Treatment of Congenital Microgastria.

INTRODUCTION: Congenital microgastria is an extremely rare birth defect. The aim of this study was to present an overview of existing literature on the treatment of microgastria. MATERIALS AND METHODS: The term "microgastria" was used in a PubMed and Medline search. Since merely case reports were found, only a narrative synthesis with limited statistical analysis can be given. Data of different treatment modalities were collected and divided into two groups: conservative or less invasive treatment (C/LT, i.e., modified diet or a gastrostomy/jejunostomy) and extensive gastric surgery (EGS, i.e., Hunt-Lawrence pouch or total esophageal gastric dissociation). Clinical outcome parameters (nutrition, growth pattern, and mortality) were compared. RESULTS: Out of 73 articles published from 1973 to 2019, 38 articles describing 51 cases were included. In four patients, microgastria was an isolated anomaly (8%). Type of treatment was described in only 46 patients, 19 were treated by C/LT. Mortality was 9/19 (47%) in the C/LT group versus 4/27 (15%) in the EGS group (chi-square = 5.829, p = 0.016, Fisher = 0.022). There was a negative correlation between the invasiveness of the treatment and both mortality (r = -0.356, p = 0.015) and comorbidity (r = -0.506, p <0.001). Patients in the C/LT group had significantly more comorbidity than in the EGS group (mean = 4.32 vs. 2.26, p = 0.001). There was a positive correlation between comorbidity and mortality (r = 0.400, p = 0.006). Median follow-up was 42 months (range: 1-240). Type and way of nutrition were poorly described. In at least 9 of the 33 surviving patients, oral feeding was reported as normal, of whom 8 belonged to the EGS group. In all patients, growth could be acknowledged, but in comparison to peers, final body length was less. There was no difference in final body length between the two treatment groups. CONCLUSION: In patients with congenital microgastria, only minimal differences in clinical outcome in terms of type of nutrition and body growth were found when C/LT was compared with treatment by EGS. Mortality was significantly higher in the first group as well as the amount of comorbidities.

Adult Presentations of Congenital Midgut Malrotation: A Systematic Review.

BACKGROUND: Adult midgut malrotation is a rare cause of an acute abdomen requiring urgent intervention. It may also present in the non-acute setting with chronic, non-specific symptoms. The objective of this study is to identify the clinical features, appropriate investigations and current surgical management associated with adult malrotation. METHODS: A systematic review was conducted according to PRISMA guidelines, identifying confirmed cases of adult malrotation. Patient demographics, clinical features, investigation findings and operative details were analysed. RESULTS: Forty-five reports met the inclusion criteria, totalling 194 cases. Mean age was 38.9 years (n = 92), and 52.3% were male (n = 130). The commonest presenting complaints were abdominal pain (76.8%), vomiting (35.1%) and food intolerance (21.6%). At least one chronic symptom was reported in 87.6% and included intermittent abdominal pain (41.2%), vomiting (12.4%) and obstipation (11.9%). Computerised tomography scanning was the most frequent imaging modality (81.4%), with a sensitivity of 97.5%. The whirlpool sign was observed in 30.9%; abnormalities of the superior mesenteric axis were the commonest finding (58.0%). Ladd's procedure was the most common surgical intervention (74.5%). There was no significant difference in resolution rates between emergency and elective procedures (p = 0.46), but length of stay was significantly shorter for elective cases. (p = 0.009). There was no significant difference in risk of mortality, or symptom resolution, between operative and conservative management (p = 0.14 and p = 0.44, respectively). CONCLUSION: Malrotation in the adult manifests with chronic symptoms and should be considered as a differential diagnosis in patients with abdominal pain, vomiting and food intolerance.

Burden of emergency pediatric surgical procedures on surgical capacity in Uganda: a new metric for health system performance.

BACKGROUND: The significant burden of emergency operations in low- and middle-income countries can overwhelm surgical capacity leading to a backlog of elective surgical cases. The purpose of this investigation was to determine the burden of emergency procedures on pediatric surgical capacity in Uganda and to determine health metrics that capture surgical backlog and effective coverage of children's surgical disease in low- and middle-income countries. METHODS: We reviewed 2 independent and prospectively collected databases on pediatric surgical admissions at Mulago National Referral Hospital and Mbarara Regional Referral Hospital in Uganda. Pediatric surgical patients admitted at either hospital between October 2015 to June 2017 were included. Our primary outcome was the distribution of surgical acuity and associated mortality. RESULTS: A combined total of 1,930 patients were treated at the two hospitals, and 1,110 surgical procedures were performed. There were 571 emergency cases (51.6%), 108 urgent cases (9.7%), and 429 elective cases (38.6%). Overall mortality correlated with surgical acuity. Emergency intestinal diversions for colorectal congenital malformations (anorectal malformations and Hirschsprung's disease) to elective definitive repair was 3:1. Additionally, 30% of inguinal hernias were incarcerated or strangulated at time of repair. CONCLUSION: Emergency and urgent operations utilize the majority of operative resources for pediatric surgery groups in low- and middle-income countries, leading to a backlog of complex congenital procedures. We propose the ratio of emergency diversion to elective repair of colorectal congenital malformations and the ratio of emergency to elective repair of inguinal hernias as effective health metrics to track this backlog. Surgical capacity for pediatric conditions should be increased in Uganda to prevent a backlog of elective cases.

Standardized Approach to Intervention for Intestinal Malrotation in Single Ventricle Patients with Heterotaxy Syndrome: Impact on Interstage Attrition and Time to Superior Cavopulmonary Connection.

Heterotaxy syndrome (HS) is a significant determinant of outcome in single ventricle (SV) physiology. Attrition rate and time-related events associated with intestinal malrotation (IM) are, yet, to be determined. We sought to evaluate hospital and interstage outcomes in relation with operative intervention for IM (IMO). Twelve SV/HS patients, who underwent IMO, from January 2004 to December 2016, were studied. Early shunt failure, time to superior cavopulmonary connection (SCPC) and interstage attrition were assessed. Since September 2014, based on a comprehensive standardized protocol, IMO was performed at the time of hospitalization for stage-I palliation (S1P) irrespective of clinical manifestations. Patients were assigned to Group A (n = 8): expectant /symptoms-driven versus Group B (n = 4): protocol-driven. At S1P 7 had systemic-to-pulmonary shunt (SPS), 1 SPS with anomalous pulmonary venous return (APVR) repair (Group A) compared to 2 SPS, 1 SPS with APVR repair and 1 Norwood operation (Group B). Median duration from S1P to IMO was 82 days (range 57-336; Group A) compared to 14 days (range 11-31; Group B); p < 0.05. Median age at IMO was 87 days (range 8-345) [Group A: 99 days (range 68-345) vs Group B: 25 days (range 8-39)] (p < 0.05). Early SPS failure occurred in 25% (2 of 8) for Group A compared to none in Group B (p < 0.05). Hospital mortality following IMO was 25% [Group A: 37.5% (3 of 8) vs Group B: 0; p < 0.05]. Interstage survival was 67% [Group A: 50% (4 of 8) vs Group B: 100%; p < 0.05]. Time to SCPC following S1P was 186 days (range 169-218) for Group A compared to 118 days (range 97-161) (Group B); p < 0.05. Operative intervention for IM in SV/HS is associated with significant interstage attrition and might impact the time to SCPC. SPS is at risk for early failure after IMO. A comprehensive standardized concept can mitigate detrimental implications.

Thinking of VACTERL-H? Rule out Fanconi Anemia according to PHENOS.

VACTERL-H association includes three of eight features: vertebral anomalies, anal atresia, congenital heart disease, tracheo-esophageal fistula, esophageal atresia, renal, limb anomalies, and hydrocephalus. The VACTERL-H phenotype among cases with FA is considered to be about 5%; the frequency of FA among patients with VACTERL-H is unknown. We examined 54 patients with FA in the National Cancer Institute Inherited Bone Marrow Failure Syndrome Cohort for features of VACTERL-H, including imaging studies (radiology and ultrasound). Eighteen of the fifty-four patients had three or more VACTERL-H features. The presence of VACTERL-H association in 33% of those with FA is much higher than the previous estimate of 5% (P < 0.0001). We created the acronym PHENOS (Pigmentation, small Head, small Eyes, central Nervous system (not hydrocephalus), Otology, and Short stature) which includes all major phenotypic features of FA that are not in VACTERL-H; these findings were more frequent in the patients with FA who had VACTERL-H. Identification of any components of the VACTERL-H association should lead to imaging studies, and to consideration of the diagnosis of FA, particularly if the patient has radial ray and renal anomalies, as well as many features of PHENOS. There was no association of the presence or absence of VACTERL-H with development of cancer, stem cell transplant, or survival. Early diagnosis will lead to genetic counseling and early surveillance and management of complications of FA. © 2016 Wiley Periodicals, Inc.

Malrotation: Age-Related Differences in Reoperation Rate.

OBJECTIVE: Intestinal malrotation classically presents in the neonatal period with bilious vomiting. However, population studies suggest that up to two-thirds of these patients are diagnosed later in childhood or in adulthood. Increased morbidity in the adult population has been reported. Local experience suggested that surgery was technically more difficult in older children and led to the hypothesis that it would be associated with increased morbidity. METHODS: A retrospective case note analysis was performed on all children presenting with intestinal malrotation to a tertiary referral center between January 2002 and November 2014. Case notes and operation records were reviewed and those who underwent laparotomy for confirmed malrotation were included. Children were grouped as infants (< 1 year) and older (> 1 year). The primary outcome was total emergency reoperation rate. Secondary outcomes were requirement for a bypass at reoperation and mortality. RESULTS: A total of 131 children with malrotation were identified (104 infants, 27 older children; 78 males; age range, 0-16 years). Overall, 13 patients had emergency reoperation following initial Ladd procedure (6 infants and 7 older children). Risk for reoperation was significantly higher in older children (p = 0.005) and additionally a bypass procedure was more often required in older children than infants (4 children, 2 infants, p = 0.016). Adhesiolysis was required on four occasions and redo Ladd procedure in two; these were evenly distributed between both groups. One child was found to have distal bowel obstruction at reoperation. There were three deaths (2.3%), all in the infant group. One was directly associated with malrotation with extensive bowel necrosis. The other two died of unrelated sepsis several months later. CONCLUSIONS: Malrotation surgery in older children is associated with a significantly higher emergency reoperation rate. The primary duodenal bypass procedure should always be considered with longstanding chronic intermittent obstruction associated with malrotation if the simple Ladd procedure is deemed inadequate.

Early prediction of complex midgut volvulus in neonates and infants.

INTRODUCTION: Prognosis of midgut volvulus in neonates and infants younger than 1 year remains poor, as diagnostic findings may not be apparent until gut infarction had occurred. To characterize factors that help to predict complex midgut volvulus early was aim of this study. METHODS: Institutionally approved retrospective analysis of all children younger than 1 year treated for midgut volvulus at the author's center from January 2002 to December 2011. Medical history, symptoms, laboratory and radiologic findings as well as sequelae of midgut volvulus were evaluated. RESULTS: In 10 years, 37 children fulfilled the inclusion criteria. Of these, 43% developed complications, and mortality rate was 16%. In 30% of the patients, the only clinical sign was a sudden worsening of the general condition and abdominal distension (complex 19% vs. simple 38%). In one child with simple midgut volvulus, all clinical, laboratory and radiologic signs were negative. CART analysis identified a base excess below -1.70 and preterm birth (<36 weeks) as the best discriminators of complex and simple midgut volvulus. A score >1pt (comprised of these two factors) was found in all children with complex and in 14% of simple midgut volvulus (p < 0.001). A positive score (>1pt) offers a sensitivity of 100% (81.7-100%), specificity of 85.7% (71.8-85.7%), a PPV of 84.2% (68.8-84.2%) and NPV 100% (83.8-100%). DISCUSSION: The study shows that midgut volvulus has a substantial morbidity and mortality. Unfortunately, not all affected children get picked up by history, laboratory and imaging. However, the proposed score helps to identify subject with increased risk of complications. It has the potential to facilitate and accelerate diagnosis of complex midgut volvulus; ultimately, it might help to reduce morbidity and mortality.

Identification of the modifier locus that suppresses neonatal lethality in (♀DDD × â™‚DH- Dh/+) F1-Dh/+ male mice.

Most F(1)-Dh/+ male mice resulting from a cross between inbred DDD strain females and DH-Dh/+ strain males exhibit growth retardation and die during the neonatal period. The lethality is caused by a combination of three independent gene loci, namely the Dh locus on chromosome 1, Grdhq1 locus on the X chromosome, and a putative Y chromosome-linked locus in some strains. Among these loci, Grdhq1 was previously mapped to a distal region of the X chromosome using progeny from♀(♀DDD × â™‚DH-+/+) F(1) × â™‚DH-Dh/+ mice. In this study, fine mapping of Grdhq1 was performed using progeny of ♀(♀DDD × â™‚CAST/EiJ) F(1) ♂DH-Dh/+ mice. Contrary to expectation, Dh/+ male pups carrying the DDD allele at DXMit135 (genetic marker nearest to Grdhq1) survived to weaning. The presence of modifier loci that suppressed the lethality by impeding the action of Grdhq1 was suggested; therefore, a genome-wide scan was performed in the surviving Dh/+ males. As a result, a significant modifier locus was identified on proximal chromosome 11. This in turn suggested that Grdhq1 was located more distally than we had expected; that is, the actual location of Grdhq1 appeared to be near and/or distal to the Mid1 locus. Thus, the results revealed that the neonatal lethality in (DDD × DH-Dh/+) F(1)-Dh/+ males was caused by the fourth gene locus on chromosome 11 in addition to the above-mentioned three gene loci on chromosomes 1, X, and Y.

[The rate and dynamics of prevalence of diseases of digestive system in north Caucasus federal okrug and Stavropolsky kray].

The article presents the results of analysis of digestive system morbidity during last decade among adult population of Stavropolsky kray as compared with North Caucasus federal okrug and Russia in general. The analysis according classes of diseases established that in the structure ofgeneral morbidity according the appealability data first place is for diseases of respiratory organs, second place is for diseases of circulatory system, third place is for diseases of urogenital system, fourth place is for diseases of musculoskeletal system, fifth place is for traumas, intoxications and other aftermath of external causes, sixth place is for diseases of digestive system and also pregnancy, delivery and puerperal period In Stavropolsky kray, gastritis and duodenitis are registered much more infrequently as compared with corresponding morbidity increase among adult population of Russia from 2001 to 2010.

Delayed presentation of anorectal malformations: the possible associated morbidity and mortality.

AIM: Anorectal malformations (ARMs) are one of the most common congenital anomalies dealt by pediatric surgeons with reported incidence of approximately 1 in 5,000 live births. The majority of patients usually presents during the neonatal period. However a significant number of patients report later during infancy or even late childhood. Such delayed presentation may affect the surgical management and contribute to functional and psychological problems for both babies and their parents. The aim of this study is to highlight the frequency of this problem, cause of delayed presentation, and how this delayed presentation of ARM contributes to morbidity and mortality. PATIENTS AND METHODS: During the 3-year study period, 104 cases with ARM (74 males and 30 females) were presented to pediatric surgery unit in Assiut university children's hospital. Delayed presentation means that the child came after the first 48 h or after 3 months of age in case of low ARM in females. RESULTS: Out of 104 cases, 20 fulfil the criteria of delayed presentation (11 females and 9 males). Their age at presentation ranged from 4 days to 14 years. Fourteen classified as low ARM (6 male and 8 female), whereas six classified as high ARM (3 male and 3 female). The causes of delayed presentation were delayed detection, wrong advice to the parents, inadequate treatment offered, and social causes. The associated morbidities encountered were chronic constipation, mega rectum, and unnecessary colostomy. Two mortalities were encountered; both were males presented on days 4 and 14 after birth. CONCLUSIONS: Delayed ARMs is not uncommon. Every neonate should be carefully examined and screened for such anomaly to avoid the possible morbidity and mortality which are directly related to this delayed presentation.

Incidence of septicemia immediately after elective gastrointestinal contrast procedures in infants: a cohort study.

BACKGROUND: Sepsis is a documented complication of gastrointestinal contrast procedures in neonates. However, the identification of preventive measures is hampered by a lack of data on its incidence and risk factors. METHODS: The study used a retrospective cohort analysis of infants with selected surgical gastrointestinal conditions admitted to a tertiary neonatal center. Risk factors were identified by logistic regression and matched case-control analyses. Contrast procedure-related bacteremia or sepsis were defined by clinical signs with or without a positive blood culture, respectively, within 48 hours after an intervention. RESULTS: The apparent incidence of contrast procedure-related sepsis was 2.7 per 100 infant procedures. Infants with contrast procedure-related sepsis were also generally of lower gestational age and birth weight and generally sicker (ie, higher incidence of hepatic cholestatic disease, and poorer weight gain). Notably, all infants with contrast procedure-related sepsis previously had necrotizing enterocolitis. Although the number of cases of sepsis directly attributable to the procedures may be lower, as suggested by a comparison with the baseline time prevalence of bacteremia in this cohort, significant associated morbidities and mortality were observed. CONCLUSIONS: This is the first study reporting the incidence of contrast procedure-related sepsis in high-risk infants with surgical gastrointestinal conditions. Based on our observations, the routine use of prophylactic antibiotics to prevent this complication in this population does not seem warranted.

Open vs laparoscopic repair of congenital duodenal obstructions: a concurrent series.

OBJECTIVE: The advantages of using laparoscopy for repair of congenital duodenal obstructions (CDO) are unclear because of scant data about complications and outcomes. Nitinol U-clips (Medtronic Surgical, Minneapolis, Minn) were developed to assist in the creation of vascular anastomoses in small vessels. Because of their ability to approximate tissue tightly with little tissue damage, we have begun to use these U-clips for laparoscopic repair of CDO. In this report, we investigate the impact of laparoscopic U-clip repair of CDO compared to the traditional open repair. METHODS: With institutional review board approval, a retrospective analysis of all patients undergoing repair of CDO from January 2003 to July 2007 was performed. During this study period, patients who underwent open repair of CDO (group 1) were compared with patients that underwent laparoscopic repair using the U-clip technique (group 2). RESULTS: Twenty-nine patients underwent repair of CDO. Fourteen patients (11 atresia, 3 stenosis) were in group 1 and 15 patients (11 atresia, 4 stenosis) in group 2. A female sex bias existed in group 1 (female-male [9:5]) compared to group 2 (female-male [7:8]). There was no difference in birth weight, age at operation, chromosomal anomalies, or congenital heart disease between the groups. There were no duodenal anastomotic leaks in either group. Operative times were similar between groups (96 vs 126 minutes; P = .06). The length of postoperative hospitalization (20.1 vs 12.9 days; P = .01), time to initial feeding (11.3 vs 5.4 days; P = .002), and time to full oral intake (16.9 vs 9 days; P = .007) were all statistically shorter in group 2. CONCLUSIONS: The laparoscopic approach to CDO repair using U-clips is safe and efficacious. In addition, patients undergoing laparoscopic repair of CDO had a shorter length of hospitalization and more rapid advancement to full feeding compared to babies undergoing the open approach. We feel that in the hands of experienced laparoscopic surgeons, the preferred technique for correction of CDO will become the laparoscopic U-clip repair.

The optimal management of malrotation diagnosed after infancy: a decision analysis.

BACKGROUND: The benefit of a prophylactic Ladd's procedure in older children and adults with malrotation is controversial. The purpose of this study was to determine the role of the Ladd's procedure in patients with asymptomatic malrotation diagnosed after infancy. METHODS: A Markov decision analysis was used to compare the quality adjusted life expectancy with and without a Ladd's procedure among patients with asymptomatic malrotation. Data obtained from the Nationwide Inpatient Sample were used to estimate the age-related probability of emergency surgery or volvulus among patients with malrotation. Estimates of the mortality of elective and emergency surgery, mortality of volvulus, and utilities of each health state were obtained from the literature. RESULTS: After infancy, the gain in quality adjusted life expectancy associated with a prophylactic Ladd's procedure was highest when asymptomatic malrotation was treated at 1 year old and steadily declined until asymptomatic malrotation was treated at 20 years old. An increasing advantage of observation over prophylactic surgery on life expectancy was observed after the second decade of life. A 2-fold increase in mortality risk for an elective Ladd's procedure decreased the age threshold to 14 years, whereas a 4-fold increase decreased the threshold to 7 years. These results were found to be robust by sensitivity analyses and Monte Carlo simulation. CONCLUSION: A Ladd's procedure should be considered for children diagnosed with asymptomatic malrotation, particularly those who are younger and with a low risk of postoperative mortality. The rare occurrence of midgut volvulus does not justify performing a prophylactic Ladd's procedure on most adults with malrotation.

Management of polycystic liver disease.

The adult forms of polycystic liver disease are characterized by autosomal dominant inheritance and numerous hepatic cysts, with or without renal involvement. Mutations in two distinct genes predispose to renal and liver cysts (PKD1 and PKD2), and mutations in two different genes yield isolated liver cysts (PRKCSH and SEC63). Mutations at certain loci of PKD1 may predispose to more severe renal cystic disease or cerebral aneurysms. Risk factors for severe hepatic cystic disease include aging, female sex, pregnancy, use of exogenous female steroid hormones, degree of renal cystic disease, or severity of renal dysfunction (in patients with mutations in PKD1 or PKD2). Although liver failure or complications of advanced liver disease is rare, some patients develop massive hepatic cystic disease and become clinically symptomatic. There is no effective medical therapy. Treatment options include cyst aspiration and sclerosis, open or laparoscopic cyst fenestration, hepatic resection, and liver transplantation.

Anorectal malformations and their impact on survival.

OBJECTIVE: To evaluate the incidence, types and the effect on outcome of associated anomalies in neonates with anorectal malformations (ARM). METHODS: This retrospective study was carried out on all neonates with ARM admitted to the neonatal surgical intensive care unit (NSICU) from 1998 through 2003. RESULTS: Of the 754 neonates admitted to the NSICU during the study period of 6 years, there were 124 (16.4%) neonates with anorectal malformations. Of these 110 were included in the study. 73 % were male and 27% female. 86% of these were high ARM (HARM) while only 14% were low ARM (LARM). Associated anomalies were seen in 68% of patients. The incidence was 72% for HARM and 50% for LARM. The major associated anomalies consisted of esophageal (13%), gastrointestinal (GIT) (11%), genitourinary (GUT) (32%), skeletal (26%), cardiac (33%) and miscellaneous 26%. The overall survival rate was 84% (82% for HARM and 94% for LARM). The survival among those with associated esophageal anomalies was 43%, GIT 67%, GUT 80%, cardiac 61%, skeletal 76% and miscellaneous 79% respectively. This difference in survival was significant only for those with esophageal (p=0.004) and cardiac anomalies (p=0.0026). The survival rates among those with one, two or more than two organ systems involved with associated anomalies were 88%, 82% and 58% respectively. This difference was significant only for more than two organ systems involvement (p=0.003). CONCLUSION: Associated anomalies are common in neonates with ARM, the incidence being similar for HARM and LARM. The survival depends upon the number and severity of associated anomalies both in patients with LARM and HARM. Neonates with more number of organ systems involved have a poorer survival specially when associated with esophageal and cardiac anomalies. All neonates with ARM merit a meticulous search for associated anomalies so that the management can be tailored for each baby.

[Advances in the treatment of newborns with congenital malformations]. / Postepy w leczeniu noworodków z wadami wrodzonymi wymagajacymi operacji bezposrednio po urodzeniu.

Authors analyzed the type and the number of treated congenital malformations in 544 newborns operated between 1992-2001 in the Department of Paediatric Surgery in the Institute of Mother and Child. The patients were divided in the following groups: digestive tract defects, abdominal wall and diaphragm defects, neural tube defects, urinary track defects, craniofacial and brain defects and others anomalies occurring rarely. Most of the operations were preformed in the first 48 hours of life. Since 1995 special newborn transport, early cardiac surgery and neonatal intensive care have been introduced. Total mortality of operated newborns and death in particular groups were analyzed. The implemented elements caused a decrease in mortality from 36 to 13 percent. In the authors' opinion improvement in treatment results is due to earlier diagnosis and better understanding of pathophysiology of the defects, introduction of noninvasive pre- and postnatal diagnostics and establishment of centres specialized in neonatal surgery and intensive care.

Malformaciones digestivas, 1996-2001 / Digestive malformations, 1996-2001

Se realizó un estudio descriptivo y retrospectivo de los 114 casos con malformaciones digestivas ocurridas en la provincia Granma; atendidas en el Hospital Provincial Docente, Carlos Manuel de Céspedes de Bayamo en el período comprendido desde el 1ro de enero de 1996 hasta el 31 de diciembre del 2001. Se encontró una incidencia de 0,8 por cada 1000 nacidos vivos, diagnosticándose por ultrasonografía prenatal 50,9 por ciento y de estas 12 por ciento se diagnóstico tardíamente, otro 12 por ciento se negó a la interrupción del embarazo. Se le realizó diagnóstico posterior al nacimiento al 49,1 por ciento de los casos. De los 56 casos operados, fallecieron 55,4 por ciento y de éstas malformaciones las más frecuentes fueron la hernia diafragmática, la atresia esofágica y atresia intestinal. Fallecieron con menos de 7 días (61,3 por ciento) de estas, la atresia esofágica con 36,8 por ciento, la hernia diafragmática con 31,6 por ciento y la atresia intestinal con 26,3 por ciento fueron los de mayor mortalidad. En los últimos 2 años se reportó una disminución de la mortalidad, siendo de 2 casos por año representado por 6,5 por ciento(AU)

Hernia diafragmática en la provincia de granma, Quinquenio 1996-2000 / Diaphragmatic hernia in Granma Province. Quinquennium 1996-2000

Se muestran los resultados de un estudio descriptivo y retrospectivo de la hernia diafragmática en la provincia de Granma, las cuales fueron atendidas en el Hospital General Docente Carlos Manuel de Céspedes de Bayamo, en el período comprendido desde el 1 de enero de 1996 hasta el 31 de diciembre del 2000. Se estudiaron diferentes variables y se encontró un incidencia de 2,9 por cada 10 000 gestaciones, se diagnosticaron por estudios prenatales 35 por ciento de los casos y 64,3 por ciento al nacer, la localización más frecuente fue la posterolateral izquierda con 66,7 por ciento, los resultados a la corrección quirúrgica fue de 33 por ciento. La hernia diafragmática (66,7 po ciento), la atresia intestinal (66,7 por ciento) y la atresia esofágica (50 por ciento) fueron las anomalías del tractus digestivo que tuvieron mayor mortalidad posquirúrgica(AU)

Hernia diafragmática congénita: nuestra experiencia retrospectiva / Congenital diaphragmatic hernia: our restrospective experience

Se presenta un informe retrospectivo de 22 casos de hernia diafragmática congénita. Se dividieron en dos grupos de acuerdo al momento del diagnóstico, siendo prenatal en 11 pacientes y posnatal en la mitad restante. En el primer grupo el diagnóstico se hizo entre las 14 y 36 semanas de embarazo y los partos ocurrieron entre las 27 y 39 semanas. Seis niños fueron sometidos a cirugía correctora, sobreviviendo sólo 2; los otros 5 fallecieron antes de ser intervenidos, mostrando una mortalidad de 81,8 por ciento. En el segundo grupo se hizo diagnóstico entre el primer día de vida y los 11 meses de edad. Todos se sometieron a cirugía y dos fallecieron en el postoperatorio, con una mortalidad de 18,2 por ciento. La mortalidad general fue de 50 por ciento (11/22). Los resultados muestran que el diagnóstico prenatal de este defecto congénito se asocia a mayor mortalidad neonatal y se discute cuáles serían las razones de ello, así como sus implicancias para el tratamiento

Alimentary tract duplications in children: report of 26 years' experience.

Duplications of the alimentary tract are one of the rare anomalies of the gastrointestinal system. Because of the wide spectrum of the signs and symptoms, preoperative diagnosis frequently cannot be made. A close familiarity with clinical and surgical characteristics provides appropriate management and treatment of duplications. A retrospective clinical study was conducted to evaluate clinical and surgical characteristics and the treatment of duplications of the alimentary tract. During a 26-year period between 1971 and 1997, 38 patients with duplications of alimentary tract underwent operation at the Hacettepe University Department of Pediatric Surgery. Forty-two duplications in 38 patients (20 male, 53%; 18 female, 47%) were encountered. Sixty-nine percent of the patients were symptomatic under the age of one year, with 24 percent presenting with symptoms in the neonatal period. There were one sublingual, nine intrathoracic (including 2 thoracoabdominal) and 32 intraabdominal duplications. Abdominal mass, abdominal distention, constipation, vomiting and respiratory distress were the most frequently encountered signs and symptoms. Plain thoracic and abdominal X-rays, ultrasonography, and computed tomography of the chest and abdomen were the most commonly used diagnostic radiological methods. Thirty-three duplications (79%) were spherical and nine (21%) were tubular. Multiple duplications were encountered in two patients (5.3%). Fourteen duplications (33%) contained heterotopic mucosa, mostly gastric type. More than one type of heterotopic mucosa in the same duplication was encountered in four duplications (10%). Additional malformations were encountered in 26 percent of patients. Six patients (15.8%) died from unrelated causes. The signs and symptoms vary among duplications. Signs and symptoms leading to diagnosis and surgery varied according to the age of patient, location of the duplication, type of mucosal lining, duration of disease and presence of complication. The ideal surgical treatment of duplication is complete excision. However, the other treatment options should be well known.