Comparison of Surgical Techniques for the Treatment of Congenital Nasal Pyriform Aperture Stenosis: A Systematic Review.

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.

A novel minimally invasive technique of inter-spinal distraction fusion surgery for single-level lumbar spinal stenosis in octogenarians: a retrospective cohort study.

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.

Congenital Nasal Pyriform Aperture Stenosis: Successful Management of Restenosis After Primary Surgery by Stent Placement.

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.

Congenital midureteral stenosis in children: a 13-year retrospective study based on data from a large pediatric medical center.

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.

Piometra secundario a estenosis cervical en una adolescente / Pyometra secondary to cervical stenosis in an adolescent

INTRODUCCIÓN: El piometra es una afección infrecuente, pero grave, que en general se diagnostica en mujeres posmenopáusicas. En adolescentes es sumamente raro, y si se acompaña de amenorrea primaria hay que tener en mente las anomalías congénitas. CASO CLÍNICO: Adolescente de 13 años, sin antecedentes personales de interés salvo amenorrea primaria, que acude con abdomen agudo y es intervenida por una peritonitis difusa causada por un piometra secundario a disgenesia (estenosis) cervical congénita. Se realizó dilatación cervical y se dejó una sonda vesical intrauterina para prevenir la reestenosis. CONCLUSIONES: Un diagnóstico precoz y un tratamiento conservador con dilatación cervical y colocación temporal de un catéter urinario son esenciales para un manejo seguro y efectivo de la estenosis cervical en adolescentes.

INTRODUCTION: Pyometra is an uncommon but serious condition that is generally diagnosed in postmenopausal women. In adolescents it is extremely rare; if accompanied by primary amenorrhea, consider congenital abnormalities. CASE REPORT: A 13-year-old adolescent, with no relevant personal history except primary amenorrhea, who presented with an acute abdomen and was operated on for diffuse peritonitis caused by pyometra secondary to congenital cervical dysgenesis (stenosis). Cervical dilation was performed and a urinary catheter was temporarily placed inside the uterus to prevent restenosis. CONLUSIONS: An early diagnosis and conservative treatment with cervical dilation and temporary placement of a urinary catheter are essential for the safe and effective management of cervical stenosis in adolescents.

Balloon dilation and rapid maxillary expansion: a novel combination treatment for congenital nasal pyriform aperture stenosis in an infant.

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.

Endoscopic incision for treatment of benign gastrointestinal strictures.

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.

Congenital soft tissue stenosis of the external auditory canal with canal cholesteatoma: Case report and literature review.

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.

Congenital midnasal stenosis: Conservative management.

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.

Congenital nasal pyriform aperture stenosis: Diagnosis, management and technical considerations.

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.

Radiologic, genetic, and endocrine findings in isolated congenital nasal pyriform aperture stenosis patients.

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.

[Congenital tracheal stenosis: from clinic to treatment.] / La sténose trachéale congénitale: de la clinique au traitement.

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.

Descending Aortic Translocation and Right Pulmonary Artery Reimplantation for Midline Descending Aorta and Crossed Pulmonary Arteries in an Infant.

Left mainstem bronchial compression by a midline descending thoracic aorta is a rare anatomic variant. Translocation of the descending thoracic aorta to the ascending aorta has recently been described to treat this condition. We performed an aortic translocation and right pulmonary artery reimplantation in a 4-month-old infant with severe pulmonary hypertension secondary to right pulmonary artery stenosis and left bronchial compression by a midline descending thoracic aorta. The procedure was successful in ameliorating the patient's left mainstem bronchial compression and pulmonary hypertension. Descending aortic translocation should be considered when the left bronchus is compressed causing respiratory symptoms.

Fluid dynamic assessment of tracheal flow in infants with congenital tracheal stenosis before and after surgery.

Tracheal flow in infants with congenital tracheal stenosis (CTS) was numerically investigated using subject-specific airway models before and after reconstructive surgery. We quantified tracheal flow based on airway resistance during inhalation, and compared it between controls and patients before and after surgery. The airway resistance in each subject was assessed using geometrical parameters of the trachea: the minimum cross-sectional area Amin, the minimum cross-sectional area normalized by the standard deviation of the cross-sectional area Amin/σA, the area ratio of the minimum and maximum cross-sectional area Amin/Amax, and ratio of the normalized standard deviation of cross-sectional area to the mean cross-sectional area σA/Amean. Our numerical results demonstrated that such geometrical parameters could be used to assess the severity of CTS. Since subjects can be more clearly categorized as controls and most preoperative patients in terms of the airway resistance, a simulation using subject-specific airway models can lead us to a precise understanding of tracheal flow, and also provide knowledge about therapeutic decision. Our numerical results also demonstrated that significant surgical expansion of cross-sectional area did not help recover tracheal flow because of expansion loss. These results will be helpful not only when making therapeutic decisions about surgery but also when assessing quality of life in postoperative patients. Graphical abstract.

Dynamic stenosis of the right main bronchus in a 3-month-old child: a tracheobronchial sleeve resection under venovenous extracorporeal membrane oxygenation.

We report the case of a boy with congenital dynamic stenosis of the right main bronchus. The operation was postponed to a later date, when the patient was in a better clinical position to tolerate surgery. A tracheobronchial sleeve resection under dual-lumen venovenous extracorporeal membrane oxygenation was performed.

Matrix metalloproteinase promotes elastic fiber degradation in ligamentum flavum degeneration.

Ligamentum flavum (LF) hypertrophy in lumbar spinal canal stenosis (LSCS) is characterized by a loss of elastic fibers and fibrosis. Chronic inflammation is thought to be responsible for the histological change but the mechanism underlying elastic fiber degradation remains unclear. Given that matrix metalloproteinase (MMP)-2 and -9 have elastolytic activity and are partly regulated by inflammatory cytokines such as interleukin (IL)-6, in this study, we investigated whether MMPs mediate LF degeneration using 52 LF samples obtained during lumbar surgery, including 31 LSCS and 21 control specimens. We confirmed by histological analysis that the LSCS samples exhibited severe degenerative changes compared with the controls. We found that MMP-2 was upregulated in LF tissue from patients with LSCS at the mRNA and protein levels, whereas MMP-9 expression did not differ between the two groups. The MMP-2 level was positively correlated with LF thickness and negatively correlated with the area occupied by elastic fibers. IL-6 mRNA expression was also increased in LF tissue from patients with LSCS and positively correlated with that of MMP-2. Signal transducer and activator of transcription (STAT)3, a component of the IL-6 signaling pathway, was activated in hypertrophied LF tissues. Our in vitro experiments using fibroblasts from LF tissue revealed that IL-6 increased MMP-2 expression, secretion, and activation via induction of STAT3 signaling, and this effect was reversed by STAT3 inhibitor treatment. Moreover, elastin degradation was promoted by IL-6 stimulation in LF fibroblast culture medium. These results indicate that MMP-2 induction by IL-6/STAT3 signaling in LF fibroblasts can degrade elastic fibers, leading to LF degeneration in LSCS.

Brida amniótica abdominal, reporte de un caso / Precordial pain in pediatrics. Update

Existe un amplio espectro de deformidades causadas por bandas amnióticas, siendo las extremidades la ubicación más común. La banda de constricción en el abdomen es un lugar raro para el síndrome de brida amniótica. Presentamos un caso de banda de constricción circunferencial congénita del abdomen sin otras malformaciones. La condición no afectó la ventilación, la alimentación, las deposiciones o el crecimiento. Se planificaron múltiples Z-Plasties en el abdomen antes del primer mes de vida. Revisamos la literatura que hace hincapié en la presentación clínica y el tratamiento de esta rara entidad clínica.

There is a wide spectrum of deformities caused by amniotic bands, being the extremities the most common location. The constriction band in the abdomen is a rare location for constriction band syndrome. We report a case of congenital circumferential constriction band of abdomen without other malformations. The condition did not affect ventilation, feeding, bowel movements, or growth. Multiple Z-plasties were planned in the abdomen before the first month from birth. We reviewed the literature emphasizing on the clinical presentation and management of this rare clinical entity.