Extended Follow-Up After Atrial Repair for Transposition of the Great Arteries: A Younger Age at Surgery Improves Late Survival

OBJECTIVE The aim of this cohort study was to describe and analyze the surgical treatment and outcome of posterior fossa arachnoid cysts (PFACs) in infants. METHODS Patients presenting with a PFAC at infancy or prenatally, between the years 2000 and 2019, and who were surgically treated before the age of 2 years, were included in this study. Patient data were retrospectively collected including baseline characteristics and surgical variables. Factors related to revision surgery were analyzed through uni- and multivariate analysis. RESULTS Thirty-five patients, of whom 54.3% were male, were included. The cyst was diagnosed prenatally in 23 patients (65.7%). Surgery was typically recommended after a mean cyst follow-up of 3.4 ± 3.9 months, with a mean age at surgery of 6.1 ± 5.1 months. In 54.3% of patients (n = 19), surgery was performed before the age of 6 months. The PFAC was treated purely neuroendoscopically in 57.1% of patients (n = 20), while 28.6% of patients underwent open cyst procedures (n = 10), 5.7% (n = 2) were treated with a shunt, and 8.6% (n = 3) underwent a combined procedure. Additional surgery was required in 31.4% of patients (n = 11; mean 2.36 ± 2.11 surgeries per patient). At the last follow-up (61.40 ± 55.33 months), no mortality or permanent morbidity was seen; radiological improvement was apparent in 83.9% of the patients. Those patients treated before the age of 6 months (p = 0.09) and who presented before surgery with a stable cyst size that was maintained throughout preoperative monitoring (p = 0.08) showed a trend toward higher revision rates after surgical treatment. CONCLUSIONS PFACs in infancy may require surgical treatment before the age of 6 months. Navigated endoscopy was a valid surgical option. Overall mortality or permanent morbidity was rare. Additional surgery was required in up to 30% of the patients; younger age and a preoperatively stable cyst might be risk factors for revision surgery.

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Radiological and clinical associations with scoliosis outcomes after posterior fossa decompression in patients with Chiari malformation and syrinx from the Park-Reeves Syringomyelia Research Consortium

Journal of Neurosurgery Pediatrics ◽

10.3171/2020.1.peds18755 ◽

2020 ◽

Vol 26 (1) ◽

pp. 53-59 ◽

Cited By ~ 2

Author(s):

Jennifer M. Strahle ◽

Rukayat Taiwo ◽

Christine Averill ◽

James Torner ◽

Jordan I. Gewirtz ◽

...

Keyword(s):

Posterior Fossa ◽

Chiari Malformation ◽

Craniocervical Junction ◽

Curve Progression ◽

Posterior Fossa Decompression ◽

Type I ◽

Younger Age ◽

Curve Magnitude ◽

The Mean

OBJECTIVEIn patients with Chiari malformation type I (CM-I) and a syrinx who also have scoliosis, clinical and radiological predictors of curve regression after posterior fossa decompression are not well known. Prior reports indicate that age younger than 10 years and a curve magnitude < 35° are favorable predictors of curve regression following surgery. The aim of this study was to determine baseline radiological factors, including craniocervical junction alignment, that might predict curve stability or improvement after posterior fossa decompression.METHODSA large multicenter retrospective and prospective registry of pediatric patients with CM-I (tonsils ≥ 5 mm below the foramen magnum) and a syrinx (≥ 3 mm in width) was reviewed for clinical and radiological characteristics of CM-I, syrinx, and scoliosis (coronal curve ≥ 10°) in patients who underwent posterior fossa decompression and who also had follow-up imaging.RESULTSOf 825 patients with CM-I and a syrinx, 251 (30.4%) were noted to have scoliosis present at the time of diagnosis. Forty-one (16.3%) of these patients underwent posterior fossa decompression and had follow-up imaging to assess for scoliosis. Twenty-three patients (56%) were female, the mean age at time of CM-I decompression was 10.0 years, and the mean follow-up duration was 1.3 years. Nine patients (22%) had stable curves, 16 (39%) showed improvement (> 5°), and 16 (39%) displayed curve progression (> 5°) during the follow-up period. Younger age at the time of decompression was associated with improvement in curve magnitude; for those with curves of ≤ 35°, 17% of patients younger than 10 years of age had curve progression compared with 64% of those 10 years of age or older (p = 0.008). There was no difference by age for those with curves > 35°. Tonsil position, baseline syrinx dimensions, and change in syrinx size were not associated with the change in curve magnitude. There was no difference in progression after surgery in patients who were also treated with a brace compared to those who were not treated with a brace for scoliosis.CONCLUSIONSIn this cohort of patients with CM-I, a syrinx, and scoliosis, younger age at the time of decompression was associated with improvement in curve magnitude following surgery, especially in patients younger than 10 years of age with curves of ≤ 35°. Baseline tonsil position, syrinx dimensions, frontooccipital horn ratio, and craniocervical junction morphology were not associated with changes in curve magnitude after surgery.

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Unilateral lateral rectus recession is an effective surgery for intermittent exotropia in young children

BMC Ophthalmology ◽

10.1186/s12886-020-01778-2 ◽

2021 ◽

Vol 21 (1) ◽

Author(s):

Oriel Spierer ◽

Abraham Spierer

Keyword(s):

Young Children ◽

Success Rate ◽

Lateral Rectus ◽

Intermittent Exotropia ◽

Age At Surgery ◽

Surgical Methods ◽

Before And After ◽

The Mean ◽

Consecutive Esotropia

Abstract Background Different surgical methods have been suggested for the correction of intermittent exotropia. Unilateral lateral rectus recession has been described as a surgical alternative for small and moderate-angle exotropia. In general, previous studies did not focus on the outcomes of unilateral lateral rectus recession in young children with intermittent exotropia. The purpose of this study is to evaluate the surgical outcomes of unilateral lateral rectus recession in the treatment of moderate-angle exotropia (≤ 25 PD (prism diopters)) in children. Methods The charts of all patients younger than 12 years of age with moderate-angle exotropia (up to 25 PD) who were operated during the years 2006–2018 were retrospectively reviewed. Fifty-eight patients underwent unilateral lateral rectus recession and had a minimum follow up of 6 months. The angle of exotropia (PD) before and after surgery and the success rate were documented. Results Mean age at surgery was 6.4 ± 1.9 (range 3.5–11.0) years. Exotropia improved from a preoperative angle of 21.4 ± 4.0 PD to 3.5 ± 5.9 PD postoperatively (p < 0.001). Success rate, defined as deviation of ≤ 10 PD, was achieved in 86.2%. There were 2 (3.4%) cases of overcorrection (consecutive esotropia). There were no intra- or postoperative complications. The mean follow-up duration after surgery was 2.3 ± 1.7 years. Conclusions In children with moderate angle exotropia, good postoperative success rate was achieved by performing unilateral lateral rectus recession.

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Vascular ring surgery – can we do better?

European Heart Journal ◽

10.1093/ehjci/ehaa946.3210 ◽

2020 ◽

Vol 41 (Supplement_2) ◽

Author(s):

G Amir ◽

N Soffair ◽

G Frenkel ◽

E Bruckheimer ◽

E Nachum ◽

...

Keyword(s):

Aortic Arch ◽

Respiratory Symptoms ◽

Ductus Arteriosus ◽

Vascular Ring ◽

Symptomatic Relief ◽

Mirror Image ◽

Vascular Rings ◽

Number Of Patients ◽

Age At Surgery

Abstract Background Vascular anomalies of the Aortic arch can cause respiratory symptoms in children due to tracheal compression. Treatment consists of division of the vascular rings, nevertheless data regarding mid- term results is scarce. The purpose of this study was to evaluate clinical results of vascular ring surgery. Methods Between2007–2014, 85 children underwent vascular ring surgery. 51 had Double Aortic Arch (DAA, 60%), 31 Right Arch with Aberrant Subclavian Artery (RAA & ALSA, 36.5%) and 3 had RAA, mirror image branching & left ductus arteriosus (3.5%). Mean age and weight at operation were 12.4±13months and 8.6±4.1 kg respectively. Mid-term follow-up included clinical follow up by a pulmonologist (38 patients, 44.7%) and a telephone questionnaire (71 patients, 83%) performed 57±25.7 months after surgery. Results In most patients, symptomatic relief occurred in less than 6 months (table 1). Mid –term follow up revealed that although most parents described a significant improvement in their child's respiratory symptoms (95%), a significant number of patients described some residual respiratory symptoms (table 2). We did not find any significant association between age at surgery (under 6 month), or vascular ring anatomy (DAA vs. RAA&ASA) and the presence of residual symptoms at follow up Conclusions Surgical division of vascular rings results in a significant clinical improvement within one year, nevertheless many patients remain symptomatic to some degree. We found no association between the age at surgery or anatomic variant to the presence of symptoms in mid- term follow up. Further evaluation whether a more aggressive surgical approach is warranted in order to decrease the incidence long-term symptoms. Funding Acknowledgement Type of funding source: None

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Long-term outcomes after corrective cartilaginous joint resection based on arthrographic findings of duplicated thumbs

Journal of Hand Surgery (European Volume) ◽

10.1177/1753193421994780 ◽

2021 ◽

Vol 46 (4) ◽

pp. 367-372

Author(s):

Kousuke Iba ◽

Akira Saito ◽

Megumi Hanaka ◽

Toshihiko Yamashita

Keyword(s):

Growth Period ◽

Joint Space ◽

Type Ii ◽

Scoring Method ◽

Joint Congruency ◽

Thumb Duplication ◽

Age At Surgery ◽

Joint Connection

We report greater than 10-year outcomes in duplicated thumbs following corrective cartilaginous resection during the growth period. We have undertaken corrective resection of cartilaginous joint connections based on intraoperative arthrographic findings to reconstruct favourable alignment in six Wassel Type II and IV thumb duplication in six patients. The age at surgery was 13 months (range 10–15), and the average post-surgical follow-up was 134 months (range 120–160). We observed five excellent and one good outcome using the Japanese Society for Surgery of the Hand scoring method. Favourable joint congruency and alignment were preserved, and no growth plate arrest or joint space narrowing was present more than 10 years after surgery. We conclude that corrective resection of the cartilaginous joint based on intraoperative arthrographic findings has long-term reliability for duplicated thumbs, especially those of Wassel Type II and IV, which have a cartilaginous joint connection. Levels of evidence: IV

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Timing of orchidopexy and its relationship to postoperative testicular atrophy: results from the ORCHESTRA study

BJS Open ◽

10.1093/bjsopen/zraa052 ◽

2021 ◽

Vol 5 (1) ◽

Author(s):

◽

C Skerritt ◽

C Bradshaw ◽

N Hall ◽

L McCarthy ◽

...

Keyword(s):

Primary Outcome ◽

Testicular Atrophy ◽

Wound Infections ◽

Atrophy Rate ◽

Younger Age ◽

Increased Risk ◽

Hospital Stays ◽

Reoperation Rates ◽

International Audit

Abstract Background In 2011 a consensus statement from the British Association of Paediatric Urologists recommended lowering the age at orchidopexy to under 1 year. There are concerns that a younger age at operation may increase postoperative testicular atrophy. The ORCHESTRA study aimed to establish the current age at orchidopexy in a multicentre, international audit and to see whether testicular atrophy was affected by age at operation. Methods The study was undertaken over a 3-month period in 28 centres in boys undergoing orchidopexy for unilateral, palpable undescended testes. Data collection was done using a standardized, predetermined protocol. The primary outcome was postoperative testicular atrophy. Secondary outcomes were wound infections, reoperations, and unplanned hospital stays related to anaesthetic events. Results A total of 417 patients were included, of whom only 48 (11.5 per cent) underwent orchidopexy before 1 year of age. There was no difference in anaesthetic complications in boys aged less than 1 year versus older patients: 0 of 48 (0 per cent) versus 6 of 369 (1.6 per cent) (P = 0.999). Complete follow-up was available for 331 patients (79.4 per cent). There was no difference in atrophy rate between those aged less than 1 year and older boys: 1 of 37 (3 per cent) versus 9 of 294 (3.1 per cent) (P = 0.999). Reoperation rates were 0 of 37 (0 per cent) and 7 of 294 (2.4 per cent) respectively (P = 1.000). There were more wound infections in boys under 1 year of age: 4 of 37 (11 per cent) versus 7 of 294 (2.4 per cent) (P = 0.025). Conclusion Only 11.5 per cent of boys underwent surgery before the age of 1 year. There was no increased risk of postoperative testicular atrophy with early surgery, although there was a higher rate of wound infection. Further study is required to demonstrate that early orchidopexy is not inferior to orchidopexy undertaken in boys aged over 1 year.

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Predictors of long-term post-thrombotic syndrome following high proximal deep vein thrombosis: a cross-sectional study

Thrombosis Journal ◽

10.1186/s12959-020-00253-8 ◽

2021 ◽

Vol 19 (1) ◽

Author(s):

Marit Engeseth ◽

Tone Enden ◽

Per Morten Sandset ◽

Hilde Skuterud Wik

Keyword(s):

Deep Vein Thrombosis ◽

Proximal Deep Vein Thrombosis ◽

Cross Sectional ◽

Clinical Criteria ◽

Vein Thrombosis ◽

Post Thrombotic Syndrome ◽

Younger Age ◽

Deep Vein

Abstract Background Post-thrombotic syndrome (PTS) is a frequent chronic complication of proximal deep vein thrombosis (DVT) of the lower limb, but predictors of PTS are not well established. We aimed to examine predictors of PTS in patients with long-term PTS following proximal DVT. Methods During 2006–09, 209 patients with a first time acute upper femoral or iliofemoral DVT were randomized to receive either additional catheter-directed thrombolysis or conventional therapy alone. In 2017, the 170 still-living participants were invited to participate in a cross-sectional follow-up study. In the absence of a gold standard diagnostic test, PTS was defined in line with clinical practice by four mandatory, predefined clinical criteria: 1. An objectively verified DVT; 2. Chronic complaints (> 1 month) in the DVT leg; 3. Complaints appeared after the DVT; and 4. An alternative diagnosis was unlikely. Possible predictors of PTS were identified with multivariate logistic regression. Results Eighty-eight patients (52%) were included 8–10 years following the index DVT, and 44 patients (50%) were diagnosed with PTS by the predefined clinical criteria. Younger age and higher baseline Villalta score were found to be independent predictors of PTS, i.e., OR 0.96 (95% CI, 0.93–0.99), and 1.23 (95% CI, 1.02–1.49), respectively. Lack of iliofemoral patency at six months follow-up was significant in the bivariate analysis, but did not prove to be significant after the multivariate adjustments. Conclusions In long-term follow up after high proximal DVT, younger age and higher Villalta score at DVT diagnosis were independent predictors of PTS.

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Biological and clinical impact of hemangioblastoma-associated peritumoral cysts in von Hippel-Lindau disease

Journal of Neurosurgery ◽

10.3171/2015.4.jns1533 ◽

2016 ◽

Vol 124 (4) ◽

pp. 971-976 ◽

Cited By ~ 17

Author(s):

Kristin Huntoon ◽

Tianxia Wu ◽

J. Bradley Elder ◽

John A. Butman ◽

Emily Y. Chew ◽

...

Keyword(s):

Clinical Impact ◽

Anatomical Location ◽

Von Hippel Lindau ◽

Study Enrollment ◽

Common Location ◽

Younger Age ◽

The Mean ◽

A Prospective Study ◽

Vhl Disease

OBJECT Peritumoral cysts are frequently associated with CNS hemangioblastomas and often underlie neurological morbidity and mortality. To determine their natural history and clinical impact, the authors prospectively analyzed hemangioblastoma-associated peritumoral cysts in patients with von Hippel-Lindau (VHL) disease. METHODS Patients with VHL disease who had 2 or more years of follow-up and who were enrolled in a prospective study at the National Institutes of Health were included. Serial prospectively acquired laboratory, genetic, imaging, and clinical data were analyzed. RESULTS One hundred thirty-two patients (of 225 in the VHL study with at least 2 years of follow-up) had peritumoral cysts that were followed for more than 2 years (total of 292 CNS peritumoral cysts). The mean age at study entrance was 37.4 ± 13.1 years ([mean ± SD], median 37.9, range 12.3–65.1 years). The mean follow-up was 7.0 ± 1.7 years (median 7.3, range 2.1–9.0 years). Over the study period, 121 of the 292 peritumoral cysts (41.4%) became symptomatic. Development of new cysts was associated with a larger number cysts at study enrollment (p = 0.002) and younger age (p < 0.0001). Cyst growth rate was associated with anatomical location (cerebellum cysts grew faster than spine and brainstem cysts; p = 0.0002 and p = 0.0008), younger age (< 35 years of age; p = 0.0006), and development of new neurological symptoms (p < 0.0001). Cyst size at symptom production depended on anatomical location (p < 0.0001; largest to smallest were found, successively, in the cerebellum, spinal cord, and brainstem). The most common location for peritumoral cysts was the cerebellum (184 cysts [63%]; p < 0.0001). CONCLUSIONS Peritumoral cysts frequently underlie symptom formation that requires surgical intervention in patients with VHL disease. Development of new cysts was associated with a larger number of cysts at study enrollment and younger age. Total peritumoral cyst burden was associated with germline partial deletion of the VHL gene.

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