologist. We compared widths between the bony inferior turbinate to the bony septum in the narrowest area of symptomatic patients, to widths in a control group of asymptomatic children. RESULTS: Nine neonates from birth to three months old presenting with nasal obstruction, severe stertor, and blocked nasal passage at the midnasal level in endoscopic examination, were diagnosed with MNS. 6/9 had CT scans. Four had isolated unilateral stenosis, two unilateral MNS and contralateral choanal atresia, and three bilateral MNS. All patients were managed conservatively, initially with nasal saline irrigation and local steroids and topical antibiotics; Median time to resolution of symptoms was 14 days. When comparing the dimensions at the midnasal narrowest area of the stenotic group with a control group of 139 healthy children, the median bony width was 1.7 mm vs. 3.2 mm, respectively (p < 0.00001). Average dimensions according to age groups until the age of 12 months are given. CONCLUSION: In neonates with nasal obstruction, when choanal atresia and pyriform aperture stenosis are excluded, stenosis of the midnasal area should be considered. Most of these neonates can be managed conservatively. LEVEL OF EVIDENCE: 4.


Subject(s)
Nasal Cavity/abnormalities , Nasal Cavity/diagnostic imaging , Nasal Obstruction/diagnostic imaging , Nasal Obstruction/etiology , Respiratory System Abnormalities/diagnostic imaging , Constriction, Pathologic/congenital , Constriction, Pathologic/diagnostic imaging , Constriction, Pathologic/etiology , Constriction, Pathologic/therapy , Endoscopy/adverse effects , Female , Humans , Infant , Infant, Newborn , Male , Nasal Cavity/pathology , Nasal Obstruction/therapy , Nasal Septum/diagnostic imaging , Respiratory System Abnormalities/complications , Respiratory System Abnormalities/therapy , Retrospective Studies , Tomography, X-Ray Computed , Turbinates/diagnostic imaging
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