Paraduodenal hernia. / Paraduodenalt brokk.

Paraduodenal hernia is a rare form of congenital internal hernia and can lead to small bowel obstruction. This case report describes the case of a young boy who was admitted with acute exacerbation of chronic abdominal pain.

A Rare Case of Small Bowel Obstruction Due to Paraduodenal Hernia.

BACKGROUND Paraduodenal hernia, or mesocolic hernia, is a rare congenital form of internal abdominal hernia, and is classified into right and left, with left paraduodenal hernia being more common than right. Patients can have non-specific symptoms and signs, which make the diagnosis challenging. However, patients may present with obstruction or ischemia requiring emergency surgery. This report is of a case of left paraduodenal hernia in a 24-year-old woman who presented with acute abdominal pain at two-weeks post-partum and who was managed with open surgery with good clinical outcome. CASE REPORT A 24-year-old Saudi woman presented with recurrent chronic abdominal pain that was managed conservatively for the previous six years. At two-weeks post-partum, she presented with acute abdominal pain, vomiting, and constipation. Computed tomography (CT) of the abdomen showed a left paraduodenal hernia. The patient underwent timely open laparotomy due to the presence of bowel dilatation. Her postoperative recovery was uneventful. CONCLUSIONS Although rare, this case has shown that paraduodenal hernia can cause intestinal obstruction. CT imaging increases the chance of early diagnosis and timely surgery. Although laparoscopic surgical repair may be preferable in some cases, this patient underwent successful open repair.

Síndrome lumbocostovertebral: primer reporte de caso en Argentina / Lumbocostovertebral syndrome: first case report in Argentina

El síndrome lumbocostovertebral se define por la presencia de hernia lumbar, hemivértebras y anomalías costales. El objetivo de este trabajo es describir el primer caso reportado en Argentina. El paciente fue comunicado a la Red Nacional de Anomalías Congénitas de Argentina. Se describe el cuadro clínico, los diagnósticos diferenciales y los posibles mecanismos patogénicos involucrados. Se sugiere que esta entidad sea considerada como una asociación. La hernia lumbar en un recién nacido es un hallazgo infrecuente y debe pesquisarse la presencia de otras anomalías asociadas.

Lumbocostovertebral syndrome is defined by the presence of lumbar hernia, hemivertebrae and costal anomalies. Our aim was to describe the first case reported in Argentina. The patient was reported to the National Registry of Congenital Anomalies of Argentina. The clinical picture, differential diagnoses and possible pathogenic mechanisms involved are described. We suggest considering this as a lumbocostovertebral association. Lumbar hernia in a newborn is an infrequent finding and other associated anomalies should be evaluated.

Isolated congenital inter-costal pulmonary hernia: a case report.

BACKGROUND: Intercostal lung herniation is a rare condition that may be congenital (20%) or acquired (80%). The isolated congenital form is exceptional, with one case reported in the literature. CASE PRESENTATION: We report a case of a 10-year-old French boy of Algeria origin, born with intermittent swelling of his right hemithorax. The swelling and pain gradually increased with age. A clinical examination revealed a localized swelling of his right hemithorax at the level of the midclavicular line and the fifth intercostal space. The swelling increased in size during respiratory movements and enlarged with Valsalva maneuvers. The intercostal lung hernia was treated by thoracoscopy. CONCLUSIONS: This is the second case of isolated congenital intercostal pulmonary hernia reported in the French and English literature. It is the first to be treated by thoracoscopy. Based on this case we performed a review of the diagnosis and therapeutic aspect of pulmonary hernias.

A multicentre study to predict neonatal survival according to lung-to-head ratio and liver herniation in fetuses with left congenital diaphragmatic hernia (CDH): Hidden mortality from the Latin American CDH Study Group Registry.

OBJECTIVE: To evaluate natural history of fetuses congenital diaphragmatic hernia (CDH) prenatally diagnosed in countries where termination of pregnancy is not legally allowed and to predict neonatal survival according to lung area and liver herniation. METHODS: Prospective study including antenatally diagnosed CDH cases managed expectantly during pregnancy in six tertiary Latin American centres. The contribution of the observed/expected lung-to-head ratio (O/E-LHR) and liver herniation in predicting neonatal survival was assessed. RESULTS: From the total population of 380 CDH cases, 144 isolated fetuses were selected showing an overall survival rate of 31.9% (46/144). Survivors showed significantly higher O/E-LHR (56.5% vs 34.9%; P < .001), lower proportion of liver herniation (34.8% vs 80.6%, P < .001), and higher gestational age at birth (37.8 vs 36.2 weeks, P < 0.01) than nonsurvivors. Fetuses with an O/E-LHR less than 35% showed a 3.4% of survival; those with an O/E-LHR between 35% and 45% showed 28% of survival with liver up and 50% with liver down; those with an O/E-LHR greater than 45% showed 50% of survival rate with liver up and 76.9% with liver down. CONCLUSIONS: Neonatal mortality in CDH is higher in Latin American countries. The category of lung hypoplasia should be classified according to the survival rates in our Latin American CDH registry.

Congenital lumbar hernia-A feature of diabetic embryopathy?

Congenital lumbar hernia is a rare anomaly consisting of protrusion of abdominal organs or extraperitoneal tissue through a defect in the lateral abdominal wall. The majority of affected patients have additional anomalies in a pattern described as the lumbocostovertebral syndrome. We report four patients born to mothers with poorly controlled diabetes with congenital lumbar hernia. All patients exhibited features of lumbocostovertebral syndrome with lumbar hernia, multiple vertebral segmentation anomalies in the lower thoracic and/or upper lumbar spine, rib anomalies, and unilateral renal agenesis. Additional anomalies present in the patients included preaxial hallucal polydactyly, abnormal situs, and sacral dysgenesis, anomalies known to be associated with diabetic embryopathy. At least 11 other patients have been previously reported with the lumbocostovertebral syndrome in the setting of maternal diabetes. We suggest that congenital lumbar hernia and the lumbocostovertebral syndrome are related to diabetic embryopathy.

Early prenatal diagnosis of a lumbo-costo-vertebral syndrome.

Lumbo-costo-vertebral syndrome (LCVS) is a rare type of lumbar hernia with associated abnormalities of the vertebral bodies, ribs, and trunk muscles. Only a few cases have been reported in the literature, all of which were diagnosed after birth. We present a case of LCVS diagnosed early in the second trimester of pregnancy using two- and three-dimensional ultrasound. In our case, the associated anomalies were: multiple costovertebral anomalies, lumbar hernia, anal imperforation, left hand supernumerary digit, and clubfoot.

Congenital Diaphragmatic Hernia with Liver Herniation into the Pericardial Sac in a 30-Week Gestation Infant.

Anterior diaphragmatic defects with pericardial involvement are extremely rare and diagnostically challenging entities encountered perinatally. While a majority of diaphragmatic defects occur in isolation, others are associated with multiple defects forming a complex of syndromes such as Pentalogy of Cantrell. Liver herniation into the pericardial sac poses a particular challenge and can mimic a pericardial tumor on prenatal ultrasound, yielding a different management course. The following case is an unusual presentation of a 30-week gestation female with an anterior midline diaphragmatic defect with liver herniation mimicking as a pericardial tumor, diagnosed at time of autopsy. Postmortem studies also found multiple congenital anomalies including an atrioventricular septal defect and midline gumline defect suggesting at least a partial Pentalogy of Cantrell or variant. Early recognition and screening for associated anomalies are essential for management in this subset of patients.

Acute gastrointestinal manifestation of situs inversus abdominus.

PURPOSE: Whilst situs inversus is associated with intestinal malrotation and volvulus particularly in infants, this is the first known report of acute intestinal obstruction in an adult patient with a situs anomaly specifically due to a congenital transmesenteric hernia. CASE: A 54-year-old woman presented with a 12-h history of progressive abdominal pain. Contrast-enhanced computed tomography scan of the abdomen revealed an incidental finding of situs inversus abdominus, ischemic small bowel obstruction and a potential occlusive thrombus of the superior mesenteric artery. At operation, the cause of intestinal obstruction was found to be secondary to herniation of the intestine through a congenital mesenteric defect. The hernia was reduced and a strong pulse was subsequently felt in the distal superior mesenteric artery. The patient required resection of a segment of compromised small bowel, and was later anastomosed at a planned second laparotomy. Her laparostomy was closed using an absorbable BioA mesh. CONCLUSION: A high index of suspicion and low threshold for exploratory surgery should be maintained in patients with major congenital abdominal anomalies presenting with an acute abdomen.

Abdomen agudo por hernia interna congénita en un preescolar. Comunicación de un caso / Acute abdomen by congenital internal hernia in a preschool child. Case report

Introducción. La hernia interna se presenta raramente, sobre todo en niños, con cuadro de dolor abdominal agudo y obstrucción intestinal. El intestino incarcerado y estrangulado, puede progresar a isquemia, necrosis y perforación, con mortalidad hasta del 50%. El diagnóstico preoperatorio es un reto, a pesar de imágenes por tomografía computarizada, y el índice de sospecha es muy bajo. Presentación del caso. Niño de 5 años, de Cuernavaca, Morelos, México. Previamente sano, con dolor abdominal intenso y vómitos de 24 horas de evolución, sin fiebre ni diarrea. Análisis, radiografías y ultrasonido orientaron a apendicitis aguda. Durante la laparotomía, se encontró isquemia intestinal de la parte herniada, en un defecto mesentérico congénito. Evolucionó a necrosis tras la primera cirugía. Requirió resección e ileostomía en la segunda y anastomosis término-terminal en la tercera. Se recuperó y fue dado de alta bien en una semana. Discusión. Existen pocos reportes de hernia interna transmesentérica congénita en niños. Por su infrecuencia, no se sospecha, y se retrasa el diagnóstico y el tratamiento, dando resultados catastróficos. Encontramos 23 casos descritos, 5 de ellos en México. Solo 4 casos son de niños en edad preescolar. Necesitamos mayor índice de sospecha para diagnosticarla, sobre todo en niños con cuadro inespecífico de abdomen agudo

Introduction. The internal hernia occurs rarely, especially in children, with picture of acute abdominal pain and intestinal obstruction. Incarcerated and strangulated bowel may progress to ischemia, necrosis and perforation, with mortality up to 50%. Preoperative diagnosis is a challenging, despite computed tomography imaging, and the index of suspicion is very low. Presentation of the case. 5 year old boy from Cuernavaca, Morelos, Mexico. Previously healthy, he presented with severe abdominal pain and vomiting lasting for 24 hours. Neither fever nor diarrhea were present. Laboratory tests, X-rays and ultrasound studies suggested acute appendicitis. Intestinal ischemia was found in a part of small bowel herniated through a congenital mesenteric defect. It evolved to necrosis after the first surgery. The child required an intestinal resection and ileostomy in the second procedure and whole anastomosis in the third. He recovered well and was discharged in a week

Jarcho-Levin Syndrome with Splenic Herniation: A Rare Presentation.

BACKGROUND Jarcho-Levin syndrome, also known as spondylothoracic dysplasia and spondylocostal dysplasia, is characterized by varieties of vertebrae and rib anomalies. Jarcho-Levin syndrome is a clinical-radiological diagnosis with clinical evidence of short neck, short trunk, normal-sized limbs, or increased arm span, and vertebral and rib defects on the skeletal survey. CASE REPORT About 400 cases have been reported in world literature and 18 in our Indian literature. We report the case of a one-day-old female baby with a short trunk, short neck, low hairline, apparently long limbs, protuberant abdomen, mild midfacial dysmorphism, low-set ears, and a high-arched palate. There was one cystic swelling over the lateral side of the left hypochondrium sized about 3×3 centimeters, nonpusatile; the skin over the swelling was normal color and free. Radiological findings showed crowding of ribs with pebble-like appearance of the vertebrae and diastematomyelia of the spinal cord (type 2). We report here the first case of Jarcho-Levin syndrome with splenic herniation. To the best of our knowledge there have been no case reports of Jarcho-Levin syndrome with splenic herniation in the literature. CONCLUSIONS Jarcho-Levin syndrome can be easily diagnosed by clinical-radiological findings in newborns, with short trunk having a high index of suspicion. Prenatal diagnosis using level 2 ultrasonography can make it easier to manage the baby after delivery. Management should be from the basic neonatal care to prevention and immediate treatment of recurrent respiratory infections. Spinal surgical intervention to improve the thoracic volume and hence decrease the pulmonary restriction has been tried.

Prenatally diagnosed severe CDH: mortality and morbidity remain high.

PURPOSE: This study sought to evaluate prenatal markers' ability to predict severe congenital diaphragmatic hernia(CDH) and assess this subgroup's morbidity and mortality. METHODS: A retrospective review was performed between 2006 and 2014. Prenatal criteria for severe CDH included: liver herniation, lung-to-head ratio (LHR) <1 on prenatal ultrasound and/or observed-to-expected LHR (o/eLHR) <25%, and/or observed-to-expected total lung volume (o/eTLV) <25% on fetal MRI. Postnatal characteristics included: mortality, ECMO utilization, patch closure, persistent suprasystemic pulmonary hypertension (PHtn), O2 requirement at discharge, and few ventilator-free days in the first 60. Statistics performed used unpaired t-test, p<0.05 significant. RESULTS: Overall, 47.5%(29/61) of patients with prenatally diagnosed, isolated CDH met severe criteria. Mean LHR: 1.04±0.35, o/eLHR: 31±10% and o/eTLV: 20±7%. Distribution was 72% LCDH, 24% R-CDH. Overall survival: 38%. ECMO requirement: 92%. Patch rate: 91%. Mean ventilator-free days in 60: 7.1±14. Supplemental oxygen at discharge was required in 27%. In this prenatally diagnosed severe cohort, 58%(15/26) had persistent PHtn post-ECMO requiring inhaled nitric oxide±epoprostenol. Comparing patients with and without PHtn: mean ECMO duration 18±10 days versus 9±7 days (p=0.01) and survival 20% versus 72% (p=0.006). CONCLUSION: A combination of prenatal markers accurately identified severe CDH patients. Outcomes of this group remain poor and persistent PHtn contributes significantly to mortality.

ALOBAR HOLOPROSENCEPHALY, CLEFT LIP/PALATE, URORECTAL SEPTUM MALFORMATION SEQUENCE AND CONGENITAL PERINEAL HERNIA IN A FETUS.

We report on a fetus with alobar holoprosencephaly, complete cleft lip and palate, urorectal septum malformation sequence and perineal hernia. To our knowledge this appears to be a novel fetal malformation syndrome.

Congenital mesenteric hernia in neonates: Still a dilemma.

Congenital transmesenteric hernia in neonates is a rare cause of intestinal obstruction with devastating outcomes and still remains a challenge to diagnose pre-operatively. Patients are often managed with emergency surgical exploration and may need bowel resection. We present 2 neonates with small bowel obstruction secondary to strangulated transmesenteric hernia through a congenital defect in the small bowel mesentery, which were managed successfully. We have also reviewed the literature about congenital transmesenteric hernia in neonates.

Cistocele escrotal incidental. Signo de la mancuerna / Incidental finding of scrotal. Cystocele dumbbell sign

No disponible

Proximal drainage plus massage of lacrimal sac improves the symptoms of congenital dacryocystoceles.

PURPOSE: To examine the effect of proximal drainage plus lacrimal sac massage in patients with congenital dacryocystoceles. METHODS: A retrospective review of 77 patients was performed. Traditional massage was performed in the traditional treatment group (35 patients) first. Proximal lacrimal sac drainage plus massage of the lacrimal sac using a Bangerter lacrimal cannula (defined as modified massage) was performed in the modified group (42 patients). Probing was undertaken if the treatments failed in both groups. Success rates of decompression of lacrimal sac and recovery rate were observed. The χ2 test was used to compare the groups. RESULTS: Success rates of decompression of lacrimal sac were 100% in the modified group and 31% in the traditional group. In the modified group, 34 patients with 37 dacryocystoceles (80%) recovered with the modified treatment within 14 days. However, in the traditional group, only 11 patients with 12 dacryocystoceles (30%) had resolution after conservative treatment. The recovery rate of the modified massage in the modified group was higher than that of massage alone in the traditional group (p<0.001). As to the individuals receiving probing, 8 patients with 9 dacryocystoceles (100%) in the modified group were successful, while 18 patients with 21 dacryocystoceles (78%) in the traditional group had a satisfying effect. CONCLUSIONS: Proximal drainage plus massage has the advantage of high efficiency and safety for the treatment of congenital dacryocystoceles.

Mesenteric Defect with Internal Herniation: A Rare Cause of Bowel Obstruction in Newborn.

Herniation through a congenital mesenteric defect is a rare cause of intestinal obstruction in the newborn. Early diagnosis and surgical treatment improves the prognosis. We present a case of a full-term infant who presented with respiratory distress at birth. Enteral feeding was not started because abdominal distension and delayed passage of meconium. Bowel obstruction was suspected. Radiological investigation did not provide a clear diagnosis. Surgical exploration revealed transmesenteric congenital hernia. After surgical repair, enteral feeding was tolerated and patient was discharged with an uneventful outcome. Diagnostic difficulties were discussed.

Divertículo vesical adquirido. Caso clínico / Acquired bladder diverticulum. A clinical case

El divertículo vesical (DV) puede ser congénito o adquirido. Consiste en una herniación de la mucosa vesical. Los adquiridos se deben a un mecanismo de hiperpresión endovesical generalmente en pacientes con obstrucción infravesical secundaria a hiperplasia benigna de próstata (HBP), patología del cuello vesical o uretral. Cuando los DV son sintomáticos o producen complicaciones se debe plantear tratamiento quirúrgico. En los últimos años la diverticulectomía laparoscópica ha presentado muy buenos resultados aunque la cirugía abierta está indicada en algunos casos más complejos. En DV adquiridos de tamaño pequeño y mediano está indicado el tratamiento transuretral. Se presenta un caso clínico y una revisión de diferentes aspectos diagnósticos y quirúrgicos del DV (AU)

The bladder diverticulum (DV) can be congenital or acquired. It consists of a herniation of the bladder mucosa. The acquired is due to a mechanism of intravesical hyperpressure usually in patients with bladder outlet obstruction secondary to BPH, cervical or urethral pathology. When DV are symptomatic or produce complications surgical treatment should be considered. In recent years laparoscopic diverticulectomy has shown very good results although open surgery is indicated in some more complex cases. In small and medium acquired DV transurethral treatment is indicated. One clinical case and a review of various diagnostic and surgical aspects of DV are presented (AU)