Inflammatory myofibroblastic tumor of caecum presenting as recurrent intussusception: A case report

Background: Inflammatory myofibroblastic tumor is an uncommon tumor. It commonly affects the lung but it can be found anywhere in the body. Case Report: We are reporting a case of caecal mass presenting as recurrent intussusceptions. Histopathology and immunohistochemistry confirmed it to be an inflammatory myofibroblastic tumor of caecum. Immunohistochemistry of tumor cells were positive for vimentin, smooth muscle actin, MIB 1, and CD 45 in lymphoid cells in the stroma of the tumor. Conclusion: Recurrent intussusception should be kept as one of the differential diagnosis in patient with caecal mass with recurrent abdominal pain. Inflammatory myofibroblastic tumor of the caecum is unusual in paediatric patients and may present as recurrent intussusception.

Spontaneous enterocutaneous fistula in an infant

Background: Enterocutaneous fistulae (ECF) most commonly occurs following intestinal surgery. Spontaneous ECF is a rare entity in infants. Early presentation to the hospital decreases complications. Case Presentation: A 2.5-month-old infant developed spontaneous ECF below the umbilicus following abdominal wall erythema and abdominal wall abscess. The bowel mucosa prolapsed out on straining efforts. Work-up was uneventful. At operation, the fistula was mobilized, and intestinal continuity was restored. Postoperative recovery was uneventful. Conclusion: Spontaneous development of ECF is a rare entity in children. Every effort should be made to identify the etiology before labeling it as spontaneous. Keywords: Enterocutaneous fistula, Necrotizing fasciitis, Primary closure.

Automatic planning of paediatric craniofacial deformities: new virtual facial-symmetry operative detection

Background: The correction of craniofacial deformities is an ongoing challenge in maxillofacial surgery. However, conventional measurement methods for treatment planning are not appropriate for craniofacial surgery. Computer-assisted approaches can improve surgical outcomes. A new, non-invasive, patient-specific automatic method, proposed here, has been tested for assisting the surgeon in preoperative planning. Case Presentation: In the case reported, the described method allows effective surgery planning that led to a significant decrease in asymmetries in the orbital region. Conclusion: The multidisciplinary collaborative approach is a central element for the construction of effective personalized procedures and for the conception of new surgical approaches. The here proposed technology offers a good level of feasibility and has an achievable potential for breakthroughs in the improvement of facial deformities surgical treatment, thus representing an overwhelmingly useful tool in a clinical setting.

Solitary intrascrotal neurofibroma in a child: A case report

Background: Neurofibroma (NF) remains a rare entity among various scrotal masses in the pediatric age group. We report a case of solitary intrascrotal extra testicular plexiform neurofibroma in a 6-year-old child with the clinical management of this rare entity and review of the literature. Case Summary: A 6-year-old male presented with incidentally noticed intrascrotal swelling. Clinical examination confirmed the presence of solitary intrascrotal swelling with normal testis. Radiological imaging showed the presence of a hypoechoic mass in the left hemiscrotum with normal both testes. Surgical exploration showed the presence of unencapsulated soft tissue mass and histopathological examination (HPE) confirmed the diagnosis of plexiform neurofibroma. Thorough evaluation ruled out features of neurofibromatosis 1 thus confirming the diagnosis of solitary intrascrotal extratesticular plexiform neurofibroma. Complete surgical excision resulted in satisfactory recovery with no recurrence on follow-up Conclusion: Neurofibroma although rare should be considered as a differential in the diagnosis of an intrascrotal mass in the pediatric age group. The benign nature of the lesion and extratesticular origin usually makes testis sparing surgical excision feasible which remains the treatment of choice with excellent prognosis and minimal chances of recurrence. A thorough histopathological examination is mandatory to rule out neurofibromatosis.

Factors affecting the outcome of neonates with anorectal malformation in a developing country

Background: The survival and outcome of neonates with anorectal malformations (ARM) have much improved in the developed countries due to optimal perioperative and postoperative care but in developing countries, sepsis, low birth weight, delayed presentation, and lack of intensive care for neonates are still important in affecting the outcome. This study was carried out to evaluate factors of poor outcome (mortality) in neonates with ARM. Method: This is a prospective analytical study. A total of 44 consecutive neonates with Anorectal malformations (ARM) presenting to the Department of Pediatric Surgery, The Children’s Hospital, Pakistan Institute of Medical Sciences, Islamabad, were included. Variables studied included age at presentation, gender, birth weight, type of malformation, sepsis at presentation, type of surgery performed, postoperative complications, and their relationship to the outcome. The statistical analysis was performed using SPSS version 21. Results: A total of 44 neonates with ARM were included in the study. In the study population, 56.8 % (25) were males and 43.2% (19) were females. The mean age at presentation was 2.1 ± 0.5 days. The mean birth weight was 2.5 ± 0.6 kg. Overall mortality was 29.5% (13) with 13.63% (6) patients died pre-operatively. The most common cause of death in postoperative patients was sepsis (40%). There was a statistically significant relationship between low birth weight (P= <0.01) and sepsis at presentation (P=0.001) with mortality. No statistically significant association was found when the outcome was compared with age at presentation (P=0.21) and postoperative complications (P=0.16). Conclusion: In developing countries, the lack of resources, lack of trained midwives/LHVs, intensive care are contributing factors to sepsis and delayed presentation, and ultimately mortality. Good antenatal care, awareness of the midwives/Lady Health Visitors to refer such patients in time, and provision of adequate intensive care can improve the outcome of surgery in ARMs.

The spectrum of congenital malformations of the lung at a rural

ABSTRACT Background: Congenital malformations of lung, such as congenital lobar emphysema, cystic adenomatoid malformation, bronchial cyst, pulmonary sequestration are of rare occurrence. However, our hospital being in a rural area attracts a large number of patients with complex malformations. Method: This retrospective analysis is done to evaluate diagnostic accuracy, safety and efficacy and outcomes of open lung resections at a rural hospital. Results: 16 children with congenital lung malformation underwent open resection in a rural hospital, with no mortality and minimal morbidity, no complications and more than 24 months follow-up. Conclusion: Open lung resections are found to be safe, effective in a rural setting also.

Abdominal tuberculosis requiring surgical intervention: A 10-year single-center experience

Background: Although uncommon in children, abdominal tuberculosis (ATB) can be a life-threatening condition with a subset requiring emergency surgical intervention. This study aims to determine the presentation, surgical procedures performed, and outcomes in children affected by abdominal tuberculosis. Methods: A retrospective chart review of all children undergoing surgical intervention for ATB from July 2007 to December 2018 was conducted. Data were analyzed using SPSS version 22. Results: Of 340 children with a diagnosis of ATB seen at the Indus Hospital’s TB clinic, 14 (4%) underwent laparotomy. Females were affected more commonly (57%), with a mean age at presentation of 11 years (range 8-14). Nine children required laparotomy for documented perforation, while 5 had an intestinal obstruction. Most children (n=10) had an established diagnosis of ATB before the surgical intervention; 2 children had completed 6–9 months anti-tuberculous treatment (ATT) courses, while 8 children had been on ATT for a mean period of 2.5 months at the time of developing acute surgical symptoms. Diversion ileostomy was made in 64%. Postoperative complications included sepsis (n=4), wound infection (n=3), abdominal collection (n=2), enterocutaneous fistula (n=2), and abdominal wound dehiscence requiring formal closure (n=2). There were 4 mortalities (29%); 10 patients were discharged after a median in-hospital stay of 12 days (range 6-35) of which 6 with ileostomies underwent reversal after completion of the ATT course. Conclusion: ATB has high morbidity and mortality. Perforation and obstruction can occur during or after the completion of ATT. Management requires early recognition and surgical intervention as indicated.

A newborn with anterolateral diaphragmatic defect: An unusual anomaly?

Background: Anterolateral defect of diaphragm is unusual and has been reported less than 5 times in literature. Case Presentation: We are reporting a case with typical initial presentation of a congenital diaphragmatic hernia. A chest x-ray raised suspicion of a cystic lesion or diaphragmatic eventration which was excluded by chest ultrasound. At operation he was found to have a localised and large anterolateral defect with a very thick sac which was plicated to bridge the diaphragmatic defect. Conclusion: A rare variant and should be differentiated from other variants using imaging.

Postoperative gastric Outlet Obstruction following hiatal hernia repair in an infant: A case report

Background: Infantile hypertrophic pyloric stenosis (IHPS) is an exceedingly rare cause of postoperative emesis in a case of hiatal hernia. Occasionally it may simulate other etiology of gastric outlet obstruction. Case Presentation: A 32-day-old male baby presented with respiratory distress and vomiting since birth. Diagnosis of eventration of left hemi diaphragm was made on CT Chest. At surgery, hiatal hernia with an intrathoracic stomach was found, which was repaired. On 5th postoperative day, the baby developed vomiting after feeding which gradually turned to be projectile in nature over a week. Contrast meal performed showed malpositioned stomach with delayed emptying. At re-operation, a well-formed olive of pylorus was encountered; Ramstedt pyloromyotomy was done. Postoperative course remained uneventful. Conclusion: IHPS is a rarely described association with hiatal hernia. Pyloric stenosis should be considered in differential diagnoses of postoperative emesis in infants with hiatal hernia.