Catheter knotting in a neonate

A 36-week preterm neonate with anal atresia and rectoperineal fistula was catheterized with a size 6F Foley’s catheter when she underwent anoplasty on the second day after birth. When the patient recovered from anesthesia, it was decided to remove the catheter. While the catheter was being removed, resistance was felt halfway through the procedure. Abdominal plain films revealed a catheter coiled in the pelvis. With copious lubricant injected into the bladder through the catheter and patient sedated, it was removed by manipulation alone using gentle traction (Figure 1). The infant had no bleeding at the urethral meatus and no obvious abnormality in urination during the 3-month of follow-up.

Case report: a de-novo 7p12.3 microduplication detected in an infant with perineal hamartoma and imperforate anus

Background Anorectal malformations (ARM) represent a wide spectrum of defects. Caudal and genitourinary malformations can associate with anorectal malformations. Genetic factors may play role in the development of anorectal malformations. Perineal masses like sacrococcygeal teratoma, rectal prolapse, or duplication cysts were reported before, but their association with perineal hamartoma and anal atresia is extremely rare. Case presentation Here, we report an 11-month-old female infant. She had 551 kb duplication at 7p12.3 with perineal hamartoma and anal atresia consisting a cystic lesion with a diameter of 4 mm at the filum terminale (L2 vertebra) on lumbar magnetic resonance imaging (MRI) in neonatal period. She presented with hypotonia. She had anorectal anomaly and external perineal mass bulging from left major labium extending across anal region with imperforate anus. There was 1 × 1 cm polyp-like protrusion on it. She was operated in neonatal period. Genetic laboratory investigations showed karyotype 46, XX. The microduplication of the chromosome 7p12.3 was detected by microarray analysis. There were not any significant homozygous or heterozygous variants determined by whole-exome sequencing. Conclusions To the best of our knowledge, this is the first report of a patient with a microduplication of the chromosome 7p12.3, and second case with perineal hamartoma and imperforate anus. Clinicians should pay attention to microdeletions and microduplications while giving genetic counseling to patients with urogenital and anorectal abnormalities.

477 Surgical Solutions and Problems in An Adult Patient with VACTERL Association

Background We report an adult patient with features of VACTERL - oesophageal and anal atresia, with cardiac malformation. In infancy he underwent colonic interposition for oesophageal atresia using the transverse colon as a conduit. As a teenager he underwent formation of a Koch continent ileostomy after previous stoma formation for anal atresia. Eventual failure of the Koch pouch nipple valve necessitated a laparotomy for attempted refashioning. Unfortunately, this operation was beset by life-threatening complications and a protracted post-operative course. He presented to our hospital with a laparostomy, high output enterocutaneous fistulae (ECF) necessitating home total parenteral nutrition (TPN), severe cachexia and deconditioning. Method Conservative therapy was utilised to improve the nutritional and physiological status of the patient. Simultaneously, attempts were made to define the anatomy of his abdomen in relation to previous surgery. However, operation notes for the original procedures (oesophageal reconstruction and Koch pouch formation) were lost due to closure of the hospital which he attended at the time. A laparotomy was subsequently performed to manage the ECFs. Most of the small intestine was unsalvageable due to multiple fistulae and adhesions, leaving 35cm of small bowel terminating in an end ileostomy. A cholecystectomy was performed to mitigate gallstone formation. Conclusions Efficient communication is imperative in the management of complex patients. The loss of original operation notes made interpretation of subsequent imaging and planning of surgery troublesome. In contrast, efficient communication between teams involved in his peri-operative care, particularly the respiratory and intensive care teams facilitated a smooth post-operative course and successful discharge from hospital.

Neonatal Respiratory Distress and Airway Emergency: Report of Two Cases

We discuss two cases of congenital airway malformations seen in our neonatal intensive care unit (NICU). The aim is to report extremely rare events characterized by immediate respiratory distress after delivery and the impossibility to ventilate and intubate the airway. The first case is a male twin born at 34 weeks by emergency caesarean section. Immediately after delivery, the newborn was cyanotic and showed severe respiratory distress. Bag-valve-mask ventilation did not relieve the respiratory distress but allowed for temporary oxygenation during subsequent unsuccessful oral-tracheal intubation (OTI) attempts. Flexible laryngoscopy revealed complete subglottic obstruction. Postmortem analysis revealed a poly-malformative syndrome, unilateral multicystic renal dysplasia with a complete subglottic diaphragm, and a tracheo-esophageal fistula (TEF). The second case is a male patient that was vaginally born at 35 weeks. Antenatally, an ultrasound (US) arose suspicion for a VACTERL association (vertebral defects, anal atresia, TEF with esophageal atresia and radial or renal dysplasia, plus cardiovascular and limb defects) and a TEF, and thus, fetal magnetic resonance (MRI) was scheduled. Spontaneous labor started shortly thereafter, before imaging could be performed. Respiratory distress, cyanosis, and absence of an audible cry was observed immediately at delivery. Attempts at OTI were unsuccessful, whereas bag-valve-mask ventilation and esophageal intubation allowed for sufficient oxygenation. An emergency tracheostomy was attempted, although no trachea could be found on cervical exploration. Postmortem analysis revealed tracheal agenesis (TA), renal dysplasia, anal atresia, and a single umbilical artery. Clinicians need to be aware of congenital airway malformations and subsequent difficulties upon endotracheal intubation and must plan for multidisciplinary management of the airway at delivery, including emergency esophageal intubation and tracheostomy.

Atresia Ani (Imperforated Anus) in Calves: Clinical, Surgical and Pathological Aspects

Background: Anal atresia is a congenital malformation, which often affects calves, and is related to the imperforation of the membrane that separates the endoderm of the posterior intestine from the ectodermal anal membrane. It is commonly associated with other congenital malformations and skeletal anomalies. The clinical signs generally appear in the first days of the animal's life, due to a retention of feces. The diagnosis is clinical and is based on observation, anamnesis and a physical examination of the animal. The only viable treatment is surgical. This paper aims to report 6 cases of anal atresia in bovine calves, 4 males and 2 females that were successfully treated surgically. Cases: This work reports 6 cases of anal atresia in 4 male calves and 2 female calves. Three presented total atresia (type II), one partial (type I) and in both female calves, anal atresia and rectovaginal fistula (type IV) were observed. The animals were all of undefined race. Five of the cases were from northern Tocantins, 4 males and 1 female (anal atresia with rectovaginal fistula), and 1 female (anal atresia with rectovaginal fistula) was from Valença, RJ. All animals were born active, by eutocic/natural birth, and assumed a quadrupedal position followed by the first feeding as normal. They were aged between 2 days and 6 months, and had a clinical history of abdominal distention and difficulty or inability defecating, and the females both also had a rectovaginal fistula, all cases compatible with anal atresia. Based on the patient's history and clinical examination, surgical treatment for anal reconstruction was decided upon. Postoperative treatment consisted of enrofloxacin [2.5 mg/kg - intramuscularly (IM), once daily (SID), for 5 days] and fluxinin meglumine [1.1 mg/kg - IM, SID, for 3 days] ; as well as a healing ointment which was applied to the area of the surgical wound, every 12 h, for 7 days. There were no trans-surgical complications. The animals showed progressive recovery after anal reconstruction and the stitches were removed in all cases on the 10th postoperative day, with no postoperative complications and no recurrence of any clinical signs from that moment on.Discussion: The study of congenital and hereditary changes enables the identification of their origins, can help prevent new cases and, some of them, are open to economically viable treatment and/or correction that can improve the well-being of the animal and prevent economic losses due to death or animal sacrifice, as reported in the present study. Anal atresia is the most common congenital defect of the lower gastrointestinal tract in calves, being an isolated abnormality, or associated with other malformations, especially of the distal spinal column such as the absence of a tail (perosumus acaudato), as one of the animals in this study. The clinical signs and physical examination are sufficient to establish the diagnosis, as demonstrated in this report, which is usually made in newborn animals, due to the lack or difficulty in defecation associated with no anal orifice and/or swelling in the perineal region. The treatment of choice for anal atresia is surgical, in order to construct an anal neo-orifice and thus avoid endotoxemic shock as well as providing relief and well-being for the animals. As observed in this study, when anal atresia is diagnosed early, and surgical treatment is properly instituted, the prognosis is favorable. The surgery is considered of low complexity, quick and it can be carried out in the field. Thus, from a commercial point of view, considering the costs of the procedures and the value of the calf at the end of weaning, such treatments are beneficial to the owners. In addition, the surgical treatment is essential for animal health and welfare in cases of anal atresia.Keywords: cattle, congenital defects, hereditary pathology, perosomus acaudato, surgery.