Incarcerated Littre's Hernia in a Neonate Presenting as Enteroscrotal Fistula.

A 27-day-old male neonate, presented with feculent discharge from the scrotum. Operative findings revealed incarcerated right inguinal hernia with perforated Meckel's diverticulum as its content, leading to enteroscrotal fistula. Resection of the Meckel's diverticulum and end-to-end ileoileal anastomosis was performed along with repair of inguinal hernia from within the abdominal cavity. The outcome was favorable. Enteroscrotal fistula due to incarceration of inguinal hernia is a rare presentation. We are adding to the literature, an extremely rare case of incarcerated Littre's hernia in the right inguinal region presenting as enteroscrotal fistula in a neonate.

Amyand's Hernia: Appendix in Hernia or Hernial Appendicitis?

Purpose: The presence of an appendix in the inguinal hernia sac is defined as Amyand's hernia (AH). This study intends to present the authors' experience in dealing with this entity and also to present a discussion on possible need of updating its definition, classification, and management. Materials and Methods: A retrospective analysis of records of all pediatric patients undergoing surgery for congenital inguinal hernia in a single unit from January 2017 to March 2021 was done. Patient's demographics, clinical presentation, preoperative investigations, peroperative findings, and postoperative outcomes were recorded and analyzed. Results: AH was found in eight patients. All were boys. The median age of presentation was 20.5 months (range 2 months to 36 months). The mean duration of symptoms was 2 days (range 2 to 4 days). All patients presented with incarcerated inguinoscrotal swelling (right sided = 5, left sided = 3); associated with pain. An abdominal radiograph and ultrasonography were done for all. All patients underwent emergency surgery. Exploration was done for all through an inguinal incision. The appendix was found inflamed for two patients, and appendectomy was done for the same. None of the patients underwent incidental appendectomy. Wound infection, secondary appendicitis, and recurrence were not seen for any of the patients. The authors have also proposed a revised definition and classification of AH. Conclusion: AH is an interesting entity and many questions like the need for incidental appendectomy remain unanswered. An updating of the definition and classification system can probably offer some solution in this regard. However, more research is warranted in this regard.

Heteropagus twins: six cases with systematic review and embryological insights.

PURPOSE: Heteropagus twinning (HT) is a rare anomaly. Six new cases along with a systematic review are described. METHODS: Six cases of HT managed at two tertiary care teaching hospitals over the last 26 years are described. A PubMed search with words: Heteropagus AND/ OR parasitic twins from 2001 to 2021 hit 183 articles. 36 were added from non-PubMed sources. Finally, 120 cases including 114 from 69 articles and 6 new cases were analysed. RESULTS: Of the new cases, 2/6 had an antenatal diagnosis. Five were males. 4 autosites had omphaloceles. Split notochord and 2 parasites attached to a single autosite were encountered. 5/6 autosites survived. On systematic review, the most frequent variant seen was rachipagus (n = 50) followed by omphalopagus (n = 46). Limbs were reported in 75 cases. Congenital heart disease was seen in 17/120(14.2%) autosites. Omphalocele and meningomyelocele were the most common extracardiac anomalies in autosites. Weight along with the anatomy and position of heteropagus twins was a better determinant of the mode of delivery than weight alone. Mortality was reported in 12 cases. CONCLUSION: Autosites in HT generally carry a good prognosis, however, final outcome depends mainly on associated major cardiac anomalies. Meticulous antenatal assessment and preoperative planning are of paramount importance. LEVEL OF EVIDENCE: IV.

Hepatobiliary malformations: proposed updation of classification system, clinicopathological profile and a report of largest pediatric giant choledochal cyst.

BACKGROUND: The present study was aimed to update the classification of hepatobiliary malformations and study the clinicopathological profile of pediatric choledochal cyst (CDC) and pediatric giant choledochal cyst (GCC) patients undergone surgery. METHODS: We have retrospectively analysed the data of 57 consecutive cases of CDCs in paediatric patients from a time period spanning from 2016 to 2020. RESULTS: Revised classification of hepatobiliary malformations was proposed and these were divided into 2 broad headings, choledochal (congenital and acquired) and extra-choledochal spectrum. 57 pediatric patients were diagnosed as having CDC with average age 4.615 years and female to male ratio of 3.7:1. We have also reported one of the largest GCC measuring 23 × 10 × 9 cm size. The classical triad was known to be more common and seen in 60% GCCs as opposed to 14.5% in CDCs. Values of serum bilirubin, SGOT, SGPT, PT/INR were elevated in CDC series and normal in GCC patients. 55 patients (96.5%) underwent cyst excision and Roux-en-Y hepaticojejunostomy. Mortality was seen in 7.01% patients (n = 4) following surgery. CONCLUSION: Simplified and broader classification system for CDCs has been proposed. Clinical studies found that GCC differs considerably from classical CDCs.

Muscle-Sparing Skin Crease Incision Posterolateral Thoracotomies in Pediatric Patients: Our Experience.

Context: Standard posterolateral muscle cutting thoracotomies in pediatric patients are associated with morbidities in terms of poor motor and esthetic outcomes and also more postoperative analgesia requirement, when compared with muscle-sparing technique. Aims: The aim of this study is to evaluate the outcomes of muscle-sparing skin crease incision posterolateral thoracotomies in pediatric patients in terms of abovementioned variables. Settings and Design: This prospective observational study was conducted over a period from January 2016 to July 2020 in a tertiary care teaching institute. Materials and Methods: All patients of stage 3 empyema and pulmonary hydatidosis were included in the study. Results: Thirty-nine patients fulfilled the inclusion criteria. There were 38 children with organized (Stage 3) empyema and one patient with bilateral lung hydatid. There were 24 males and 15 females; age ranging from 6 months to 15 years. Right thoracotomy was performed in 28, left in 10, and bilateral in one patient with lung hydatid. Adequate exposure was achieved in all cases. Decortication with complete excision of thickened parietal pleura with stripping of the visceral peel and release of the entrapped lung was performed in all 38 patients. In 5 children along with decortication, repair of bronchopleural fistula due to necrotizing pneumonia was performed. Bilateral thoracotomies with pericystectomies were performed with a gap of 3 weeks for lung hydatid. Lung expansion assessment at 3 months was satisfactory. Ultrasound assessment of the latissimus dorsi muscle and serratus anterior muscle at 4 weeks revealed complete integrity in all the cases. Most of the patients achieved satisfactory motor and esthetic outcomes. Conclusions: Muscle-sparing skin crease incision posterolateral thoracotomy is a viable alternative to standard posterolateral muscle cutting thoracotomy, especially in a resource-challenged setting. The technique is easy to perform with satisfactory exposure.

Spachelated Intussusception a Forgotten Entity: Historical Review and a Case Report.

Intussusception of one or another type is frequently seen in children and is the most common cause of acute bowel obstruction. This pathology is usually promptly recognized and treated accordingly. But if untreated, this entity can have either of the two outcomes. The first one is death from peritonitis and resulting toxemia, which is the most common one. The second one is spontaneous elimination followed by auto-anastomosis and is extremely infrequent. We report one such case of this rare progression who was found to have old healed small bowel intussusception with luminal narrowing on exploration for acute bowel obstruction. We have also reviewed the world literature to understand the mechanism and circumstances under which this progression occurs.

Consensus on the Management of Posterior Urethral Valves from Antenatal Period to Puberty.

The need for successful management of posterior urethral valves always captivates the minds of pediatric surgeons. Its success, however, depends on several factors ranging from prenatal preservation of upper tracts to postoperative pharmacological compliance. Regardless of measures available, some cases do not respond and progress to end stage. The management depends on several issues ranging from age and severity at presentation to long-term follow-up and prevention of secondary renal damage and managing valve bladder syndrome. This article is based on a consensus to the set of questionnaires, prepared by research section of Indian Association of Paediatric Surgeons and discussed by experienced pediatric surgeons based in different institutions in the country. Standard operating procedures for conducting a voiding cystourethrogram and cystoscopy were formulated. Age-wise contrast dosage was calculated for ready reference. Current evidence from literature was also reviewed and included to complete the topic.

Congenital Pouch Colon: Role of Genetics or Environmental Influence?

BACKGROUND: Congenital pouch colon (CPC), a high type of anorectal malformation, is a sporadic disease and several environmental factors are known to be involved in its pathology. To the best of our knowledge, no familial incidence of CPC has been reported anywhere in the literature so far. AIM: In the present study, which is first of its kind, we have reported the familial incidences of CPC and also tried to elucidate the role of genetics in this pathology. METHODS: We have reported 1 familial pedigree of CPC and 2 incidences of dizygotic twins (DZ), out of them one is affected and another one is normal. Highly comprehensive microarray CytoScan HD from Affymetrix was employed to understand the defects underlying submicroscopic genomic imbalance like segment duplication and deletion of the twin patients vis-à-vis their parents and unaffected siblings in these DZ twins. RESULTS: A total of 21 copy number variations (CNVs) were reported in the patient samples that did not overlap with the CNVs in normal parents and healthy sibling, including 5 loss, 3 LOH and 13 gain with size varied from 95 bp to 77 kbp. Genetic analysis revealed involvement of 12 potential genetic loci on Chr 1, 2, 3, 4, 6, 11, and 16. CONCLUSION: Genetic study found that CPC could be a developmental disorder. These findings are important for further elucidating genetic causes of CPC pathogenesis.

Precision medicine in asthma: the role of bronchial thermoplasty.

PURPOSE OF REVIEW: The inflammatory makeup of severe asthma is heterogeneous. Identification of the predominant cellular endotype via biomarkers can aid in the selection of more advanced therapies. This review is clinically focused on how to use these biomarkers to help select between biologic agents and/or bronchial thermoplasty. RECENT FINDINGS: Several Th2 biomarkers exist for the detection of eosinophilic disease; however, the best biomarker for clinical practice is debatable depending upon local resources. Currently, there are three federal drug agency-approved biologic agents (omalizumab, mepolizumab and reslizumab) to treat severe asthma with frequent exacerbations despite standard medical therapy. Several others are either in clinical trials or in the development phase for the treatment of eosinophilic asthma. To date, agents targeting neutrophilic inflammation have been largely unsuccessful. Bronchial thermoplasty has emerged as an option for the treatment of severe asthma. SUMMARY: The appropriate selection of patients through the use of eosinophilic biomarkers has led to significant reductions in exacerbations with the use of mAb therapy. Bronchial thermoplasty has also shown reductions in asthma exacerbations and improved quality of life; however, it is unclear which patients may respond best to this intervention.

Histopathological and Immunohistochemical Findings in Congenital Pouch Colon: A Prospective Study.

BACKGROUND: There is a paucity of literature on the histopathological aspects of congenital pouch colon (CPC) and immunohistochemical (IHC) assessment has not been reported. So we planned to study the histopathological and IHC findings within the spectrum of CPC and compare the findings with the normal colon. METHODS: This is a descriptive prospective study on CPC patients. There were 49 cases of CPC (42 males and 7 females) and 13 controls. Histological examination was done using hematoxylin and eosin and Masson trichrome stain. IHC analysis was done with actin, myosin, and desmin antibodies, and neuron-specific enolase and S100 markers for counting ganglionic cells. RESULTS: Histologically, congestion, edema and hemorrhage were seen in mucosa, submucosa, and serosa. Muscle layers were disrupted and divided into bands. An additional muscle coat inside of the muscularis propria was seen in CPC types 1 and 2. Mature ganglionic cells were reduced and muscle layers showed reduced and patchy positivity for smooth muscle actin, myosin, and desmin compared to a normal colon. CONCLUSIONS: Histopathological and IHC findings suggest that CPC has distinct defects in the neuromusculature.

Congenital Pouch Colon with Rectal Atresia Revisited.

We are adding two cases of congenital pouch colon with rectal atresia (RA) to the already published five cases. The presentation of RA is often delayed owing to the presence of normal anus, however, clinical evaluation, plain abdominal radiograph, and urine examination for meconuria, may help in detecting this rare association early.

Advances in pleural diseases: what is the future for medical thoracoscopy?

PURPOSE OF REVIEW: Medical thoracoscopy provides the physician a window into the pleural space. The procedure allows biopsy of the parietal pleura under direct visualization with good accuracy. In addition, it achieves therapeutic goals of fluid drainage, guided chest tube placement, and pleurodesis. RECENT FINDINGS: Comparable diagnostic yield is achieved with the flexi-rigid pleuroscope even though pleural biopsies are smaller using the flexible forceps as compared to rigid thoracoscopy. Flexi-rigid pleuroscopy is extremely well tolerated and can be performed safely as an outpatient procedure. Biopsy quality can be further enhanced with accessories that are compatible with the flex-rigid pleuroscope such as the insulated tip knife and cryoprobe. SUMMARY: With more sensitive tools to image the pleura such as contrast-enhanced computed tomography, MRI, ultrasonography, PET, increased yield with image-guided biopsy as well as advances in cytopathology, what lies in the future for medical thoracoscopy remains to be seen. However, it is the authors' opinion that medical thoracoscopy will evolve with time, complement novel techniques, and continue to play a pivotal role in the evaluation of pleuropulmonary diseases.

Whole Exome-Trio Analysis Reveals Rare Variants Associated with Congenital Pouch Colon.

Anorectal malformations (ARM) are individually common, but Congenital Pouch Colon (CPC) is a rare anorectal anomaly that causes a dilated pouch and communication with the genitourinary tract. In this work, we attempted to identify de novo heterozygous missense variants, and further discovered variants of unknown significance (VUS) which could provide insights into CPC manifestation. From whole exome sequencing (WES) performed earlier, the trio exomes were analyzed from those who were admitted to J.K. Lon Hospital, SMS Medical College, Jaipur, India, between 2011 and 2017. The proband exomes were compared with the unaffected sibling/family members, and we sought to ask whether any variants of significant interest were associated with the CPC manifestation. The WES data from a total of 64 samples including 16 affected neonates (11 male and 5 female) with their parents and unaffected siblings were used for the study. We examined the role of rare allelic variation associated with CPC in a 16 proband/parent trio family, comparing the mutations to those of their unaffected parents/siblings. We also performed RNA-Seq as a pilot to find whether or not the genes harboring these mutations were differentially expressed. Our study revealed extremely rare variants, viz., TAF1B, MUC5B and FRG1, which were further validated for disease-causing mutations associated with CPC, further closing the gaps of surgery by bringing intervention in therapies.

Risk factors for bronchopleural fistula after right pneumonectomy: does eliminating the stump diverticulum provide protection?

PURPOSE: Bronchopleural fistula (BPF) remains an important source of morbidity and mortality after right pneumonectomy. We reviewed our 18-year institutional experience with right pneumonectomy to identify risks factors for BPF. METHODS: From 1992 to 2010, a total of 145 patients who underwent right pneumonectomy were identified from an institutional database. Median age was 56 years. Most patients (66.2%) underwent surgery for non-small cell lung cancer. Sixty-seven patients (46.2%) received either chemotherapy or radiotherapy before surgery. Medical records were reviewed for 14 variables potentially predictive for BPF, including two airway closure techniques (standard bronchial closure and carinal closure). Variables predictive of BPF by univariate analysis were entered into a logistic regression model. RESULTS: The overall mortality rate was 13.1% (n=19), with 15.9 and 10.5% mortality in the bronchial closure and carinal closure groups, respectively (P=0.33). The overall BPF rate was 7.6% (n=11), with a 3.9% (3 of 76) rate in the carinal closure group compared to 11.6% (8 of 69) in the bronchial closure group (P=0.08). Seven of eight bronchial closure patients who developed BPF required operative repair. Only one of three patients who developed BPF after carinal closure did not spontaneously heal after open drainage. Multivariate analysis identified preoperative radiation dose (P=0.042) and bronchial closure (P=0.041) as independent risk factors for BPF, while the length of postoperative ventilation before development of BPF approached significance (P=0.057). CONCLUSIONS: In our experience, higher preoperative radiation doses are a risk factor for BPF after right pneumonectomy, while carinal closure exerts a protective effect.

Inferring bona fide Differentially Expressed Genes and Their Variants Associated with Vitamin K Deficiency Using a Systems Genetics Approach.

Systems genetics is key for integrating a large number of variants associated with diseases. Vitamin K (VK) is one of the scarcely studied disease conditions. In this work, we ascertained the differentially expressed genes (DEGs) and variants associated with individual subpopulations of VK disease phenotypes, viz., myocardial infarction, renal failure and prostate cancer. We sought to ask whether or not any DEGs harbor pathogenic variants common in these conditions, attempt to bridge the gap in finding characteristic biomarkers and discuss the role of long noncoding RNAs (lncRNAs) in the biogenesis of VK deficiencies.

Spontaneous Esophageal Perforation in an Infant: A Rare Case.

Spontaneous perforation of the esophagus is an emergency that requires early diagnosis and management. It may be fatal and delay in treatment can cause an increase in morbidity and mortality. Despite of being very rare in infants, we have to be watchful whenever we encounter signs and symptoms related to it. Only 7 cases of spontaneous esophageal perforation in infants have been report in the literature to the best of our knowledge. Here we are reporting a rare case of spontaneous esophageal rupture in an infant.

Advances in thoracoscopy: 100 years since Jacobaeus.

Thoracoscopy provides the physician a window into the pleural space, and enables the biopsy of the parietal pleura under direct visual guidance, chest tube placement and pleurodesis for recurrent pleural effusions or pneumothoraces in selected patients. In this review, we discuss the advances that have been achieved in thoracoscopy since its inception more than a century ago.

Nurse-administered propofol sedation: feasibility and safety in bronchoscopy.

BACKGROUND: Propofol is a fast-acting intravenous sedative that has advantages as a procedural sedative over traditional regimens. It has been shown to have a similar safety profile to traditional sedating medications in the setting of gastroenterologic endoscopy. Nurse-administered propofol sedation is given by a specially-trained nurse, without anesthesiologist involvement. OBJECTIVES: We have used nurse-administered propofol sedation in our bronchoscopy suite for several years. In this report, we summarize our experience with nurse-administered propofol sedation, and demonstrate it to be feasible and safe for bronchoscopic procedures. METHODS: Procedure reports and nursing notes for 588 bronchoscopic procedures performed between July 2006 and June 2008 were retrospectively reviewed. Patient demographics, procedure type and indication, procedure time, medication doses, and adverse events were noted and analyzed. RESULTS: Nurse-administered propofol sedation was used in 498/588 (85%) procedures. Patients utilizing nurse-administered propofol sedation had an average age of 53 years (range 18-86) with an average weight of 80 kg. 56% of the patients were male, and 57% of the procedures were performed on outpatients. Average procedure duration was 25 min (range 3-123). The average propofol dose was 3.13 mg/kg (range 0.12-20 mg/kg). Adverse events attributable to sedation were noted in 33 (6.6%) procedures. Of the 14 (2.8%) major adverse events (death, need for intubation, ICU stay, or hospitalization), only 6 (1.2%) were potentially attributable to the sedation regimen. There were 2 deaths, neither of which was related to sedation. CONCLUSIONS: Nurse-administered propofol sedation is a feasible and safe sedation method for bronchoscopic procedures.

Role of plain abdominal radiographs in predicting type of congenital pouch colon.

BACKGROUND: Congenital pouch colon (CPC) is a rare form of high ano-rectal malformation (ARM) in which part of or the entire colon is replaced by a pouch with a fistula to the genito-urinary tract. According to the Saxena-Mathur classification CPC is divided into five types. Although plain abdominal radiographs are taken in infants with suspicion of CPC to detect large dilatation of the pouch, the determination of the type of CPC is made during surgical exploration. Since large variations in the length of normal colon are present in the various types, management strategy options can be determined only at the time of surgery. OBJECTIVE: The aim of this study was to review abdominal radiographs of children with congenital pouch colon (CPC) and evaluate their value in determining the type of CPC prior to surgical exploration to assist pre-operative planning. MATERIALS AND METHODS: Over a 12-year period (1995-2007), CPC was documented in 80 children (52 boys and 28 girls, age range 1 day-9 years, median 2.4 days) and retrospective analysis of plain abdominal radiographs of 77 children at the time of presentation was performed. Radiographic findings were correlated with surgical findings. RESULTS: Of 77 children, 5 were excluded from the study since the pouch colon was perforated. The direction of the pouch apex was correlated with surgical findings to determine the CPC type (P<0.0001, Fisher exact test). Type 1 (17/18) and type 2 CPC (18/18) were characterized by a single large pouch with the apex positioned in the left hypochondrium. In type 3 CPC (2/2) the pouch apex was directed towards the right hypochondrium. In type 4 CPC the apex of the pouch was directed towards the right hypochondrium (28/33); however in 5 children it was towards the left hypochondrium. In type 5 CPC (n=1) the radiograph was inconclusive. CONCLUSION: Plain abdominal radiographs have a predictive value in determining the type of CPC and obviating the need for an invertogram.