Tatsuo Nakaoka

Indications for surgical treatment of funnel chest by chest radiograph

Abstract. Forty-seven children with funnel chest (FC) who underwent sternal elevation and 210 normal children were examined to determine the indications for surgical treatment using the vertebral index (VI) and frontosagittal index (FSI). In normal children VI gradually increased and FSI gradually decreased with age. Both indices changed significantly at 3 years of age. Although the VI of FC patients decreased significantly from 33.8 ± 7.6 (n=40) to 24.4 ± 3.9 (n=38) postoperatively (P < 0.0001), it was significantly larger than that of normal children over 3 years of age (20.2 ± 2.2, n=150) (P < 0.0001), and although the FSI of FC patients increased significantly from 22.0 ± 7.0 (n=40) to 34.5 ± 6.5 (n=38) postoperatively (P < 0.0001), it was significantly smaller than that of normal children over 3 years of age (41.1 ± 4.0, n=150) (P < 0.0001). Since many patients had a thin and flat chest despite excellent correction, their postoperative indices were not normal. There was a correlation between VI and FSI in normal children and a high degree of correlation between VI and FSI both before and after operation in FC patients. We conclude that a VI of more than 27 and/or a FSI of less than 29 are indications for surgical treatment based on the mean VI + 3SD and FSI − 3SD of normal children over 3 years of age. These values are almost equal to the mean VI − SD and FSI + SD of patients with physical, cosmetic, and/or psychological disturbances. However, it is not necessary to measure both indices simultaneously. Postoperative VI and FSI did not always reflect the degree of chest-wall depression in FC patients because of their flat chests.

Does overgrowth of costal cartilage cause pectus excavatum? A study on the lengths of ribs and costal cartilages in asymmetric patients

PURPOSE The cause of pectus excavatum has been hypothesized to be overgrowth of the costal cartilage. According to this theory, the length of costal cartilages must be longer in the side of deep depression in asymmetric patients. To challenge this hypothesis, we measured the lengths of ribs and costal cartilages and investigated lateral differences. SUBJECTS AND METHODS Twenty-four adolescent and adult patients with asymmetric pectus excavatum (14-30 years of age) with no history of surgery were investigated in this study. The fifth and sixth ribs and costal cartilages were individually traced to measure their full lengths on 3-dimensional computed tomographic (CT) images. As an index of asymmetry, sternal rotation angle was measured in the chest CT images. Patients with a 21 degrees or greater angle of sternal twist were designated as an asymmetric group and those with an angle of smaller than 20 degrees as a symmetric group. Lateral differences in the fifth and sixth costal and costal cartilage lengths were compared between the groups. RESULTS On comparison of the costal and costal cartilage lengths in the asymmetric group, the right fifth ribs and costal cartilages were significantly shorter than the left (P = .02 and .03, respectively), and right sixth ribs were also significantly shorter than the left (P = .004), but right sixth costal cartilages were not (P = .31). In the symmetric group, the lengths of the left and right fifth ribs and costal cartilages were showing no significant difference (P = .20 and P = .80, respectively), and those of the sixth ribs and costal cartilage were also showing no significant difference (P = .97 and P = .64, respectively). DISCUSSION The ribs and costal cartilages on the right side with severer depression were significantly shorter or not different than those on the contralateral side. Based on these findings, the theory of costal cartilage overgrowth is contradictory. The etiology of asymmetric chest deformity should be reevaluated.

Indications for surgical repair of funnel chest based on indices of chest wall deformity and psychological state.

AbstractPurpose. We examined the surgical indications for funnel chest, taking psychological factors into consideration. Methods. We assessed 36 young people with funnel chest who were seen as outpatients, including 31 boys and 5 girls aged from 1 to 22 years old. Respondents were asked whether they suffered psychological distress, and if they wanted surgery. The severity of the deformity was evaluated using the Vertebral Index (VI) and the Frontosagittal Index (FSI) calculated from chest roentgenograms. Results. The VI in 11 patients without distress (23.7 ± 4.1) was lower than that in 25 patients with distress (32.8 ± 8.2), and the FSI in the patients without distress (33.5 ± 5.3) was higher than that in the patients with distress (23.6 ± 8.6). The VI in 19 patients who did not want surgery (26.9 ± 7.9) was lower than that in 17 patients who did (33.5 ± 7.5), and the FSI in the patients who did not want surgery (30.4 ± 8.1) was higher than that in the patients who did (22.4 ± 8.1). The distressed patients suffered many psychological problems, such as being the object of bullying. Conclusion. The severity of the deformity affected the patients psychological state. We consider that a VI ≫28 or an FSI ≪28 are indications for surgery, based on the mean VI + SD and the mean FSI-SD of patients not suffering distress.

Evaluation of the Nuss procedure using pre- and postoperative computed tomographic index

BACKGROUND Objective assessment of the chest in patients with pectus excavatum after the Nuss procedure has not been published. This study evaluated the results of the Nuss procedure using computed tomographic (CT) index (CTi). METHODS We have performed the Nuss procedure in 382 patients since 1998, and 150 patients who underwent bar removal were included in this study. Computed tomographic scans were obtained before the Nuss procedure and after bar removal, and then preoperative CTi (pre-CTi) and postoperative CTi (post-CTi) were calculated. Computed tomographic scans of 62 age-matched patients without chest deformity were collected as controls. Patients were divided at 10 years of age into the younger and older groups, and groups with mild and severe deformity were defined using a pre-CTi value of 5 as border. These CT indices were compared and statistically analyzed. RESULTS Mean pre-CTi in all cases was 5.97 +/- 3.31 and improved to 3.08 +/- 0.64. Postoperative CTi was not significantly different from that of the control (2.47 +/- 0.32, P = .17). In the group with mild depression, pre-CTi was 4.15 +/- 0.62, and post-CTi was 2.88 +/- 0.50. Preoperative CTi in the group with severe deformity (7.44 +/- 3.82) improved to 3.25 +/- 0.69. Postoperative CTi values between the severe and mild groups were not significantly different (P = .75). Computed tomographic index of the young group improved from 6.20 +/- 3.58 to 2.93 +/- 0.49 and in older group from 5.50 +/- 2.64 to 3.40 +/- 0.79. These 2 post-CTi values were not significantly different (P = .73). CONCLUSION Postoperative CT scan could provide objective evaluation of sternal elevation. Mean CTi after the Nuss procedure was statically equivalent to that of the control cohort. Good sternal elevation can be achieved with the Nuss procedure regardless of the severity of chest depression or age.

Overgrowth of costal cartilage is not the etiology of pectus excavatum

PURPOSE The etiology of pectus excavatum (PE) has not been clarified. In 1944, Sweet (Sweet RH. Pectus excavatum. Ann Surg 1944;119:922-934) mentioned about the possibility of the overgrowth of costal cartilage being involved. However, no additional report that supports his hypothesis is available. In this study, we investigated whether the overgrowth of costal cartilage was an actual cause of PE through measurement of the costal cartilage length in PE patients and healthy controls. MATERIALS AND METHODS We investigated the length of the fifth and sixth costal cartilages and ribs in PE patients from reconstructed images of 3-dimensional computed tomography. To examine the relative costal cartilage length, we calculated the C/R ratio, defined as the quotient of the costal cartilage length divided by the adjacent rib length, and compared it between PE patients and healthy controls. RESULTS In PE patients, the C/R ratios were not larger than in healthy controls at any level. At the left sixth, the C/R ratio was significantly smaller in patients than in the healthy control group. DISCUSSION The results revealed that, in PE patients, relative costal cartilage lengths were not longer than in healthy controls. We conclude that the overgrowth of costal cartilage is not the etiology of PE.

Familial Currarino syndrome associated with Hirschsprung disease: two cases of a mother and daughter.

Currarino syndrome with Hirschsprung disease (CS-HD) is extremely rare. We present the first family with CS-HD. Case 1: A 28-year-old woman was admitted with severe abdominal distension and dyspnea. She was diagnosed with anal stenosis, hemisacrum, anterior sacral meningocele (ASM), tethered cord (TC), and short-segment aganglionosis. She underwent the modified Duhamel operation after meningocele repair and cord detethering. A bicornuate uterus, bilateral ovarian dermoid cysts, and small rectal duplication were also noted intraoperatively. Case 2: The daughter of case 1 was admitted for abdominal distension and anal stenosis at the age of 17 days. Studies revealed a hemisacrum, ASM, TC, presacral mass, atrial septal defect, polyp in the right nasal cavity, right vesicoureteral reflux, and short-segment aganglionosis. She underwent the modified Soave operation at the age of 1 year and 4 months after meningocele repair, cord detethering, and resection of the presacral mass (epidermoid cyst). In both cases, the aganglionic segments were confirmed by preoperative rectal suction biopsy and postoperative pathological examination on full-thickness rectal specimens. Some causal genes for Currarino syndrome (CS) and Hirschsprung disease (HD) are currently investigated. Thus far, 10 CS-HD cases have been reported, including 6 cases of familial CS. However, all the patients had sporadic HD. Recent reports suggest that anomalies of the enteric nerve system contribute to postoperative constipation in CS cases.

Early Detection and Treatment of Neuroblastic Tumor with Opsoclonus-Myoclonus Syndrome Improve Neurological Outcome: A Review of Five Cases at a Single Institution in Japan

INTRODUCTION Opsoclonus-myoclonus syndrome (OMS) is a paraneoplastic neurological disorder associated with neuroblastic tumor (NT) in childhood. Half of patients have neurological sequelae after the neurological and oncological treatment. We reviewed the neurological and oncological outcomes of NT with OMS, and discussed whether the treatment of NT would contribute to improving the neurological prognosis. METHODS We retrospectively assessed NT patients with OMS from January 2001 to December 2013 at a single institution in Japan. Demographic data, neurological and oncological status, histopathology, treatments, prognosis, and diagnosis and treatment timing were retrospectively reviewed from the records. The timings assessed were the interval between OMS onset and NT detection, initial NT therapy, and initial OMS therapy, the interval between NT therapy and OMS remission, and duration of OMS. RESULTS A total of 73 patients with NT were treated during the study period, and 5 of 73 patients were diagnosed as having NT with OMS. The median age at onset of OMS was 22 months (range, 18-30 months). The median age at detection of NT was 29 months (range, 21-33 months). Three of five cases showed no uptake on meta-iodobenzylguanidine scintigraphy. The tumor histopathology was neuroblastoma in two patients, ganglioneuroblastoma in two patients, and ganglioneuroma in one patient. Primary resection was performed in three cases. All patients survived. Two of five cases presented with atypical neurological symptoms without opsoclonus. The initial neurological therapy was started within a mean of 20 days (range, 3-76 days) from the onset of OMS in all cases. Four patients received intravenous immunoglobulin, and one with persistent neurological problems received rituximab. Neurological symptoms resolved in three cases. The mean interval between the onset of OMS and the detection of NT in case without neurological sequelae was 57 days (range, 25-113 days), while in case with neurological sequelae it was 365 days (range, 271-458 days). The mean interval between onset of OMS and initial therapy for NT in case without neurological sequelae was 88 days (range, 47-145 days), while in case with neurological sequelae it was 389 days (range, 292-486 days). CONCLUSION The interval between the onset of OMS and the detection and initial therapy of NT tended to be longer in patients with neurological sequelae than in those without neurological sequelae. This study suggested that early detection and treatment of NT with OMS might improve the neurological outcomes.

Successful reconstruction of communicating bronchopulmonary foregut malformation associated with laryngotracheoesophageal cleft

A full-term newborn male infant presented with dyspnea and cleft lip and palate. He was thought to have esophageal atresia with tracheoesophageal fistula. He underwent bronchoscopy before operation that showed a laryngotracheoesophageal cleft (LTEC) type III. The left main bronchus originated from the lower esophagus. His diagnosis was communicating bronchopulmonary foregut malformation (CBPFM) type IA associated with LTEC type III. Enhanced chest computed tomographic scan showed the left pulmonary artery originated from the descending aorta. Staged operations were indicated. At first, reconstruction of the left pulmonary artery was done at 3 months of age. Then at 6 months of age, operations for LTEC (tracheoplasty and esophagostomy) and CBPFM left bronchoplasty were performed. Reconstruction of esophagus was performed at age of 1 year. He is now 3 years old and doing well with a mild degree of bronchomalacia. This is the first report of total reconstruction of CBPFM type IA associated with LTEC.

Ultrasound-guided hydrostatic enema for meconium obstruction in extremely low birth weight infants: a preliminary report

Meconium obstruction (MO) in extremely low birth weight (ELBW) infants is a challenging disease to treat. We performed ultrasound-guided hydrostatic enema on six ELBW infants diagnosed with MO. We consider this procedure to be safe and effective, and recommend it as a treatment for MO in ELBW infants.

Implantable central venous access device in infants: Long-term results

The management of central venous access device (CVAD) is extremely difficult in babies and small infants (BSI). We therefore compared the long‐term results and complications of CVAD in BSI with those in children.