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1.
J Pediatr Surg ; 59(4): 616-620, 2024 Apr.
Artigo em Inglês | MEDLINE | ID: mdl-38161090

RESUMO

PURPOSE: To validate the effectiveness of Deflux® treatment for vesicoureteral reflux (VUR) following pediatric renal transplantation (RT), based on our single-institution experience. METHOD: A retrospective study was conducted using the medical records of pediatric patients who underwent Deflux® treatment for VUR after RT from April 2008 to March 2022. RESULTS: Sixty-eight pediatric patients underwent RT. VUR was subsequently detected in 22 (32 %) of these patients. Seven of the 22 patients (32 %) underwent Deflux® treatment to avoid renal dysfunction due to urinary infection (UTI). The median age at the time of RT was 4 years (range:2-12). All 7 patients had urinary UTIs before Deflux® treatment. The median estimated glomerular filtration rate (eGFR) before Deflux® treatment was 67 ml/min/1.73 m2 (range:42-138 ml/min/1.73 m2). After Deflux® treatment, VUR was downgraded in three cases (43 %). Four patients (57 %) experienced postoperative UTI, two of who underwent a second Deflux® treatment, one underwent submuscular tunnel reconstruction, and the other one experienced UTI without VUR after 1st Deflux® treatment but did not reoccur. All seven patients continued prophylactic medication after Deflux® treatment, without any history of recurrent UTIs during the observation period after treatment (median 37 months [range 7-86 months]). Furthermore, the eGFRs did not significantly decrease after Deflux® treatment (median eGFR 58 ml/min/1.73 m2 [range:33-99 ml/min/1.73 m2], p > 0.1). CONCLUSION: Deflux® treatment for VUR after RT is technically challenging because the new ureteral orifice is ventrally anastomosed at the bladder. We believe our results indicate the possibility of reducing the frequency of UTIs and contributing to preservation of the renal function after RT. TYPE OF STUDY: Retrospective Study. LEVEL OF EVIDENCE: Level III.


Assuntos
Transplante de Rim , Infecções Urinárias , Refluxo Vesicoureteral , Criança , Humanos , Lactente , Pré-Escolar , Refluxo Vesicoureteral/etiologia , Refluxo Vesicoureteral/cirurgia , Transplante de Rim/efeitos adversos , Estudos Retrospectivos , Dextranos/uso terapêutico , Ácido Hialurônico/uso terapêutico , Infecções Urinárias/etiologia , Infecções Urinárias/prevenção & controle , Infecções Urinárias/tratamento farmacológico
2.
Pediatr Int ; 65(1): e15666, 2023.
Artigo em Inglês | MEDLINE | ID: mdl-37888751

RESUMO

BACKGROUND: The aim of this study was to clarify the characteristics and outcomes of pediatric patients with solid pseudopapillary neoplasms (SPNs) who underwent pancreatectomy. METHODS: Pediatric patients with SPNs who underwent pancreatectomy at our institution between 1995 and 2020 were included in the study. RESULTS: During the period under review, 12 patients underwent pancreatectomy for SPNs (median age: 10 years; range: 6-15 years). The surgical procedures included pancreatoduodenectomy (n = 2; 16.6%), distal pancreatectomy (n = 3; 25%), and enucleation (n = 7; 58.3%). The most common postoperative complication was postoperative pancreatic fistula (n = 6; 50%). Patients who underwent enucleation tended to have higher postoperative complication rates compared with those who underwent other procedures. All patients were alive without recurrence at the end of the study period. CONCLUSIONS: SPN is associated with a good prognosis, regardless of the surgical procedure. If surgeons select enucleation for pediatric SPNs, they should bear in mind that it is associated with a higher complication rate.


Assuntos
Neoplasias Pancreáticas , Humanos , Criança , Neoplasias Pancreáticas/diagnóstico , Neoplasias Pancreáticas/cirurgia , Prognóstico , Pâncreas , Complicações Pós-Operatórias/epidemiologia , Complicações Pós-Operatórias/etiologia , Resultado do Tratamento , Estudos Retrospectivos
3.
Surg Case Rep ; 9(1): 191, 2023 Oct 31.
Artigo em Inglês | MEDLINE | ID: mdl-37903968

RESUMO

BACKGROUND: Sacrococcygeal teratomas (SCTs) are known to cause urological complications, but urethrovaginal (UV) fistula as a complication of SCT is rare. We herein report a case of SCT with UV fistula and hydrocolpos. CASE PRESENTATION: A 1-day-old female neonate presented to our department with prominent swelling in the sacrococcygeal region. She was born at 37 gestational weeks via spontaneous vaginal delivery from a 39-year-old woman. The weight of the baby was 2965 g, and her Apgar scores were 4/10 (at 1 and 5 min). An MRI examination confirmed an 11 × 11 cm Altman classification typeII SCT associated with hydrocolpos, a dilated urinary bladder, and bilateral hydronephrosis. When she was 5 days, the SCT was excised totally and a coccygectomy was performed. After the operation, as her urinary output appeared unstable, a cystoscopic examination was performed on the third postoperative day. This revealed that the UV fistula was located approximately 1 cm from the urethral opening. In addition, the proximal urethra was unobstructed and connected to the bladder. The cystoscope allowed for the passage of a urinary catheter through the urethra. After 1 month of catheter placement, she was discharged from the hospital at 57 days of age. Follow-up was uneventful, with neither urinary infection nor retention. CONCLUSIONS: SCTs are associated with not only trouble with rectal function and lower extremity movement but also urinary complications. The pathogenesis of this UV fistula is thought to be the rapid growth of the SCT that developed in the fetal period, resulting in obstruction of the urethra by the tumor and the pubic bone, which in turn caused urinary retention and the formation of a fistula as an escape route for the pressure. Because SCTs can cause a variety of complications depending on the course of the disease, careful examination and follow-up are necessary.

4.
J Pediatr Surg ; 58(7): 1246-1251, 2023 Jul.
Artigo em Inglês | MEDLINE | ID: mdl-36914460

RESUMO

PURPOSE: The aim of this study was to clarify the appropriate management after birth for congenital biliary dilatation (CBD, choledochal cyst) patients with a prenatal diagnosis. METHOD: Thirteen patients with a prenatal diagnosis of CBD who underwent liver biopsy during excision surgery were divided into two groups and retrospectively analyzed: group A, with liver fibrosis above F1 and group B, without liver fibrosis. RESULTS: Excision surgery was performed earlier in group A (F1-F2), at a median of 106 days old (p = 0.04). There were significant differences between the two groups in the presence symptoms and sludge, the cyst size, and the level of serum bilirubin and gamma glutamyl transpeptidase (GGT) before excision surgery (p < 0.05). Especially, in group A, prolonged serum GGT elevation and larger cysts were consistently observed from birth. The cut-off values of predictions for the presence of liver fibrosis in serum GGT and cyst size were 319 U/l and 45 mm. No significant differences were observed in the postoperative liver function or complications during the follow-up period. CONCLUSION: In patients with prenatally diagnosed CBD, the postnatal serial changes of serum GGT values and cyst size, in addition to symptoms, could help to prevent progressive liver fibrosis. LEVEL OF EVIDENCE: Ⅲ. TYPE OF STUDY: Treatment Study.


Assuntos
Cisto do Colédoco , Gravidez , Feminino , Humanos , Cisto do Colédoco/diagnóstico , Cisto do Colédoco/cirurgia , Estudos Retrospectivos , Diagnóstico Pré-Natal , Biópsia , gama-Glutamiltransferase , Cirrose Hepática
5.
J Pediatr Surg ; 58(7): 1252-1257, 2023 Jul.
Artigo em Inglês | MEDLINE | ID: mdl-36898878

RESUMO

BACKGROUND: There is no standard timing for switching to surgical management for children with adhesive small bowel obstruction (ASBO) who initially receive conservative treatment. We hypothesized that an increased gastrointestinal drainage volume may indicate the need for surgical intervention. METHODS: The study population included 150 episodes in the patients less than 20 years of age who received treatment for ASBO in our department from January 2008 to August 2019. Patients were divided into two groups: the successful conservative treatment group (CT) and the eventual surgical treatment group (ST). Following the analysis of all episodes (Study 1), we limited our analysis to only first ASBO episodes (Study 2). We retrospectively reviewed their medical records. RESULTS: There were statistically significant differences in the volume on the 2nd day in both Study 1 (9.1 ml/kg vs. 18.7 ml/kg; p < 0.01) and study 2 (8.1 ml/kg vs. 19.7 ml/kg; p < 0.01). The cut-off value was the same for both Study 1 and Study 2 (11.7 ml/kg). CONCLUSIONS: The gastrointestinal drainage volume on the 2nd day in ST was significantly larger than that in CT. Accordingly, we considered that the drainage volume may predict eventual surgical intervention for children with ASBO who initially receive conservative treatment. LEVEL OF EVIDENCE: Level IV.


Assuntos
Obstrução Intestinal , Criança , Humanos , Aderências Teciduais/complicações , Aderências Teciduais/cirurgia , Estudos Retrospectivos , Obstrução Intestinal/etiologia , Obstrução Intestinal/cirurgia , Obstrução Intestinal/patologia , Intestino Delgado/cirurgia , Intestino Delgado/patologia , Descompressão , Resultado do Tratamento
6.
DEN Open ; 3(1): e155, 2023 Apr.
Artigo em Inglês | MEDLINE | ID: mdl-35898823

RESUMO

Gastrointestinal bleeding or perforation following influenza infection is rare. We encountered a pediatric case of hemorrhagic duodenal ulcer following influenza A infection. The patient was a 1-year and 4-month-old boy who was diagnosed with influenza A infection and treated with laninamivir octanoate. After inhalation, he had diarrhea, poor appetite, and melena. The next day, he had hematochezia and developed hemorrhagic shock. Contrast-enhanced computed tomography showed extravasation in the descending part of the duodenum. Esophagogastroduodenoscopy revealed spurting bleeding from a Dieulafoy's lesion on the oral side of the major papilla, and he underwent hemostasis by clipping. From the bulb to the descending part of the duodenum, the mucosa appeared atrophic with spotty redness on the circular folds and multiple and irregularly shaped erosions. Almost all mucosal lesions had healed by the eighth day, and he was monitored as an outpatient for more than one year without re-bleeding. Intestinal ischemia, viral invasion, and drug reaction of laninamivir octanoate may be involved in duodenal mucosal injury. Acute duodenal ulcers may occur in children with influenza infection, especially young children.

7.
DEN Open ; 3(1): e128, 2023 Apr.
Artigo em Inglês | MEDLINE | ID: mdl-35898836

RESUMO

Objectives: Pyriform sinus cyst (PSC) and pyriform sinus fistula (PSF) is a rare congenital malformation that arises from the third or fourth branchial structure. In our study, we describe the safety and the utility of endoscopic electrocauterization against PSC/PSF. Methods: We retrospectively reviewed the records of patients who underwent endoscopic electrocauterization for PSC/PSF at our hospital. The internal opening of the fistula was identified under general anesthesia using a flexible endoscope (XQ-260 or H-290; Olympus, Tokyo, Japan), and the DualKnifeJ (KD-655L; Olympus) was used to ablate the internal opening. Results: We experienced three PSF and three PSC patients. The postoperative course was uneventful in all cases. The patients declared no pain in the neck, and there were no cases showing recurrent nerve paralysis. Five in six cases (83%), the closure of fistula was archived in the first cauterization. One case (16.6%) required repeated cauterization. No recurrence was found during the follow-up period ( median: 1 year) in any cases. Conclusions: Owing to its rarity in neonates, the diagnosis and treatment of PSC remains complicated and not clearly described. Complete removal of the fistula and the cyst with or without affected thyroid tissue was previously the most commonly used treatment. From our experience, we believe that endoscopic electrocauterization can be the first choice not only for PSF but also for neonatal PSC. In conclusion, endoscopic electrocauterization is feasible even for neonatal PSC. Further investigations including multicenter analyses are needed.

8.
Pediatr Surg Int ; 38(12): 1829-1838, 2022 Dec.
Artigo em Inglês | MEDLINE | ID: mdl-36169671

RESUMO

PURPOSE: This study aims to clarify the relationship between changes in skeletal muscle mass during treatment and prognosis of pediatric malignant solid tumors. METHODS: Patients with pediatric malignant solid tumors who were treated at Kyushu University Hospital from 2007 to 2017 were divided into two groups: the progression-free survival (PFS) group and the relapse/death (R/D) group; the psoas major muscle volume (PMV) was then compared. We also measured the PMV and psoas muscle area (PMA) of pediatric patients with no complications who underwent surgery for acute appendicitis (control) and compared the values with those of patients with malignant tumors. RESULTS: No significant differences were observed in the PMV and PMA between patients with appendicitis and those with malignant tumors. Significant differences were found in the rate of change in PMV between the PFS (1.424) and R/D groups (1.071) (P = 0.0024). When the cut-off value of the rate of change in the PMV was 1.20, patients whose rate of change in PMV was ≥ 1.20 had longer PFS (P = 0.0231) and overall survival (P = 0.0229) than those whose rate of change was < 1.20. CONCLUSION: Pediatric patients with malignant solid tumors and increased skeletal muscle mass during treatment have a good prognosis.


Assuntos
Apendicite , Neoplasias , Sarcopenia , Humanos , Criança , Sarcopenia/complicações , Sarcopenia/patologia , Apendicite/complicações , Estudos Retrospectivos , Prognóstico , Músculos Psoas/diagnóstico por imagem , Músculos Psoas/patologia , Neoplasias/cirurgia , Neoplasias/complicações
9.
Pediatr Surg Int ; 38(12): 1855-1860, 2022 Dec.
Artigo em Inglês | MEDLINE | ID: mdl-36136118

RESUMO

PURPOSE: The number of accessible central veins (CVs) affects the prognosis of patients with intestinal failure (IF). The loss of residual CVs should be avoided. We, therefore, evaluated the efficacy of a new CV catheter-exchange technique using a subcutaneous fibrous sheath (FS) in pediatric IF patients. METHODS: We retrospectively collected the CV catheter (CVC) data of pediatric IF patients managed from January 2009 to December 2019. The data were divided into two groups; Groups 1 (CVCs placed with the FS method) and Group 2 (CVCs placed by the primary or another insertion). The main outcome was the CVC indwelling time. RESULTS: Eighty-five CVCs were analyzed. The FS method was attempted in 47 cases and succeeded in 40 (85%). No significant difference was observed between the groups regarding characteristics. A log-rank test revealed an equivalent CVC indwelling time between the two groups (Group 1: 268 [126-588] days vs. Group 2: 229 [126-387] days, p = 0.256). CONCLUSIONS: The FS method is highly recommended for pediatric IF patients, as its attempt showed a high success rate with an indwelling time equivalent to primary insertion. The FS method leads to the prolonged use of a single CV and thereby contributes to improving the outcomes of pediatric IF patients.


Assuntos
Infecções Relacionadas a Cateter , Cateterismo Venoso Central , Cateteres Venosos Centrais , Insuficiência Intestinal , Criança , Humanos , Cateterismo Venoso Central/métodos , Estudos Retrospectivos
10.
Front Psychol ; 13: 852866, 2022.
Artigo em Inglês | MEDLINE | ID: mdl-35529561

RESUMO

Introduction: Wind instrument players sometimes suffer from erosion of the mucous membrane of the lip. This is caused by the action and pressure of the mouthpiece of the wind instrument against teeth. To address this problem, a lip shield is fitted over the dental arch to prevent direct contact between the lips and teeth. However, there are a few studies on the influence of the lip shield on the acoustics of wind instruments. The purpose of this study was to analyze the psychoacoustics of a clarinet performance with the player wearing a custom-made soft lip shield to prevent mucosal erosion of the lower lip. Case Description: A lip shield was custom-made with a soft thermoplastic material for a female clarinetist who complained of mucosal erosion and pain of the lower lip. The psychoacoustics of her musical performance played in different dynamics, fortissimo, mezzo forte and pianissimo were analyzed, including loudness and sharpness. A self-evaluation questionnaire with items rated on a 10-point scale was administered. After wearing the lip shield, the patient reported that the mucosal erosion and pain of her lower lip when playing clarinet resolved. The lip shield had little effect on the loudness. There was a slight decrease in sharpness when the lip shield was worn compared to when it was not, describing the reduction of high frequencies. Furthermore, fewer variations in sharpness between the tones were observed. Conclusion: The results suggest that lip shields made of soft materials can eliminate mucosal erosion and pain of the lower lip while having little effect on performance, although, a slight change in timbre is possible.

11.
J Laparoendosc Adv Surg Tech A ; 31(12): 1501-1506, 2021 Dec.
Artigo em Inglês | MEDLINE | ID: mdl-34748410

RESUMO

Background: We actively use novel endoscopic surgical approaches with complete curability and good cosmetic outcomes to facilitate the removal of resected tumors from the body via a small incision. Patients and Methods: This retrospective study reviewed the medical records of patients who underwent endoscopic surgery for treating solid tumors in the abdominal, thoracic, and urogenital regions between April 2013 and March 2020. Results: At our institution, minimally invasive surgery (MIS) is performed for malignant tumors with a maximum diameter of ≤5 cm and nonmalignant tumors without diameter restrictions, although both need to have no vascular encasement. In total, 135 pediatric solid tumor resections were performed at our institution during the aforementioned period, among whom 37 patients satisfied the MIS criteria. Among them, 28 patients underwent endoscopic surgeries, whereas 9 underwent open surgeries. The median surgical durations were 192 and 138 minutes in the MIS and open groups, respectively (P = .096). The median volume of blood loss was 1 and 8 mL in the MIS and open groups, respectively (P = .086). The median lengths of hospital stay were 8 and 7 days in the MIS and open groups, respectively (P = .178). One patient in each group had Clavien-Dindo grade ≥Ⅲ complications. However, there was no surgery-related death. All patients receiving MIS had satisfactory operative scarring, early recovery, and good cosmetic outcomes. Conclusion: MIS can be used for pediatric solid tumors, considering the patient's quality of life while allowing complete curability and providing endoscopic surgical advantages.


Assuntos
Neoplasias , Qualidade de Vida , Criança , Endoscopia , Humanos , Procedimentos Cirúrgicos Minimamente Invasivos , Neoplasias/cirurgia , Estudos Retrospectivos , Resultado do Tratamento
12.
Pediatr Int ; 63(9): 1095-1102, 2021 Sep.
Artigo em Inglês | MEDLINE | ID: mdl-33417724

RESUMO

BACKGROUND: Acetylcholinesterase (AChE) histochemistry has been widely performed for the histopathological diagnosis of Hirschsprung's disease (HD). However, we occasionally come across diagnostic difficulties. We conducted concurrent AChE histochemistry and hematoxylin and eosin (HE) staining to validate the ancillary value of this technique. METHODS: Of 177 patients diagnosed using AChE histochemistry from January 2014 to December 2016, 90 patients underwent formalin-fixed paraffin-embedded HE staining. The histopathological findings and diagnostic abilities were investigated and compared retrospectively. RESULTS: The sensitivity, specificity, accuracy, and kappa index of AChE histochemistry and HE staining were 94.1%, 100%, 98.9%, and 0.964 and 76.5%, 84.9%, 83.3%, and 0.530, respectively. The specificity, accuracy and kappa index of AChE histochemistry were significantly higher than those of HE staining (P < 0.001, <0.001, and <0.05). Hematoxylin and eosin staining supported the suspected diagnosis of total colon aganglionosis at the initial biopsy; furthermore, HE staining helped confirm the distinct shape of ganglion cells and hypertrophic nerve bundles. CONCLUSION: We re-confirmed that AChE histochemistry is an excellent method for diagnosing HD. Although the diagnostic ability of HE staining is limited, it has acceptable utility as an ancillary method. Thus, AChE staining is a useful test and it should be performed together with HE staining.


Assuntos
Acetilcolinesterase , Doença de Hirschsprung , Adolescente , Adulto , Idoso , Biópsia , Criança , Pré-Escolar , Amarelo de Eosina-(YS) , Feminino , Hematoxilina , Doença de Hirschsprung/diagnóstico , Humanos , Lactente , Recém-Nascido , Masculino , Pessoa de Meia-Idade , Reto , Estudos Retrospectivos , Coloração e Rotulagem , Adulto Jovem
13.
Pediatr Int ; 63(1): 88-93, 2021 Jan.
Artigo em Inglês | MEDLINE | ID: mdl-32568432

RESUMO

BACKGROUND: Interval appendectomy (IA) is a common treatment of acute appendicitis (AA) with inflammatory appendiceal mass (IAM). However, the management of patients with IAM is still controversial. The aim of this study was to assess the outcomes in patients with this condition. METHODS: We retrospectively evaluated 244 patients with AA for their clinical characteristics and outcomes. RESULTS: Forty-three patients had IAM at the first medical examination. The mean age was significantly younger and the C-reactive protein level significantly higher (12.6 vs 3.1 mg/dL) in patients with IAM. Thirty-four patients received IA, and nine received emergency appendectomy (EA). In the IA group, the diameter of the abscess was larger than in the EA group (31.4 vs 16.1 mm). The total length of hospitalization was longer in the IA group than the EA group (20.6 vs 7.0 days), although the operative time was longer in the EA group because of adhesion (101.1 vs 192.1 min). Furthermore, most IA patients received a reduced-port appendectomy (74% vs 11%). Recurrence occurred in approximately 15% of patients awaiting IA. There were no complications in either group. CONCLUSIONS: Although each treatment approach has its advantages and disadvantages, both IA and EA can be the first option for the treatment of AA with IAM.


Assuntos
Apendicectomia , Apendicite , Abscesso , Doença Aguda , Apendicite/patologia , Apendicite/cirurgia , Tratamento de Emergência , Humanos , Estudos Retrospectivos
14.
Surg Today ; 51(2): 181-186, 2021 Feb.
Artigo em Inglês | MEDLINE | ID: mdl-32577882

RESUMO

Hirschsprung's disease (HD) is a congenital disease manifesting various degrees of functional bowel obstruction caused by the absence of enteric ganglion cells, which are usually absent in the colonic segment of the HD patient. Because the aganglionic segment of HD always includes the rectum, pathological diagnosis can be made using a rectal sample. HD should be diagnosed as early as possible because serious complications, such as acute enterocolitis or toxic megacolon, can develop without a definitive diagnosis and appropriate treatment. In the mid-1900s, HD was diagnosed by HE staining of specimens obtained by full-thickness biopsy. Since then, the combination of rectal mucosal biopsy and rubeanic acid-amplificated AChE staining has been brought about by the following milestones: the discovery that the submucosal plexus and the intermuscular plexus had the same level of nerve migration; the findings of research on acetylcholine (ACh) and acetylcholinesterase (AChE) in the intestinal tract; and the establishment of a rubeanic acid amplification method. Consequently, the diagnostic rate of HD improved dramatically in the 1980s. This review outlines the history of diagnostic methods for HD, the roles of ACh and AChE in the intestine, and the method of AChE staining.


Assuntos
Acetilcolinesterase/análise , Doença de Hirschsprung/diagnóstico , Doença de Hirschsprung/patologia , Mucosa Intestinal/metabolismo , Mucosa Intestinal/patologia , Reto/metabolismo , Reto/patologia , Coloração e Rotulagem/métodos , Biomarcadores/análise , Biópsia/instrumentação , Biópsia/métodos , Humanos , Recém-Nascido , Tioamidas
15.
J Laparoendosc Adv Surg Tech A ; 30(9): 1029-1035, 2020 Sep.
Artigo em Inglês | MEDLINE | ID: mdl-32716256

RESUMO

Aim: We evaluated a series of late-presenting congenital diaphragmatic hernia (Late-CDH) cases and assessed the reliability and risks of laparoscopic and thoracoscopic approaches for Late-CDH at a single institution. Materials and Methods: From 2005 to 2017, we experienced totally 11 patients with Late-CDH who received endoscopic repairs. We retrospectively surveyed the approach, defect size, operating time, and postoperative outcomes, including recurrence. Results: Eleven patients (Bochdalek, n = 10; Morgagni, n = 1) underwent a total of 14 endoscopic repairs (laparoscopy, n = 10; thoracoscopy, n = 4). The average defect size was ∼3.1 × 1.5 cm. In all 14 endoscopic repairs, patients received intracorporeal interrupted nonabsorbable stitches and extracorporeal knot tying were applied, without the use of an artificial patch. In the laparoscopic repairs, 7 patients received left-handed suturing when closing the diaphragmatic defect, because the reduced viscera lay directly below the posterior rim of the diaphragmatic defect, making it difficult to confirm the rim. In contrast, in the thoracoscopic repairs, the viscera were reduced over the diaphragmatic defect, so the surgeons could easily perform suturing. The average operating time was 172 minutes for laparoscopy and 194 minutes for thoracoscopy. No major intraoperative or postoperative complications occurred in association with either of the approaches. Among the 11 patients, 2 experienced a total of 3 recurrences (all after laparoscopic repairs). Conclusion: Although there were few differences between the laparoscopic and thoracoscopic approaches, because of the technical difficulty of the procedure and the possibility of recurrence with the laparoscopic approach, a thoracoscopic approach may be better for the repair of Late-CDH.


Assuntos
Hérnias Diafragmáticas Congênitas/cirurgia , Laparoscopia , Técnicas de Sutura , Toracoscopia , Adolescente , Feminino , Humanos , Lactente , Laparoscopia/efeitos adversos , Laparoscopia/métodos , Masculino , Duração da Cirurgia , Complicações Pós-Operatórias/etiologia , Recidiva , Estudos Retrospectivos , Toracoscopia/efeitos adversos , Toracoscopia/métodos
16.
BMC Med Genet ; 21(1): 80, 2020 04 15.
Artigo em Inglês | MEDLINE | ID: mdl-32295525

RESUMO

BACKGROUND: Pierson syndrome (PS) is a rare autosomal recessive disorder, characterized by congenital nephrotic syndrome and microcoria. Advances in renal replacement therapies have extended the lifespan of patients, whereas the full clinical spectrum of PS in infancy and beyond remains elusive. CASE PRESENTATION: We present the case of a 12-month-old boy with PS, manifesting as the bilateral microcoria and congenital nephrotic syndrome. He was born without asphyxia, and was neurologically intact from birth through the neonatal period. Generalized muscle weakness and hypotonia were recognized from 3 months of age. The infant showed recurrent vomiting at age 5 months of age, and was diagnosed with gastroesophageal reflux and intestinal malrotation. Despite the successful surgical treatment, vomiting persisted and led to severely impaired growth. Tulobuterol treatment was effective in reducing the frequency of vomiting. Targeted sequencing confirmed that he had a compound heterozygous mutation in LAMB2 (NM_002292.3: p.Arg550X and p.Glu1507X). A search of the relevant literature identified 19 patients with severe neuro-muscular phenotypes. Among these, only 8 survived the first 12 months of life, and one had feeding difficulty with similar gastrointestinal problems. CONCLUSIONS: This report demonstrated that severe neurological deficits and gastrointestinal dysfunction may emerge in PS patients after the first few months of life.


Assuntos
Anormalidades Múltiplas/genética , Laminina/genética , Síndromes Miastênicas Congênitas/genética , Síndrome Nefrótica/genética , Distúrbios Pupilares/genética , Anormalidades Múltiplas/patologia , Trato Gastrointestinal/metabolismo , Trato Gastrointestinal/patologia , Humanos , Lactente , Masculino , Mutação , Síndromes Miastênicas Congênitas/patologia , Síndrome Nefrótica/patologia , Fenótipo , Distúrbios Pupilares/patologia
17.
Surg Case Rep ; 5(1): 167, 2019 Nov 04.
Artigo em Inglês | MEDLINE | ID: mdl-31686265

RESUMO

BACKGROUND: Esophageal hiatal hernia and gastroesophageal reflux have been recognized as inevitable complications after the definitive gastroschisis operation. Patients with refractory gastroesophageal reflux require anti-reflux surgery; however, the surgical adhesions may complicate subsequent surgical therapy, especially in the cases treated by staged repair. CASE PRESENTATION: A male infant who showed a severe gastroesophageal reflux due to hiatal hernia after staged abdominal fascial closure of gastroschisis. In spite of continuous conservative management, frequent vomiting and hematemesis had become progressively worse at the age of 8 months. Laparoscopic Nissen fundoplication was attempted and completed with no adverse events. CONCLUSIONS: Laparoscopic fundoplication may be applied, as a first-line approach, for the treatment of gastroesophageal reflux in this difficult group of patients, after the repair of congenital abdominal wall defect.

18.
J Pediatr Surg ; 54(12): 2546-2549, 2019 Dec.
Artigo em Inglês | MEDLINE | ID: mdl-31727384

RESUMO

BACKGROUND/AIM: Although the mucosectomy-commencing points on transanal endorectal pull-through (TAEPT) differ among reports, the optimal point is unclear. This study assessed the outcomes among different mucosectomy-commencing points. METHODS: We conducted a nationwide survey from 2008 to 2012. The data of 1,087 Hirschsprung's disease patients were collected, and data on those who underwent TAEPT were extracted. The patients were divided according to the mucosectomy-commencing points into two groups: in Group A, mucosectomy was started ≥5 mm from the dentate line (DL), and in Group B, mucosectomy was started <5 mm from the DL. The extent of the aganglionic segment and postoperative complications in the month after TAEPT were compared. RESULTS: The data of 327 patients were extracted (Group A, n=155; B, n=172). Aganglionosis extending to the sigmoid colon was the most frequent in both groups. Regarding postoperative complications, the patients of each group experienced enterocolitis (Group A: 8.4%; B: 7.6%) and incontinence (A: 3.9%; B: 2.9%). The incidence of rectal mucosal prolapse was significantly greater in Group B (4.1%); (A: 0%, p=0.02). CONCLUSIONS: Although the outcomes of TAEPT were comparable in both groups, rectal mucosal prolapse was significantly frequent in patients in whom the commencing point was <5 mm from the DL. TYPE OF STUDY: Retrospective study LEVEL OF EVIDENCE: Level III.


Assuntos
Doença de Hirschsprung/cirurgia , Mucosa Intestinal/cirurgia , Complicações Pós-Operatórias/etiologia , Adolescente , Adulto , Criança , Pré-Escolar , Procedimentos Cirúrgicos do Sistema Digestório/efeitos adversos , Procedimentos Cirúrgicos do Sistema Digestório/métodos , Enterocolite/etiologia , Incontinência Fecal/etiologia , Humanos , Lactente , Recém-Nascido , Japão , Pessoa de Meia-Idade , Prolapso Retal/etiologia , Estudos Retrospectivos , Inquéritos e Questionários , Adulto Jovem
19.
J Pediatr Surg ; 54(12): 2574-2578, 2019 Dec.
Artigo em Inglês | MEDLINE | ID: mdl-31575415

RESUMO

BACKGROUND: Indocyanine green (ICG) fluorescence imaging is a promising tool for intraoperative decision-making. The aim of this study was to evaluate the utility of near-infrared fluorescence cholangiography (NIR-FCG) with ICG in primary surgery for biliary atresia (BA). METHODS: We performed NIR-FCG with ICG in 10 BA patients and observed the fluorescence of their hilar micro-bile ducts and hilar exudate in order to assess the appropriate level at which to dissect the hilar fibrous corn. We compared the jaundice outcome of 10 patients using NIR-FCG (Group A) to that of 35 historical patients in whom NIR-FCG had not been used (Group B). RESULTS: The mean age of patients was 74.8 days. The classification of BA was type I in two cases and type-III in eight cases. NIR-FCG visualized the hilar micro-bile ducts, and the incidence of positive fluorescence was 80%. The ratio of postoperative normalization of hyperbilirubinemia in Group A was significantly higher than that in Group B (1.0 vs. 0.65, p < 0.05). CONCLUSION: NIR-FCG provided important objectifiable information about the biliary structures in surgery of BA. Although the number of cases was small, our results suggest that NIR-FCG may be useful for improving the outcome of primary surgery for BA. TYPE OF STUDY: Study of Diagnostic Test. LEVEL OF EVIDENCE: Level III.


Assuntos
Ductos Biliares , Atresia Biliar , Colangiografia/métodos , Verde de Indocianina/uso terapêutico , Espectroscopia de Luz Próxima ao Infravermelho/métodos , Ductos Biliares/diagnóstico por imagem , Ductos Biliares/cirurgia , Atresia Biliar/diagnóstico por imagem , Atresia Biliar/cirurgia , Feminino , Humanos , Lactente , Masculino , Imagem Óptica/métodos , Estudos Retrospectivos , Cirurgia Assistida por Computador/métodos
20.
J Laparoendosc Adv Surg Tech A ; 29(10): 1334-1341, 2019 Oct.
Artigo em Inglês | MEDLINE | ID: mdl-31313947

RESUMO

Purpose: This study aimed to evaluate the learning effects of continuous training with a disease-specific endoscopic surgical simulator for young pediatric surgeons. Materials and Methods: Participants trained with a laparoscopic fundoplication simulator for 1 hour every 10 days. At the start of each training session, we assessed the participant's surgical technique using the surgical skill evaluation system, which evaluates the following items: (1) task time, (2) right-left balance of suturing, (3) suture spacing between the three ligatures, (4) total path length traveled by forceps, (5) velocity of the forceps tips, (6) length of the wrap, (7) number of ligature failures, and (8) comparison of improvements according to assessment point. We examined the learning effects of this continuous training program. Results: Task time, right-left balance of suturing, and sum of the distance traveled by each forceps showed significant progressive improvements (P < .05). Suture spacing and average velocity of forceps tips did not change significantly with training (P = .5781, P = .0781, respectively). However, the ratio of traveled distance between left and right forceps significantly improved (P < .05). There was a significant trend for the wrap length to approach the target value (P < .05). According to the linear mixed-effects model, the number of training sessions required for learning was not uniform and varied depending on the skill. Conclusion: This simulator training program can help pediatric surgeons to acquire surgical skills easily, economically, and safely. In the future, we need to evaluate how surgical skills acquired during this continuous training are reflected in clinical operations.


Assuntos
Fundoplicatura/educação , Laparoscopia/educação , Pediatria/educação , Treinamento por Simulação/métodos , Criança , Competência Clínica/estatística & dados numéricos , Feminino , Humanos , Laparoscopia/métodos , Curva de Aprendizado , Estudos Longitudinais , Masculino
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