Minimally invasive surgery for pilonidal disease: Outcomes of the Gips technique-A systematic review and meta-analysis.

BACKGROUND: Pilonidal disease is a common inflammatory condition that significantly impacts quality of life. Currently, there is a tendency to favor minimally invasive procedures. The present review aims to summarize the evidence and assess the outcomes of the Gips procedure. METHODS: A systematic review was conducted on MEDLINE/Pubmed, Scopus, Web of Science, and Cochrane Library databases until December 2022. Eligible studies included patients with pilonidal disease submitted to the Gips procedure, reporting at least 1 of the following outcomes: wound complications, wound healing time, time to resume daily activities, and recurrence (International Prospective Register of Systematic Reviews protocol: CRD42023389269). The National Institutes of Health assessment tool was used for risk of bias evaluation. Meta-analysis was performed using OpenMeta[Analyst] and R software, and a subgroup analysis was performed when applicable. RESULTS: Thirteen observational studies with a total of 4,286 patients submitted to Gips were included. The pooled wound complications rate was 7.8% (95% confidence interval: 5.1-10.6), the median time to resume daily activities was 1 day (95% confidence interval: 1-2), and the mean wound healing time was 4.7 weeks (95% confidence interval: 3.0-6.4). Subgroup analysis showed that pooled recurrence rate was 6.5% (95% confidence interval: 5.2-7.8) up to 2 years and 38.9% (95% confidence interval: 27.1-50.7) after more than 2 years of surgery. Most results showed substantial heterogeneity across studies. CONCLUSION: Despite apparent favorable outcomes of the Gips procedure, there is a high recurrence rate over time. Because included studies had an observational nature and unstandardized methodologies, comparative randomized controlled trials with longer follow-ups are needed for high-level evidence regarding these outcomes.

Short and Medium-term Outcomes of Omphalocele and Gastroschisis: A Survey from a Tertiary Center.

OBJECTIVE: To characterize and compare the outcomes of omphalocele and gastroschisis from birth to 2 years of follow-up in a recent cohort at a tertiary center. METHODS: This is a retrospective clinical record review of all patients with gastroschisis and omphalocele admitted to the Neonatal Intensive Care Unit between January 2009 and December 2019. RESULTS: There were 38 patients, 13 of whom had omphalocele, and 25 of whom had gastroschisis. Associated anomalies were present in 6 patients (46.2%) with omphalocele and in 10 (41.7%) patients with gastroschisis. Compared with patients with omphalocele, those with gastroschisis had younger mothers (24.7 versus 29.6 years; p = 0.033), were born earlier (36 versus 37 weeks, p = 0.006), had lower birth weight (2365 ± 430.4 versus 2944.2 ± 571.9 g; p = 0.001), and had a longer hospital stay (24 versus 9 days, p = 0.001). The neonatal survival rate was 92.3% for omphalocele and 91.7% for gastroschisis. Thirty-four patients were followed-up over a median of 24 months; 13 patients with gastroschisis (59.1%) and 8 patients with omphalocele (66.7%) had at least one adverse event, mainly umbilical hernia (27.3% vs 41.7%), intestinal obstruction (31.8% vs 8.3%), or additional surgical interventions (27.3% vs 33.3%). CONCLUSION: Despite the high proportion of prematurity, low birth weight, and protracted recovery, gastroschisis and omphalocele (without chromosomal abnormalities) may achieve very high survival rates; on the other hand, complications may develop in the first years of life. Thus, a very positive perspective in terms of survival should be transmitted to future parents, but they should also be informed that substantial morbidity may occur in the medium term.

OBJETIVO: Caracterizar e comparar os desfechos do onfalocelo e gastrosquisis desde o nascimento até aos 2 anos de seguimento numa coorte recente de um centro terciário. MéTODOS: Este é um estudo retrospectivo em que foi feita uma revisão dos registos clínicos de todos os pacientes com gastrosquisis e onfalocelo que foram internados na unidade de cuidados intensivos neonatais, entre janeiro de 2009 e dezembro de 2019. RESULTADOS: Identificamos 38 pacientes, 13 dos quais tinham onfalocelo e 25 dos quais tinham gastrosquisis. Anomalias associadas estavam presentes em 6 pacientes (46.2%) com onfalocelo e 10 (41.7%) com gastrosquisis. Comparativamente com os pacientes com onfalocelo, os pacientes com gastrosquisis tinham mães mais jovens (24.7 versus 29.6 anos; p = 0.033), nasceram mais precocemente (36 versus 37 semanas, p = 0.006), com menor peso ao nascimento (2,365 ± 430.4 versus 2,944.2 ± 571.9 g; p = 0.001), e o internamento teve uma duração mais longa (24 versus 9 dias, p = 0.001). A taxa de sobrevivência neonatal foi de 92.3% para o onfalocelo e 91.7% para a gastrosquisis. Trinta e quatro pacientes foram seguidos durante um tempo mediano de seguimento de 24 meses: 13 com gastrosquisis (59.1%) e 8 com onfalocelo (66.7%) apresentaram pelo menos um evento adverso, sobretudo hérnia umbilical (27.3% vs 41.7%), obstrução intestinal (31.8% vs 8.3%) ou intervenções cirúrgicas adicionais (27.3% vs 33.3%). CONCLUSãO: Apesar da alta proporção de prematuridade, de baixo peso e de recuperação lenta, os gastrosquisis, assim como os onfalocelos (sem anomalias cromossómicas), podem ter uma taxa de sobrevivência muito alta; por outro lado, nos primeiros anos de vida, podem surgir complicações não desprezíveis. Assim, aos futuros pais pode ser transmitida uma perspectiva muito positiva em termos de sobrevivência, embora eles também devam ser informados de que pode ocorrer morbidade substancial no médio prazo.

Neonatal Urinary Tract Infection and Renal Nodular Lesion: A Rare Case of Xanthogranulomatous Pyelonephritis.

Xanthogranulomatous pyelonephritis (XPN) is an uncommon variant of chronic pyelonephritis with a poorly understood pathogenesis and a challenging diagnosis. It is rare in pediatric patients, particularly in the neonatal period. We report the case of an 18-day-old female neonate admitted to the emergency room due to macroscopic hematuria and poor feeding. Urinalysis revealed leukocyturia and she was initially admitted under the clinical suspicion of acute pyelonephritis. Renal ultrasound and magnetic resonance imaging (MRI) revealed a progressive nodular lesion in the middle third of the left kidney. Given the suspicion of renal abscess or neoplasm, the patient was transferred to our tertiary hospital. Urinary catecholamines and tumor markers had normal values. Percutaneous kidney biopsy confirmed XPN. Posterior computed tomography scan excluded extension to neighboring structures. A conservative management with systemic antibiotic therapy was decided. She completed 7 weeks of systemic antibiotic therapy (ampicillin and cefotaxime) with progressive reduction of lesion size and posterior calcification. Follow-up at 3 years was uneventful. The lipid profile and study of neutrophil function were normal. Voiding cystourethrography excluded vesicoureteral reflux. The authors intend to highlight the importance of a high index of suspicion of XPN to allow preoperative diagnosis. Histopathological assessment is mandatory to confirm XPN and exclude other entities mimicked by focal and unilateral progressive disease. There are only a few published cases of optimal clinical evolution solely with broad-spectrum antibiotics; however, this may allow a beneficial nephron-sparing approach in selected patients.

Short and Medium-term Outcomes of Omphalocele and Gastroschisis: A Survey from a Tertiary Center / Resultados a curto e médio prazo do onfalocelo e gastrosquisis: uma investigação de um centro terciário

Abstract Objective To characterize and compare the outcomes of omphalocele and gastroschisis from birth to 2 years of follow-up in a recent cohort at a tertiary center. Methods This is a retrospective clinical record review of all patients with gastroschisis and omphalocele admitted to the Neonatal Intensive Care Unit between January 2009 and December 2019. Results There were 38 patients, 13 of whom had omphalocele, and 25 of whom had gastroschisis. Associated anomalies were present in 6 patients (46.2%) with omphalocele and in 10 (41.7%) patients with gastroschisis. Compared with patients with omphalocele, those with gastroschisis had younger mothers (24.7 versus 29.6 years; p=0.033), were born earlier (36 versus 37 weeks, p=0.006), had lower birth weight (2365±430.4 versus 2944.2±571.9 g; p=0.001), and had a longer hospital stay (24 versus 9 days, p=0.001). The neonatal survival rate was 92.3% for omphalocele and 91.7% for gastroschisis. Thirty-four patients were followed-up over a median of 24 months; 13 patients with gastroschisis (59.1%) and 8 patients with omphalocele (66.7%) had at least one adverse event, mainly umbilical hernia (27.3% vs 41.7%), intestinal obstruction (31.8% vs 8.3%), or additional surgical interventions (27.3% vs 33.3%). Conclusion Despite the high proportion of prematurity, low birth weight, and protracted recovery, gastroschisis and omphalocele (without chromosomal abnormalities) may achieve very high survival rates; on the other hand, complications may develop in the first years of life. Thus, a very positive perspective in terms of survival should be transmitted to future parents, but they should also be informed that substantial morbidity may occur in the medium term.

Resumo Objetivo Caracterizar e comparar os desfechos do onfalocelo e gastrosquisis desde o nascimento até aos 2 anos de seguimento numa coorte recente de um centro terciário. Métodos Este é um estudo retrospectivo em que foi feita uma revisão dos registos clínicos de todos os pacientes com gastrosquisis e onfalocelo que foram internados na unidade de cuidados intensivos neonatais, entre janeiro de 2009 e dezembro de 2019. Resultados Identificamos 38 pacientes, 13 dos quais tinham onfalocelo e 25 dos quais tinham gastrosquisis. Anomalias associadas estavam presentes em 6 pacientes (46.2%) com onfalocelo e 10 (41.7%) com gastrosquisis. Comparativamente com os pacientes com onfalocelo, os pacientes com gastrosquisis tinham mães mais jovens (24.7 versus 29.6 anos; p=0.033), nasceram mais precocemente (36 versus 37 semanas, p=0.006), com menor peso ao nascimento (2,365±430.4 versus 2,944.2±571.9 g; p=0.001), e o internamento teve uma duração mais longa (24 versus 9 dias, p=0.001). A taxa de sobrevivência neonatal foi de 92.3% para o onfalocelo e 91.7% para a gastrosquisis. Trinta e quatro pacientes foram seguidos durante umtempo mediano de seguimento de 24meses: 13 com gastrosquisis (59.1%) e 8 com onfalocelo (66.7%) apresentaram pelo menos um evento adverso, sobretudo hérnia umbilical (27.3% vs 41.7%), obstrução intestinal (31.8% vs 8.3%) ou intervenções cirúrgicas adicionais (27.3% vs 33.3%). Conclusão Apesar da alta proporção de prematuridade, de baixo peso e de recuperação lenta, os gastrosquisis, assim como os onfalocelos (sem anomalias cromossómicas), podem ter uma taxa de sobrevivência muito alta; por outro lado, nos primeiros anos de vida, podem surgir complicações não desprezíveis. Assim, aos futuros pais pode ser transmitida uma perspectiva muito positiva em termos de sobrevivência, embora eles também devam ser informados de que pode ocorrermorbidade substancial no médio prazo.

Massive sacrococcygeal teratoma in a preterm infant.

In extreme preterm infants, massive congenital sacrococcygeal teratomas with great hemodynamic commitment may be a situation for limitation of care.

Herlyn-Werner-Wunderlich Syndrome: Report of a Prenatally Recognised Case and Review of the Literature.

Herlyn-Werner-Wunderlich syndrome, defined by the triad of uterus didelphys, obstructed hemivagina and ipsilateral renal agenesis, is a rare Mullerian malformation, usually diagnosed after menarche, when symptoms related to hematocolpos arise. Rarely, this malformation is diagnosed in the neonatal period, normally following prenatal diagnosis of renal agenesis. Herein, a case recognized on prenatal imagiology that underwent surgery on the fourth day of life is reported. The records of prepubertal cases were also collected, addressing the clinical and imagiological features. In the presence of a solitary kidney and/or a pelvic mass on prenatal ultrasound, Herlyn-Werner-Wunderlich syndrome should be considered, enabling neonatal treatment.

Minimal access surgery in the management of pediatric urolithiasis.

BACKGROUND: In contrast to adult patients, a relatively large number of open surgical procedures are still needed in the treatment of urolithiasis in children. Since almost all open surgical techniques may be reproduced by minimal access surgery (MAS), there is a rationale to apply the latter in the management of pediatric urolithiasis. Our study aimed to assess the feasibility and outcome of MAS in the treatment of pediatric urinary calculi. METHODS: The charts of patients with urolithiasis submitted to MAS between 1994 and 2007 were retrospectively reviewed. The inclusion criteria were contraindication for and failure of lithotripsy or endourology techniques. Demographic data, lithiasis characterization (location, dimension, composition), predisposing factors (anatomic or metabolic) and surgical approach (technique and outcome) were evaluated. RESULTS: Fifteen consecutive patients (eight girls, seven boys) with a median age of 108 months (range: 10-297 months) were elected for MAS. Eleven (73%) children had associated urogenital malformations and three (20%) presented metabolic abnormalities. A total of 17 procedures were performed laparoscopically: three nephrolithotomy (one transperitoneal, two by retroperitoneoscopy), four pyelolithotomies (retro), three ureterolithotomy (trans) and seven cystolithotomies (suprapubic approach). Five patients underwent concomitant correction of urological anomalies (three calyceal diverticula, one obstructive megaureter, one ureteropelvic junction obstruction). Complete removal of calculi was accomplished in 14 (82%) procedures. There were two perioperative complications (one intraperitoneal vesical perforation and one perivesical urinoma). At a median follow up of 4 years (range, 1 month to 11 years), four patients have developed recurrence. CONCLUSIONS: MAS is an effective and safe approach for urolithiasis in children who are not candidates for minimally invasive modalities.

Complete Ureteral Duplication: Outcome of Different Surgical Approaches.

INTRODUCTION: The surgical management of complete ureteral duplication anomalies is not consensual. OBJECTIVE: To characterize the pediatric population who underwent surgery for complete ureteral duplication and assess the outcomes of different approaches. MATERIAL AND METHODS: Clinical records from patients treated between January 2008 and June 2014 were retrospectively reviewed. Epidemiology, diagnosis, clinical manifestations and surgical procedures were collected and analysed. Ureteral units were divided into two groups: A, with ureterocele; and B, without it. RESULTS: Forty-one ureteral units from 32 patients with complete duplication underwent surgery. In group A (n = 18), the selected primary procedure was: ureterocele punction (12); ureter reimplantation (3); pyelopyelostomy (2); heminephrectomy (1). A reintervention was required in 3 of the 12 units submitted to punction: heminephrectomy (1), ureteroureterostomy (1), and ureteric reimplantation (1). In group B (n = 23), STING was performed in 10 units, ureteric reimplantation in 3, pyelopyelostomy in 3, ureteroureterostomy in 1, and heminephrectomy in 6; two cases required reintervention. DISCUSSION: A conservative primary approach was favoured in cases with ureterocele and/or reflux in hemisystems worth preserving (53.7%); it was effective per se in 75% (n = 9/12) units in group A and 80% (n = 8/10) in group B. An ablative primary procedure was adopted in 17% (n = 7/41) cases, 5.6% of group A (n = 1/18) and 26.1% of group B (n = 6/23). CONCLUSIONS: A conservative approach is effective as a primary and isolated procedure in the majority of cases with ureterocele or vesicoureteral reflux. Further studies are needed to establish the advantages over primary invasive or ablative approaches.

Introdução: O tratamento cirúrgico das duplicações ureterais completas não é consensual. Objetivos: Caraterizar a população pediátrica submetida a cirurgia para tratamento de duplicações ureterais completas e avaliar resultados de diferentes abordagens. Material e Métodos: Processos clínicos de doentes tratados entre janeiro de 2008 e junho de 2014 foram retrospetivamente revistos. Dados acerca de epidemiologia, diagnóstico, manifestações clínicas e procedimentos cirúrgicos foram recolhidos. As unidades ureterais foram divididas em dois grupos: A, com ureterocelo; e B, sem ureterocelo. Resultados: Quarenta e uma unidades ureterais de 32 doentes com duplicação completa foram intervencionados. No grupo A (n = 18), o procedimento primário selecionado foi: punção de ureterocelo (12); reimplantação de ureter (3); pielopielostomia (2) e heminefrectomia (1). Foi necessário reintervir em três dos 12 casos submetidos a punção: heminefrectomia (1), ureteroureterostomia (1) e reimplantação (1). No grupo B (n = 23), foi efetuado STING em 10 unidades, reimplantação ureteral em três, pielopielostomia em três, ureteroureterostomia em um, e heminefrectomia em seis; dois casos necessitaram de reintervenção. Discussão: Foi favorecida uma abordagem primária conservadora para tratamento de ureterocelo ou refluxo em hemissistemas a preservar (53,7%; n = 22/41), tendo sido eficaz per se em 75% (n = 9/12) unidades do grupo A e 80% (n = 8/10) do grupo B. Uma abordagem ablativa primária foi adotada em 17% (n = 7/14) casos, 5,6% do grupo A (n = 1/18) e 26,1% do grupo B (n = 6/23). Conclusão: Uma abordagem conservadora é eficaz como procedimento primário isolado na maioria dos casos com ureterocelo ou refluxo. Mais estudos são necessários para estabelecer as suas vantagens sobre abordagens primárias invasivas ou ablativas.

Lung maturity in esophageal atresia: Experimental and clinical study.

INTRODUCTION: Esophageal atresia and tracheoesophageal fistula (EA-TEF) survivors suffer respiratory morbidity of unclear pathogenesis. Defective lung morphogenesis has been described in the rat model. This study examined fetal lung growth and maturity in rats and patients with EA-TEF. METHODS: Pregnant rats received either adriamycin or vehicle. Control and adriamycin-exposed lungs, with and without EA-TEF, were weighed and processed for RT-PCR, DNA quantification, immunofluorescence and immunoblot analysis of TTF1, VEGF, Sp-B, and α-sma. Twenty human lungs were also processed for immunofluorescence and Alcian-blue staining. RESULTS: Lungs from fetuses with EA-TEF (E21) showed decreased total DNA; FGF7 and TTF1 mRNA expressions were upregulated at E15 and E18, respectively. Protein expression and immunofluorescent distribution of maturity markers were similar. Lungs from stillborns with EA-TEF showed decreased epithelial expression of Sp-B and VEGF whereas those from newborns tended to have less Sp-B and more VEGF and mucous glands. DISCUSSION: The lungs of rats with EA-TEF were hypoplastic but achieved near-normal maturity. Stillborns with EA-TEF exhibited an apparently disturbed differentiation of the airway epithelium. Newborns with EA-TEF demonstrated subtle differences in the expression of differentiation markers, and increased number of mucous glands that could influence postnatal respiratory adaptation and explain some respiratory symptoms of EA-TEF survivors.

Defective upper gastrointestinal function after repair of combined esophageal and duodenal atresia.

BACKGROUND: Both esophageal atresia (EA) and duodenal atresia (DA) involve deficient anti-reflux barrier, poor esophageal function and eventually, duodenogastric reflux. This study aims at examining the upper gastrointestinal functional status in a cohort of patients with both EA and DA. METHODS: A retrospective survey of patients treated for EA and DA between 1965 and 2012 was conducted. Clinical charts, office visits, imaging, upper gastrointestinal endoscopy and esophageal pH metry/impedance were used to assess the long-term condition of the esophagus, the presence of gastroesophageal reflux disease (GERD) and/or the need for fundoplication. RESULTS: Twenty out of 581 patients treated for EA had associated DA. Ten/twenty children survived; 1 had primary esophageal replacement. With a median follow-up of 9 years, 8/9 had complicated outcomes and 5 still suffered digestive ailments: 2 GER; 1 eosinophilic esophagitis; 1 nodular gastritis, and 1 wrap herniation. A total of 10 procedures were performed: 8 fundoplications, 1 esophagogastric dissociation and 1 replacement with colon. DISCUSSION: The association of EA with DA involves a poor upper digestive function with high risks of GERD and fundoplication failure. The lifelong synergistic play of esophageal, gastric and duodenal dysfunctions in these patients prompts long-term follow-up, and eventually active treatment.

Omental infarction: a reappraisal of conservative management in children.

INTRODUCTION: Accurate recognition of omental infarction has resulted in increasing supporters of non-operative approach. In order to assess the efficacy and safety of conservative management, we surveyed the cases of omental infarction treated at a single institution. MATERIAL AND METHODS: Primary omental infarction treated between 2004 and 2011 were reviewed. Cases recognized by imaging studies were submitted to conservative treatment that consisted of intravenous analgesics and antibiotics. Demographics, clinical presentation, laboratory findings, imaging diagnosis and outcome were analyzed. RESULTS: There were 9 cases of omental infarction. Eight patients (4 males; median age, 8.5 years) presented at initial course of disease; all had right-sided abdominal pain and a normal or lightly increased leukocyte count. Six cases, diagnosed by CT scan after US suspicion in four, were managed conservatively, recovered uneventfully, and were discharged after a median hospital stay of 3 days. Two patients were only recognized at surgery. An additional case presented with intestinal obstruction due to an internal hernia and was successfully resolved by laparoscopy. DISCUSSION: Imaging techniques were diagnostic of omental infarction in the majority of cases, enabling a conservative approach to be adopted. Non-operative management was successful with no complications in all patients presenting at the initial course of disease. One patient presented with a harmful complication that required operative treatment. CONCLUSION: In the absence of a standard approach for omental infarction, conservative management is an effective noninvasive alternative but it claims for active surveillance.

Introdução: A melhoria da acuidade diagnóstica do enfarte do omento resultou num incremento da atitude conservadora no seu tratamento. Com o objectivo de avaliar a eficácia e segurança da abordagem não-operatória, analisámos os casos de enfarte do omento tratados num hospital terciário. Material e Métodos: Incluímos os casos de enfarte do omento primários tratados entre 2004 e 2011. Os enfartes do omento diagnosticados por imagiologia foram submetidos a tratamento conservador que constou de analgésicos e antibioterapia intravenosa. Avaliámos os dados demográficos, apresentação clínica, dados laboratoriais, imagiologia e resultado. Resultados: Ocorreram nove casos de enfarte do omento. Oito pacientes (4 rapazes), com uma idade mediana de 8,5 anos, apresentaram- se na fase inicial da doença por dor abdominal à direita; a contagem leucocitária era normal ou ligeiramente elevada. Seis casos, diagnosticados por TC após ecografia suspeita de enfarte do omento em quatro, foram trados conservadoramente sem complicações, tendo alta ao terceiro dia (mediana). Dois doentes foram apenas diagnosticados durante a intervenção cirúrgica por suposta apendicite. O nono caso apresentou-se com uma obstrução intestinal devida a hérnia interna que foi resolvida por laparoscopia. Discussão: A imagiologia foi diagnóstica na maioria dos casos de enfarte do omento, permitindo a adoção de uma abordagem conservadora. O tratamento não-operatório foi eficaz e sem complicações em todos os doentes que se apresentaram na fase inicial da doença. Um doente apresentou-se com uma complicação grave que requereu intervenção cirúrgica. Conclusão: Na ausência de um tratamento consensual para o enfarte do omento, a abordagem não-operatória é uma alternativa não invasiva e eficaz, mas requer uma vigilância clínica ativa.

Abnormal Sonic hedgehog signaling in the lung of rats with esophageal atresia induced by adriamycin.

BACKGROUND: Abnormal lung development was recently described in the rat model of esophageal atresia and tracheoesophageal fistula (EA-TEF). Since in this condition the ventral-to-dorsal switch of Shh expression in the foregut is disturbed, the present study tested the hypothesis that this abnormal expression at the emergence of the tracheobronchial bud might be translated into the developing lung. METHODS: Pregnant rats received either 1.75 mg/kg i.p. adriamycin or vehicle from E7 to E9. Three groups were studied: control and adriamycin-exposed with and without EA-TEF. Embryos were recovered and the lungs were harvested and processed for reverse transcription polymerase chain reaction and immunofluorescence analysis of the Shh signaling cascade. RESULTS: Shh signaling was downregulated at the late embryonic stage of lung development (E13) in embryos with EA-TEF. Throughout the subsequent stages of development, the expression of both Shh and its downstream components increased significantly and remained upregulated throughout gestation. Immunofluorescent localization was consistent with these findings. CONCLUSION: Defective Shh signaling environment in the foregut is present beyond the emergence of lung buds and probably impairs lung development. Later in gestation, lungs exhibited a remarkable ability to upregulate the Shh cascade, suggesting a compensatory response. These findings may be relevant to understand pulmonary disease suffered by children with EA-TEF.

The multifactorial origin of respiratory morbidity in patients surviving neonatal repair of esophageal atresia.

Esophageal atresia with or without tracheoesophageal fistula (EA ± TEF) occurs in 1 out of every 3000 births. Current survival approaches 95%, and research is therefore focused on morbidity and health-related quality of life issues. Up to 50% of neonates with EA ± TEF have one or more additional malformations including those of the respiratory tract that occur in a relatively high proportion of them and particularly of those with vertebral, anal, cardiac, tracheoesophageal, renal, and limb association. Additionally, a significant proportion of survivors suffer abnormal pulmonary function and chronic respiratory tract disease. The present review summarizes the current knowledge about the nature of these symptoms in patients treated for EA ± TEF, and explores the hypothesis that disturbed development and maturation of the respiratory tract could contribute to their pathogenesis.

Maternal hyperthyroidism increases the prevalence of foregut atresias in fetal rats exposed to adriamycin.

PURPOSE: Gastrointestinal malformations such as esophageal atresia with tracheoesophageal fistula (EA/TEF) and duodenal atresia (DA) have been reported in infants born to hyperthyroid mothers or with congenital hypothyroidism. The present study aimed to test whether maternal thyroid status during embryonic foregut division has any influence on the prevalence of EA/TEF and DA in an accepted rat model of these malformations. METHODS: Pregnant rats received either vehicle or 1.75 mg/kg i.p. adriamycin on gestational days 7, 8 and 9. Transient maternal hyper or hypothyroidism was induced by oral administration of levothyroxine (LT4, 50 µg/kg/day) or propylthiouracil (PTU, 2 mg/kg/day), respectively, on days 7 to 12 of gestation. Plasma cholesterol, total T3, free T4 and TSH were measured at gestational days 7, 12, and 21. At the end of gestation, the mothers were sacrificed and embryo-fetal mortality was recorded. Fetuses were dissected to determine the prevalence of esophageal and intestinal atresias. RESULTS: At gestational day 12, mothers treated with LT4 or PTU had hyper or hypothyroid status, respectively; plasma cholesterol levels were similar. In the adriamycin-exposed fetuses from hyperthyroid mothers, the embryonal resorption rate and the prevalence of both EA/TEF and DA were significantly higher than in the other groups; maternal hypothyroidism during the same period did not have significant effect on the prevalence of atresias. CONCLUSIONS: Maternal hyperthyroidism during the embryonic window corresponding to foregut cleavage increased the prevalence of both EA/TEF and duodenal atresia in fetal rats exposed to adriamycin. This suggests that maternal thyroid hormone status might be involved in the pathogenesis of foregut atresias and invites further research on this likely clinically relevant issue in humans.

Abnormal control of lung branching in experimental esophageal atresia.

PURPOSE: Esophageal atresia and tracheo-esophageal fistula (EA-TEF) result from abnormal division of the foregut into esophagus and trachea thus, it may influence airway branching and lung development. The present study examined lung morphogenesis in fetuses with EA-TEF focusing in the expression of FGF10 and its receptor FGFR2 IIIb. METHODS: Pregnant rats received either 1.75 mg/kg i.p. adriamycin or vehicle on E7, E8 and E9. Embryos were recovered at E15, E18 and E21 and lungs processed for immunohistochemistry and RT-PCR. Three groups were studied: control, adriamycin-exposed with EA-TEF, and adriamycin-exposed without EA-TEF. Comparisons were performed with Mann-Whitney or t tests (significance level, 5 %). RESULTS: Lung weight at E15 and E18 were significantly lower in adriaEA fetuses in which the relative mRNA levels of FGF10 were significantly higher. These differences disappeared near term. The receptor FGFR2 IIIb messenger was only significantly increased in adria noEA fetuses at E15. Immunohistochemical study was consistent with these findings. CONCLUSIONS: Abnormal expression of FGF10 during earlier stages of development, when the lungs are smaller than controls, suggests a compensatory response aimed at "catching up" delayed tracheobronchial branching. Whether similar changes take place in the human condition and influence respiratory physiology remain to be determined.

Abdominal inflammatory myofibroblastic tumor a clinicopathologic study with reappraisal of biologic behavior.

BACKGROUND AND PURPOSE: Inflammatory myofibroblastic tumor (IMT) is a proliferative lesion of controversial nosology and uncertain prognosis. In an attempt to acquire further understanding of pathogenesis and biologic behavior, we surveyed abdominal IMTs managed over the last 12 years at a single institution. METHODS: Intra-abdominal IMTs treated between 1995 and 2007 were reviewed concerning demographic, clinical, and pathologic features as well as therapeutic management and outcome. All specimens were reevaluated by histologic examination and immunohistochemistry. RESULTS: There were 7 patients (4 males; age range, 28 days to 14 years). Five lesions were located in alimentary tract: 1 gastric presenting with bleeding, 1 hepatic presenting with a thoracic wall mass, 1 pancreatic and 2 colonic presenting with obstructive symptoms. One splenic IMT was found incidentally. The remaining case arose from the adrenal gland and presented with a palpable mass. The gastric and adrenal IMTs had evidence of a previous or concomitant infectious setting. Five lesions were excised. The pancreatic IMT underwent a drainage procedure followed by steroid administration, and the hepatic lesion received antibiotics. Histopathology revealed characteristic findings of IMT. Expression of anaplastic lymphoma kinase was negative in all cases. At a median follow-up of 6 years (range, 3-15), all children were asymptomatic with no recurrences. The hepatic and pancreatic IMT displayed complete and near total regression, respectively. CONCLUSION: A benign behavior of abdominal IMTs was observed even in patients not undergoing surgical excision. Although IMT remains a surgical disease, a conservative approach may be reasonable in select cases.

Gastric emptying and antireflux surgery.

PURPOSE: Absence of consistent data on the outcome of gastric emptying after fundoplication raises concerns about preoperative workup and surgical management. This study assessed how gastric emptying evolves after isolated fundoplication in order to determine whether a preoperative investigation and/or a concurrent gastric drainage procedure are justified. METHODS: Eleven children with GERD underwent both pre- and post-operative gastric emptying scintigraphy. No gastric drainage procedures were added to fundoplication. Scintigraphy was performed according to a standardized protocol (labeled meal, 300 ml + 500 µCi (99m)Tc/m(2) bsa). Half-emptying time (t (1/2)), emptying rate and fractions of retention are expressed as the median followed by the [range]; statistical significance level was set at 5%. RESULTS: Postoperative t (1/2) (min) showed a much smaller dispersion and was significantly lower than preoperative one (76 [56;101] vs. 107 [57;186], p = 0.04) with a variation of -48 [-105;19]; there was no individual correlation between pre- and post-operative measurements (r = 0.25). The proportion of patients with delayed gastric emptying (t (1/2) > 100) was significantly lower in the postoperative scintigraphy (9 vs. 55%, p = 0.03). The postoperative emptying rate (%/h) was significantly faster (35.1 [18.9;41.7] vs. 28.5 [16.3;38.7], p = 0.05). The retentions (%) at 30 and 60 min were similar; at 90 and 120 min were significantly lower after surgery (45 [35;54] and 32 [24;46] vs. 53 [33;78] and 41 [25;66], p = 0.03 and 0.05). CONCLUSION: Gastric emptying accelerates significantly after fundoplication with a shift to normal values in the vast majority of patients. Scintigraphy does not predict the postoperative outcome. Therefore, concomitant drainage procedures seem unnecessary and preoperative gastric emptying study not useful.

[Transhiatal esophagectomy with gastric transposition for esophageal replacement in post-corrosive stricture in children]. / Esofagectomia transhiatal com transposição gástrica na substituição esofágica por estenose cáustica em crianças.

BACKGROUND & PURPOSE: Despite potential advantages of gastric transposition there are some concerns about this surgical technique of esophageal substitution in children. In the current study the morbidity and functional outcome are surveyed in a series of patients undergoing gastric transposition due to extensive post-corrosive esophageal strictures. METHODS: Retrospective analysis of children proposed for esophageal replacement from September 2003 through April 2007 after endoscopic dilations failure. Demographic and pathological features, intra and postoperative complications and clinical outcome were assessed. Continuous variables are expressed as median [range]. RESULTS: There were six children (age: 4.3 [2.5;14.4] years) with esophageal structures secondary to corrosive alkali ingestion; five had been submitted to dilation (n = 8 [7;27]) and one had undergone gastrostomy. Esophagectomy without thoracotomy plus gastric transposition were uneventfully performed in all cases. There was no mortality. On postoperative course there was one hypertensive pneumothorax and one pneumonia. With a follow-up of 50 [38;80] months, two children experienced mild dysphagia that was promptly solved by one or two sessions of dilations of esophagogastric anastomosis; both weight and height were between 5th and 75th centiles; none presented gastrointestinal or recurrent respiratory symptoms, anemia or substitute dilation. CONCLUSION. Transhiatal esophagectomy with gastric transposition in posterior mediastinum presented low morbidity and excellent functional outcome with no impairment of growth or respiratory function, at least on short/medium term.