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1.
Ann Surg ; 276(6): e969-e975, 2022 12 01.
Artículo en Inglés | MEDLINE | ID: mdl-33156070

RESUMEN

OBJECTIVE: To determine the impact of tumor characteristics and treatment approach on (1) local recurrence, (2) scoliosis development, and (3) patient-reported quality of life in children with sarcoma of the chest wall. SUMMARY OF BACKGROUND DATA: Children with chest wall sarcoma require multimodal therapy including chemotherapy, surgery, and/or radiation. Despite aggressive therapy which places them at risk for functional impairment and scoliosis, these patients are also at significant risk for local recurrence. METHODS: A multi-institutional review of 175 children (median age 13 years) with chest wall sarcoma treated at seventeen Pediatric Surgical Oncology Research Collaborative institutions between 2008 and 2017 was performed. Patient-reported quality of life was assessed prospectively using PROMIS surveys. RESULTS: The most common diagnoses were Ewing sarcoma (67%) and osteosarcoma (9%). Surgical resection was performed in 85% and radiation in 55%. A median of 2 ribs were resected (interquartile range = 1-3), and number of ribs resected did not correlate with margin status ( P = 0.36). Local recurrence occurred in 23% and margin status was the only predictive factor(HR 2.24, P = 0.039). With a median follow-up of 5 years, 13% developed scoliosis (median Cobb angle 26) and 5% required corrective spine surgery. Scoliosis was associated with posteriorrib resection (HR 8.43; P= 0.003) and increased number of ribs resected (HR 1.78; P = 0.02). Overall, patient-reported quality of life is not impaired after chest wall tumor resection. CONCLUSIONS: Local recurrence occurs in one-quarter of children with chest wall sarcoma and is independent of tumor type. Scoliosis occurs in 13% of patients, but patient-reported quality of life is excellent.


Asunto(s)
Sarcoma , Escoliosis , Oncología Quirúrgica , Neoplasias Torácicas , Pared Torácica , Niño , Humanos , Adolescente , Pared Torácica/cirugía , Pared Torácica/patología , Calidad de Vida , Estudios Retrospectivos , Neoplasias Torácicas/cirugía , Neoplasias Torácicas/patología , Sarcoma/cirugía , Sarcoma/patología
2.
J Surg Res ; 253: 115-120, 2020 09.
Artículo en Inglés | MEDLINE | ID: mdl-32353636

RESUMEN

BACKGROUND: Pleuropulmonary blastoma (PPB) is the most common primary lung cancer in children. While rare, these tumors are highly aggressive. Tumor recurrence and overall survival are dependent on histologic grade and extent of surgical resection. We sought to examine our institutional experience with PPB to determine the effect of gross total resection (GTR) on recurrence and patient outcomes. MATERIALS AND METHODS: After IRB approval, a retrospective chart review from 1998 to 2018 was performed. Cases were confirmed by histology and Dehner Grade (I to III). Data collection included demographics, treatment, extent of surgical resection, and patient outcomes. RESULTS: Eight patients with nine procedures were identified. Histologically, three cases were type 1, 2 type 2, and four poor prognosis type 3. Three patients received neoadjuvant chemotherapy to facilitate surgical resection. The operative goal was to achieve GTR (>95%), and to this end, three partial lobectomies, five lobectomies, and one pneumonectomy were performed. All nine cases achieved GTR, of which eight had negative microscopic margins. Two patients with type III disease recurred (one locally, one distant) and died. One type 3 patient had a positive microscopic hilar margin not amenable to further resection. The patient recurred (distant) but is in remission. With respect to patient outcomes, the event-free survival was 2.3 y with an overall survival of 3.3 y. CONCLUSIONS: From our experience, GTR of PPB is associated with minimal surgical morbidity and good overall survival. Multi-institutional studies are needed to determine if positive surgical margins affect outcomes given the morbidity of mediastinal dissection.


Asunto(s)
Neoplasias Pulmonares/terapia , Recurrencia Local de Neoplasia/epidemiología , Neumonectomía/métodos , Blastoma Pulmonar/terapia , Quimioterapia Adyuvante/métodos , Quimioterapia Adyuvante/estadística & datos numéricos , Preescolar , Supervivencia sin Enfermedad , Femenino , Humanos , Lactante , Recién Nacido , Pulmón/patología , Pulmón/cirugía , Neoplasias Pulmonares/mortalidad , Neoplasias Pulmonares/patología , Masculino , Márgenes de Escisión , Terapia Neoadyuvante/métodos , Terapia Neoadyuvante/estadística & datos numéricos , Clasificación del Tumor , Recurrencia Local de Neoplasia/prevención & control , Neumonectomía/estadística & datos numéricos , Pronóstico , Blastoma Pulmonar/mortalidad , Blastoma Pulmonar/patología , Estudios Retrospectivos , Factores de Tiempo
3.
J Surg Res ; 252: 63-68, 2020 08.
Artículo en Inglés | MEDLINE | ID: mdl-32234570

RESUMEN

BACKGROUND: Postoperative hypocalcemia because of hypoparathyroidism is the most common complication of total thyroidectomy in children. We hypothesized that most children with postoperative hypocalcemia would be eucalcemic by 12 mo and sought to define risk factors for permanent hypoparathyroidism. METHODS: We retrospectively reviewed children who underwent total thyroidectomy at a single children's hospital from 2012 to 2019. Patients with prior neck surgery were excluded. Indication for operation, final pathologic diagnosis, and postoperative serum calcium up to 12 mo were recorded. Permanent hypoparathyroidism was defined as supplemental calcium requirement beyond 1 y postoperatively. RESULTS: Sixty-eight patients underwent total thyroidectomy. Graves' disease was the most common benign indication for surgery (38 patients). Twenty-six patients (38%) had cancer on final pathology. Central lymph node dissection (CLND) was performed in 12 cancer patients. Twenty-eight patients (41%) had postoperative hypocalcemia. Eight patients (12%) had hypocalcemia at 6 mo. Risk factors for hypoparathyroidism at 6 mo were a cancer diagnosis (odds ratio [OR] 6.7; P = 0.02), CLND (OR 12.6; P < 0.01), and parathyroid tissue in the surgical specimen on pathologic analysis (OR 19.5; P < 0.01). Only two patients (3%) developed permanent hypoparathyroidism, both of whom had thyroidectomy for cancer and underwent CLND. CONCLUSIONS: Children with thyroid cancer are at high risk for postoperative hypocalcemia after total thyroidectomy. The risk is further increased by CLND, which should be performed selectively. A majority of patients with hypoparathyroidism at 6 mo postoperatively regain normal parathyroid function by 1 y. Permanent hypoparathyroidism in children after total thyroidectomy at a pediatric endocrine surgery center is rare.


Asunto(s)
Enfermedad de Graves/cirugía , Hipocalcemia/epidemiología , Hipoparatiroidismo/epidemiología , Complicaciones Posoperatorias/epidemiología , Neoplasias de la Tiroides/cirugía , Tiroidectomía/efectos adversos , Adolescente , Calcio/sangre , Niño , Preescolar , Femenino , Humanos , Hipocalcemia/sangre , Hipocalcemia/diagnóstico , Hipocalcemia/etiología , Hipoparatiroidismo/sangre , Hipoparatiroidismo/diagnóstico , Hipoparatiroidismo/etiología , Masculino , Glándulas Paratiroides/lesiones , Glándulas Paratiroides/patología , Hormona Paratiroidea/sangre , Complicaciones Posoperatorias/sangre , Complicaciones Posoperatorias/diagnóstico , Complicaciones Posoperatorias/etiología , Periodo Posoperatorio , Recuperación de la Función , Estudios Retrospectivos , Factores de Riesgo , Glándula Tiroides/patología , Glándula Tiroides/cirugía , Neoplasias de la Tiroides/patología , Adulto Joven
4.
J Surg Res ; 249: 8-12, 2020 05.
Artículo en Inglés | MEDLINE | ID: mdl-31918331

RESUMEN

BACKGROUND: Pelvic neuroblastomas are rare and often present in children as massive tumors whose surgical resection can be associated with significant morbidity, given sacral nerve root involvement and close proximity to pelvic vascular structures. We sought to examine the characteristics of patients with pelvic neuroblastoma and the effect of extent of surgical resection on survival and surgical outcomes. MATERIALS AND METHODS: After institutional review board approval, a retrospective chart review was performed at Children's Hospital Los Angeles from 2000 to 2018. Collected data included tumor location, size, image-defined risk factors histology, stage and risk classification, amplification of the oncogene MYCN or N-myc, use of preoperative chemotherapy, and extent of surgical resection. Outcome variables included postoperative complications and survival. RESULTS: Ten patients with primary pelvic neuroblastoma tumors were identified. The median age at diagnosis was 4.2 y (3 mo to 11 y). Four patients presented with a localized pelvic tumor (stage I or stage II) and underwent upfront tumor resection. Six patients presented with advanced disease (stage III or stage IV) and underwent neoadjuvant chemotherapy, followed by partial resection (30%-90% debulked). One patient experienced a complication: lower extremity hypotonia after tumor resection. One patient died from extensive metastatic disease for which no resection was attempted. The mean postoperative follow-up was 3.9 y with 90% overall survival. CONCLUSIONS: Our data show that patients who undergo gross total resection for localized pelvic neuroblastoma or neoadjuvant chemotherapy, followed by partial resection for advanced disease have excellent survival. We recommend that small localized pelvic neuroblastoma undergo gross total resection and large unresectable tumors undergo neoadjuvant chemotherapy, followed by partial debulking resection to avoid neurovascular morbidity.


Asunto(s)
Protocolos de Quimioterapia Combinada Antineoplásica/uso terapéutico , Procedimientos Quirúrgicos de Citorreducción/métodos , Terapia Neoadyuvante/métodos , Neuroblastoma/terapia , Neoplasias Pélvicas/terapia , Niño , Preescolar , Procedimientos Quirúrgicos de Citorreducción/efectos adversos , Femenino , Estudios de Seguimiento , Humanos , Lactante , Masculino , Estadificación de Neoplasias , Neuroblastoma/diagnóstico , Neuroblastoma/mortalidad , Neuroblastoma/patología , Neoplasias Pélvicas/diagnóstico , Neoplasias Pélvicas/mortalidad , Neoplasias Pélvicas/patología , Pelvis/irrigación sanguínea , Pelvis/diagnóstico por imagen , Pelvis/cirugía , Complicaciones Posoperatorias/epidemiología , Complicaciones Posoperatorias/etiología , Estudios Retrospectivos , Medición de Riesgo , Factores de Riesgo , Análisis de Supervivencia , Tomografía Computarizada por Rayos X , Resultado del Tratamiento
5.
J Surg Res ; 247: 380-386, 2020 03.
Artículo en Inglés | MEDLINE | ID: mdl-31753554

RESUMEN

BACKGROUND: Anterior exposures for lumbar spine surgery are increasingly common for treating various spinal pathologies. A retroperitoneal approach via a paramedian incision has grown rapidly in popularity, but little is known about the risk of incisional hernia development with this technique. We sought to assess the incidence of paramedian incisional hernia development and identify risk factors that are associated with occurrence. MATERIALS AND METHODS: We conducted a retrospective review of all patients who underwent anterior lumbar spine exposure by a paramedian approach between 2012 and 2017 at a single, tertiary medical center. The primary outcome was the development of postoperative paramedian incisional hernia. RESULTS: Of the 735 patients included in the study, 445 (60.5%) were women, and the mean (standard deviation) age of all patients was 60 y (12.4). Nearly all (97.4%) paramedian approaches were performed with a vascular surgeon present. Median follow-up time was 10 mo (interquartile range 3.5-19.9). Postoperative paramedian hernia developed in 20 patients (2.7%), of which 14 underwent repair. The mean (standard deviation) size of the hernia was 13.5 cm (5.5); 9 of 14 (64%) were repaired with synthetic mesh, whereas 3 of 14 (21%) required bowel resection. On multivariate analysis, risk factors associated with hernia development were male gender (0.045), higher American Society of Anesthesiologists class (0.039), history of abdominal surgery (P = 0.013), and postoperative intensive care unit admission (P = 0.02). CONCLUSIONS: A paramedian approach for anterior lumbar spine exposure resulted in a low rate of incisional hernia with minimal morbidity. Surgeons involved in these collaborative procedures should consider the risk factors that predispose patients to develop these hernias.


Asunto(s)
Hernia Ventral/epidemiología , Hernia Incisional/epidemiología , Vértebras Lumbares/cirugía , Fusión Vertebral/efectos adversos , Reeemplazo Total de Disco/efectos adversos , Anciano , Femenino , Estudios de Seguimiento , Hernia Ventral/etiología , Humanos , Incidencia , Hernia Incisional/etiología , Unidades de Cuidados Intensivos/estadística & datos numéricos , Masculino , Persona de Mediana Edad , Periodo Posoperatorio , Estudios Retrospectivos , Factores de Riesgo , Factores Sexuales , Fusión Vertebral/métodos , Centros de Atención Terciaria/estadística & datos numéricos , Reeemplazo Total de Disco/métodos
6.
Pediatr Surg Int ; 36(3): 317-324, 2020 Mar.
Artículo en Inglés | MEDLINE | ID: mdl-31760443

RESUMEN

PURPOSE: Intercostal nerve cryoblation during the Nuss procedure for pectus excavatum decreases pain, opiate requirement, and hospital length of stay (LOS) compared to thoracic epidural analgesia. However, long-term complications of cryoablation, including neuropathic pain development, are not well studied. METHODS: We conducted a multi-institutional retrospective review of patients following intercostal nerve cryoablation during Nuss bar insertion (11/2015-7/2018). Patients completed the Leeds Assessment of Neuropathic Symptoms and Signs, a validated questionnaire for detecting neuropathic symptoms. Primary outcome was neuropathic pain development. Secondary outcomes included duration of chest numbness and LOS. T test was performed; p < 0.05 is significant. RESULTS: 43 patients underwent intercostal cryoablation during the Nuss procedure. Ages at repair ranged 11-47 years (median 16). Patients were grouped by age: ≤ 21 years (30 patients) or older (13 patients). Mean LOS was shorter for the younger group, 2.0 versus 3.9 days (p = 0.03). No patients in the younger group, and three in the older, experienced neuropathic pain. Mean time to numbness resolution was shorter for the younger group, 3.4 versus 10.8 months (p = 0.003). CONCLUSION: In pediatric patients, intercostal cryoablation provides effective analgesia following the Nuss procedure with minimal risk of post-operative neuropathic pain. Adult patients are at greater risk of experiencing neuropathic pain and prolonged numbness.


Asunto(s)
Criocirugía/efectos adversos , Tórax en Embudo/cirugía , Neuralgia/epidemiología , Adolescente , Adulto , Analgesia Epidural/métodos , Analgésicos Opioides/uso terapéutico , Niño , Femenino , Humanos , Incidencia , Masculino , Persona de Mediana Edad , Neuralgia/etiología , Neuralgia/terapia , Manejo del Dolor , Dolor Postoperatorio/epidemiología , Dolor Postoperatorio/etiología , Dolor Postoperatorio/terapia , Estudios Retrospectivos , Estados Unidos/epidemiología , Adulto Joven
7.
Am Surg ; : 31348241248789, 2024 Apr 29.
Artículo en Inglés | MEDLINE | ID: mdl-38684325

RESUMEN

BACKGROUND: Gastrostomy creation is a common pediatric surgical procedure, but the time to initiation of feeds and to goal feeding volumes postoperatively varies greatly. Delays in reaching goal feeding volumes promote malnutrition and may prolong hospital length of stay. We hypothesized that implementing an accelerated, standardized post-gastrostomy feeding protocol would allow patients to reach goal feeding volumes sooner, without increasing postoperative complications. METHODS: We conducted a retrospective cohort study of children who underwent gastrostomy tube placement between 1/1/2022 and 11/30/2023. The feeding protocol was implemented on 11/16/2022, with patients separated into pre- and post-protocol cohorts. Abstracted data included comorbidities, time to initiation of enteral feeds, time to goal feeding volume, and postoperative complications. RESULTS: 322 patients were included: 166 pre-protocol and 156 post-protocol. The post-protocol cohort had a greater proportion of patients with gastrointestinal and/or cardiac comorbidities (P < .001). Through the protocol, postoperative enteral feeds were initiated significantly faster (5.4 hrs [IQR 43-7.7] vs 7.0 hrs [IQR 5.6-14.3]; P < .001). The post-protocol cohort also achieved goal feeding volumes sooner (12.8 hrs [IQR 9.1-25.3] vs 26.3 hrs [IQR 21.6-38.9]; P < .001). Postoperative complication rates did not differ between cohorts. Sub-analysis of children with complex cardiac conditions also demonstrated faster time to goal nutrition without an associated increase in postoperative events. DISCUSSION: These findings demonstrate that our accelerated post-gastrostomy feeding protocol was effective in achieving goal enteral nutrition earlier without increasing postoperative adverse outcomes. This protocol may be used by other centers to safely expedite time to goal enteral feeds in children postoperatively.

8.
Lab Invest ; 93(12): 1265-75, 2013 Dec.
Artículo en Inglés | MEDLINE | ID: mdl-24126890

RESUMEN

The intestinal barrier becomes compromised during systemic inflammation, leading to the entry of luminal bacteria into the host and gut origin sepsis. Pathogenesis and treatment of inflammatory gut barrier failure is an important problem in critical care. In this study, we examined the role of cyclooxygenase-2 (COX-2), a key enzyme in the production of inflammatory prostanoids, in gut barrier failure during experimental peritonitis in mice. I.p. injection of LPS or cecal ligation and puncture (CLP) increased the levels of COX-2 and its product prostaglandin E2 (PGE2) in the ileal mucosa, caused pathologic sloughing of the intestinal epithelium, increased passage of FITC-dextran and bacterial translocation across the barrier, and increased internalization of the tight junction (TJ)-associated proteins junction-associated molecule-A and zonula occludens-1. Luminal instillation of PGE2 in an isolated ileal loop increased transepithelial passage of FITC-dextran. Low doses (0.5-1 mg/kg), but not a higher dose (5 mg/kg) of the specific COX-2 inhibitor Celecoxib partially ameliorated the inflammatory gut barrier failure. These results demonstrate that high levels of COX-2-derived PGE2 seen in the mucosa during peritonitis contribute to gut barrier failure, presumably by compromising TJs. Low doses of specific COX-2 inhibitors may blunt this effect while preserving the homeostatic function of COX-2-derived prostanoids. Low doses of COX-2 inhibitors may find use as an adjunct barrier-protecting therapy in critically ill patients.


Asunto(s)
Inhibidores de la Ciclooxigenasa 2/administración & dosificación , Mucosa Intestinal/efectos de los fármacos , Peritonitis/tratamiento farmacológico , Pirazoles/administración & dosificación , Sulfonamidas/administración & dosificación , Animales , Celecoxib , Dinoprostona/metabolismo , Modelos Animales de Enfermedad , Íleon/efectos de los fármacos , Íleon/enzimología , Mucosa Intestinal/enzimología , Ratones , Ratones Endogámicos C57BL , Permeabilidad/efectos de los fármacos
9.
Ann Vasc Surg ; 27(8): 1183.e1-4, 2013 Nov.
Artículo en Inglés | MEDLINE | ID: mdl-23988540

RESUMEN

Endofibrosis of the external iliac artery is a rare cause of performance-limiting claudication in elite athletes. We describe a 47-year-old male competitive cyclist and a 52-year-old female former international triathlete, with unilateral and bilateral external artery occlusions, respectively, who presented with disabling claudication and an inability to cycle or run. Due to a long-segment occlusion, both were treated with Dacron bypass grafting. Both were able to return to competitive racing postoperatively.


Asunto(s)
Arteriopatías Oclusivas/cirugía , Implantación de Prótesis Vascular , Arteria Ilíaca/cirugía , Resistencia Física , Arteriopatías Oclusivas/diagnóstico , Arteriopatías Oclusivas/etiología , Arteriopatías Oclusivas/fisiopatología , Ciclismo , Prótesis Vascular , Implantación de Prótesis Vascular/efectos adversos , Implantación de Prótesis Vascular/instrumentación , Femenino , Fibrosis , Oclusión de Injerto Vascular/etiología , Oclusión de Injerto Vascular/cirugía , Humanos , Arteria Ilíaca/diagnóstico por imagen , Arteria Ilíaca/patología , Claudicación Intermitente/etiología , Claudicación Intermitente/cirugía , Masculino , Persona de Mediana Edad , Tereftalatos Polietilenos , Diseño de Prótesis , Recuperación de la Función , Reoperación , Carrera , Natación , Factores de Tiempo , Tomografía Computarizada por Rayos X , Resultado del Tratamiento
10.
J Laparoendosc Adv Surg Tech A ; 33(10): 994-1004, 2023 Oct.
Artículo en Inglés | MEDLINE | ID: mdl-37462727

RESUMEN

Background: Cryoanalgesia uses the application of cold temperatures to temporarily disrupt peripheral sensory nerve function for pain control. This review outlines the principles of cryoablation, clinical applications, and clinical data for its use in pediatric thoracic surgery. Methods: A comprehensive PubMed search was performed using the principal terms and combinations of cryoablation, cryoanalgesia, Nuss, Nuss repair, pectus, pectus excavatum, thoracic surgery, thoracotomy, and chest wall. Pediatric articles were reviewed and included if relevant. Adult articles were reviewed for supporting information as needed. Reference lists of included articles were reviewed for possible additional sources. Discussion: The scientific and clinical principles of cryoablation are outlined, followed by a focused review of current clinical application and outcome data. Conclusion: Postoperative pain is a major challenge following thoracic surgery. Cryoanalgesia is emerging as an adjunct in pediatric thoracic surgery, particularly for the Nuss procedure or minimally invasive repair of pectus excavatum. It effectively controls pain, decreasing postoperative opioid use and hospital length of stay with few short-term complications. Although more long-term studies are needed, early evidence suggests there is reliable return of sensation to the chest wall and long-term neuropathic pain is rare.


Asunto(s)
Criocirugía , Tórax en Embudo , Cirugía Torácica , Pared Torácica , Niño , Humanos , Criocirugía/métodos , Tórax en Embudo/cirugía , Nervios Intercostales/cirugía , Procedimientos Quirúrgicos Mínimamente Invasivos/métodos , Dolor Postoperatorio/prevención & control , Dolor Postoperatorio/cirugía
11.
Sci Rep ; 13(1): 19915, 2023 11 14.
Artículo en Inglés | MEDLINE | ID: mdl-37964011

RESUMEN

C-C motif chemokine ligand 2 (CCL2) is a monocyte chemoattractant that promotes metastatic disease and portends a poor prognosis in many cancers. To determine the potential of anti-CCL2 inhibition as a therapy for recurrent metastatic disease in neuroblastoma, a mouse model of minimal residual disease was utilized in which residual disease was treated with anti-CCL2 monoclonal antibody with etoposide. The effect of anti-CCL2 antibody on neuroblastoma cells was determined in vitro with cell proliferation, transwell migration, and 2-dimensional chemotaxis migration assays. The in vivo efficacy of anti-CCL2 antibody and etoposide against neuroblastoma was assessed following resection of primary tumors formed by two cell lines or a patient-derived xenograft (PDX) in immunodeficient NOD-scid gamma mice. In vitro, anti-CCL2 antibody did not affect cell proliferation but significantly inhibited neuroblastoma cell and monocyte migration towards an increasing CCL2 concentration gradient. Treatment of mice with anti-CCL2 antibody combined with etoposide significantly increased survival of mice after resection of primary tumors, compared to untreated mice.


Asunto(s)
Neuroblastoma , Humanos , Animales , Ratones , Etopósido/farmacología , Etopósido/uso terapéutico , Ligandos , Neoplasia Residual/tratamiento farmacológico , Ratones Endogámicos NOD , Neuroblastoma/patología , Quimiocinas , Quimiocina CCL2 , Línea Celular Tumoral
12.
J Pediatr Surg ; 56(5): 1062-1067, 2021 May.
Artículo en Inglés | MEDLINE | ID: mdl-33982660

RESUMEN

BACKGROUND/PURPOSE: Cervicofacial lymphatic malformations (CFLM) are rare, potentially life-threatening vascular anomalies, yet reports on multidisciplinary treatment strategies are lacking. We evaluated outcomes for CFLMs following sclerotherapy, surgical resection, and/or medical management. METHODS: We identified children with a CFLM at a vascular anomalies center from 2004 to 2019. EXCLUSION CRITERIA: retro-orbital malformations, untreated malformations, patients without follow-up. Primary clinical outcome was contour improvement, with significance defined as LM volume reduction of >50% by cross-sectional imaging. RESULTS: Sixty-three children met inclusion criteria: 35 with macrocystic CFLMs, six with microcystic CFLMs, and 22 with mixed-type malformations. Mean post-intervention follow-up was 27.5 months. Fifty-eight patients underwent sclerotherapy (median: two treatments). Doxycycline and/or bleomycin were used in 95% of patients. After sclerotherapy, 97% of macrocystic CFLMs improved significantly compared to 82% of mixed and 67% of microcystic lesions. Sixteen children underwent surgical resection with 75% significantly improving; two additional patients were successfully treated with sclerotherapy after debulking surgery. Six children received sirolimus for microcystic disease, of which 33% significantly improved. CONCLUSION: Sclerotherapy is very effective for macrocystic components of CFLMs, albeit less so for microcystic disease. Microcystic CFLMs frequently require surgical resection. Sirolimus is a helpful therapeutic adjunct, particularly for microcystic lesions, but more study is needed. LEVEL OF EVIDENCE: Level II, prognosis study.


Asunto(s)
Linfangioma Quístico , Anomalías Linfáticas , Bleomicina/uso terapéutico , Niño , Humanos , Lactante , Anomalías Linfáticas/tratamiento farmacológico , Estudios Retrospectivos , Soluciones Esclerosantes/uso terapéutico , Escleroterapia , Resultado del Tratamiento
13.
J Vasc Surg Venous Lymphat Disord ; 9(3): 781-784, 2021 05.
Artículo en Inglés | MEDLINE | ID: mdl-32687897

RESUMEN

We describe a 17-year-old boy with capillary malformation-arteriovenous malformation syndrome and a massive vascular malformation of the right chest wall, shoulder, and upper arm. Persistent growth of the malformation caused cutaneous ulcerations and recurrent massive bleeding episodes. We proceeded with a modified shoulder disarticulation preceded by ligation of the subclavian artery and innominate vein by median sternotomy. After a staged debulking resection of the residual chest wall arteriovenous malformation with rotational transverse rectus abdominis myocutaneous flap coverage, the patient was discharged home safely. This report demonstrates that a multidisciplinary approach is critical for management of life-threatening complications in capillary malformation-arteriovenous malformation patients.


Asunto(s)
Brazo/irrigación sanguínea , Malformaciones Arteriovenosas/terapia , Capilares/anomalías , Desarticulación , Hemorragia/terapia , Técnicas Hemostáticas , Colgajo Miocutáneo , Mancha Vino de Oporto/terapia , Hombro/irrigación sanguínea , Pared Torácica/irrigación sanguínea , Procedimientos Quirúrgicos Vasculares , Adolescente , Malformaciones Arteriovenosas/complicaciones , Malformaciones Arteriovenosas/diagnóstico , Transfusión Sanguínea , Embolización Terapéutica , Hemorragia/etiología , Humanos , Masculino , Mancha Vino de Oporto/complicaciones , Mancha Vino de Oporto/diagnóstico , Recurrencia , Resultado del Tratamiento
14.
World J Gastrointest Surg ; 12(2): 45-54, 2020 Feb 27.
Artículo en Inglés | MEDLINE | ID: mdl-32128028

RESUMEN

BACKGROUND: Sodium level is an important clinical predictor of complex biliary disease. Hyponatremia has been observed in conjunction with biliary disease, however the nature of this association remains unclear. AIM: To investigate the association between serum sodium and severe biliary disease. METHODS: Of 920 patients with gallstone disease treated at the SFVA Hospital from 1989-2019 were studied. We conducted multivariate analyses of correlation between sodium level and biliary disease severity, the presence/location of biliary bacteria, and other factors. Minimum sodium level pre-intervention was collected. Gallstones, bile, and blood (as relevant) were cultured. Illness severity was characterized: (1) None (no infectious manifestations); (2) Systemic inflammatory response syndrome; (3) Severe illness (gangrenous cholecystitis, cholangitis, necrotizing pancreatitis); and (4) Multiple organ dysfunction syndrome (bacteremia, hypotension, organ failure). Comorbidity was defined using the Charlson Comorbidity Index (CCI). RESULTS: Decreased sodium level significantly correlated with worsening illness severity, ascending bacterial infection, gangrenous changes, elevated CCI score, increasing age, male sex, and glucose. On multivariate analysis, all factors, except age, gender and glucose, independently correlated with sodium level and factors were additive. CONCLUSION: This unique study is the first to explore, with such granularity, the relationship between biliary disease and sodium. No prior studies have examined specific culture and clinical data. It illustrates an inverse, independent correlation between illness severity and sodium. Culture data demonstrate that sodium decreases as infection ascends from gallstone colonization to bactibilia to bacteremia. Patient comorbidity and gangrenous changes also independently correlate with sodium on multivariate analysis. Sodium level is an important clinical indicator of disease severity for patients with biliary disease.

15.
Semin Pediatr Surg ; 29(3): 150922, 2020 Jun.
Artículo en Inglés | MEDLINE | ID: mdl-32571507

RESUMEN

Benign thyroid disease in children represents a wide spectrum of disease. While most benign thyroid disorders may be either monitored or managed medically, surgery is an important treatment, particularly for Graves' disease or large multinodular goiters. Multidisciplinary teams including specialists in pediatric endocrinology, genetics, radiology, pathology and surgery at high volume centers offer the safest and most effective management.


Asunto(s)
Bocio/cirugía , Enfermedad de Graves/cirugía , Tiroidectomía/métodos , Niño , Terapia Combinada , Bocio/diagnóstico , Bocio/terapia , Enfermedad de Graves/diagnóstico , Enfermedad de Graves/terapia , Humanos , Selección de Paciente , Atención Perioperativa/métodos
16.
Am J Surg ; 219(6): 947-951, 2020 06.
Artículo en Inglés | MEDLINE | ID: mdl-31757439

RESUMEN

BACKGROUND: Congenital hyperinsulinism (CHI) and insulinomas are the most common causes of medically-refractory pediatric hyperinsulinism. METHODS: Children with CHI or insulinoma treated from 1/1/2014-1/1/2019 at an academic center were retrospectively analyzed. Primary outcome was persistent intravenous dextrose requirement at discharge. RESULTS: Eleven patients were identified: six with diffuse-type CHI, three with focal-type CHI, two with insulinoma. Median age at diagnosis was 20 days (1 day-16 years). Preoperative functional imaging (18F-Fluoro-l-DOPA PET-CT scan) accurately localized 66% of focal-type CHI lesions. All patients with focal-type CHI and insulinoma were cured by local resection. All patients with diffuse-type CHI underwent near-total pancreatectomy (NTP): four patients were cured of hyperinsulinism, of which 2 developed insulin-dependent diabetes, while two patients were palliated to home enteral glucose infusion. CONCLUSIONS: Localized resection cures children with focal, insulin-secreting lesions. NTP may cure diffuse-type CHI; potential complications include diabetes, exocrine insufficiency, and persistent hypoglycemia from residual hypersecreting pancreatic tissue. SUMMARY: Congenital hyperinsulinism (CHI) and insulinomas are the most common causes of medically-refractory pediatric hyperinsulinism, causing potential complications including permanent brain injury. 18F-Fluoro-l-DOPA PET-CT scan can be used to localize focal insulin-secretion lesions preoperatively. Focal-type CHI and insulinoma are cured by localized resection. Diffuse-type CHI requires near-total pancreatectomy for cure, but complications include diabetes, exocrine insufficiency, or persistent hypoglycemia from residual foci of hypersecreting pancreatic tissue.


Asunto(s)
Hiperinsulinismo Congénito/cirugía , Insulinoma/cirugía , Pancreatectomía , Neoplasias Pancreáticas/cirugía , Adolescente , Niño , Preescolar , Femenino , Humanos , Lactante , Recién Nacido , Masculino , Pancreatectomía/métodos , Estudios Retrospectivos
17.
J Immunother Cancer ; 8(2)2020 12.
Artículo en Inglés | MEDLINE | ID: mdl-33428582

RESUMEN

BACKGROUND: Immunotherapy with anti-disialoganglioside dinutuximab has improved survival for children with high-risk neuroblastoma (NB) when given after induction chemotherapy and surgery. However, disease recurrence and resistance persist. Dinutuximab efficacy has not been evaluated when initiated before primary tumor removal. Using a surgical mouse model of human NB, we examined if initiating dinutuximab plus ex vivo-activated natural killer (aNK) cells before resection of the primary tumor improves survival. METHODS: In vitro, human NB cells (SMS-KCNR-Fluc, CHLA-255-Fluc) were treated with dinutuximab and/or aNK cells and cytotoxicity was measured. In vivo, NB cells (SMS-KCNR-Fluc, CHLA-255-Fluc, or COG-N-415x PDX) were injected into the kidney of NOD-scid gamma mice. Mice received eight intravenous infusions of aNK cells plus dinutuximab beginning either 12 days before or 2 days after resection of primary tumors. Tumors in control mice were treated by resection alone or with immunotherapy alone. Disease was quantified by bioluminescent imaging and survival was monitored. aNK cell infiltration into primary tumors was quantified by flow cytometry and immunohistochemistry at varying timepoints. RESULTS: In vitro, aNK cells and dinutuximab were more cytotoxic than either treatment alone. In vivo, treatment with aNK cells plus dinutuximab prior to resection of the primary tumor was most effective in limiting metastatic disease and prolonging survival. aNK cell infiltration into xenograft tumors was observed after 1 day and peaked at 5 days following injection. CONCLUSION: Dinutuximab plus aNK cell immunotherapy initiated before resection of primary tumors decreases disease burden and prolongs survival in an experimental mouse model of NB. These findings support the clinical investigation of this treatment strategy during induction therapy in patients with high-risk NB.


Asunto(s)
Anticuerpos Monoclonales/uso terapéutico , Inmunoterapia/métodos , Células Asesinas Naturales/inmunología , Neuroblastoma/tratamiento farmacológico , Animales , Anticuerpos Monoclonales/farmacología , Humanos , Ratones , Neuroblastoma/mortalidad , Análisis de Supervivencia
18.
Semin Pediatr Surg ; 28(4): 150821, 2019 Aug.
Artículo en Inglés | MEDLINE | ID: mdl-31451177

RESUMEN

Diagnosis and management of congenital lung malformations has evolved dramatically over the past several decades. Advancement in imaging technology has enabled earlier, more definitive diagnoses and, consequently, more timely intervention in utero or after birth, when indicated. These advancements have increased overall survival rates to around 95% from historical rates of 60%. However, further refinement of diagnostic technique and standardization of treatment is needed, particularly as the increased sensitivity of diagnostic imaging results in more frequent diagnoses. In this article, we provide an updated review of the diagnostic strategies, management, and prognosis of congenital lung malformations.


Asunto(s)
Quiste Broncogénico/diagnóstico por imagen , Secuestro Broncopulmonar/diagnóstico por imagen , Malformación Adenomatoide Quística Congénita del Pulmón/diagnóstico por imagen , Enfisema Pulmonar/diagnóstico por imagen , Quiste Broncogénico/congénito , Quiste Broncogénico/terapia , Secuestro Broncopulmonar/terapia , Malformación Adenomatoide Quística Congénita del Pulmón/terapia , Humanos , Imagen por Resonancia Magnética , Atención Posnatal , Diagnóstico Prenatal , Enfisema Pulmonar/congénito , Enfisema Pulmonar/terapia , Ultrasonografía Prenatal
19.
JAMA Surg ; 154(1): 33-39, 2019 01 01.
Artículo en Inglés | MEDLINE | ID: mdl-30325977

RESUMEN

Importance: Sutureless gastroschisis repair offers an alternative to the traditional sutured method and has been associated with decreased intubation time. Published study results are inconsistent regarding the advantages of sutureless closure. Objective: To compare the clinical outcomes of sutureless and sutured gastroschisis repair. Design, Setting, and Participants: A single-center cohort review was performed of all consecutive patients (n = 97) who underwent gastroschisis repair from February 1, 2007, to April 30, 2017, at the University of California, San Francisco. Patients' medical records were evaluated for clinical characteristics and outcomes. Cases with incomplete data during initial hospitalization were excluded. Main Outcomes and Measures: Length of hospital stay, time to full enteral feeds, total parenteral nutrition duration, days requiring intravenous analgesia, days intubated, wound infection rate, antibiotic treatment duration, rate of umbilical hernias that required an operation, and readmission rate. Results: In total, 97 patients (47 [48%] were female and 50 [52%] were male with a mean [SD] age of 2.8 [2.8] days) underwent gastroschisis repair, of which 7 were excluded for incomplete medical record. Of the 90 patients included in the study, 50 (56%) underwent sutured closure and 40 (44%) underwent sutureless closure. No statistical difference was found between the sutured and sutureless groups in length of hospital stay (mean [SD] days, 43.9 [40.4] vs 36.7 [21.2]; P = .71), time to full enteral feeds (mean [SD] days, 31.4 [20.2] vs 27.9 [17.3]; P = .22), total parenteral nutrition duration (mean [SD] days, 33.5 [29.8] vs 27.4 [18.2]; P = .23), wound infection rates (14 [28%] vs 10 [25%]; P = .81), and readmission rates (5 [10%] vs 7 [18%]; P = .36). The sutureless group, compared with the sutured group, had substantially fewer days receiving antibiotics (mean [SD], 7.2 [6.4] vs 12.4 [13.2]; P = .003), fewer days intubated (mean [SD], 2.8 [3.3] vs 6.8 [1.3]; P = .001), fewer days receiving intravenous analgesia (mean [SD], 4.2 [4.0] vs 7.1 [4.5]; P = .003), and fewer patients that required silo reduction (25 [63%] vs 48 [96%]; P < .001). Sutureless closures, compared with the sutured technique, had considerably more umbilical hernias requiring surgical repair (5 [13%] vs 0; P = .02). Conclusions and Relevance: Sutureless repair of gastroschisis appears to be associated with a statistically significant reduction in mechanical ventilation duration and pain medication requirements but may increase umbilical hernia risk. Multicenter randomized clinical trials are necessary to determine the true advantages of the sutureless approach.


Asunto(s)
Gastrosquisis/cirugía , Técnicas de Sutura , Nutrición Enteral/estadística & datos numéricos , Femenino , Humanos , Recién Nacido , Tiempo de Internación/estadística & datos numéricos , Masculino , Cuidados Posoperatorios/métodos , Estudios Retrospectivos , San Francisco , Resultado del Tratamiento , Técnicas de Cierre de Heridas/estadística & datos numéricos
20.
J Pediatr Surg ; 54(11): 2250-2256, 2019 Nov.
Artículo en Inglés | MEDLINE | ID: mdl-30935731

RESUMEN

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


Asunto(s)
Analgésicos Opioides/uso terapéutico , Criocirugía , Tórax en Embudo/cirugía , Nervios Intercostales/cirugía , Tiempo de Internación/estadística & datos numéricos , Criocirugía/efectos adversos , Criocirugía/métodos , Criocirugía/estadística & datos numéricos , Humanos , Procedimientos Ortopédicos/efectos adversos , Procedimientos Ortopédicos/métodos , Procedimientos Ortopédicos/estadística & datos numéricos , Dolor Postoperatorio/tratamiento farmacológico
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