Minimally invasive repair of pectus excavatum in patients with Marfan syndrome and marfanoid features

Purpose

The presence of a pectus excavatum (PE) requiring surgical repair is a major skeletal feature of Marfan syndrome. Marfanoid patients have phenotypic findings but do not meet all diagnostic criteria. We sought to examine the clinical and management differences between Marfan syndrome patients and those who are marfanoid compared with all other patients undergoing minimally invasive PE repair.

Methods

A retrospective institutional review board-approved review was conducted of a prospectively gathered database of all patients who underwent minimally invasive repair of PE. Patients were grouped according to diagnosis of Marfan syndrome (MAR), Marfanoid appearance (OID), and all others (ALL). Patient demographics, preoperative imaging and testing, operative strategy, complications, and postoperative surveys were evaluated. Fisher's Exact test and χ² were applied for statistical analysis.

Results

From June 1987 to September 2008, 1192 patients underwent minimally invasive PE repair (MAR = 33, OID = 212, ALL = 947). There was a significantly higher proportion of females with either MAR or OID who underwent repair (21.5%vs 15.5%, P = .04). The MAR patients had significantly more severe PE determined by computed tomography index (MAR = 8.75, OID = 5.82, ALL = 4.94, P < .0001) and required multiple pectus bars (≥2) to be placed during operation (MAR = 58%, OID = 36%, ALL = 29%, P = .001). There was a trend toward higher wound infection rates in MAR patients (MAR = 6%, OID = 1.4%, ALL = 1.3%, P = .07). The recurrence rate was similar among all groups (MAR = 0%, OID = 2%, ALL = 0.7%, P = .12). Successful outcome from surgeon perspective in either MAR or OID patients was similar to ALL (98%vs 98%, P = .88) and correlated well with patient satisfaction after repair (96%vs 95%, P = .43).

Conclusions

Minimally invasive PE repair is safe in patients with Marfan syndrome or marfanoid features with equally good results. Patients with Marfan syndrome have clinically more severe PE requiring multiple bars for chest repair and may have slightly higher wound infection rates. Patients are satisfied with minimally invasive repair despite a phenotypically more severe chest wall defect.     

Intraoperative intercostal nerve cryoablation During the Nuss procedure reduces length of stay and opioid requirement: A randomized clinical trial

PurposeMinimally-invasive repair of pectus excavatum by the Nuss procedure is associated with significant postoperative pain, prolonged hospital stay, and high opiate requirement. We hypothesized that intercostal nerve cryoablation during the Nuss procedure reduces hospital length of stay (LOS) compared to thoracic epidural analgesia.DesignThis randomized clinical trial evaluated 20 consecutive patients undergoing the Nuss procedure for pectus excavatum between May 2016 and March 2018. Patients were randomized evenly via closed-envelope method to receive either cryoanalgesia or thoracic epidural analgesia. Patients and physicians were blinded to study arm until immediately preoperatively.SettingSingle institution, UCSF-Benioff Children's Hospital.Participants20 consecutive patients were recruited from those scheduled for the Nuss procedure. Exclusion criteria were age < 13 years, chest wall anomaly other than pectus excavatum, previous repair or other thoracic surgery, and chronic use of pain medications.Main outcomes and measuresPrimary outcome was postoperative LOS. Secondary outcomes included total operative time, total/daily opioid requirement, inpatient/outpatient pain score, and complications. Primary outcome data were analyzed by the Mann–Whitney U-test for nonparametric continuous variables. Other continuous variables were analyzed by two-tailed t-test, while categorical data were compared via Chi-squared test, with alpha = 0.05 for significance.Results20 patients were randomized to receive either cryoablation (n = 10) or thoracic epidural (n = 10). Mean operating room time was 46.5 min longer in the cryoanalgesia group (p = 0.0001). Median LOS decreased by 2 days in patients undergoing cryoablation, to 3 days from 5 days (Mann–Whitney U, p = 0.0001). Cryoablation patients required significantly less inpatient opioid analgesia with a mean decrease of 416 mg oral morphine equivalent per patient (p = 0.0001), requiring 52%–82% fewer milligrams on postoperative days 1–3 (p < 0.01 each day). There was no difference in mean pain score between the groups at any point postoperatively, up to one year, and no increased incidence of neuropathic pain in the cryoablation group. No complications were noted in the cryoablation group; among patients with epidurals, one patient experienced a symptomatic pneumothorax and another had urinary retention.Conclusions and relevanceIntercostal nerve cryoablation during the Nuss procedure decreases hospital length of stay and opiate requirement versus thoracic epidural analgesia, while offering equivalent pain control.Type of studyTreatment study.Level of evidenceLevel I.     

Fontan类手术治疗心房异构、内脏异位合并心脏畸形的效果

目的 评价Fontan类手术治疗心房异构、内脏异位合并心脏畸形的效果.方法 2002年4月至2010年12月,25例心房异构、内脏异位合并心脏畸形共行手术治疗28次.男10例,女15例,年龄2～18岁,平均(9.1±5.5)岁.右心房异构18例,左心房异构7例.左心室双入口合并肺动脉狭窄15例,左心室双入口和双出口合并肺动脉狭窄5例,右心室双出口合并肺动脉闭锁2例,合并肺动脉狭窄1例,二尖瓣和三尖瓣闭锁合并肺动脉狭窄各1例.Fontan手术16例次,其中15例次行心外管道Fontan手术,1例次行心房内侧隧道Fontan手术;双向腔肺分流术8例次,3例次为双上腔静脉肺动脉分流;全腔静脉肺动脉分流4例次(Kawashima手术).结果 术后早期死亡2例,均为术后严重低心排血量综合征.术后室上性心动过速5例,结性心律2例.术后经皮血氧饱和度0.78 ～ 1.00,平均0.86±0.07.随访6个月以上者15例,最长7年,经皮血氧饱和度0.68 ～0.97,平均0.82±0.08;心室射血分数＞0.55者13例,＜0.50者2例;结性心律4例.结论 Fontan类手术是治疗心房异构、内脏异位合并心内复杂畸形的主要手术方式,术后早、中期效果较好.选择Fontan手术的术式,特别是心外管道,妥善修复心内合并畸形是提高效果的关键.