Congenital mixed giant hiatal hernia in a four-month-old infant. / Hernia hiatal mixta congénita gigante en lactante de cuatro meses.

INTRODUCTION: Congenital hiatal hernia is a rare pathology, presenting at 28 months of age on average. Paraesophageal/mixed hernias cause recurrent respiratory infections, vomiting, anemia, and growth failure. CLINICAL CASE: Four-month-old infant, with irritability since birth and partial feeding intolerance in the last 24 hours. A partial intrathoracic stomach was evidenced in the esophago-gastro-duodenal contrast study. A thoraco-abdominal CT scan was carried out, with giant mixed hiatal hernia, right posterolateral diaphragmatic hernia, and congenital short esophagus being considered as potential diagnoses. A giant mixed hiatal hernia was noted during surgery. Laparoscopic herniorrhaphy and Nissen fundoplication were performed. DISCUSSION: In the pediatric population, Bochdalek's hernia and Morgagni's hernia are the most frequent congenital diaphragmatic hernias. Hiatal hernia is rare and causes gastrointestinal symptoms more frequently. Surgery is the treatment of choice, with the objective of preventing or minimizing these symptoms as well as gastric volvulus.

INTRODUCCION: La hernia hiatal congénita es poco frecuente, con una edad media de presentación a los 28 meses de vida. Las hernias paraesofágicas/mixtas provocan más frecuentemente infecciones respiratorias repetitivas, vómitos, anemia y fallo de medro. CASO CLINICO: Nos encontramos ante un lactante de 4 meses que presenta irritabilidad desde el nacimiento y rechazo parcial de las tomas en las últimas 24 horas. En el tránsito esófago-gastro-duodenal se evidencia un estómago parcialmente intratorácico. Tras realizarse una tomografía computarizada toraco-abdominal, se plantean como diagnósticos hernia hiatal mixta gigante vs. hernia diafragmática posterolateral derecha vs. esófago corto congénito. Intraoperatoriamente se visualiza hernia hiatal mixta gigante, realizándose herniorrafia laparoscópica y funduplicatura de Nissen. COMENTARIOS: Las hernias con afectación diafragmática más frecuentes en Pediatría son la hernia de Bochdalek y de Morgagni. La hernia hiatal produce más frecuentemente síntomas gastrointestinales; así, el tratamiento es quirúrgico, con el objetivo de evitar o minimizar dichos síntomas y prevenir las consecuencias de la volvulación gástrica.

Congenital intrathoracic stomach can be safely managed laparoscopically.

PURPOSE: Congenital intrathoracic stomach (CIS) is an uncommon pediatric surgical diagnosis where > 2/3rd of the stomach lies within the chest through a hiatus defect. We reviewed our recent experience with this condition. METHODS: A retrospective single-center review of children with a diagnosis of CIS (2007-2018) was performed. Patient demographics, presentation, imaging and management were assessed. Results are expressed as median (range). RESULTS: Eleven patients (6 girls) were identified with onset of symptoms at 2 (0-26) months of age. Presenting symptoms were vomiting (8/11), respiratory symptoms (4/11) and failure to thrive (2/11). Two patients had Marfan's syndrome. An upper gastrointestinal contrast study demonstrated gastric herniation in all. All were corrected laparoscopically with hiatus repair and fundoplication [age at surgery 10.5 (1.5-34.5) months]. A concurrent gastrostomy was done in children ≤ 6 months (n = 5). Enteral feeds were commenced on post-operative day one in 9 and second post-operative day in 2. At 7 (0-95) months follow-up, all were on full enteral feeds. One patient had a recurrence 6 months post-operatively, which was re-operated laparoscopically without any further recurrence. CONCLUSION: This is the largest reported series of children with CIS. All could be managed laparoscopically with no conversions and a low recurrence.

Radiographic confirmation of feeding tube placement: a diagnostic tool identifying gastrointestinal anomalies.

Feeding tubes are commonly used in neonatal intensive care units, and their abnormal position seen on radiographs may indicate underlying serious problems. We recently cared for two infants who presented with clinical deterioration. An abnormally placed feeding tube seen on the chest radiograph revealed underlying serious conditions. The first case was an infant 29 weeks of age who presented with right-sided pneumothorax after birth. By history and a right-side-displaced orogastric (OG) tube, iatrogenic esophageal perforation was diagnosed. The second case was a 16-day-old infant who presented with recurrent vomiting. An OG tube extending into a cystic mass at the right cardiophrenic angle resulted in diagnosis of a herniated stomach with organoaxial-type volvulus, which required surgical repair. Both cases recovered uneventfully. As illustrated in these two rare cases, feeding tube position is not only important for feeding practice, but it also has diagnostic implications in newborn infants.

Laparoscopic treatment of type III para-oesophageal hernia.

Type III congenital para-oesophageal hernia is a rare condition in children and is characterised by the herniation of both a substantial portion of the stomach and the gastro-oesophageal junction into the chest. This report describes the laparoscopic repair of 4 para-oesophageal hernias in children between 2002 and 2010. All hernias were treated successfully using the laparoscopic method. There were no recurrences. The laparoscopic repair of a para-oesophageal hernia is technically challenging, but is feasible and safe in the hands of paediatric surgeons familiar with laparoscopic anti-reflux surgery.

Gastric dissociation for the treatment of congenital microgastria with paraesophageal hiatal hernia.

Microgastria is a rare but well-described congenital anomaly of the alimentary tract that presents in the neonatal period with vomiting, aspiration, and failure to thrive. Based on a relatively small number of case reports, gastric augmentation with a double-barrel loop of jejunum, known as a Hunt-Lawrence pouch, has been advocated as the reconstructive procedure of choice in affected children who fail nonoperative management. In this report, we present a novel method of foregut reconstruction in an infant with congenital microgastria and a paraesophageal hiatal hernia. In this procedure, the stomach was transected 1 cm below the gastroesophageal junction with construction of a straight Roux-en-Y jejunal anastomosis to the gastric fundic cuff. A feeding gastrostomy tube was placed into the distal remnant stomach for enteral access. The patient did well and eventually transitioned to full oral feeds by 3 years of age.

Laparoscopic management of neonatal paraesophageal hernia with intrathoracic gastric volvulus.

Intrathoracic gastric volvulus associated with neonatal paraesophageal hernia is very rare in the newborn period. We report a case of a 3-week-old term infant who presented to the hospital with a history of non-bilious vomiting. Workup for hypertrophic pyloric stenosis eventually revealed the presence of a congenital hiatal hernia and intrathoracic gastric volvulus requiring urgent surgical management. The infant underwent successful laparoscopic repair. We discuss the diagnosis and management of this extremely rare surgical cause of neonatal nonbilious emesis.

Congenital para esophageal hernia: a 10 year experience from Saudi Arabia.

OBJECTIVE: Para esophageal hiatal hernia is a rare childhood condition and reported series have had scant number of children which makes diagnosis a challenge. The authors sought to study the presentation and the outcome of treatment of congenital para esophageal hernias (CPEH) over a period of 10 years from a single tertiary care hospital in Saudi Arabia. METHODS: The records of 9 patients presenting between 1997 and 2007, were retrospectively analyzed for demographics, presenting features, referral diagnoses, investigations, management including operative procedures, their outcome and follow-up. RESULTS: Nine patients (3 males and 6 females) aged between 8 days to 34 months were seen. Respiratory distress (n=6), vomiting (n=5) and frequent respiratory tract infections (n=3) were the most common presentations. Cyanosis (n=2), cough and excessive crying were the other important symptoms. The referral diagnoses in these patients included congenital Bochdalek's hernias, lung abscess, bronchogenic cyst, pneumatocoele, bronchiolitis, and pneumonias which reflected a misinterpretation of their clinical findings and chest X-rays. Seven of these patients had other associated congenital anomalies. Three had cardiovascular abnormalities and 2 had lesions of the central nervous system. A pair of siblings had Marfan's syndrome. All the patients had abnormal chest C-rays and an UGS (upper GI series) proved to be diagnostic in 8 patients. The CT scans done in 4 patients corroborated the findings of the UGS. A laparotomy was done on most patients (n=8) which comprised of reduction of the stomach, resection of the hernial sac, tightening of the hiatus and a gastropexy or a gastrostomy. One patient, who underwent thoracotomy died of surgical complications. Two others died of causes unrelated to the surgery. The remaining six operated patients have been followed up for a median of 3.5 years and are doing well. CONCLUSION: CPEH is uncommon in children, presented with respiratory tract symptoms and vomiting, and may be associated with Martan's syndrome. It should be considered in the workup of a child with vomiting or frequent chest infections. Abnormal chest X-rays may indicate the diagnosis and a subsequent UGS, is confirmatory. The present study found the aparotomya good approach for repair of the wide hiatus. A gastropexy and a floppy fundoplication were added to prevent reherniation and post operative reflux though given the small numbers it is not possible to determine the place of either of these procedures. CPEH may be frequently associated with other congenital problems which may impact survival.

Hiatal hernia in the dog: a clinical report of four Chinese shar peis.

Four Chinese shar pei littermate puppies were presented for vomiting, regurgitation, hypersalivation, and poor growth. Diagnosis of hiatal hernia was made for each of the four dogs based on survey radiographs and barium esophagram studies. All dogs initially underwent medical therapy, to which only one dog responded. All dogs underwent surgical treatment, which included manual hernia reduction followed by phrenoplasty, esophagopexy, and left incisional gastropexy, 5 to 40 days following initiation of medical therapy. The three surgical techniques described eliminated the need to enter the pleural cavity and offered excellent long-term results with no recurrence of clinical signs following surgery.

An unusual case: a giant paraesophageal hiatal hernia with intrathoracic spleen, preduodenal portal vein, malrotation, and left inferior vena cava.

A giant paraesophageal hiatal hernia with preduodenal portal vein, nonrotating gut, intrathoracic spleen, and left inferior vena cava has not been reported to date. This set of complex anomalies can have significant clinical implications. Awareness of these anomalies is essential to avoid further complications.

Impact of concomitant thoracic interventions on feasibility of Nuss procedure.

BACKGROUND: This study aimed to analyze the feasibility of subsequent minimally invasive pectus repair, particularly modified Nuss procedure, combined with simultaneous thoracic procedures for different underlying intrathoracic diseases and conditions. METHODS: A total of 110 patients, who underwent minimally invasive pectus repair in Nuss technique over a 5-year period, were retrospectively analyzed concerning complications, cosmetic results, and satisfaction. Six patients (5%) underwent the Nuss procedure with concomitant thoracic interventions. Patients with prior cardiac surgery or planned redo pectus repair were not examined and were excluded. The mean age of 6 patients (3 male and 3 female) was 11 years (range, 5.5-17.2). Two patients with former left-sided transabdominal diaphragmatic hernia repair and 1 with former lobectomy of the left lower lobe underwent thoracoscopic adhesiolysis. Two underwent thoracotomy: one for closure of a recurrent left-sided diaphragmatic hernia with fundoplication owing to a large hiatal hernia, another for lobectomy of the right middle lobe owing to recurrent infections and bronchodysplasia. One patient presented with anterior mediastinal mass, which was suspected to be benign, and underwent thoracoscopic complete resection. All patients underwent a 1-stage procedure with subsequent simultaneous Nuss procedure. RESULTS: Simultaneous Nuss procedure was feasible without intraoperative complications in all patients (100%). Thoracoscopic adhesiolysis did not affect the feasibility of the Nuss procedure in 3 patients with former diaphragmatic hernia repair, particularly former lobectomy in one. Thoracotomy with middle-lobe lobectomy, as well as repair of recurrent diaphragmatic hernia and fundoplication in 2, did not affect modified Nuss technique and dispensed thoracoscopic guidance. Histopathologic analysis in one patient with a removed anterior mediastinal mass revealed Hodgkin lymphoma (stage IA), and the patient received multiagent chemotherapy. The postoperative course was uneventful in 5 of 6 patients. One patient required intermittent drainage of pleural effusion after simultaneous lobectomy. At follow-up (6 months-5 years), 6 patients had excellent cosmetic results and good quality of life confirmed by a questionnaire. There was no evidence of recurrent malignancy in one patient after 6 months. CONCLUSIONS: Thoracic surgery and subsequent Nuss procedure can be performed simultaneously. Underlying conditions, such as prior repair of congenital diaphragmatic hernia or diaphragmatic eventration, as well as former lobectomy, had no impact on feasibility. Open thoracotomy can be combined with Nuss procedure dispensing thoracoscopy.

Congenital paraesophageal hiatal hernia: pitfalls in the diagnosis and treatment.

PURPOSE: The aim of this study was to analyze pitfalls in the diagnosis and treatment of congenital paraesophageal hiatal hernia (PEHH). METHODS: Between 1992 and 2004, the records of 5 infants with PEHH were retrospectively reviewed for age, sex, presenting symptoms, radiological studies, operative findings and approaches, and outcomes. RESULTS: All cases (3 male, 2 female) had right-sided hernias. They had clinical features of recurrent chest infections and intermittent vomiting that were present since birth in 3. Three presented acutely ill with findings of respiratory distress and vomiting. Three were referred with misdiagnoses of reflux disease, thoracic mass, and bronchopneumonia. On the chest x-rays of 3 cases, there were paracardiac opacities suggesting a mass lesion. According to the upper gastrointestinal series and/or computed tomography findings, 4 cases had a combination of sliding and paraesophageal hernia, and the remainder one had pure rolling hiatus hernia. Three had obstruction owing to organoaxial volvulus and required an emergency operation. All cases had a large hernia orifice. Four had gastroesophageal junction (GEJ) displaced into the thorax, and in 3, the stomach was found to be twisted, and transverse colon with omentum was also in the thorax in 2. In the remainder, the GEJ was in its normal position with herniated stomach. None of the cases had normal gastrosplenic and gastrocolic ligaments. Surgical repair included resection of the sac, closure of the hiatal defect, and Thal procedure. Two had intestinal malrotation, with right ovarian torsion and ventricular septal defect, respectively. Postoperative ventilation was required in one who later died. At a mean follow-up of 2 years, the other 4 had no symptoms related to the disease, and no evidence of recurrence or reflux was noted on control upper gastrointestinal series. CONCLUSION: Congenital PEHH may be difficult to diagnose. It is frequently complicated and associated with morbidity and even mortality. If the defect is large and associated with displacement of GEJ into the thorax, adding an antireflux procedure to the repair is appropriate.

Prenatal diagnosis of intrathoracic stomach (gastric herniation).

Intrathoracic stomach is a rare and serious congenital abnormality. The anomaly may be complicated by gastric volvulus and can lead to ischemic gastric infarction in the neonate. If diagnosed antenatally, neonatal management can be planned in advance so as to reduce morbidity. This anomaly must be differentiated from the more common congenital diaphragmatic hernia, as associated pulmonary hypoplasia is common in the latter and rare with gastric herniation. We report an infant born to a mother with Marfan's syndrome with the antenatal diagnosis of intrathoracic stomach. The ultrasound and magnetic resonance imaging features of this congenital abnormality are described. A review of the literature would indicate that this is the first case report of gastric volvulus diagnosed in utero.

Prenatal sonographic diagnosis of congenital hiatal hernia.

OBJECTIVES: To report a rare case of congenital hiatal hernia illustrating the importance of its prenatal diagnosis as well as to discuss the prenatal sonographic criteria. CASE REPORT: A case of congenital hiatal hernia was diagnosed by ultrasound at 33 weeks of gestation. After a normal second-trimester morphologic ultrasound examination, a hypoechogenic mass was detected in the posterior mediastinum juxtaposed to the vertebral body and seemed to be in continuity with the intra-abdominal stomach bubble. Congenital hiatal hernia was suspected mainly because of the dynamic position of the stomach during the examination, without mediastinal shift, and normal appearance of the diaphragm on parasagittal sections of the thorax. Postnatal management was planned with no urgency and surgery was successfully performed, confirming the diagnosis. CONCLUSION: This rare case illustrates the importance of prenatal diagnosis of congenital hiatal hernia for prenatal counseling and postnatal management. The ultrasound criterion for prenatal diagnosis is the presence of a herniated stomach in the posterior mediastinum, sometimes having a dynamic position during examination, with no mediastinal shift associated with normal diaphragm appearance on parasagittal sections of the thorax.

Neonatal intrathoracic stomach in Marfan's syndrome: report of two cases.

The authors report experience with 2 neonates with the unusual combination of intrathoracic stomach and Marfan's syndrome.

Congenital narrowing of the intrapharyngeal opening in a dog with concurrent oesophageal hiatal hernia.

A one-year-old, male, smooth-haired standard dachshund was presented with a history of chronic hypersalivation, dysphagia, puffing of the cheeks on expiration, and inspiratory stridor. Oral examination revealed a moderately thickened tongue radix and that the normal intrapharyngeal opening was obliterated. A 7 mm long, midline palatal slit was the only communication between the naso- and oropharynx. The soft palate was fused to the caudal pharyngeal wall. A concurrent hiatal hernia was diagnosed on thoracic radiographs. The soft palate abnormality was surgically corrected and the hiatal hernia was managed medically. On follow-up evaluations, the clinical signs had markedly improved, and the hiatal hernia was no longer visible on survey thoracic radiographs.

Laparoscopic intervention for intrathoracic stomach in infants.

BACKGROUND: Intrathoracic stomach is an uncommon condition in infants. We report our experience managing such a condition successfully by laparoscopy in four patients. METHODS: Patients' ages at the time of operation ranged from 30 days to 14 months. In all cases, the intrathoracic stomach was easily pulled down into the abdominal cavity. The phrenoesophageal ligament was completely resected, and the enlarged hiatus was narrowed by intraabdominal suturing technique. The esophagus was wrapped with the mobilized fundus in a floppy fundoplication. Anchoring sutures were placed between the wrapping cuff and crura. RESULT: One patient had paraesophageal hernia (type 2), whereas the other had combined hiatal hernia (type 3). No adverse complications were observed in any of the cases. Symptomatic gastroesophageal reflux and radiographic recurrence of hernia were not seen in any case. The cosmesis was excellent in all cases. CONCLUSIONS: We conclude that laparoscopic repair for intrathoracic stomach is a safe and feasible method when preoperative evaluation is conducted adequately.

Laparoscopic repair of an extrahiatal hernia caused by congenital dysplasia: a case report.

A 40-year-old woman with complaints of relapse in the upper abdomen and dysphagia was referred for laparascopic hiatal hernia repair. Chest radiograph, barium-swallow, and upper endoscopy revealed a paraesophageal hernia. Esophageal manometry and 24-h-pH study showed no pathological findings. A laparoscopic gastropexy was planned. Intraoperatively, in contradiction to the preoperative findings, an extrahiatal hernia containing most of the stomach was found. After resection of the hernia sac, the beating heart without covering pericardium was seen. These findings were confirmed by an additional thoracoscopy at the end of the operation. The defect was closed by direct suturing. The postoperative course and 2-month follow-up were uneventful. The resected parts of the hernia sac showed an embryonic and dysgenetic etiology. This rare malformation has been reported in combination with complex syndromes, which appear with serious clinical and morphological signs in the neonatal period. In adults, the pericardial aplasia can be observed during diagnostic or surgical interventions. In these patients, complaints are usually not caused by the malformation but may be due to the occasional herniation of abdominal organs. We consider laparoscopic repair to be a gentle and safe procedure for the treatment of extrahiatal hernias.

Serial sonographic findings in a fetus with congenital hiatal hernia.

A continuum of prenatal findings in a case of hiatal hernia is described. Second-trimester scans showed absence of fetal stomach and polyhydramnios suggestive of esophageal atresia. Third-trimester scans revealed a dilated tubular structure in the thoracic cavity with intermittent visualization of an intra-abdominal small stomach. A diagnosis of hiatal hernia was entertained. After birth, the diagnosis of a dilated esophagus with the stomach herniated into the thoracic cavity through a very lax esophageal hiatus was confirmed and the baby underwent corrective surgery.