Esophageal Atresia and Associated Duodenal Atresia: A Cohort Study and Review of the Literature

Abstract Introduction Esophageal atresia (EA) is associated with duodenal atresia (DA) in 3 to 6% of cases. The management of this association is controversial and literature is scarce on the topic. Materials and Methods We aimed to (1) review the patients with EA + DA treated at our institution and (2) systematically review the English literature, including case series of three or more patients. Results Cohort study: Five of seventy-four patients with EA had an associated DA (6.8%). Four of five cases (80%) underwent primary repair of both atresia, one of them with gastrostomy placement (25%). One of five cases (20%) had a delayed diagnosis of DA. No mortality has occurred. Systematic Review: Six of six-hundred forty-five abstract screened were included (78 patients). Twenty-four of sixty-eight (35.3%) underwent primary correction of EA + DA, and 36/68 (52.9%) underwent staged correction. Nine of thirty-six (25%) had a missed diagnosis of DA. Thirty-six of sixty-eight underwent gastrostomy placement. Complications were observed in 14/36 patients (38.9 ± 8.2%). Overall mortality reported was 41.0 ± 30.1% (32/78 patients), in particular its incidence was 41.7 ± 27.0% after a primary treatment and 37.0 ± 44.1% following a staged approach. Conclusion The management of associated EA and DA remains controversial. It seems that the staged or primary correction does not affect the mortality. Surgeons should not overlook DA when correcting an EA.

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Triple Atresia, Triple Threat? An Unusual Constellation of Primary Surgical Abnormalities

Pediatric Reports ◽

10.3390/pediatric13020026 ◽

2021 ◽

Vol 13 (2) ◽

pp. 189-196

Author(s):

Raef Jackson ◽

Carmen Francis ◽

Karim Awad ◽

Semiu E. Folaranmi

Keyword(s):

Literature Review ◽

Neonatal Period ◽

Oesophageal Atresia ◽

Case Series ◽

Duodenal Atresia ◽

Vacterl Association ◽

Term Survival ◽

Staged Approach ◽

Definitive Surgery

We present a case series of two patients with tracheo-oesophageal fistula with oesophageal atresia (TOF/OA), duodenal atresia (DA) and ano-rectal malformation (ARM). This constellation of abnormalities, dubbed triple atresia (TA), is a rare combination with few described cases in the literature. Here we describe our management of these cases, as well as the results of our literature review. Both of our cases had staged surgical procedures and were initially managed with thoracotomy for repair of TOF/OA on day two of life. They subsequently underwent laparotomy for management of their abdominal pathology at day five and seven of life. Both have survived the neonatal period and are awaiting definitive surgery for ARM. Literature review yielded seven cases of TA involving a TOF, DA, and ARM. Four patients underwent staged repair, while three patients underwent repair of TOF/OA, DA and colostomy for ARM at the same time. Of these three patients, two died, representing 22% of the overall cohort. Triple atresia remains a rare subset of patients suspected to have VACTERL association, however mortality may be significantly higher. Our data would suggest a staged approach to be optimal for long term survival.

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Congenital high airway obstruction syndrome complicated with foregut malformation and high airway fistula to the alimentary tract – a case series with four distinct types

Case Reports in Perinatal Medicine ◽

10.1515/crpm-2019-0064 ◽

2020 ◽

Vol 9 (1) ◽

Author(s):

Yutaka Kanamori ◽

Kazunori Tahara ◽

Michinobu Ohno ◽

Kotaro Tomonaga ◽

Yohei Yamada ◽

...

Keyword(s):

Mortality Rate ◽

Airway Obstruction ◽

Esophageal Atresia ◽

Tracheoesophageal Fistula ◽

Alimentary Tract ◽

Correct Diagnosis ◽

Case Series ◽

Duodenal Atresia ◽

Case Presentation ◽

Foregut Malformation

AbstractBackgroundCongenital high airway obstruction syndrome (CHAOS) is a rare disease and recently has been noticed to show typical prenatal images, such as hyperinflated lungs and flattened or inverted diaphragms. However, in some cases correct diagnosis may be difficult and in such cases the mortality rate increases.Case presentationWe report four cases of CHAOS complicated with a high airway fistula to the alimentary tract and foregut malformation. The patients did not show the typical features of CHAOS in the fetus. This may be attributed to the high airway fistula acting as a decompression route for the accumulated lung fluids to the alimentary tract.ConclusionThe combination of CHAOS, foregut malformation and a high airway fistula is very rare and classified into four distinct types: (1) CHAOS with a high airway fistula but not with a foregut malformation; (2) CHAOS with esophageal atresia and tracheoesophageal fistula; (3) CHAOS with a high airway fistula and duodenal atresia; and (4) CHAOS with esophageal atresia, tracheoesophageal fistula and duodenal atresia. It may be useful for treating physicians to be aware of these four distinct types and the typical characteristics of each type.

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Intraorbital wooden foreign bodies: case series and literature review

International Journal of Ophthalmology ◽

10.18240/ijo.2021.10.21 ◽

2021 ◽

Vol 14 (10) ◽

pp. 1619-1627

Author(s):

Ya-Yan You ◽

◽

Xin-Yan Wang ◽

Jin Chen ◽

Zheng-Rong Wang ◽

...

Keyword(s):

Foreign Bodies ◽

Case Reports ◽

Delayed Diagnosis ◽

Clinical Manifestations ◽

Case Series ◽

Aged People ◽

Imaging Characteristics ◽

Close Observation ◽

Missed Diagnosis ◽

Magnetic Resonance Imaging Mri

Intraorbital wooden foreign bodies (IOWFBs) constitute a relatively rare ocular trauma, which occupy a special type of intraorbital foreign bodies (IOFBs). Data regarding IOWFBs must be obtained from case reports or small case series due to their rarity. Here, we reported 5 cases of IOWFBs and reviewed the related literatures, which could provide comprehensive information regarding the clinical manifestations, diagnosis, and surgical treatment of IOWFBs. Combined with the published literature, a total of 51 independent cases were counted after we added 5 cases. Among them, the number of male and female patients was 35 and 16 respectively; the mean age was 27.3±18.2 (range 1-66)y. Obviously, the disorder seemed to occur mainly in young and middle-aged people. Because of the diversity in the clinical manifestations and imaging characteristics of IOWFBs, misdiagnosis and missed diagnosis often occur during the initial visit. Delayed diagnosis may lead to a high risk of orbital infection caused by IOWFBs. Surgery is the treatment of choice for most patients; however, the missed diagnosis and residue of foreign bodies after previous surgery cannot be ignored. Therefore, an accurate diagnosis is governed by the detailed trauma history, careful ocular examination, close observation of clinical manifestations, correct imaging diagnosis [e.g., magnetic resonance imaging (MRI) or computerized tomography (CT)], and timely and completely elimination of IOWFBs.

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Macular buckling versus vitrectomy on macular hole associated macular detachment in eyes with high myopia: a randomised trial

British Journal of Ophthalmology ◽

10.1136/bjophthalmol-2020-317800 ◽

2021 ◽

pp. bjophthalmol-2020-317800

Author(s):

Xiujuan Zhao ◽

Yonghao Li ◽

Wei Ma ◽

Ping Lian ◽

Xiling Yu ◽

...

Keyword(s):

Success Rate ◽

Macular Hole ◽

High Myopia ◽

Randomised Trial ◽

Case Series ◽

Primary Treatment ◽

Closure Rate ◽

Macular Detachment ◽

The Difference ◽

Myopic Maculopathy

AimTo compare the efficacy of macular buckling (MB) and pars plana vitrectomy (PPV) for full-thickness macular holes (FTMH) and associated macular detachment (MD) in highly myopic eyes.MethodsProspective interventional case series of eyes undergoing PPV or MB for FTMH and MD.Main outcome measuresBest-corrected visual acuity (BCVA) at postoperative month 24. Other measured outcomes include the initial surgical success rate, macular hole closure rate and the progression of myopic maculopathy.ResultsA total of 53 eyes from 53 participants were included in this study (26 participants receiving MB and 27 participants receiving PPV), and finally 49 eyes from 49 participants (25 participants in the MB group and 24 participants in the PPV group) were analysed. At postoperative month 24, the BCVA had improved significantly in those that underwent either MB (p<0.001) or PPV (p=0.04). The difference between the groups was not significant (p=0.653). The surgical failure rate after the primary treatment was significantly higher in the PPV group than the MB group (25.00% vs 4.00%, respectively; p=0.04). The macular closure rate was higher in the MB group compared with the PPV group, but the difference was not statistically significant (64.00% vs 58.33%, respectively; p=0.45). Myopic maculopathy development may be more severe following PPV than following MB surgery.ConclusionPatients with high myopia obtained anatomical and functional improvements from either MB or PPV. However, MB achieved a significantly higher success rate in retinal reattachment compared with PPV.Trial registration numberNCT03433547.

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Clinical, Radiographic, and Histomorphometric Evaluation of a Vertical Ridge Augmentation Procedure Using a Titanium-Reinforced Microporous Expanded Polytetrafluoroethylene Emrane: A Prospective Case Series with 1-Year Follow-Up

Materials ◽

10.3390/ma14143828 ◽

2021 ◽

Vol 14 (14) ◽

pp. 3828

Author(s):

Jung-Gu Ji ◽

Jung-A Yu ◽

Seong-Ho Choi ◽

Dong-Woon Lee

Keyword(s):

Case Series ◽

Graft Material ◽

Ridge Augmentation ◽

Expanded Polytetrafluoroethylene ◽

Implant Placement ◽

Significant Difference ◽

Staged Approach ◽

Height Change ◽

Prospective Case Series ◽

Augmentation Procedure

Vertical ridge augmentation for long-term implant stability is difficult in severely resorbed areas. We examined the clinical, radiological, and histological outcomes of guided-bone regeneration using novel titanium-reinforced microporous expanded polytetrafluoroethylene (MP-ePTFE) membranes. Eighteen patients who underwent implant placement using a staged approach were enrolled (period: 2018–2019). Vertical ridge augmentation was performed in areas with vertical bone defects ≥ 4 mm. Twenty-six implant fixtures were placed in 14 patients. At implant placement six fixtures had relatively low stability. On cone-beam computed tomography, the average vertical changes were 4.2 ± 1.9 (buccal), 5.9 ± 2.7 (central), and 4.4 ± 2.8 mm (lingual) at six months after vertical ridge augmentation. Histomorphometric analyses revealed that the average proportions of new bone, residual bone substitute material, and soft tissue were 34.91 ± 11.61%, 7.16 ± 2.74%, and 57.93 ± 11.09%, respectively. Stable marginal bone levels were observed at 1-year post-loading. The residual bone graft material area was significantly lower in the exposed group (p = 0.003). There was no significant difference in the vertical height change in the buccal side between immediately after the augmentation procedure and the implant placement reentry time (p = 0.371). However, all implants functioned well regardless of the exposure during the observation period. Thus, vertical ridge augmentation around implants using titanium-reinforced MP-ePTFE membranes can be successful.

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Sinonasal mucosal melanoma: treatment strategies and survival rates for a rare disease entity

Wiener klinische Wochenschrift ◽

10.1007/s00508-021-01847-6 ◽

2021 ◽

Author(s):

Alexandros Andrianakis ◽

Peter Kiss ◽

Markus Pomberger ◽

Axel Wolf ◽

Dietmar Thurnher ◽

...

Keyword(s):

Rare Disease ◽

Survival Data ◽

Survival Rates ◽

Delayed Diagnosis ◽

Distant Metastases ◽

Primary Treatment ◽

Mucosal Melanoma ◽

Disease Entity ◽

Improve Outcome ◽

Initial Symptoms

Summary Background Sinonasal mucosal melanoma (SNMM) is a rare disease entity comprising 0.4–1.3% of all melanomas. Surgery with free margins has been the primary treatment over decades. Neither the addition of radiotherapy nor chemotherapy could significantly improve outcome rates of this devastating malignancy. This study presents our clinical experience with SNMM over a 19-year period and summarizes the current body of literature on SNMM. Methods This retrospective analysis included 12 patients with SNMM treated from 2001 to 2019 at an academic center. Additionally, a literature review of the last 29 years on treatment and survival data of SNMM was conducted. Results Main initial symptoms were epistaxis and nasal obstruction. Of the patients 9 underwent endoscopic surgery, 6 received adjuvant therapy. 3 patients who did not undergo surgery, received chemoradiotherapy, radiotherapy alone, and chemotherapy alone, respectively. At the time of diagnosis 2 patients had distant metastases and 4 patients developed distant metastases during the course of the disease. Mean overall survival (OS) was 30.6 months, 3‑year and 5‑year OS were 25%, and 18.2%, respectively. Conclusion Unspecific symptoms and hidden anatomic locations lead to delayed diagnosis and increased rates of metastatic dissemination. Distant metastasis is the main treatment failure in SNMM. Surgery with free margins remains the primary treatment for SNMM. Adjuvant radiotherapy might improve local control in individual cases but efficient systemic therapy is needed to improve outcome rates. To evaluate and define more effective targeted treatment options and improve outcome rates, homogeneous data and prospective multicentric analysis are needed.

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Impact of SARS-COV-2 Pandemic on Kidney Cancer Management

Kidney Cancer ◽

10.3233/kca-210112 ◽

2021 ◽

pp. 1-14

Author(s):

Melissa Bersanelli ◽

Camillo Porta

Keyword(s):

Survival Rates ◽

Delayed Diagnosis ◽

Case Series ◽

Cost Benefit ◽

Original Data ◽

Immune Checkpoint Blockade ◽

Retrospective Case ◽

Renal Masses ◽

Term Survival ◽

Cancer Management

Background: The SARS-CoV-2 pandemic still has a huge impact on the management of many chronic diseases such as cancer. Few data are presently available reagarding how the management of renal cell carcinoma (RCC) has changed due to this unprecedented situation. Objective: To discuss the challenges and issues of the diagnosis and treatment of RCC in the COVID-19 era, and to provide recommendations based on the collected literature and our personal experience. Methods: Systematic review of the available Literature regarding the management of RCC during the SARS-CoV-2 pandemic. Results: Our review showed a prevalence of narrative publications, raising the issue of the real relevance of the evidence retrieved. Indeed, the only original data about RCC and COVID-19 found were a small retrospective case series and two surveys, providing either patients’ or physicians’ viewpoints. Conclusions: The expected delayed diagnosis of RCC could lead to an increase of advanced/metastatic cases; thus, proper therapeutic choices for patients with small renal masses should be carefully evaluated case by case, in order to avoid negative effects on long-term survival rates. The controversial interaction between immune checkpoint blockade and COVID-19 pathogenesis is more hypothetical than evidence-based, and thus immunotherapy should not be denied, whenever appropriate. To avoid treatments which won’t have an impact on patients’ survival, a honest and accurate evaluation of the cost/benefit ratio of each treatment option should be always performed. Finally, SARS-CoV-2 swab positivity should not prevent the continuation of ongoing active treatments in asymptomatic cases, or or after symptoms’ resolution.

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