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2.
Cir Pediatr ; 36(1): 12-16, 2023 Jan 01.
Artigo em Inglês, Espanhol | MEDLINE | ID: mdl-36629343

RESUMO

INTRODUCTION: Hypertrophic pyloric stenosis (HPS) is a frequent pathology in neonates, with extramucosal pyloromyotomy being a healing surgery. It may be performed through a transverse subcostal incision (TSI) or a transumbilical incision (TUI). OBJECTIVE: To compare complications, operating times, hospital stay, and esthetic results between both techniques. MATERIALS AND METHODS: A retrospective, descriptive study of patients undergoing HPS surgery between January 2010 and January 2020 was carried out. Qualitative variables (sex and complications) were expressed as absolute frequency and percentage, whereas quantitative variables (age at surgery, operating time, hospital stay, and scar esthetic assessment scales: MVSS [Modified Vancouver Scar Scale] and P-SAS [Patient Scar Assessment Scale]) were expressed as median and interquartile range. RESULTS: 107 patients were analyzed: TSI (60.7%, n = 65) vs. TUI (39.3%, n = 42). Male patients: 89.2%, n = 58 vs. 83.3%, n = 35; age (days): 31 (24.5-39.5) vs. 34.5 (29.5-47.25); operating time (minutes): 41 (33.75-60) vs. 46 (38.5-60); and hospital stay (days): 2 (2-4) vs. 3 (2-3). Clavien-Dindo grade II complications were more frequent in the TUI Group (1.54%, n = 1 vs. 23.81%, n = 10; p <0.001), with most of them being wound infections. The opinion regarding the scar according to the MVSS scale was better in the TUI Group (1.5 [0-4] vs. 0 [0-2]; p = 0.022). No significant differences were found in the P-SAS scale (10 (6-18) vs. 6 (6-9); p = 0.060). CONCLUSIONS: TUI is preferred from an esthetic point of view, and even though surgical wound infections are more frequent, it is not associated with longer operating times, longer hospital stay, or severe complications.


INTRODUCCION: La estenosis hipertrófica de píloro (EHP) es una patología frecuente en neonatos donde la piloromiotomía extramucosa es una cirugía curativa. Puede realizarse a través de una incisión transversa subcostal (IT) o una incisión transumbilical (ITU). OBJETIVO: Comparar complicaciones, tiempo quirúrgico y de hospitalización y resultado estético entre ambas técnicas. MATERIAL Y METODOS: Estudio descriptivo retrospectivo en pacientes intervenidos de EHP entre enero 2010-2020. Variables cualitativas (sexo y complicaciones) expresadas mediante frecuencia absoluta y porcentaje; y cuantitativas (edad en cirugía, tiempo operatorio, días de hospitalización y escalas de estética de cicatrices: MVSS (Modified Vancouver Scar Scale) y P-SAS (Patient Scar Assessment Scale)) expresadas mediante mediana y rango intercuartílico. RESULTADOS: Se analizaron 107 pacientes: IT (60,7%, n = 65) vs. ITU (39,3%, n = 42): varones (89,2%, n = 58 vs. 83,3%, n = 35), días de vida (31 [24,5-39,5] vs. 34,5 [29,5-47,25]), tiempo quirúrgico (41 [33,75-60] vs. 46 [38,5-60] minutos) y días de hospitalización (2 [2-4] vs. 3 [2-3]). Las complicaciones Clavien-Dindo II fueron más frecuentes en el grupo ITU (1,54%, n = 1 vs. 23,81%, n = 10; p <0,001), siendo la mayoría infecciones de la herida. En el grupo ITU presentaban una mejor opinión sobre la cicatriz en la escala MVSS (1,5 [0-4] vs. 0 [0-2]; p = 0,022). La escala P-SAS no alcanzó diferencias significativas (10 [6-18] vs. 6 [6-9]; p = 0,060). CONCLUSIONES: La ITU es mejor aceptada a nivel estético y, aunque presenta más infecciones de herida quirúrgica, no precisa más tiempo quirúrgico o de ingreso, ni asocia complicaciones graves.


Assuntos
Estenose Pilórica Hipertrófica , Piloromiotomia , Recém-Nascido , Humanos , Masculino , Lactente , Estenose Pilórica Hipertrófica/cirurgia , Cicatriz , Estudos Retrospectivos , Piloromiotomia/métodos , Infecção da Ferida Cirúrgica
3.
Cir. pediátr ; 36(1): 12-16, Ene. 2023. ilus, tab
Artigo em Espanhol | IBECS | ID: ibc-214574

RESUMO

Introducción: La estenosis hipertrófica de píloro (EHP) es una patología frecuente en neonatos donde la piloromiotomía extramucosa es una cirugía curativa. Puede realizarse a través de una incisión transversa subcostal (IT) o una incisión transumbilical (ITU). Objetivo. Comparar complicaciones, tiempo quirúrgico y de hospitalización y resultado estético entre ambas técnicas. Material y métodos: Estudio descriptivo retrospectivo en pacientes intervenidos de EHP entre enero 2010-2020. Variables cualitativas (sexo y complicaciones) expresadas mediante frecuencia absoluta y porcentaje; y cuantitativas (edad en cirugía, tiempo operatorio, días de hospitalización y escalas de estética de cicatrices: MVSS [Modified Vancouver Scar Scale] y P-SAS [Patient Scar Assessment Scale]) expresadas mediante mediana y rango intercuartílico. Resultados:S e analizaron 107 pacientes: IT (60,7%, n = 65) vs. ITU (39,3%, n = 42): varones (89,2%, n = 58 vs. 83,3%, n = 35), días de vida (31 [24,5-39,5] vs. 34,5 [29,5-47,25]), tiempo quirúrgico (41 [33,75-60] vs. 46 [38,5-60] minutos) y días de hospitalización (2 [2-4] vs. 3 [2-3]). Las complicaciones Clavien-Dindo II fueron más frecuentes en el grupo ITU (1,54%, n = 1 vs. 23,81%, n = 10; p < 0,001), siendo la mayoría infecciones de la herida. En el grupo ITU presentaban una mejor opinión sobre la cicatriz en la escala MVSS (1,5 [0-4] vs. 0 [0-2]; p = 0,022). La escala P-SAS no alcanzó diferencias significativas (10 [6-18] vs. 6 [6-9]; p = 0,060). Conclusiones: La ITU es mejor aceptada a nivel estético y, aunque presenta más infecciones de herida quirúrgica, no precisa más tiempo quirúrgico o de ingreso, ni asocia complicaciones graves.(AU)


Introduction: Hypertrophic pyloric stenosis (HPS) is a frequent pathology in neonates, with extramucosal pyloromyotomy being a healing surgery. It may be performed through a transverse subcostal incision (TSI) or a transumbilical incision (TUI). Objective: To compare complications, operating times, hospital stay, and esthetic results between both techniques. Materials and methods: A retrospective, descriptive study of patients undergoing HPS surgery between January 2010 and January 2020 was carried out. Qualitative variables (sex and complications) were expressed as absolute frequency and percentage, whereas quantitative variables (age at surgery, operating time, hospital stay, and scar esthetic assessment scales: MVSS [Modified Vancouver Scar Scale] and P-SAS [Patient Scar Assessment Scale]) were expressed as median and interquartile range. Results: 107 patients were analyzed: TSI (60.7%, n = 65) vs. TUI (39.3%, n = 42). Male patients: 89.2%, n = 58 vs. 83.3%, n = 35; age (days): 31 (24.5-39.5) vs. 34.5 (29.5-47.25); operating time (minutes): 41 (33.75-60) vs. 46 (38.5-60); and hospital stay (days): 2 (2-4) vs. 3 (2-3). Clavien-Dindo grade II complications were more frequent in the TUI Group (1.54%, n = 1 vs. 23.81%, n = 10; p < 0.001), with most of them being wound infections. The opinion regarding the scar according to the MVSS scale was better in the TUI Group (1.5 [0-4] vs. 0 [0-2]; p = 0.022). No significant differences were found in the P-SAS scale(10 (6-18) vs. 6 (6-9); p = 0.060). Conclusions: TUI is preferred from an esthetic point of view, and even though surgical wound infections are more frequent, it is not associated with longer operating times, longer hospital stay, or severe complications.(AU)


Assuntos
Humanos , Masculino , Feminino , Recém-Nascido , Estenose Pilórica Hipertrófica , Piloromiotomia , Cirurgia Geral , Ferida Cirúrgica , Epidemiologia Descritiva , Estudos Retrospectivos
4.
Neurogastroenterol Motil ; 35(2): e14529, 2023 02.
Artigo em Inglês | MEDLINE | ID: mdl-36594414

RESUMO

BACKGROUND: The pylorus plays a key role in the control of gastric content outflow. Impairment of pyloric physiology has been observed in gastroparesis, particularly when associated with diabetes mellitus or opioid intake or after antireflux surgery. New tools have been developed to identify pyloric dysfunction in routine care, including functional luminal impedance planimetry (FLIP). As such, a new therapeutic strategy targeting the pylorus, namely endoscopic pyloromyotomy (G-POEM), has received increasing attention and emerged as a promising treatment for gastroparesis. PURPOSE: The present review details the involvement of the pyloric pathophysiology in gastroparesis, as well as clinical results of G-POEM according to the current literature.


Assuntos
Gastroparesia , Piloromiotomia , Humanos , Piloro/cirurgia , Piloromiotomia/métodos , Gastroparesia/cirurgia , Resultado do Tratamento , Gastroscopia/métodos , Esvaziamento Gástrico
5.
J Surg Res ; 285: 142-149, 2023 05.
Artigo em Inglês | MEDLINE | ID: mdl-36669393

RESUMO

INTRODUCTION: In order to define optimal resources and outcome standards for infant pyloromyotomy, we sought to perform a contemporary analysis of surgical approach (laparoscopic versus open) and outcomes. METHODS: The National Surgical Quality Improvement Project Pediatrics Participant Use File (NSQIP PUF) was queried from 2016 to 2020. Utilization of laparoscopy was trended over time. Complication rates and length of stay were compared by operative approach. RESULTS: 9752 pyloromyotomies were included in the analysis. The utilization of laparoscopy steadily increased over the study time period (66% to 79%) and was associated with a shorter operative time. On multivariate regression, the utilization of laparoscopy was associated with a lower risk of overall complications, length of stay, and superficial surgical site infections. Overall complication rates were lower than previously reported (2.02%). The most common complication was superficial infection (1.2%). CONCLUSIONS: In facilities reporting to pediatric National Quality Improvement Project, utilization of laparoscopy has steadily increased, and complication rates are lower than previously reported. Complication rates and length of stay were lower with the laparoscopic approach in this contemporary cohort. These results offer benchmarks for quality improvement initiatives. The laparoscopic approach should be standard in facilities performing this procedure.


Assuntos
Laparoscopia , Estenose Pilórica Hipertrófica , Piloromiotomia , Lactente , Humanos , Criança , Estenose Pilórica Hipertrófica/cirurgia , Piloro/cirurgia , Piloromiotomia/efeitos adversos , Laparoscopia/efeitos adversos , Duração da Cirurgia , Tempo de Internação , Complicações Pós-Operatórias/etiologia
6.
J Laparoendosc Adv Surg Tech A ; 32(11): 1134-1143, 2022 Nov.
Artigo em Inglês | MEDLINE | ID: mdl-35939274

RESUMO

Gastric emptying delay may be caused with both functional and anatomic derangements. Gastroparesis is suspected in patients presenting with certain foregut symptoms without anatomic obstruction. Data are still emerging regarding the best treatment of this condition. In cases where large paraesophageal hernias alter the upper gastrointestinal anatomy, it is difficult to know if gastroparesis also exists. Management of hiatal hernias is also still evolving, with various strategies to reduce recurrence being actively investigated. In this article, we present a systematic review of the existing literature around the management of gastroparesis and the management of paraesophageal hernias when they occur separately. In addition, since there are limited data to guide diagnosis and management of these conditions when they are suspected to coexist, we provide a rational strategy based on our own experience in patients with paraesophageal hernias who have symptoms or studies that raise suspicion for a coexisting functional disorder.


Assuntos
Toxinas Botulínicas Tipo A , Gastroparesia , Hérnia Hiatal , Piloromiotomia , Humanos , Piloromiotomia/efeitos adversos , Esvaziamento Gástrico , Hérnia Hiatal/complicações , Hérnia Hiatal/cirurgia , Gastroparesia/etiologia , Gastroparesia/cirurgia , Resultado do Tratamento , Estudos Retrospectivos
7.
Surg Endosc ; 36(12): 9254-9261, 2022 Dec.
Artigo em Inglês | MEDLINE | ID: mdl-35851820

RESUMO

BACKGROUND AND AIMS: G-POEM is an emerging method for treatment of severe gastroparesis. Safe mucosal closure is necessary to avoid adverse events. The aim of this study was to compare the efficacy of two closure methods: clips and endoscopic suturing (ES) after G-POEM. METHODS: We performed a single center, prospective study. The closure method was assigned at the discretion of an endoscopist prior to the procedure. The main outcome was the proportion of subjects with successful closure. Unsuccessful closure was defined as a need for a rescue method, or a need for an additional intervention or incomplete closure-related adverse events. Secondary outcomes were the easiness of closure (VAS score 1 = very difficult, 10 = easy), closure time, and cost. RESULTS: A total of 40 patients [21 female; mean age, range 47.5; (20-74)] were included; 20 received ES and 20 clips [mean number of clips 6; range (4-19)]. All 20 patients with ES (100%, 95% CI 84-100%) and 18 patients with clips (89%, 95% CI 70-97%) had successful closure (p = 0.49). One patient needed a rescue method (KING closure) and the other patient an additional clipping on POD1. Closure with clips was quicker [mean time 9.8 (range 4-20) min vs. 14.1 (5-21) min; p = 0.007] and cheaper [mean cost 807 USD (± 402) vs. 2353 USD (± 145); p < 0.001]. Endoscopist assessed the easiness of ES and clips as comparable [mean VAS, range 7.5 (3-10) (ES) vs. 6.9 (3-10) (clips); p = 0.3]. CONCLUSIONS: Both ES and clips are effective methods for mucosal closure in patients undergoing G-POEM. However, centres using clips should have a rescue closure method available as clips may fail in some patients. Closure with ES is more costly than with clips.


Assuntos
Acalasia Esofágica , Gastroparesia , Piloromiotomia , Humanos , Feminino , Piloromiotomia/métodos , Estudos Prospectivos , Gastroparesia/cirurgia , Endoscopia , Instrumentos Cirúrgicos , Resultado do Tratamento , Acalasia Esofágica/cirurgia
9.
Chin Med J (Engl) ; 135(12): 1432-1437, 2022 Jun 20.
Artigo em Inglês | MEDLINE | ID: mdl-35866346

RESUMO

BACKGROUND: Gastroparesis is a debilitating medical condition with limited treatment options. Gastric per-oral pyloromyotomy (G-POEM) has emerged as a promising treatment option with remarkable short-term clinical success shown in multiple studies. While the post-procedure protocol is not standardized across many centers, the majority of the centers observes these patients in the hospital after the procedure for monitoring. In this single-center prospective study, we evaluated the safety and feasibility of same day discharge after the G-POEM procedure. METHODS: All the patients with refractory gastroparesis undergoing G-POEM from October 2019 to March 2020 were enrolled. A total of 25 patients were enrolled in the procedure. Based on the pre-defined criteria, patients were either discharged on the same day after the procedure or admitted to the hospital for further observation. The patient and procedure-related data were extracted from the chart review. Univariate analysis was performed (chi-squared test) on categorical variables after organizing categorical variables as numeric counts or percentages. The student t test was performed on continuous variables after reporting as mean and standard deviation. For analysis with a smaller sample size, Fisher exact and Mann-Whitney tests were used. RESULTS: A total of 25 patients were enrolled. The technical success of G-POEM was 100% and clinical success was 80% (20/25) at 1-month follow-up. Of the 25 patients, 9 patients (36%) were discharged on the same day according to the procedure from the recovery unit. Of the remaining 16 patients who were admitted to the hospital post-procedure, 10 (40%) were admitted due to procedure-related causes while other admissions were either pre-planned or due to social reasons. The average Charlson comorbidity index was lower in the same day discharge group ( P   <  0.05). The number of patients requiring double myotomy was higher in the same day discharge group ( P  < 0.05). The overall complication rate of G-POEM in the study cohort was 12% (3/25) with all complications being mild without any severe adverse events. CONCLUSION: G-POEM is a safe and effective method of treatment for refractory GP with higher clinical success in short-term follow-up. The same day discharge after G-POEM is safe and feasible in >50% of patients with close periprocedural monitoring.


Assuntos
Gastroparesia , Piloromiotomia , Estudos de Viabilidade , Esvaziamento Gástrico , Gastroparesia/cirurgia , Gastroscopia/métodos , Humanos , Alta do Paciente , Projetos Piloto , Estudos Prospectivos , Piloromiotomia/efeitos adversos , Piloromiotomia/métodos , Resultado do Tratamento
10.
BMC Surg ; 22(1): 218, 2022 Jun 07.
Artigo em Inglês | MEDLINE | ID: mdl-35672844

RESUMO

PURPOSE: A new novel technique for the treatment of pediatric hypertrophic pyloric stenosis (HPS), transumbilical single-site laparoscopic pyloromyotomy with a single instrument (TUSSLP), was introduced. TUSSLP was compared with the transabdominal three-site laparoscopic pyloromyotomy (TATSLP) procedure. METHODS: Patients with HPS who underwent TUSSLP and TATSLP between January 2016 and September 2020 were assigned to group A and group B, respectively. The descriptive variables, perioperative clinical characteristics and postoperative follow-up results were retrospectively analyzed and compared between the 2 groups. The primary outcome of this study was the rate of switching to conventional pyloromyotomy. RESULTS: Sixty-four patients were enrolled in this study. Of these patients, 29 (22 males, 7 females, 54.4 ± 22.6 days) who received TUSSLP were assigned to group A. The remaining 35 (28 males, 7 females, 54.5 ± 27.6 days) who received TATSLP were assigned to group B. The data of preoperative patient variables were comparable between the 2 groups (P > 0.05). The mean operative time (ORT) was 28.1 ± 5.6 min in group A, which was not significantly different from 25.8 ± 3.1 min in group B (P = 0.25). The other perioperative features were not significantly different between the 2 groups (P > 0.05). During follow-up (39.1 ± 14.7 m in group A and 35.4 ± 16.1 m in group B, P = 0.51), no significant difference was observed in the overall incidence of vomiting between the 2 groups (P = 0.26). CONCLUSIONS: TUSSLP is a feasible and reliable minimally invasive method for HPS. It has the advantages of an improved cosmetic appearance. The postoperative follow-up results of TUSSLP are comparable with those of TATSLP.


Assuntos
Laparoscopia , Estenose Pilórica Hipertrófica , Piloromiotomia , Criança , Feminino , Humanos , Lactente , Laparoscopia/métodos , Masculino , Estenose Pilórica Hipertrófica/cirurgia , Piloromiotomia/métodos , Piloro/cirurgia , Estudos Retrospectivos
11.
Gut Liver ; 16(6): 825-839, 2022 11 15.
Artigo em Inglês | MEDLINE | ID: mdl-35670120

RESUMO

Gastroparesis, once regarded as a rare disease, is difficult to diagnose and challenging to treat; there were many breakthrough advances in the 2010s, shifting the paradigm of the understanding of this complex entity and its management. Similar to diabetes, its increasing prevalence reflects increased accessibility to diagnostic modalities and suggests that gastroparesis was underacknowledged in the past. Major developments in the three main aspects of the disease include the discovery of smooth muscle cells, interstitial cells of Cajal, PDGFRα+ cells syncytium, rather than interstitial cells of Cajal alone, as the main gastric pacemaker unit; the development of validated point-of-care diagnostic modalities such as a wireless motility capsule, the carbon 13-labeled breath test, and impedance planimetry; and the introduction of novel minimally invasive therapeutic options such as newer pharmacologic agents and gastric peroral endoscopic pyloromyotomy. All aspects of these advances will be discussed further in this review.


Assuntos
Acalasia Esofágica , Gastroparesia , Piloromiotomia , Humanos , Gastroparesia/diagnóstico , Gastroparesia/etiologia , Gastroparesia/terapia , Acalasia Esofágica/cirurgia , Esfíncter Esofágico Inferior , Testes Respiratórios , Resultado do Tratamento
13.
J Pediatr Surg ; 57(11): 736-739, 2022 Nov.
Artigo em Inglês | MEDLINE | ID: mdl-35577606

RESUMO

PURPOSE: Pyloromyotomy for pyloric stenosis is one of the more common surgical procedures performed on infants. The long-term effects of the procedure are however unclear. The purpose of this study was to study the effects into adult life, compare them with controls and to see if there is a need for structured follow up of patients. METHODS: Of the 101 patients operated for pyloric stenosis between 1972 and 1974 at our tertiary referral center 91 could be traced. They were all invited to participate in the study and were sent validated questionnaires (PAGI-SYM, GIQLI) as well as a study-specific questionnaire examining the use of antacid drugs, incidence of gastroscopy and abdominal surgery. Sixty patients responded (66%, mean age 45 years, 46 male) and were included. Thereafter, 600 age and sex-matched controls were sent the same questionnaires. 132 responded (22%, 90 male) and were includes as controls. RESULTS: No significant differences could be found in any of the examined parameters when looking at the whole material or the male patients. Female patients had higher PAGI-SYM-scores for post prandial fullness (mean 1.11 vs 0.43, P = 0.035) and heartburn (mean 0.59 vs 0.14, P = 0.043) when compared to controls. CONCLUSIONS: The present study shows that most patients operated for pyloric stenosis during infancy experience no negative effects into adulthood. The finding in the female patient group is interesting but is unlikely to have any clinical implications. The results from this study strongly implicate that there is no need for follow up of patients into adulthood. LEVEL OF EVIDENCE: Level III.


Assuntos
Estenose Pilórica Hipertrófica , Estenose Pilórica , Piloromiotomia , Adulto , Antiácidos , Feminino , Humanos , Incidência , Lactente , Masculino , Pessoa de Meia-Idade , Estenose Pilórica/etiologia , Estenose Pilórica/cirurgia , Estenose Pilórica Hipertrófica/cirurgia , Piloromiotomia/efeitos adversos , Piloromiotomia/métodos , Piloro/cirurgia , Inquéritos e Questionários
14.
J Gastrointest Surg ; 26(8): 1585-1595, 2022 08.
Artigo em Inglês | MEDLINE | ID: mdl-35585422

RESUMO

INTRODUCTION: The incidence of adenocarcinoma of esophageal-gastric junction (AEJ) has been increasing in recent years. Esophagogastrostomy after proximal gastrectomy (PG-EG) is the most commonly used surgical method for this disease which causes a constant spasm of the pyloric sphincter by cutting the vagus nerve around the esophagus, so H-M pyloroplasty (Heineke-Mikulicz pyloroplasty) is often operated after PG-EG to prevent delayed gastric emptying. However, H-M pyloroplasty destroys anti-reflux structure of pylorus and leads to serious bile reflux. The present study was designed to compare pyloromyotomy and H-M pyloroplasty in proximal subtotal gastrectomy through clinical studies and animal experiments. METHODS: We retrospectively evaluated the outcomes of 73 AEJ patients (39 underwent PG-EG with an H-M pyloroplasty and 34 underwent PG-EG with a pyloromyotomy) between January 2016 and August 2020, and perioperative variables were compared. In the animal experiment, 48 rats were randomly divided into four groups (n = 12): vagotomy group (V group), H-M pyloroplasty group (HM group), pyloromyotomy group (PM group), and control group (O group). Gastric emptying and bile reflux were evaluated in each group. RESULTS: In the retrospective clinic study, pyloromyotomy and H-M pyloroplasty could all prevent delayed gastric emptying effectively, and the incidence of bile reflux found by electronic gastroscopy in the PM group was significantly lower than that in the HM group (HM, 14/39; PM, 4/34; P = 0.028). In the animal experiment, there was no significant between-group difference of gastric emptying rate (%) in the HM group and PM group (HM, 70.6 ± 16; PM, 72.3 ± 12; P = 0.68) while the gastric emptying rate (%) was significantly lower in the V group than in the HM, PM, and control group (P values were 0.037, 0.021, and 0.001 respectively). The gastric mucosa bile acid concentration was significantly higher in the HM group than other group (P values were all less than 0.001). CONCLUSIONS: The pyloromyotomy could prevent delayed gastric emptying effectively after PG-EG for types II and III AEJ and reduce bile reflux compared to Heineke-Mikulicz pyloroplasty.


Assuntos
Adenocarcinoma , Refluxo Biliar , Gastroparesia , Piloromiotomia , Adenocarcinoma/cirurgia , Animais , Esôfago , Gastrectomia , Esvaziamento Gástrico , Gastroparesia/cirurgia , Piloro/cirurgia , Ratos , Estudos Retrospectivos
15.
J Pediatr Hematol Oncol ; 44(7): 409-411, 2022 10 01.
Artigo em Inglês | MEDLINE | ID: mdl-35398868

RESUMO

Hereditary fructose intolerance is a rare autosomal recessive metabolic disorder characterized by liver failure, renal tubulopathy, growth retardation, and occasionally death upon exposure to fructose. We present a 2-month-old male infant diagnosed with pyloric stenosis who developed disseminated intravascular coagulopathy following pyloromyotomy. Unexplained persistent coagulopathy, acute liver failure, and metabolic dysfunction led to whole-exome sequencing, which revealed compound heterozygous variants in ALDOB (p.Arg60Ter and p.Ala150Pro), diagnostic of hereditary fructose intolerance. Shortly after initiating a fructose-free diet, our patient had resolution of his coagulopathy, hepatic, and metabolic dysfunction.


Assuntos
Intolerância à Frutose , Piloromiotomia , Dieta , Intolerância à Frutose/diagnóstico , Humanos , Lactente , Fígado , Masculino
16.
ANZ J Surg ; 92(5): 1153-1158, 2022 05.
Artigo em Inglês | MEDLINE | ID: mdl-35393697

RESUMO

BACKGROUND: Our aims were to determine if the diagnostic threshold for diagnosing hypertrophic pyloric stenosis (HPS) on ultrasound scan (USS) should be adjusted based on birth weight (BW), current weight (CW), gestational age (GA), chronological age (CA) or corrected gestational age (CGA). METHODS: All patients who underwent either an USS and pyloromyotomy (Group 1) or an USS for possible HPS (Group 2) at our tertiary centre between July 2013 and June 2019 were identified. Ideal threshold values are identified by measuring Youden's Index (J = sensitivity + specificity - 1; higher is better). Mean maximum Youden's Index for stratified results was compared to that for combined results. RESULTS: Two hundred and eighty-four patients were included (142 patients in both Group 1 and Group 2). Combined maximum Youden's Index for all patients was 0.92 for pyloric canal thickness (PMT) and 0.87 for pyloric canal length (PCL). Mean maximum Youden's Index was higher when patients were stratified by GA, CGA, BW or CW, and equivalent for CA. For pyloric canal length (PCL), mean maximum Youden's Index was lower for all variables when stratified compared to combined. There was no visual trend observed in the diagnostic thresholds between groups. CONCLUSION: Stratifying USS PMT diagnostic thresholds values based on age and weight is statistically more accurate than a single threshold in diagnosing HPS. However, the lack of visual correlation indicates a larger dataset is required to validate these results.


Assuntos
Estenose Pilórica Hipertrófica , Piloromiotomia , Constrição Patológica , Humanos , Lactente , Estenose Pilórica Hipertrófica/diagnóstico por imagem , Estenose Pilórica Hipertrófica/cirurgia , Piloro/diagnóstico por imagem , Ultrassonografia
17.
Gut ; 71(11): 2170-2178, 2022 11.
Artigo em Inglês | MEDLINE | ID: mdl-35470243

RESUMO

OBJECTIVE: Endoscopic pyloromyotomy (G-POEM) is a minimally invasive treatment option with promising uncontrolled outcome results in patients with gastroparesis. DESIGN: In this prospective randomised trial, we compared G-POEM with a sham procedure in patients with severe gastroparesis. The primary outcome was the proportion of patients with treatment success (defined as a decrease in the Gastroparesis Cardinal Symptom Index (GCSI) by at least 50%) at 6 months. Patients randomised to the sham group with persistent symptoms were offered cross-over G-POEM. RESULTS: The enrolment was stopped after the interim analysis by the Data and Safety Monitoring Board prior to reaching the planned sample of 86 patients. A total of 41 patients (17 diabetic, 13 postsurgical, 11 idiopathic; 46% male) were randomised (21 G-POEM, 20-sham). Treatment success rate was 71% (95% CI 50 to 86) after G-POEM versus 22% (8-47) after sham (p=0.005). Treatment success in patients with diabetic, postsurgical and idiopathic gastroparesis was 89% (95% CI 56 to 98), 50% (18-82) and 67% (30-90) after G-POEM; the corresponding rates in the sham group were 17% (3-57), 29% (7-67) and 20% (3-67).Median gastric retention at 4 hours decreased from 22% (95% CI 17 to 31) to 12% (5-22) after G-POEM and did not change after sham: 26% (18-39) versus 24% (11-35). Twelve patients crossed over to G-POEM with 9 of them (75%) achieving treatment success. CONCLUSION: In severe gastroparesis, G-POEM is superior to a sham procedure for improving both symptoms and gastric emptying 6 months after the procedure. These results are not entirely conclusive in patients with idiopathic and postsurgical aetiologies. TRIAL REGISTRATION NUMBER: NCT03356067; ClinicalTrials.gov.


Assuntos
Gastroparesia , Piloromiotomia , Feminino , Esvaziamento Gástrico , Gastroparesia/etiologia , Gastroparesia/cirurgia , Gastroscopia/métodos , Humanos , Masculino , Estudos Prospectivos , Piloromiotomia/efeitos adversos , Piloromiotomia/métodos , Resultado do Tratamento
18.
Gastrointest Endosc ; 96(3): 500-508.e2, 2022 09.
Artigo em Inglês | MEDLINE | ID: mdl-35413333

RESUMO

BACKGROUND AND AIMS: Limited data exist concerning the long-term efficiency of gastric peroral endoscopic myotomy (G-POEM) as a treatment of refractory gastroparesis. This study evaluated the 3-year results of G-POEM in patients with refractory gastroparesis. METHODS: This was a prospective multicenter study of all G-POEM operations performed in 2 expert French centers for 46 patients with refractory gastroparesis with at least 3 years of follow-up. RESULTS: Clinical success was 65.2% at 36 months. There was significant improvement in symptom severity. Median Gastroparesis Cardinal Symptom Index decreased from 3.33 to 1.80 (P < .0001), with improvement in all subscales. We created a predictive score concerning G-POEM success (G-POEM predictive score) to which points were assigned as follows: nausea subscale <2: predictive of success, 1 point; satiety subscale >4: predictive of success, 1 point; bloating subscale >3.5: predictive of success, 1 point; percentage of gastric retention at 4 hours on scintigraphy >50%: 1 point. A threshold of 2 was identified by receiver operating characteristic curve analysis with an area under the curve of .825 that predicted clinical success with a sensitivity of 93.3% (95% confidence interval [CI], .77-.99), specificity of 56.3% (95% CI, .33-.77), positive predictive value of 80% (95% CI, .67-.93), negative predictive value of 81.8% (95% CI, .59-1.00), and accuracy of 80.4% (95% CI, .69-.92). Patients with a score ≥2 were significantly more likely to be responders at 3 years than were patients with a score <2 (80% and 18%, respectively; P = .0004). CONCLUSIONS: The clinical success of G-POEM for refractory gastroparesis was 65.2% at 36 months. Our predictive score offers an easy tool that needs to be confirmed in other studies.


Assuntos
Acalasia Esofágica , Gastroparesia , Piloromiotomia , Acalasia Esofágica/cirurgia , Esfíncter Esofágico Inferior , Esvaziamento Gástrico , Gastroparesia/etiologia , Gastroparesia/cirurgia , Humanos , Seleção de Pacientes , Estudos Prospectivos , Piloromiotomia/métodos , Resultado do Tratamento
19.
Surg Endosc ; 36(11): 8012-8020, 2022 Nov.
Artigo em Inglês | MEDLINE | ID: mdl-35437639

RESUMO

BACKGROUND AND AIMS: There has been interest in the use of pyloric therapies for the treatment of refractory gastroparesis. However, data on endoscopic pyloric dilation are scarce. We aimed to assess the efficacy and safety of this procedure in refractory gastroparesis. METHODS: We performed a retrospective analysis of 47 patients referred for refractory gastroparesis, confirmed by gastric emptying scintigraphy, and treated with endoscopic pyloric through-the-scope balloon dilation. The primary endpoint was the effectiveness of the procedure, evaluated with the Gastric Cardinal Symptom Index (GCSI) at 2 and 6 months. RESULTS: A clinical response, defined by a 1.0 point decrease in the GCSI score, was observed in 25 patients at 2 months (53%) and in 19 patients at 6 months (40%). The mean GCSI score decreased significantly at 2 and 6 months compared to the preoperative score (3.9 ± 0.87 vs 2.3 ± 1.37 and 3.9 ± 0.87 vs 2.9 ± 1.27, respectively; p < 0.0001). No complication was observed. Nine patients had a delayed relapse at 1 year. A second dilation was performed for eight patients and it was effective in five of them (63%). The mean follow-up time of the patients was 27.0 ± 10.4 months. At 2 years, 15 patients still experienced improvement following this treatment (32%). No predictive factor of clinical response was identified. CONCLUSION: The efficacy of pyloric dilation is 53% at 2 months, with sustained improvement in one third of patients at 2 years. This treatment should be considered as an alternative option to pyloromyotomy.


Assuntos
Gastroparesia , Piloromiotomia , Humanos , Gastroparesia/etiologia , Gastroparesia/cirurgia , Estudos Retrospectivos , Dilatação , Resultado do Tratamento , Piloromiotomia/efeitos adversos , Piloromiotomia/métodos , Esvaziamento Gástrico
20.
Endoscopy ; 54(4): v14, 2022 04.
Artigo em Inglês | MEDLINE | ID: mdl-35350078
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