Neonatal acute appendicitis: a strangulated appendix in an incarcerated inguinal hernia

A 4-day-old infant with acute appendicitis (AA) due to incarceration of a right inguinal hernia is presented. Although appendicitis is the most common condition requiring abdominal surgery, the reported occurrence of AA subsequent to neonatal hernia incarceration is exceptionally rare. Accepted: 25 January 2001     

Inguinal hernia in very low birthweight children: A continuing risk to age 8 years

The prevalence of inguinal hernia repair up to 8 years of age was determined in 205 inborn children of birthweight less than 1500 g who had survived to the age of 8 years; 37 (18.0%) of the children required surgery for inguinal hernia, significantly more frequently for boys than girls (32.0% and 3.9% respectively). The cumulative percentage of inguinal hernia operations in boys was 8.7% during primary hospitalization, 20.4% by 2 years, 30.1% by 5 years and 32.0% by 8 years; 36.4% (12 of 33) hernias in boys occurred beyond 2 years of age. The risk of hernia in boys was only marginally higher in those who required assisted ventilation and in those of birthweight less than 1000 g. Very low birthweight infants, particularly boys, are at risk of developing an inguinal hernia not only in infancy but also to at least 8 years of age.     

Vaginal foreign body in childhood: A multidisciplinary approach

A 5-year-old girl referred to our division for evaluation of persisting foul-smelling brown vaginal discharge with pruritus for over 2 years, not corresponding to antibiotic therapy. Transabdominal ultrasound identified a heterogeneous mass intensely fixed in the upper third of vaginal wall. Vaginography revealed a filling defect in the upper part of vagina, confirming the diagnosis of an intravaginal foreign body. The foreign body was grasped and removed by traction; it turned out to be a thin double-wrapped piece of sponge. Psychological assessment of the girl was undertaken, in order to illuminate the potential of an underlying emotional and behavioural problem and revealed the existence of psychological disturbances mostly affecting social competence and adaptiveness.     

儿童上消化道异物致消化道损伤217例回顾性分析

目的 了解儿童上消化道异物致消化道损伤的临床特点及治疗转归。方法 收集2011年1月至2016年12月行胃镜检查诊断为上消化道异物,且出现消化道损伤的217例患儿的临床资料,回顾性分析其临床特点、胃镜下表现、并发症及转归。结果 217例患儿中,1~3岁患儿占比最高（114例,52.5%）。异物以硬币最常见（99例,45.6%）,其次为硬质/尖锐食物（45例,20.7%）及金属（35例,16.1%）。消化道黏膜损伤以溃疡最多见（43.8%）,其次为糜烂（33.2%）。纽扣电池较其他异物更易引起食管穿孔（P < 0.01）。食管为最常见受损部位（207例,95.4%）。217例患儿中,24例（11.1%）并发感染;异物致穿孔患儿较异物致溃疡患儿感染发生率明显增高（P=0.003）。217例患儿中,204例（94.0%）内镜下成功取出异物。98例因黏膜损伤较重需住院治疗,予以抗感染、抑酸治疗及鼻胃管肠内营养和/或静脉营养支持治疗。10例患儿自动出院而失访,其余患儿均好转出院。结论 幼儿期为上消化道异物高发年龄段;儿童上消化道常见异物为硬币、硬质/尖锐食物及金属;纽扣电池易造成食管穿孔;消化道黏膜损伤越重继发感染率越高;内镜下异物取出术结合鼻胃管肠内营养等处理患儿预后良好。     

Acute Leukemia in an Infant with Marfan's Syndrome: A Case Report

We describe a familial case of Marfan's syndrome with associated intrathoracic stomach detected during the neonatal period. The patient developed a primitive leukemia at 3 months of age. Acute leukemia in a patient with Marfan's syndrome has not previously been reported.     

Laryngeal foreign bodies in children: first stop before the right main bronchus

Laryngeal foreign bodies may produce either complete or incomplete airways obstruction. In complete airways obstruction the presentation is with calamitous respiratory difficulty. However incomplete laryngeal obstruction may present with less severe symptoms, resulting in possible misdiagnosis and confusion with other causes of upper airway obstruction such as infectious croup. This report describes three cases of incomplete laryngeal obstruction secondary to inhaled foreign bodies. In each case, the diagnosis of an inhaled foreign body was initially missed, resulting in delay in diagnosis and in one case prolonged recovery. The importance of considering laryngeal foreign bodies, both in cases of suspected foreign body inhalation and clinical cases of incomplete laryngeal obstruction are discussed.     

The treatment of an unusual complication associated with a HeartMate II LVAD in an adolescent

The HeartMate II LVAD has provided a bridge to heart transplantation or a permanent fixture for destination therapy for patients with heart failure. LVAD infections are associated with significant morbidity even when treated with explantation, device exchange, or a salvage procedure. We present an unusual complication following the placement of the HeartMate II device in an adolescent, whereby a pocket infection resulted in a large soft tissue defect overlying and surrounding the device. The novel use of a VRAM flap was successfully used to repair the defect and salvage the device.     

Glandular Inclusions in Inguinal Hernia Sacs: A Clinicopathological Study of Six Cases

Glandular inclusions in inguinal hernia sacs are not frequent. We present six cases of inguinal hernia with this finding, which represents an incidence of 2.6% in males and shows a predominance in the prepubertal stage. Five patients showed cryptorchidism and two cases were related to congenital malformations of the single umbilical artery type and 47,XY chromosome disorder with chromosomal marker. The most important differential diagnosis must be made with normal histological structures such as the vas deferens or epididymis. The mean diameter of the inclusions was 0.1988 mm and there was a significant difference in size between the inclusions and the vas deferens, but not the epididymis. Differentiation from the latter is based on the absence of a well-developed muscular coat in the wall of the inclusions. It is important to recognize that these inclusions can occur in hernia sacs because of the clinical and medicolegal implications that arise if they are confused with true epididymis or vas deferens. They may arise from paratesticular embryonal remnants.     

Prevention of unnecessary contralateral exploration using the silk glove sign (SGS) in pediatric patients with unilateral inguinal hernia

Introduction The issue of how to best detect an occult contralateral patent processus vaginalis (PPV) in a child who presents with a unilateral inguinal hernia has been debated for over 50 years. The ideal diagnostic tool would eliminate unnecessary contralateral exploration. The “silk glove sign” (SGS) or palpating the processus vaginalis over the pubic tubercle is the simplest preoperative diagnostic test for diagnosing contralateral PPV. Materials and methods In an attempt to determine whether it is possible with the SGS to evaluate the contralateral inguinal region to ascertain if a contralateral PPV is present, we prospectively reviewed our own experience at a single institution within a 5-year period. Between January 2001 and December 2005, 1,040 pediatric patients with inguinal hernias were treated by one surgeon at Chang Gung Children’s Hospital. Of these, with the exception of 120 patients with clinical bilateral inguinal hernia, 920 patients (746 boys and 174 girls) with unilateral inguinal hernia underwent examination. A total of 180 (19.8%), of whom 154 were boys and 26 were girls, were SGS positive and underwent bilateral surgery. Results A PPV or true hernia sac was found in 164 (91.1%). The sensitivity of diagnosis by SGS was 93.1%. In the remaining 740 SGS-negative patients, a metachronous hernia developed in 20. The specificity of diagnosis by SGS was 97.3%. The SGS could detect a contralateral hernia in patients regardless of age, but the accuracy of detection in girls was lower than in boys. Conclusion SGS is a simple, noninvasive method of preoperative evaluation of the groins. Accuracy through repeated and careful examination is about 91%. Our policy of contralateral exploration based on the SGS has been reliable in minimizing unnecessary surgical exploration.     

An unusual complication of nasal foreign body in a pediatric patient: A case report

Foreign body (FB) of nasal cavity is a common clinical incidence in Pediatric Otorhinolaryngology. Inferior meatus is a rare location for FB lodgment inside the nasal cavity. In case of nasal FB, patients usually present with nasal obstruction, foul smell discharge and epistaxis. FB at the inferior meatus blocking the nasolacrimal outflow is an extremely rare complication of nasal FB leading to dacryocystitis. X-ray skull will help to rule out the radio-opaque FB in the nasal cavity. Diagnostic nasal endoscopy will help for accurate diagnosis of nasal FB. Early diagnosis and safe removal of FB from nasal cavity help to reduce complications. We present an unusual case of nasal FB in a 10-year-old boy presented with chronic dacryocystitis. Chronic dacryocystitis is an extremely rare presentation due to FB of nasal cavity.     

Bronchial foreign body: should bronchoscopy be performed in all patients with a choking crisis?

The aspiration of a bronchial foreign body (FB) remains a common pediatric problem with serious and sometimes fatal sequelae. The diagnosis is often delayed or overlooked. With the aim of determining a reliable clinical and/or radiologic finding to indicate the requirement for bronchoscopy, 100 patients admitted to our hospital because of FB aspiration who underwent rigid bronchoscopy were retrospectively studied. The clinical and radiologic data were compared with the bronchoscopy findings, which revealed that the history of a choking crisis was the clinical parameter that showed the highest sensitivity (97%) with high specificity (63%), and that other symptoms and radiology, even those with high sensitivity (88% and 85%, respectively), had low specificity (9%). We conclude that bronchoscopy should be performed in all patients with a history of a choking crisis even if they have normal radiologic findings and few symptoms.     

Two unusual tumours of the gastrointestinal tract in a patient with tuberous sclerosis

A 16 year old girl with an established diagnosis of tuberous sclerosis presented with a 1 year history of swelling of the left cheek. A 2 cm diameter tumour was excised which showed histological features of a solid variant of a minor salivary gland basal cell adenoma. One year later during laparotomy and excision of multiple renal angiomyolipomas, a 5 cm diameter subserosal tumour was found at the hepatic flexure of the colon. Examination of biopsy material revealed a leiomyoma. This case is presented to demonstrate two tumours that have not to the authors' knowledge been previously described in a young patient with tuberous sclerosis. Although the association may be coincidental, these tumours could represent two rare associations of tuberous sclerosis.     

Tyrosinemia type II: Novel mutations in TAT in a boy with unusual presentation

Tyrosinemia type II is a rare autosomal recessive disorder caused by deficiency of tyrosine aminotransferase (TAT). It may occur with ocular and cutaneous symptoms with or without mental retardation, but epileptic seizure is a rare presentation of this disease. Herein we report the clinical, biochemical and genetic features of a 4‐year‐old boy who presented with afebrile seizure and photophobia. Genomic DNA was obtained from peripheral blood leukocytes from the whole family. Sequencing analysis was performed using the MiSeq next‐generation sequencing platform. Sequencing of TAT indicated two new homozygous mutations p.L312P (c.935T>C) and p.T408M (c.1223C>T) for the proband and his asymptomatic sister. During a 2 year follow‐up period, the patient had overall poor compliance with protein‐restricted diet, but his asymptomatic sister had good compliance with the diet. Cognitive function of the patient worsened steadily, but his asymptomatic sister maintained normal mental status. Tyrosinemia type II should be considered in the differential diagnosis of children presenting with epileptic seizure and photophobia; furthermore, early diagnosis and protein‐restricted regimen are important to reduce the risk of long‐term complications of tyrosinemia type II such as mental disability.     

Ingested foreign bodies causing complications and requiring hospitalization in European children: Results from the ESFBI study

Background:  In young children, particularly those aged 1–3 years, aerodigestive tract foreign bodies (FB) are a common pediatric problem. The aim of the present study was therefore to characterize the risk of complications and prolonged hospitalization due to FB in the upper digestive tract in terms of the characteristics of the injured patients (age, gender), typology and features of the FB, the circumstances of the accident and hospitalization details.
Methods:  A retrospective study was done in 19 hospitals in 19 corresponding European countries of 186 cases of injury due to the presence of an FB in the mouth, esophagus and stomach (ICD935), out of the 2103 overall cases of FB reported in other locations.
Results:  Complications arose in 14 cases and hospitalization was required in 164 cases. No deaths were observed. A higher incidence of hospitalization in male patients (61%) was observed. Median age for children who experienced complications was 2 years old. The most common FB removal technique was esophagoscopy. In the majority of cases the children were treated by the ENT Department. The most common FB were coins, batteries and fish bones among food.
Conclusion:  Because batteries, as well as coins and fish bones among food were the most common type of FB encountered, and because recent development of technology has accelerated broad use of disk-type batteries, parents should be aware of this hazard, and an educational campaign for public education for this serious problem is advisable.     

Right diaphragmatic hernia after liver transplant in pediatrics: A case report and review of the literature

Diaphragmatic hernias (DH) are an unusual complication after pediatric liver transplantation; however, they have been reported with increased frequency in the past few years. DHs are responsible for nearly half of the small bowel obstructions requiring surgical intervention in this patient population. It has been suggested that the use of a left lobe liver graft, surgical trauma, malnourishment, elevated intra‐abdominal pressures, and mTor inhibitors may predispose to development of DH. The use of a segmental graft may increase the recognition of diaphragmatic hernia because the surgically damaged right hemi‐diaphragm often remains exposed to underlying viscera, instead of being covered by the right hepatic lobe. Treatment is surgical reduction, with up to 20% of the patients requiring resection of the herniated intestine. Herein we describe a case of DH after left segmental liver transplant in a two‐ yr‐old boy that presented one month post left lobe split liver transplant with abdominal pain, anorexia, and respiratory distress. Just like in the majority of the reported cases, an urgent laparotomy with primary repair was performed. No resection of the herniated segment of intestine was required. For pediatric patients with otherwise unexplained respiratory or gastrointestinal symptoms after a left lateral segment liver transplant, right‐sided diaphragmatic hernias should always be high in the differential diagnosis.     

Incarcerated femoral hernia with ovary and fallopian tube torsion in an infant: a rare occurrence

Incarcerated femoral hernia with ovary and fallopian tube torsion is very rare in children. We herein report a case of incarcerated femoral hernia with ovary and fallopian tube torsion in a 7-month-old female who was diagnosed on the basis of operative findings. We released the torsion of the left fallopian tube. However, the ovary remained discolored. Left salpingo-oophorectomy and McVay repair for femoral hernia were performed. She has experienced no recurrence of femoral hernia for 3 years. We must consider incarcerated femoral hernia in the differential diagnosis for infants with groin redness and swelling.     

Effect of maternal dexamethasone treatment on the type II pneumocytes in hypoplastic lung by oligohydramnios: an ultrastructural study

A previous study documented the effects of maternal corticosteroid treatment on structural growth and functional development in fetal lungs associated with pathogenic conditions such as oligohydramnios using immunohistochemical and morphometric analyses. The purpose of the present study was to examine the effect of maternal dexamethasone treatment the expression of lamellar body in type II pneumocytes of the fetal rabbit lungs with hypoplasia induced by oligohydramnios using electron microscopy. Using an amniotic shunting rabbit model, pregnant rabbits were injected intravenously with either 0.1 ml of saline or 0.25 mg/kg/day of dexamethasone in 0.1 ml of saline 48 and 24 h before the delivery of fetuses, at day 30 of gestation. The number of lamellar bodies per type II pneumocyte was counted in each group using electron micrographs. The number of lamellar bodies per type II pneumocyte in the lungs of the shunted group that received saline was consistently and significantly less than that of the other three groups (5.49 ± 2.07 vs. 7.34 ± 2.27: shunted group that received dexamethasone, 7.58 ± 2.08: non-shunted group that received saline, 7.79 ± 1.90: non-shunted group that received dexamethasone; P < 0.01). These results suggest that maternal dexamethasone treatment accelerates the maturation of the surfactant system, especially the expression of lamellar bodies in type II pneumocytes, even in hypoplastic lungs induced by oligohydramnios.     

Glioblastoma in a boy with fragile X: an unusual case of neuroprotection

Glioblastomas in children usually occur in the brainstem and are often untreatable and associated with an extremely poor prognosis. Such tumours may however occur in supratentorial locations more typically associated with adult gliomas, where the outcome is more variable, but still poor. We report the case of a boy with fragile X who also developed an inoperable midbrain glioblastoma, but who has survived 8 years after diagnosis.     

The psuedo-Chediak-Higashi anomaly: an unusual staining pattern in an Indian child with acute myeloid leukemia

The authors describe the psuedo-Chediak-Higashi anomaly in a 12-year-old boy with acute myeloid leukemia (AML-M2). There were large purple granules in the blasts, promyelocytes, and myelocytes. Instead of the previously described patterns, the authors observed a unique rim pattern staining of the granules in both the May-Grunwald-Giemsa and the myeloperoxidase stains. Moreover, many of the granules had central vacuoles with strong myeloperoxidase positivity at the periphery. The bone marrow had a much higher positivity for these mega-granules as compared to the peripheral blood. On remission, these granules were no longer seen. To the best of the authors' knowledge, this pattern of staining has not been previously reported in the literature.