Intercostal Nerve Cryoablation or Epidural Analgesia for Multimodal Pain Management after the Nuss Procedure: A Cohort Study.

BACKGROUND: Nuss procedure for pectus excavatum is a minimally invasive, but painful procedure. Recently, intercostal nerve cryoablation has been introduced as a pain management technique. MATERIALS AND METHODS: In this cohort study, we compared the efficacy of multimodal pain management strategies in children undergoing a Nuss procedure. The effectiveness of intercostal nerve cryoablation combined with patient-controlled systemic opioid analgesia (PCA) was compared with continuous epidural analgesia (CEA) combined with PCA. The study was conducted between January 2019 and July 2022. Primary outcome was length of stay (LOS), and secondary outcomes were operation room time, postoperative pain, opioid consumption, and gabapentin use. RESULTS: Sixty-six consecutive patients were included, 33 patients in each group. The cryoablation group exhibited lower Numeric Rating Scale (NRS) pain scores on postoperative day 1 and 2 (p = 0.002, p = 0.001) and a shorter LOS (3 vs. 6 days (p < 0.001). Cryoablation resulted in less patients requiring opioids at discharge (30.3 vs. 97.0%; p < 0.001) and 1 week after surgery (6.1 vs. 45.4%; p < 0.001)). In the CEA group, gabapentin use was more prevalent (78.8 vs. 18.2%; p < 0.001) and the operation room time was shorter (119.4 vs. 135.0 minutes; p < .010). No neuropathic pain was reported. CONCLUSIONS: Intercostal nerve cryoablation is a superior analgesic method compared with CEA, with reduced LOS, opioid use, and NRS pain scores. The prophylactic use of gabapentin is redundant.

Pediatric thoracoscopic lung resections: a comprehensive analysis of congenital lesion cases.

BACKGROUND: Congenital lung lesions in pediatric patients may be managed conservatively or by video assisted thoracoscopic surgery (VATS). This study aimed to determine the complications after VATS for congenital lung lesions in children. METHODS: All children undergoing a lung resection between January 2009 and June 2022 were retrospectively identified. Children undergoing a primary open lobectomy or a resection other than a congenital lung lesion were excluded. Both early (<30 days) and late postoperative pulmonary complications were determined. The primary endpoint was postoperative complications within 30 days. RESULTS: In total, 56 patients were included, with a median age of 13 months (IQR 9-37). A VATS lobectomy were performed in 46 patients (82%), an extralobar sequestration in 8 patients (14%), an wedge resection in 1 patient and a segment resection in 1 patient. During the COVID pandemic, fewer resections were performed with an increase in symptomatic patients. A conversion to open occurred in 6 patients (11%), of which a preoperative lung infection was associated with an increased risk thereof(p = 0.004). The median follow-up was 22 months (IQR 7-57) and all patients were alive. A postoperative complication (Clavien Dindo ≥3) occurred in 9 patients and complications without the need of intervention in 6 patients. During follow-up a pneumonia occurred in 11 patients. CONCLUSION: There seems to be a shift towards delayed surgery with an increase in symptomatic congenital lung malformations, which might lead to an increase in postoperative complications.

Children with an Anorectal Malformation Going to Primary School: The Parent's Perspective.

BACKGROUND: Continence problems occur often in children with anorectal malformations (ARM). The aim of this study was to evaluate parental experiences with toilet facilities at Dutch primary schools and their experience with how schools deal with ARM children. METHODS: This survey was developed in collaboration with the national patient advocacy group (PAG). Recruitment for participation was conducted by the PAG (email listing and social media) and one expertise center for ARM. Participants were parents of school-attending ARM children aged 3 to 12 years. RESULTS: Sixty-one participants (31.9%) responded to the survey. The median age of the children was 7.0 years (IQR 5.0-9.0). Schools were often located in a village (63.9%) and encompassed 100-500 children (77.0%). In total, 14 parents (23.0%) experienced difficulties in finding a primary school. Experiences with the school were described as solely positive (37.7%), solely negative (9.8%), positive and negative (34.4%), and neither positive nor negative (16.4%). Regarding school toilet facilities, 65.6% of the toilets were reported clean and 78.7% were easily accessible. CONCLUSIONS: About 25% of parents reported difficulties in enrolling their children into primary school, and 45% reported negative experiences. This highlights the need for improved guidance and the optimization of education in schools when dealing with ARM children.

Spinal cord anomalies in children with anorectal malformations: a retrospective cohort study.

PURPOSE: First, to assess the number of spinal cord anomalies (SCA), specifically tethered spinal cord (TSC) in patients with anorectal malformations (ARM), identified with spinal cord imaging (i.e. spinal cord US and/or MRI). Second, to report outcomes after TSC treatment. METHODS: A retrospective mono-center study was performed. All ARM patients born between January 2000 and December 2021 were included. Screening for SCA consisted of spinal cord US and/or MRI. Radiology reports were scored on presence of SCA. Data were presented with descriptive statistics. RESULTS: In total, 254 patients were eligible for inclusion, of whom 234 (92.1%) underwent spinal cord imaging. In total, 52 (22.2%) patients had a SCA, diagnosed with US (n = 20, 38.5%), MRI (n = 10, 19.2%), or both US and MRI (n = 22, 42.3%), of whom 12 (23.5%) with simple, 27 (52.7%) intermediate, and 12 (23.5%) complex ARM types. TSC was identified in 19 patients (8.1%), of whom 4 (21.1%) underwent uncomplicated neurosurgical intervention. CONCLUSIONS: SCA were present in 22% of ARM patients both in simple, as well as more complex ARM types. TSC was present in 19 patients with SCA, of whom 4 underwent uncomplicated neurosurgical intervention. Therefore, screening for SCA seems to be important for all ARM patients, regardless of ARM type. LEVEL OF EVIDENCE: Level III.

Outcomes after Surgical Treatment for Rectal Atresia in Children: Is There a Preferred Approach? A Systematic Review.

Rectal atresia (RA) affects only 1 to 2% of all children with anorectal malformations. No consensus on optimal treatment strategy is yet achieved. Therefore, the aim of this systematic review is to summarize all surgical interventions for RA and outcomes described in the current literature. A literature search was conducted in PubMed, Embase, Web of Science, and Cochrane Library on January 24, 2022. All studies describing treatment for RA in children (< 18 years) were included. Operation technique and postoperative complications were listed. Only descriptive analysis was anticipated. Quality of the studies was assessed using Johanna Briggs Institute critical appraisal checklist for case reports and series. The search yielded 6,716 studies of which, after duplicate removal, 4,028 were excluded based on title and abstract screening. After full-text assessment, 22 of 90 studies were included, yielding 70 patients. Posterior sagittal anorectoplasty (PSARP) and pull-through were most performed (43/70 and 18/70 patients, respectively). Four patients experienced postoperative complications: anal stenosis (n = 1), anastomotic stenosis (n = 2), and death due to a pulmonary complication (n = 1). In the low-quality literature available, most patients with RA are treated with PSARP or pull-through technique. A low complication rate of both has been described but follow-up was often not mentioned. Larger well-designed studies should be performed to determine optimal treatment strategy for children with RA. This study reflects level of evidence V.

The Importance of Screening for Additional Anomalies in Patients with Anorectal Malformations: A Retrospective Cohort Study.

BACKGROUND: In children with anorectal malformations (ARM), additional anomalies can occur within the VACTERL-association. Routine screening is of great importance for early identification and potential treatment. However, uniformity in screening protocols is lacking and only small cohorts have been described in literature. The aim of this study was to assess and describe a unique large cohort of ARM patients who underwent VACTERL screening in the neonatal period. METHODS: A retrospective mono-center cohort study was performed. Included were all neonates born between January 2000 and December 2020 who were diagnosed with ARM and screened for additional anomalies. Full screening consisted of x-ray and ultrasound of the spine, cardiac and renal ultrasound, and physical examination for limb deformities, esophageal atresia, and ARM. Criteria for VACTERL-classification were predefined according to the EUROCAT-definitions. RESULTS: In total, 216 patients were included, of whom 167 (77.3%) underwent full VACTERL-screening (66% in 2000-2006 vs. 82% in 2007-2013 vs. 86% in 2014-2020). Median age at follow-up was 7.0 years (IQR 3.0-12.8). In 103/167 patients (61.7%), additional anomalies were identified. Some 35/216 patients (16.2%) fulfilled the criteria of a form of VACTERL-association. In 37/216 patients (17.1%), a genetic cause or syndrome was found. CONCLUSIONS: The majority of ARM patients underwent full screening to detect additional anomalies (77%), which improved over time to 86%. Yet, approximately a quarter of patients was not screened, with the potential of missing important additional anomalies that might have severe consequences in the future. Forms of VACTERL-association or genetic causes were found in 16% and 17% respectively. This study emphasizes the importance of routine screening. LEVEL OF EVIDENCE: III.

Dynamic Compression Therapy for Pectus Carinatum in Children and Adolescents: Factors for Success.

BACKGROUND: Pectus carinatum (PC) is a congenital chest wall deformity. In childhood, it is increasingly treated with dynamic compression therapy. Factors for success for dynamic brace therapy are relatively unknown. METHODS: Between 2013 and 2020, 740 patients treated with the Dynamic Compression System (DCS), were studied. This included the effect of age, gender, pectus height, symmetry and pectus rigidity on treatment time and symptoms with linear multiple regression analyses. RESULTS: Carinatum height and high pressure of initial correction at the start of treatment were associated with a prolonged duration of treatment. For each cm increase in carinatum height, the total treatment duration increased with 1.9 months (p-value= 0.002, 95% CI: 0.70-3.13). An initial correction pressure of ≥7.6 pounds per square inch (psi), increased the treatment duration with 3.5 months (p-value 0.006, 95% CI: 1.04-6.01) compared to an initial correction pressure of ≤5.0 psi. A high initial pressure of correction of ≥7.6 psi increased the odds of having somatic symptoms with 1.19 (p-value= 0.012, 95% CI: 1.04-1.45) and psychosocial symptoms with 1.13 (p-value= 0.04, 95% CI: 1.01-1.27) compared to a low initial pressure of correction of ≤5.0 psi. An initial pressure of correction of 5.1-7.5 psi increased the odds of having somatic symptoms with 1.14 (p-value 0.046, 95% CI: 1.00-1.29) compared to an initial pressure of correction of ≤5.0 psi. Patients with asymmetric chests were more likely to abandon therapy CONCLUSIONS: High carinatum height and high initial pressure of correction are associated with prolonged bracing treatment and a higher failure rate. LEVEL OF EVIDENCE: III.

Ravitch Surgery or Dynamic Compression Bracing for Pectus Carinatum: A Retrospective Cohort Study.

BACKGROUND: Pectus carinatum is a pediatric condition that can be treated by dynamic compression system (DCS) bracing or surgery. Several publications on DCS bracing or surgery are available; however, they do not compare both treatments. METHODS: Over a 10-year period, 738 patients with pectus carinatum were treated at the Amsterdam Pectus Center (Amsterdam, The Netherlands). This study describes this 10-year experience and the results of both treatments. RESULTS: Of the 631 patients who underwent DCS bracing treatment, 553 finished treatment, and 78 patients are still under treatment. A total of 73.8% (n = 408) of these patients finished treatment successfully, 13.6% (n = 75) experienced treatment failure, and 12.7% (n = 70) were lost to follow-up. The success rate decreased with an increasing pressure of initial compression (84.2%-67.3%). Marfan syndrome and Poland syndrome were associated with unfavorable results. Ravitch surgery was performed in 105 patients, with a success rate of 92.4%. Complications occurred in 32.4% of patients, and 6.7% of patients had complications for which surgery was needed. No relationship was found between osteotomy or sternal fixation and outcomes or complications. The Abramson procedure was performed in 2 patients. CONCLUSIONS: DCS bracing should be the treatment of choice in patients with pectus carinatum because of its noninvasiveness, good results, and lower complication rate compared with surgery. Besides pressure of initial correction, motivation is an important factor influencing outcomes, and compliance remains a major challenge in treating pectus carinatum using DCS bracing. Bracing patients before their growth spurt should be discouraged. Patients with a higher pressure of initial compression (>8.0-8.5 psi) and Marfan syndrome or Poland syndrome have poorer outcomes. In those patients, surgery may be considered.

Treatment strategies for anterior cutaneous nerve entrapment syndrome in children: A systematic review.

BACKGROUND: Anterior cutaneous nerve entrapment syndrome (ACNES) is a frequently overlooked cause of chronic abdominal pain in children. Currently, both nonsurgical and surgical treatment options are available to treat this disease. The objective was to give insight into the success rate of different treatment strategies for children with ACNES, and provide treatment recommendations for physicians based on the published evidence. METHOD: A literature search of PubMed, Embase.com and the Wiley/Cochrane Library was conducted for studies published up to 25 February 2020. Randomized controlled trials, prospective or retrospective cohort studies, meta-analyses and literature reviews describing the outcome of different treatment strategies for children (<18 years old) with ACNES with a follow-up duration of at least four weeks were included. RESULTS: Six studies, involving 224 patients, were included with an overall quality reported to be between fair and poor. Treatment success of local injections with an anesthetic agent into the trigger point ranged from 38% to 87% with a follow-up ranging from 4 weeks to 39 months. In addition, treatment success of anterior neurectomy ranged from 86% to 100%, with a follow-up duration ranging from 4 weeks to 36 months. CONCLUSION: A step-up treatment strategy should be applied when treating pediatric patients with ACNES. This strategy starts with an injection with a local anesthetic agent, reserving surgery (anterior neurectomy) as a viable option in case of persistent pain. LEVEL OF EVIDENCE: II.

Interventions with Music in PECTus excavatum treatment (IMPECT trial): a study protocol for a randomised controlled trial investigating the clinical effects of perioperative music interventions.

INTRODUCTION: Pectus excavatum repair is associated with substantial postoperative pain, despite the use of epidural analgesia and other analgesic regimens. Perioperative recorded music interventions have been shown to alleviate pain and anxiety in adults, but evidence for children and adolescents is still lacking. This study protocol describes a randomised controlled trial that evaluates the effects of recorded music interventions on postoperative pain relief in children and adolescents after pectus excavatum repair. METHODS: A multicentre randomised controlled trial was set up comparing the effects of perioperative recorded music interventions in addition to standard care with those of standard care only in patients undergoing a Nuss procedure for pectus excavatum repair. One hundred and seventy subjects (12-18 years of age) will be included in three centres in the Netherlands. Patient inclusion has started in November 2018, and is ongoing. The primary outcome is self-reported perceived pain measured on the visual analogue scale. Secondary outcomes are anxiety level, analgesics consumption, vital parameters such as heart rate, blood pressure and respiratory rate, length of hospital stay, postoperative complications, quality of life and cost-effectiveness. ETHICS AND DISSEMINATION: This study is being conducted in accordance with the Declaration of Helsinki. The Medical Ethics Review Board of Erasmus University Medical Centre Rotterdam, The Netherlands, has approved this protocol. Results will be disseminated via peer-reviewed scientific journals and conference presentations. TRIAL REGISTRATION NUMBER: NL6863.

Sternocostal instability after Ravitch repair in adolescents; 3 case-reports and a review of surgical techniques in the literature.

Ravitch repair is a common surgical procedure to correct chest wall deformities. In this procedure, a subperichondreal cartilage resection of the deformed parasternal cartilage, and if necessary a repositioning of the sternum, is performed. Insufficient regeneration of the resected cartilage may result in sternocostal instability or even floating sternum. This rare complication presents with symptoms of pain and exercise intolerance. METHODS: We describe sternocostal instability in 3 adolescent patients after the Ravitch procedure for pectus carinatum and reviewed the literature on this topic. RESULTS: Our patients suffered different degrees of instability. In all cases, we eventually achieved a satisfactory outcome. There is little literature on sternocostal instability. It is a rare complication, mainly occurring after reoperation by damaging the perichondrium. CONCLUSIONS: Malunion of costal cartilage is a rare complication of open pectus repair. To achieve the best regeneration and stability of the sternum, less extended resection of cartilage should be performed and the number of cartilages resected should be limited. The perichondrium must be kept intact. Autologous grafts, growth-enhancing materials, and metal or bioabsorbable struts may contribute to stabilization and regeneration of the cartilage.

Histopathologic examination of resected nerves from children with anterior cutaneous nerve entrapment syndrome: Clues for pathogenesis?

BACKGROUND: Anterior cutaneous nerve entrapment syndrome (ACNES) has been described as a possible cause for chronic pain in the pediatric population. However, the exact pathophysiology of ACNES is unknown. It may be caused by compression or traction of cutaneous nerve branches of intercostal nerves, or it may be the result of an infection. Therefore, we present histopathological evidence to determine the pathophysiology of ACNES. METHODS: A total of seven pediatric patients underwent a neurectomy for ACNES. All specimens were sent for histopathological evaluation, including immunohistochemical staining, to evaluate if there were any signs of infection, inflammation or compression. RESULTS: Seven out of seven (100%) histopathological specimens showed non-specific nerve degeneration. Immunohistochemical evaluation showed there were several CD68-positive macrophages present in the specimens. Four out of seven (57%) specimens showed the presence of a few CD3-positive T-cells, however, this was not suggestive for inflammation or infection. CONCLUSION: Our study supports the hypothesis that ACNES is caused by compression of the nerves rather than inflammation. LEVEL OF EVIDENCE: III.

[Dynamic compression brace for pectus carinatum: 5 years on]. / Dynamische-compressiebrace bij pectus carinatum.

Pectus carinatum is a congenital chest-wall deformity with a prevalence of 0.3-0.7%. This chest-wall deformity can lead to functional, cosmetic and psychosocial problems. For many years, the only available treatment was an invasive surgical procedure. A less-invasive treatment, namely external compression of the sternum with a brace, showed disappointing results due to discomfort, a high percentage of skin complications and low compliance. The introduction of the dynamic compression brace has meant that the pressure of sternal compression can be measured and adjusted, which has improved comfort and compliance and resulted in an increased success rate of 92% and a decrease in the percentage of skin complications to just 8%. Despite the fact that wearing a compression brace for a longer period of time is not an easy undertaking for an adolescent, the use of the dynamic compression brace results in a high success rate, and is a safe and an efficient treatment.

Measured dynamic compression for pectus carinatum: A systematic review.

BACKGROUND: Patients with pectus carinatum have been treated with orthotic braces since the 1970s. By applying external pressure to the anterior chest wall, a normal chest shape can be restored. However, high patient treatment dropout rates were reported because of the subjectively high pressures applied to the patient´s skin. Measured dynamic compression allows measurement and adjustments of the brace's pressure on the thoracic wall, leading to a controlled correction. METHODS: We performed an electronic database search (Pubmed and Cochrane) of the medical literature on measured dynamic compression. A total of 14 studies were found and eight studies between 2008 and 2018 were included. Study designs ranged from retrospective chart reviews to cross-sectional cohort studies. RESULTS: From the 8 studies, 1185 patients were included. The median age was 14 years (range 2-28) and 87% were male. The mean study follow up period was 16 months; 44% of patients were still under treatment, 29% of patients successfully completed treatment. 6% dropped out and 21% were lost to follow-up. Dropout was mainly caused social discomfort (7.2%) and failure of treatment (5.8%). Complications were infrequent. Mild chest discomfort or tightness was reported in 12% and skin lesions occurred in 5.1%. The overall recurrence rate was 2.6%. CONCLUSIONS: Several studies are available on measured dynamic compression. Dynamic compression appears to be a safe, non-invasive and efficient treatment to correct pectus carinatum in patients with a non-rigid thorax. Patients experience less discomfort, which in turn results in better compliance. However, accurate selection of patients based on age, pressure of initial correction and motivation is important and an objective scoring system to assess the esthetic and long-term physical and psychological results of the treatment is needed.

The Dynamic Compression Brace for Pectus Carinatum: Intermediate Results in 286 Patients.

BACKGROUND: Dynamic brace compression is a novel treatment for patients with pectus carinatum. The dynamic compression system contains a device to measure the flexibility of the thoracic wall and regulate the pressure of the brace. METHODS: Patients referred to our pediatric surgical center were screened for treatment with the dynamic compression brace. Patients with a pressure of initial correction (PIC) of 10.0 pounds per square inch or less were offered treatment with the brace. Patients with a PIC above 10.0 pounds per square inch were offered surgical correction. Between March 2013 and April 2016, 286 patients were treated with the brace; 260 were male (91%) and 26 were female (9%). Their mean age was 14 years (range, 4 to 21 years). RESULTS: Seventy-eight patients completed brace treatment; the mean treatment time was 14 months. Twenty-seven patients abandoned treatment because of lack of motivation, loss to follow-up, persistent protrusion of the sternal bone or flaring that required surgical correction, failure of treatment because of a bifid rib, fear of locking the brace, and delayed correction. One hundred eighty-one patients are still wearing the brace, either in the active or in the retainer phase. Patients with a high PIC also showed improvement when they were compliant. Adverse events were minor and included skin lesions (n = 4, 1%) and vasovagal reactions at the start of therapy (n = 3, 1%). CONCLUSIONS: These data show that brace therapy can be considered a valuable treatment option to correct pectus carinatum in patients with a flexible thorax.

[Mechanical ileus in children with no prior history of abdominal surgery]. / Ileus bij kinderen zonder eerdere buikoperaties.

In children with no prior history of abdominal surgery and no signs of intussusception or incarcerated inguinal hernia, mechanical ileus may have a congenital cause such as malrotation with volvulus or a persistent omphalomesenteric duct. Acquired causes include sigmoid volvulus. We present two cases of mechanical ileus in children. The first case involved a 6-year-old boy who presented with acute abdominal pain and vomiting. An emergency laparotomy was performed, with resection of the omphalomesenteric duct. Recovery was uneventful. The other case concerned a 9-year-old boy who presented with increasing abdominal pain, bilious vomiting and general clinical deterioration. An emergency laparotomy was performed, revealing malrotation with volvulus and intestinal ischaemia. Children with no prior abdominal history who present with symptoms that may be caused by mechanical obstruction should be managed with a view to surgery and without delay, in order to prevent a catastrophic outcome resulting from a congenital or acquired mechanical obstruction.

[Gastric volvulus in children]. / Maagvolvulus bij kinderen.

BACKGROUND: Gastric volvulus in children is rare and can be life-threatening if not recognised in time. CASE DESCRIPTION: A 3.5-year-old boy presented to his GP with severe, non-bilious vomiting, abdominal distension and abdominal pain. Additional diagnostic tests showed hypochloraemic alkalosis and abdominal ultrasonography suggested gastric volvulus. A barium study showed slow passage of contrast into the duodenum combined with craniomedial displacement of the duodenum. This image matches that of mesenteroaxial gastric volvulus. The diagnosis was confirmed during surgery and gastropexy was successfully performed. CONCLUSION: The diagnosis of gastric volvulus can be difficult. Easily accessible additional diagnostics in the form of ultrasonography, possibly followed by a barium study, may be helpful.

[Dynamic compression brace for pectus carinatum]. / Dynamische-compressiebrace bij pectus carinatum.

Pectus carinatum occurs in one of 1500-1700 people and may lead to physical, cosmetic and psychosocial problems. Surgical treatment of pectus carinatum is performed using the 'Ravitch procedure'. An alternative and less invasive treatment consists of external compression with a brace. Results are often disappointing because of skin complications, a low level of comfort of the brace and low compliance. Nowadays a new brace (dynamic compression brace) exists with which the comfort and thus the compliance can be increased by measuring and regulating the pressure of correction during the treatment. This leads to better results and fewer complications. In 80-90% of patients the results are good to excellent and an operation can be avoided. This new brace is therefore a less invasive, cheap and safe alternative to the Ravitch procedure in the treatment of pectus carinatum in children.

[A newborn with an ovarian cyst]. / Een pasgeborene met een ovariumcyste.

BACKGROUND: An ovarian cyst is one of the most common types of cysts found in neonates. As a result of the introduction of the 20-week screening ultrasound, the detection of ovarian cysts will likely increase. CASE DESCRIPTION: An abdominal cyst was detected by ultrasound in a female foetus in her 20th gestational week. This simple cyst remained smaller than 5 cm in diameter so an expectative course was opted for. Six months after her birth, however, the cyst's echographic appearance changed. A necrotic left ovary was resected during a diagnostic laparoscopy. CONCLUSION: We recommend taking an expectative approach to asymptomatic simple or complex ovarian cysts, because they usually regress spontaneously in the first year of life. It is important to monitor the cyst by ultrasound and to repeatedly measure tumour marker concentrations as necessary.