Bronchogenic Cyst: Lessons Learned in 20 Years of Experience at a Tertiary Pediatric Center.

BACKGROUND: Bronchogenic cysts are benign congenital malformations of the primitive ventral foregut. The aim of this study is to analyze and report 20 years of experience in the diagnosis and treatment of bronchogenic cysts at a tertiary pediatric center. METHODS: A retrospective review was conducted of all patients diagnosed with a bronchogenic cyst between 2000-2020. Presence of symptoms, cyst location, surgical technique, postoperative complications, need for pleural drainage, and recurrence were reviewed. RESULTS: Forty-five children were included in the study. In 37 patients a partial resection of the cyst was done, followed by cauterization or chemical obliterateration with iodopovidone of the mucosa of the remaining cyst wall that was adherent to the airway. A lobectomy was done in patients who had intrapulmonary cysts (n = 8). Cyst location was subcarinal in 23 (51.1%), paratracheal in 14 (31.1%), and intrapulmonary in eight patients (17.8%). The majority of subcarinal and paratracheal cysts (90%) were approached by thoracoscopy. Complications occurred in seven patients (15%): subcutaneous emphysema after pleural drain removal in one, extubation failure in two, reoperation due to bleeding in one, surgical site infection in one, bronchopleural fistula in one, and pneumothorax in one. Reoperation due to cyst recurrence was necessary in two patients (4.4%). Mean follow-up was 56 months (range, 0-115). CONCLUSION: A minimally invasive approach is a safe option for the management of paratracheal and subcarinal bronchogenic cysts with no history of infection in specialized pediatric surgery center. Thoracoscopic partial resection is a feasible option in most patients with subcarinal and paratracheal bronchogenic cysts with a low complication and reoperation rate. LEVEL OF EVIDENCE: IV.

In a Setting of Esophageal Replacement, Total Gastric Pull-Up has Fewer Complications than Partial Gastric Pull-Up.

AIM: The main indications for an esophageal replacement (ER) are unresolved complex esophageal atresia (EA) and caustic strictures (CS). The use of different organs for replacement has been described. When the stomach is chosen, there are two ways to do a gastric pull-up: a partial (PGP) or a total pull-up (TGP). Few studies have been published comparing the different techniques. The aim of this study was to compare the outcomes of patients who underwent ER by PGP or by TGT. METHODS: The medical records of all patients who underwent gastric pull-up for ER in the last 18 years at the National Pediatric Hospital Prof. Dr. Juan P. Garrahan were reviewed. The study is comparative, retro-prospective and longitudinal. Patients were divided in two groups according to the ER technique (PGP or TGP). We compared the following outcomes: duration of the operation, days of hospitalization in the intensive care unit (ICU), days of total hospitalization, time to initiation of oral feedings and rate of anastomosis dehiscence, incidence of anastomotic stenosis, need for re-operations, incidence of gastroesophageal reflux disease (GERD), incidence of tracheo-esophageal fistulas (TEF), incidence of dumping syndrome, incidence of gastric necrosis and mortality. RESULTS: There were 92 patients included in the study: 70 in the PGP group (76%) and 26 in the TGP group (24%). The two groups were demographically equivalent. Patients in the TGP group had a statistically significant lower incidence of anastomotic dehiscence (22,7% versus 54,3%; p = 0.01) and dumping syndrome (13,6% versus 37,1%; p = 0.038). Patients in the TGP had lower incidence of anastomotic stenosis, although the difference was not statistically significant. There were no statistically significant differences between the groups in terms of duration of the operation, postoperative days in the ICU, time to oral feedings, GERD, TEF or overall hospital stay. There were no cases of gastric necrosis. There were 3 deaths in the PGP group and one in the TGP group. CONCLUSIONS: We observed benefits in the TGP group versus the PGP approach in terms of anastomotic dehiscence and dumping syndrome, as well as a trend toward a lower incidence of anastomotic stenosis. Based on this experience, we recommend the TGP approach for patients who need an esophageal replacement by a gastric pull-up. LEVELS OF EVIDENCE: According to the Journal of Pediatric Surgery this research corresponds to type of study level III for retrospective comparative study.

Surgical treatment of thoracic outlet syndrome in pediatrics.

AIM: Thoracic outlet syndrome (TOS) is a condition that occurs when the cervical neuro-vascular bundle becomes compressed at one of the three narrow areas of the thoraco-cervico-axillary region. Conservative management is the first line of treatment. Patients who do not respond to conservative management should be treated surgically. The aim of this review is to present our experience with the surgical management of TOS in pediatric patients. METHODS: We retrospectively reviewed the outcomes of all patients with TOS operated at our Hospital between 2001 and 2020. We collected all demographic data, clinical features, imaging data, type of operation performed, intraoperative findings, complications and recurrence. RESULTS: We operated 9 patients within the study period. The median age at surgery was 14 (7 to 17) years. A transaxillary approach was used in 7 patients and a supraclavicular approach in 2. There was only one minor intraoperative complication (violation of the pleural space). There were no postoperative complications. The median length of stay was 3 (2 to 4) days. All patients were extubated in the operating room. Two patients developed symptoms on the contralateral side. One of these underwent a successful contralateral transaxillary Roos operation. The follow-up was 4 months to 20 years. All patients are asymptomatic. CONCLUSION: We believe that the Roos operation is a safe and effective treatment with excellent long-term outcomes for children with TOS that fail conservative management.

Nefroma mesoblastico congénito: reporte de un caso / Congenital mesoblastic nephroma: case report

El nefroma mesoblastico congénito es un tumor renal infrecuente, pero común en el periodo neonatal. De comportamiento benigno y pronóstico excelente en su variedad clásica pero en el subtipo celular puede presentar recurrencia local, invasión retroperitoneal y metástasis pulmonares y cerebrales. El tratamiento es la nefrectomía radical. Los factores pronósticos son la edad menor a tres meses y la resección quirúrgica completa. Paciente de 17 días nacida a las 35 semanas por polihidramnios, con diagnóstico prenatal de masa abdominal. Al examen físico masa en flanco derecho e hipertensión arterial (121/79 mm Hg, TAM 77mm Hg). Ecografía abdominal con riñón derecho aumentado de tamaño y voluminosa formación sólida, de 4,4 x 4,3 x 4,8 cm, heterogénea y vascularizada al Doppler en polo inferior. Tomografía de abdomen con formación nodular heterogénea de 5,0 x 4,1 x 5,1 cm en polo inferior del riñón derecho. Se realizó nefrectomía radical derecha sin complicaciones. Evolución en los últimos 20 meses satisfactoria y libre de enfermedad.

Congenital mesoblastic nephroma is an uncommon renal tumor, but frequent in neonates. Classical variety usually benign with excellent prognosis. Cellular cases may be recurrent with retroperitoneal invasion, brain and lung metastasis. Radical nephrectomy is the treatment of choice. Relevant prognostic factors are diagnosis before three months of age and complete resection. A 17 day old patient, borned at 35 weeks due to polyhydramnios, with prenatal diagnosis of abdominal mass. On examination, right flank abdominal mass and arterial hypertension (121/79 mm Hg, MAP 77 mm Hg). Abdominal ultrasound showed an enlarged right kidney with a voluminous solid heterogeneous mass, 4.4 x 4.3 x 4.8 cm in the lower pole, vascularized on Doppler scan. Computed tomography showed a big 5.0 x 4.1 x 5.1 cm, nodular, heterogeneous mass in the lower pole of the right kidney. Right radical nephrectomy was performed with an uneventful recovery. Follow up for the last 20 months satisfactory and free of disease.

Biological versus synthetic patch for the repair of congenital diaphragmatic hernia: 8-year experience at a tertiary center.

PURPOSE: The aim of this study is to compare outcome between small intestinal submucosal (SIS) and polytetrafluoroethylene (PTFE) patches in patients with congenital diaphragmatic hernia (CDH) with large muscular defects. MATERIAL AND METHODS: We performed a retrospective review of prospectively collected data of patients who underwent CDH repair from January 2011 to June 2019, with a minimum follow-up of 1 year. Data were collected regarding length of stay (LOS), mortality and recurrence. Kaplan-Meier analyses was used. RESULTS: In the study period, 195 patients with CDH were admitted, 176 underwent surgical repair and 19 died before surgery. Sixty-two (35%) required a patch. Over the last 5 years, our institution transitioned from the use of all SIS to all PTFE. SIS was used in 18 cases and PTFE in 25. Overall survival rate was 89% (174/195). There was no significant difference regarding LOS and mortality. Nine patients (50%) of SIS and 1 (4%) of PTFE repairs recurred (P < 0.01; log-rank test P <  0.01), with a time course of recurrence of 18 (SD = 13) and 8 months, respectively. Median follow-up was 60 months (SD: 23) for the SIS group and 26 (SD: 10) for the PTFE group. CONCLUSION: A significant lower recurrence was found using PTFE patches instead of SIS for the repair of large CDH. LEVEL OF EVIDENCE: Level 3. Retrospective comparative study.

Giant omphalocele: Delayed closure using the San Martin technique following epithelialization of the membrane.

AIM: The management of patients with congenital anterior abdominal wall defects remains challenging, particularly in cases of giant omphalocele. In 1948, San Martín described a surgical technique for the repair of large midline incisional hernias in adults without the need for a mesh. The purpose of this report is to describe our experience with this technique for the delayed closure of giant omphaloceles. METHODS: We retrospectively reviewed the outcomes of all patients with giant omphalocele managed with the San Martin technique between September 2013 and March 2019. Data collected included birth weight, gestational age, associated malformations, neonatal hospital stay, age at the time of the abdominal wall closure, days on mechanical ventilation (MV) after the closure, time to initiation of enteral feedings, intra- and postoperative complications, and postoperative hospital stay. RESULTS: A total of 8 patients were included in the study. The median birth weight was 3.190 (2.150 to 3.400) grams. The median gestational age was 35 (32 to 38) weeks. The median age at surgery was 6 (5 to 13) years. The median postoperative days on MV was 3 (3 to 11) days. Enteral feeding were initiated postoperatively at a median of 4 (2 to 4) days. There was one intraoperative complication (minor vascular injury). There were no short-term or long-term complications directly related to the surgical technique. The median postoperative hospital stay was 10 (6 to 16) days. The follow-up was 18 months to 8 years. CONCLUSION: We believe that the San Martín technique is a valid alternative for the delayed closure of giant omphaloceles. LEVEL OF EVIDENCE: According to the Journal of Pediatric Surgery this research corresponds to type of study level IV for case series with no comparison group.

Pediatric airway tumors: A report from the International Network of Pediatric Airway Teams (INPAT).

OBJECTIVE: Primary tracheobronchial tumors (PTTs) are rare heterogeneous lesions arising from any part of the tracheobronchial tree. Nonspecific symptoms may lead to delayed diagnosis that requires more aggressive surgical treatment. An analysis of cases collected by the International Network of Pediatric Airway Team was undertaken to ensure proper insight into the behavior and management of PTTs. METHODS: Patients <18 years of age with a histological confirmation of PTT diagnosed from 2000 to 2015 were included in this multicenter international retrospective study. Medical records, treatment modalities, and outcomes were analyzed. The patient presentation, tumor management, and clinical course were compared between malignant and benign histotypes. Clinical and surgical variables that might influence event-free survival were considered. RESULTS: Among the 78 children identified, PTTs were more likely to be malignant than benign; bronchial carcinoid tumor (n = 31; 40%) was the most common histological subtype, followed by inflammatory myofibroblastic tumor (n = 19; 25%) and mucoepidermoid carcinoma (n = 15; 19%). Regarding symptoms at presentation, wheezing (P = 0.001) and dyspnea (P = 0.03) were more often associated with benign growth, whereas hemoptysis was more frequently associated with malignancy (P = 0.042). Factors that significantly worsened event-free survival were age at diagnosis earlier than 112 months (P = 0.0035) and duration of symptoms lasting more than 2 months (P = 0.0029). CONCLUSION: The results of this international study provide important information regarding the clinical presentation, diagnostic workup, and treatment of PTTs in children, casting new light on the biological behavior of PTTs to ensure appropriate treatments. LEVEL OF EVIDENCE: NA Laryngoscope, 130:E243-E251, 2020.

Disseminated visceral Kaposi´s sarcoma in a pediatric bilateral lung transplant recipient.

Cancer is a major adverse outcome of solid organ transplantation, and risks are especially high for malignancies caused by viral infections. HHV-8 is the etiologic agent of Kaposi´s sarcoma (KS). We report a case of visceral KS occurring in a 15-year-old patient after lung transplantation. The evolution was dramatically fast and interestingly, KS lesions were diffusely observed, but not in the skin. The autopsy showed the presence of numerous tumoral lesions in many organs. Microscopically, they all had very similar features, regardless of the organ affected. KS presented without cutaneous involvement. The girl was not tested for HHV-8 prior to transplantation as it was not part of our protocol. The donor was negative. The aim of the report is to alert other teams, especially those working in pediatrics, about this rare but potential complication in the setting of solid organ transplantation.

Primary lung tumors in children: 24 years of experience at a referral center.

PURPOSE: Primary lung tumors are rare during childhood and encompass a wide variety of histological types. Each has a different biology and a different therapeutic approach. The aim of this article is to review the experience of a pediatric referral center with this kind of tumors during the last 24 years. METHODS: A retrospective chart review was performed for patients with diagnosis of primary lung tumor between the years 1990-2014. The variables analyzed were age, sex, course of the disease, symptoms, localization, surgery, histology and outcome. RESULTS: Between 1990 and 2014, 38 patients with primary lung tumors were treated at our institution. Age at presentation was 6.6 ± 5.2 years (r 0.91-16.58) and the female:male relationship was 1.37. Inflammatory myofibroblastic lung tumor (n = 13), carcinoid tumor (n = 6) and pleuropulmonary blastoma (n = 6) were the most frequent histological types. Persistent radiographic abnormality was the most frequent presenting sign (34 %). Global mortality was 15.8 % varying according to histology. CONCLUSION: Although the diagnosis of primary lung tumor is rare, the persistence of a radiographic abnormality in spite of adequate treatment for inflammatory processes forces us to evaluate further. The age of the patient is an important factor in the decision of the diagnostic work-up.

[Late diagnosis bilateral congenital diaphragmatic hernia: a case report]. / Hernia diafragmática congénita bilateral de diagnóstico tardío: reporte de un caso.

Congenital diaphragmatic hernia (CDH) is a rare malformation, usually unilateral and left sided. Bilateral CDH (B-CDH) represents 1% of all CDH. Mortality is higher in right side and bilateral defects. We report a case of a patient with Bilateral CDH, initially diagnosed and treated as unilateral right CDH. At 8 months during a laparoscopic Ladd procedure a left side CDH was found.

La hernia diafragmática congénita (HDC) es una malformación rara, habitualmente unilateral y más frecuente del lado izquierdo. La HDC bilateral representa el 1% de todas las HDC. La mortalidad es mayor en las HDC derechas y en las bilaterales. Reportamos el caso de un paciente con HDC bilateral, cuya malformación inicialmente se diagnosticó y trató como una HDC derecha. Luego se encontró la presencia de HDC izquierda durante la cirugía de Ladd laparoscópica a los 8 meses de edad.

Hernia diafragmática congénita bilateral de diagnóstico tardío: reporte de un caso / Late diagnosis bilateral congenital diaphragmatic hernia: a case report

La hernia diafragmática congénita (HDC) es una malformación rara, habitualmente unilateral y más frecuente del lado izquierdo. La HDC bilateral representa el 1% de todas las HDC. La mortalidad es mayor en las HDC derechas y en las bilaterales. Reportamos el caso de un paciente con HDC bilateral, cuya malformación inicialmente se diagnosticó y trató como una HDC derecha. Luego se encontró la presencia de HDC izquierda durante la cirugía de Ladd laparoscópica a los 8 meses de edad.

Congenital diaphragmatic hernia (CDH) is a rare malformation, usually unilateral and left sided. Bilateral CDH (B-CDH) represents 1% of all CDH. Mortality is higher in right side and bilateral defects. We report a case of a patient with Bilateral CDH, initially diagnosed and treated as unilateral right CDH. At 8 months during a laparoscopic Ladd procedure a left side CDH was found.

Reconstrucción laringotraqueal con aloinjerto de aorta criopreservada como rescate de una complicación de resección cricotraqueal en pediatría / Laryngotracheal reconstruction with cryopreserved aortic allograft as a salvage technique when cricotracheal resection complications occur in paediatrics

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Umbilical arteriovenous malformation in a healthy neonate with umbilical hernia.

We describe the case of a neonate with an umbilical hernia and persistent wet umbilicus. Examination revealed a pulsatile umbilical cord with palpable thrill. Doppler ultrasound suspected umbilical arteriovenous malformation and contrast-enhanced computed tomography was performed leading to a definitive diagnosis. Surgery was successfully performed on day 27.

[Surgical treatment of tracheal stenosis in pediatrics]. / Tratamiento quirúrgico de la estenosis traqueal en pediatría.

Twenty patients with tracheal stenosis were surgically treated between July 2005 and May 2008; ten patients had a congenital stenosis and ten an acquired one. Global survival was 85%. Three patients died: 1 with acquired stenosis and 2 with congenital stenosis. From 17 alive patients, 15 remain asymptomatic; 2 patients operated on due to congenital stenosis, are under continuous follow-up (both with stents placed in the trachea). Surgical treatment of acquired stenosis presents less severe complications, requires less ventilatory support and hospital stay. Conversely, congenital stenosis often requires more than one procedure to control the disease, and presents a higher mortality rate.

Tratamiento quirúrgico de la estenosis traqueal en pediatría / Surgical treatment of tracheal stenosis in pediatrics

Veinte pacientes con estenosis de tráquea fueron intervenidos quirúrgicamente entre julio de 2005y mayo de 2008, diez con estenosis congénita y diez de origen adquirido.La sobrevida global fue de 85 por ciento. Fallecieron tres pacientes, uno con estenosis adquirida y dos conestenosis congénita.De los diecisiete sobrevivientes, quince se encuentran asintomáticos; dos pacientes operados por estenosis congénita requieren controlesperiódicos (ambos con tutores endoluminales [stents] colocados).La estenosis traqueal adquirida presenta menos complicaciones, requiere menos asistencia respiratoria y menor estadía hospitalaria en el posoperatorio. Los pacientes con estenosis traquealcongénita necesitan, generalmente, másde un procedimiento terapéutico y presentan mayor mortalidad.(AU)

Tratamiento quirúrgico de la estenosis traqueal en pediatría / Surgical treatment of tracheal stenosis in pediatrics

Veinte pacientes con estenosis de tráquea fueron intervenidos quirúrgicamente entre julio de 2005y mayo de 2008, diez con estenosis congénita y diez de origen adquirido.La sobrevida global fue de 85 por ciento. Fallecieron tres pacientes, uno con estenosis adquirida y dos conestenosis congénita.De los diecisiete sobrevivientes, quince se encuentran asintomáticos; dos pacientes operados por estenosis congénita requieren controlesperiódicos (ambos con tutores endoluminales [stents] colocados).La estenosis traqueal adquirida presenta menos complicaciones, requiere menos asistencia respiratoria y menor estadía hospitalaria en el posoperatorio. Los pacientes con estenosis traquealcongénita necesitan, generalmente, másde un procedimiento terapéutico y presentan mayor mortalidad.

Extrathoracic esophageal elongation (Kimura's technique): a feasible option for the treatment of patients with complex esophageal atresia.

AIM: The aim of this study was to evaluate the outcome of all patients who underwent an extrathoracic esophageal elongation (EEE) (Kimura's technique) and determine its role, among other surgical options, for the treatment of patients with complex esophageal atresia (EA) who have a previously created esophagostomy. METHODS: Between March 1997 and September 2008, we performed 20 EEEs. Twelve patients were males and 8 were females. The diagnoses were type C EA (n = 12), type A EA (n = 5), type B EA (n = 2), and type D EA (n = 1). Mean age at the initiation of the EEE was 10 months (range, 3-25 months). RESULTS: At the time of this report, 15 of the 20 patients have finished the treatment, 4 patients are still in the process of elongation, and one patient (premature, with a birth weight of 685 g) died before the final esophageal reconstruction. Of the 15 patients who finished the treatment, 12 (80%) completed it satisfactorily and 3 (20%) had to be prematurely interrupted. (In 2 patients, despite multiple attempts, the upper pouch could not be adequately elongated, and in one patient, an early perforation of the upper pouch precluded further elongations.) Of the 12 patients who completed the treatment satisfactorily, 10 (83%) are asymptomatic and exclusively on oral alimentation, whereas 2 (17%) have a pseudodiverticulum and esophageal dysmotility (requiring supplemental alimentation through a gastrostomy). Five of the 12 patients have gastroesophageal reflux (2 required a Nissen fundoplication and 3 are being treated medically). CONCLUSIONS: We believe that the EEE is a useful surgical option for a selected group of patients with complex long-gap EA who required a primary esophagostomy and also for patients with any type of EA who developed severe complications after a primary repair and required a secondary esophagostomy. With this technique, we avoided an esophageal replacement in 80% of cases, and given that the EEE does not invalidate a later esophageal replacement, we believe that the EEE is a feasible initial option for these patients.