Gastrointestinal polyposis in infancy and childhood

The clinical and pathological features in 12 children with symptomatic gastrointestinal polyposis are presented. Five children with Peutz-Jeghers syndrome all presented with intussusception. Three children with Gardner syndrome presented with multiple large epidermoid cysts of the scalp. The other 4 with juvenile polyposis all presented with anaemia, often associated with growth retardation and extragastrointestinal abnormalities. One of these 4, as well as her mother, had Osler disease with pulmonary arteriovenous malformation. Some degree for overlap in the histology was noted between the polyps in the three conditions, and confusion may arise if only small areas are examined. The histological evolution of small lesions in juvenile polyposis and the surgical observation of friability of adjacent non-polypoid mucosa and association with multiple disorders, local as well as generalised, mechanical as well as inflammatory, suggest these to be the result of non-specific ulceration followed by granulation tissue formation, inflammatory infiltration, and irregular epithelial regeneration.     

儿童系统性红斑狼疮的消化系统表现及急重症分析

目的 分析系统性红斑狼疮(SLE)患儿消化系统受累的临床表现,尤其是急重症表现,以提高儿科临床医师对SLE消化系统表现,尤其是急重症的认识.方法 回顾性分析2010年1月至2013年3月在北京协和医院明确诊断为SLE,且诊断年龄在16岁以下共计119例患儿的临床资料,对SLE消化系统受累临床资料进行分析.SLE诊断均符合1997年美国风湿病协会关于SLE的诊断标准,并无硬皮病和重叠综合征的临床表现.结果 SLE消化系统受累病例共24例,发生率为20.2％,平均年龄(13.5±2.0)岁(6～15岁).男∶女为1.0∶2.4,分别占同时期诊断SLE同性别患儿的26.9％和18.3％.其中有4例患儿消化系统表现为SLE的首发症状(4/24例,16.6％).SLE患儿消化系统受累最常见的表现为腹痛(13/24例,54.2％),其次为恶心呕吐(11/24例,45.8％)、腹胀(4/24例,16.6％)、腹泻(3/24例,12.5％).另有8例无明显症状,辅助检查发现肝功能异常,但无病毒感染等其他导致肝功能异常的证据.消化系统急重症表现病例共9例,6例为假性肠梗阻(2例同时合并双肾盂、输尿管扩张);1例诊断为腹膜炎,行手术剖腹探查;1例表现为蛋白丢失性肠病;1例为急性胰腺炎.所有病例经足量激素及环磷酰胺治疗后病情好转.消化系统受累的SLE患儿合并肾盂输尿管扩张的比例高,清蛋白水平差异有统计学意义,而在是否有红细胞沉降率增快、肾脏、血液系统、中枢神经系统受累差异无统计学意义.结论 目前对儿童SLE消化系统受累认识不足,低估了SLE消化系统受累的概率及严重程度.部分患儿可以消化系统受累为首发症状,假性肠梗阻、蛋白丢失性肠病、急性胰腺炎是较为少见,但需提高认识的SLE累及消化系统重症表现.及早诊断,联合肾上腺皮质激素和环磷酰胺治疗对于消化系统急重症治疗非常重要.     

Neonatal gastrointestinal perforations.

Fourteen neonates presented with clinical and radiological evidence of gastrointestinal perforation, of which 13 were treated by operative intervention. No infant was formula fed, had rectal bleeding or radiological evidence of pneumatosis intestinalis. Operative findings revealed localized perforation in ten of which three were gastric, two distal ileal, three cecal and two in sigmoid colon. Gangrene with extensive perforation of jejunum, ascending colon and transverse colon were seen in one each. Blood cultures were positive in four, one grew E. coli and three Klebsiella while peritoneal cultures were positive in six, one grew E. coli and five Klebsiella. Ten neonates survived and are being followed up. Three of the four cases who died had white blood cell count greater than 25 x 109/L and grew Klebsiella on peritoneal culture. Factors predisposing to gastrointestinal perforations in neonates are discussed, emphasis is made on the cautious use of umbilical, gastric and rectal catheters, and the need for early surgical intervention.     

Pharyngeal and esophageal perforations in newborn infants

In three newborn infants pharyngeal or esophageal perforations were due to injury caused by the obstetrician's finger in the infant's mouth to help deliver the head, by orogastric feeding tube, or by endotracheal tube. They all had in common respiratory distress and right-sided pneumothorax. The patients were treated medically with antibiotics, antifungal therapy and total parenteral nutrition. All perforations healed without surgical repair.     

Colorectal perforations in neonates with anorectal malformations

 Colorectal perforations in neonates with anorectal malformations (ARM) are rarely reported. Two cases, one each with a low and high ARM is presented. Delayed patient presentation and “closed-loop” intestinal obstruction seem to be possible causes of perforation in these cases. Both patients survived following surgical intervention. The pertinent literature is reviewed to emphasize the overall management of such cases. Accepted: 17 December 1999     

Thermometers and rectal perforations in the neonate.

Three neonates with rectal perforations probably caused by the use of the rectal thermometer, are reported. The dangers of this condition and the difficulty of diagnosis even at laporatomy are emphasised. It is suggested that since the axillary temperature in the neonate is an adequate reflection of the core temperature the routine use of the rectal thermometer, except in exceptional circumstances, should cease.     

Gastrointestinal intervention in children

Interventional radiology is a rapidly growing discipline in paediatrics. Many non-vascular interventional techniques may be used in the gastrointestinal tract in children. The technically simpler and more common of these may be adopted by any paediatric radiologist with an interest in interventional radiology. Other rarer and more complex techniques are currently restricted to specialist centres with a higher overall caseload. This review emphasizes the common procedures such as oesophageal dilatation, gastrostomy, insertion of transgastric jejunal feeding tubes and biopsy. Less common salivary, hepatobiliary, pancreatic and intestinal interventions are also described.     

Gastrointestinal malacoplakia in children

Four children, whose ages ranged from 1 to 13 years, with malacoplakia of the gastrointestinal tract were treated at King Faisal Specialist Hospital between 1979 and 1983. All patients had either a preceding or a coexisting chronic illness. In one patient, malacoplakia was an incidental finding, while the remaining three patients presented with bloody diarrhea, abdominal pain, recurrent fever, and severe malnutrition. Colonoscopy in two patients revealed markedly inflamed and friable mucosa with focal ulceration alternating with patches of normal mucosa and pseudopolyposis. They were treated with antibiotics and cholinergic agonists. Three patients responded favorably, while one patient continued to have extensive active disease. Although the response to therapy is unpredictable, patients may respond if the treatment is continued on a long-term basis.     

A rare case of multiple duodenal perforations in early infancy

Duodenal perforation in early infancy is an uncommon condition. We describe a case of duodenal perforation from suspected ulcer. A premature boy was born at the gestational age of 26 weeks with a birth weight of 764 g. The Apgar score at 1 min was 3 and at 5 min had decreased to 2. He was given intermittent mandatory ventilation for one month after the birth. Ninety-eight days after birth, the infant's abdomen became distended. A supine and cross-table lateral radiograph of the abdomen revealed massive pneumoperitoneum. An exploratory laparotomy was performed, which revealed two perforations in the anterior wall of the first portion of the duodenum. The operation procedure was direct closure and intra-abdominal drainage. On the postoperative first day, he had central urorrhagia from hematencephalon. The patient's growth after surgery has been normal, with no recurrence of duodenal ulcer.