Optimal treatment of pneumothorax in adolescents with Marfan syndrome.

PURPOSE: Pneumothorax often develops in patients with Marfan syndrome (MFS). Here, we examined the effects of conservative and surgical pneumothorax treatments in children with MFS. METHODS: In this study, 23 patients, less than 20 years old, diagnosed with both MFS and pneumothorax between 1999 and 2019 were included. All data were collected retrospectively from patients' medical records. RESULTS: In total, 18 of 23 patients (78%) had relapsed pneumothorax either on the ipsilateral or contralateral side. Among these 18 patients, 6 (26%) patients had multiple relapses. Conservative and surgical treatments of pneumothorax were attempted in 33 and 29 lungs, respectively. The conservative treatment was attempted as a definitive therapy in 21 lungs. Twelve conservative treatments (57%) failed, which required surgical intervention. In 9 lungs (43%) with successful conservative treatment, 6 (67%) had ipsilateral relapses. In contrast to the above findings, only 4 (13%) ipsilateral relapses were observed in 29 surgical treatments. CONCLUSIONS: Our study revealed a low response and high relapse rate when MFS adolescents who diagnosed pneumothorax were subjected to the conservative treatment modality. Thus, we recommend surgical intervention as the first line of therapy to treat pneumothorax in adolescents diagnosed with MFS. LEVEL OF EVIDENCE: Ⅲ (Treatment Study).

Prophylactic innominate artery transection to prevent tracheoinnominate artery fistula: a retrospective review of single institution experiences.

PURPOSE: This study aimed to investigate the optimal indication and availability of prophylactic innominate artery transection (PIAT). METHODS: We retrospectively analyzed the medical records of the patients with neurological or neuromuscular disorders (NMDs) who underwent PIAT. Meanwhile, we originally defined the tracheal flatting ratio (TFR) and mediastinum-thoracic anteroposterior ratio (MTR) from preoperative chest computed tomography imaging and compared these parameters between non-PIAT and PIAT group. RESULTS: There were 13 patients who underwent PIAT. The median age was 22 years. PIAT was planned before in one, simultaneously in five, and after tracheostomy or laryngotracheal separation in seven patients. Image evaluations of the brain to assess circle of Willis were performed in all patients. Appropriate skin incisions with sternotomy to expose the innominate artery were made in four patients. All patients are still alive except one late death without any association with PIAT. No neurological complications occurred in any patients. As significant differences (p < 0.01) between two groups were observed for TFR and MTR, objective validity of the indication of PIAT was found. CONCLUSIONS: PIAT is safe and tolerable in case of innominate artery compression of the trachea with NMDs. TFR and MTR are useful objective indexes to judge the indication of PIAT.

Is intraoperative anal endosonography necessary during laparoscopy-assisted anorectoplasty for high/intermediate type imperforate anus?

PURPOSE: The aim of this study was to evaluate the relevance of anal endosonography (AES) during Georgeson laparoscopy-assisted anorectoplasty (GLA) for high/intermediate type imperforate anus (HIIA) using a continence evaluation questionnaire (CEQ) and postoperative AES (post-AES). METHODS: Fifteen HIIA patients who underwent GLA were enrolled in this prospective study. Six had intraoperative AES (op-AES) to confirm the accuracy of the positioning of the pull-through (PT) canal and 9 did not (no-AES). All subjects were reviewed prospectively by one surgeon and all had post-AES to measure the external sphincter (ES) and puborectalis (PR) at 3 and 9 o'clock to confirm that the PT colon was central. A CEQ (5 parameters: frequency of motions, severity of staining, severity of perianal erosions, anal shape, and requirement for medications; maximum score: 10) was administered to 9 subjects (4 from op-AES and 5 from no-AES) followed-up for over 3 years to assess continence. RESULTS: Mean age at GLA for both groups was not statistically different. There were no statistical differences between the two groups for thickness of the ES and PR on post-AES. Mean annual CEQ scores for the op-AES group 1, 2, 3, 4, and 5 years after GLA were 5.3, 6.3, 7.4, 8.4, and 8.5, respectively, while for the no-AES group were 5.9, 7.1, 8.1, 8.2, and 8.2, respectively; all differences were not statistically significant. CONCLUSIONS: Our results suggest that op-AES is not necessary during GLA because there is no difference in clinical and functional status that can be attributed to op-AES.

Midterm postoperative clinicoradiologic analysis of surgery for high/intermediate-type imperforate anus: prospective comparative study between laparoscopy-assisted and posterior sagittal anorectoplasty.

PURPOSE: The objective of this study is to analyze the outcome of surgery for high/intermediate-type imperforate anus using anal endosonography (AES), magnetic resonance imaging (MRI), and a continence evaluation questionnaire (CEQ). METHODS: In this study, 24 cases of high/intermediate-type imperforate anus were studied. Fifteen of 24 had Georgeson's laparoscopy-assisted anorectoplasty (GLA), and 9 of 24 had Pena's posterior sagittal anorectoplasty (PPA). All subjects had AES and MRI postoperatively. On AES, differences in thickness of the external sphincter (ES) and puborectalis (PR) at 3 and 9 o'clock were compared, and if pull-through colon was central, AES was 0. On MRI, differences in thickness were analyzed semiquantitatively and scored; if muscles were of even thickness, the score was 0; slight difference was 1, and marked difference was 2. A 5-parameter CEQ questionnaire (maximum score, 10) was administered to 16 of 24 subjects followed up for more than 3 years (9 GLA, 7 PPA). Surgical stress was assessed using mean febrile period, duration of raised white blood cell count, and peak C-reactive protein level. RESULTS: Mean age at surgery and mean postoperative period for both groups were not statistically different. There were no differences in mean muscle thickness for ES or PR on AES according to procedure (ES: GLA = 0.19 +/- 0.15 mm, PPA = 0.16 +/- 0.09 mm, P = .59; PR: GLA = 0.19 +/- 0.19 mm, PPA = 0.22 +/- 0.15 mm, P = .69). Magnetic resonance imaging scores were also not significantly different according to procedure (GLA: 0.77 +/- 0.83; PPA: 0.75 +/- 0.50, P = .97). When CEQ were compared annually, scores for GLA were generally higher throughout the study but only statistically significant at 3 and 4 years (P < .05). Differences in parameters of surgical stress were not significant. CONCLUSION: Although there were no differences in muscle thickness and parameters of surgical stress observed according to technique, GLA would appear to provide better outcome based on CEQ scores.

Retrosternal revision of jejunum interposed in the anterior sternal space for the treatment of esophageal atresia.

Esophageal replacement continues to be a challenging operation associated with significant morbidity. We report the case of a 5-year-old boy with esophageal atresia treated by jejunal interposition in the anterior sternal space who had revision of the interposed jejunum into the retrosternal space for cosmetic reasons.