Chronic Constipation Unmasking as Hirschsprung Disease in a Preadolescent: Delayed Presentation or Delayed Diagnosis?

Hirschsprung disease, a rare genetic disorder affecting the enteric nervous system, is characterized by the absence of ganglion cells in the myenteric plexus. Typically identified in neonates due to the failure to pass meconium, diagnosis beyond the first year of life is considered delayed. Common clinical manifestations in children with late-onset Hirschsprung disease include abdominal distension, abdominal pain, vomiting, fever, and abnormal bowel sounds. Sigmoid volvulus, though uncommon, can complicate Hirschsprung disease, potentially leading to misdiagnosis and severe complications such as intestinal perforation, hemorrhage, sepsis, and even mortality. Non-surgical interventions such as antibiotic therapy, intestinal decompression, and fluid resuscitation are preferred initial treatments to stabilize the patient. This case involves a 9-year-old boy who has presented with abdominal distension since birth and a lengthy history of irregular bowel habits. The diagnosis of Hirschsprung disease was confirmed at our institution, and the patient underwent a two-stage repair procedure, which was completed without any intraoperative or postoperative complications. The patient experienced an uneventful recovery, was discharged with stable vital signs, and regained normal bowel function. This case highlights the challenges of delayed diagnosis at nine years and underscores the importance of prompt management.

Surgical Management of Omphalocele With Concurrent Ileal Atresia: A Case Report.

Omphalocele, a congenital anomaly characterized by the protrusion of abdominal viscera through the umbilical ring, often presents challenges in surgical management, especially when concurrent with other anomalies such as intestinal atresia. We presented a case of a female infant weighing 2.6 kg born with omphalocele and concurrent ileal atresia. The child was successfully managed through prompt surgical intervention. Preoperative investigations revealed signs suggestive of intestinal obstruction, necessitating immediate surgical exploration. Intraoperatively, meticulous reduction of the omphalocele sac and resection of the atretic segment were performed. Postoperative care in the neonatal intensive care unit ensured optimal recovery. This case underscored the importance of timely intervention and multidisciplinary collaboration in managing complex congenital anomalies in neonates.

A Female Infant with Rectovestibular Fistula and Imperforate Hymen.

In a female neonate with anorectal malformation (ARM), the diagnosis is usually simple. But when there are two openings in the introitus and absent anal opening at the normal site, the scenario poses a diagnostic challenge. Careful and detailed evaluation of anomaly is therefore necessary before planning definitive correction. Imperforate hymen, although is not commonly associated with ARM should always be kept in mind as a differential diagnosis and other vaginal anomalies like Mayer-Rokitansky-Küster-Hauser syndrome should be ruled out before definitive surgical correction.

Intra-Uterine Perforation Presented as a Giant Cystic Abdominal Mass in a Neonate: A Giant Meconium Pseudocyst.

A meconium pseudocyst is formed following meconium peritonitis. At present, antenatal diagnosis and planned management of meconium pseudocyst have reduced the mortality rate significantly. We presented a case of a neonate with abdominal distension and non-passage of meconium who experienced respiratory distress and was taken for exploratory laparotomy at a tertiary care center due to suspected bowel perforation. The neonate was diagnosed with a meconium pseudocyst intraoperatively as maternal ultrasound and ultrasound of the abdomen of the neonate after birth failed to make a definitive diagnosis; even an X-ray abdomen did not reveal pathognomonic egg-shell calcification. An interesting aspect of this case is the mother's complex obstetric history, which compelled us to conjecture whether it was possible to predict the chances of meconium peritonitis and take steps to prevent it. It must be noted that, despite rigorous research, the researchers could not find reliable literature co-relating the obstetric history of the mother with the formation of a meconium pseudocyst in the neonate.

Successful Outcome With a Multimodal Approach in the Management of Pediatric Necrotizing Fasciitis: A Report of a Rare Case.

Necrotizing fasciitis is a commonly encountered modality by a practicing clinician which needs urgent and vigilant management. To decrease the potential morbidity and mortality of the pathology, an aggressive multimodal approach shows promising results. In our report, we present a case of a three-year-old male child who presented to us with the findings consistent with necrotizing fasciitis. The case report highlights the importance of multimodal approach in such a case.

A Rare Case Presentation on Total Colonic Aganglionosis in a Female Infant of Indian Origin.

Total colonic aganglionosis, also called total colonic Hirschsprung's disease, is a known congenital disorder caused by the migration of abnormal embryonic neuroblasts. RET, NRG1, and L1CAM genes are reported as pathological gene variants associated with the incidence of different variants of Hirschsprung's disease. Major clinical presentations are well documented as inefficiency to pass stools, vomiting, fever, persistent crying, and other features of intestinal obstruction. We present here the case of a two-day-old female infant of Indian origin and its diagnostic, clinical, and case management data.

Importance of Second-Look Surgery in a Newborn Presenting With Early Malrotation.

Intestinal malrotation refers to the abnormal positioning of the intestines due to a deviation from normal developmental stages. Volvulus is seen in 60%-70% of neonates diagnosed with intestinal malrotation. We are reporting a case of s six-day-old male who presented with multiple episodes of bilious vomiting and constipation and had malrotation of intestines with midgut volvulus. After a contrast upper GI study, the patient was taken for exploratory laparotomy, and extensive patches of an early stage of bowel ischemia were observed; resection was avoided at this stage. In the second-look surgery, all the gangrenous bowel loops were resected, and anastomosis was done.

Small Bowel Injury Due to Electrocautery Excision of Umbilical Granuloma in a Four-Month-Old Child.

Many surgeons are familiar with small bowel perforation-a breach in the continuity of the bowel wall resulting in spillage of contents into the peritoneal cavity. Usually, patients present with severe abdominal pain, and radiological investigations suggest pneumoperitoneum. However, intestinal perforation secondary to electrocautery used for umbilical granuloma excision is rare. We report a case of a 4-month-old boy who presented with primary concerns of constipation, severe abdominal pain, and multiple episodes of vomiting three days following an electrocautery excision of umbilical granuloma. An exploratory laparotomy revealed a perforation of the terminal ileum. Primary repair of the ileal perforation was done, which saved the infant's life. As this case illustrates, even a minor surgical procedure can lead to a major intraperitoneal injury, and appropriate evaluation based on clinical signs and symptoms is imperative. This case is also a reminder to handle an electrosurgical instrument with proper skill, training, and technical assistance.

Pelvico-Vesicostomy for Horseshoe Kidney With Severe Right Ureteric Stricture and Bilateral Hydronephrosis.

Ureteropelvic junction (UPJ) obstruction is the most common renal anomaly observed in infants with congenital hydronephrosis. The present study presents a five-month-old infant with severe right ureteropelvic obstruction. Anderson-Hynes pyeloplasty intervention significantly improved renal function. The study concludes that early surgical intervention is the definitive treatment for avoiding kidney impairment and its complications.

Management of Liver Abscess in Children: Our Experience.

INTRODUCTION: Liver abscess is common in pediatric population in India. Children have unique set of predisposing factors and clinical features. Liver abscesses are infectious, space-occupying lesions in the liver; the two most common abscesses being pyogenic and amebic. Its severity depends on the source of the infection and the underlying condition of the patient. MATERIALS AND METHODS: A total of 34 patients less than 12 years were assessed in a retrospective study from January 2012 to 2016. Patients were assessed in terms of age of presentation, etiology, bacteriology, diagnosis, and modality of treatment. RESULTS: The mean age of presentation was 6.3 years. Average volume of abscess was 164 cc. Nine patients (26.4%) underwent percutaneous needle aspiration under ultrasound guidance with wide bore needle (18 G disposable needle). Three patients required more than two sittings of aspiration. Patients with volume more than 80 cc were treated with catheter drainage. Twenty patients (58.8%) underwent ultrasound-guided percutaneous catheter drainage. Two patients required catheter drainage for large abscess and needle aspiration for the smaller abscess. CONCLUSION: Antimicrobial therapy along with percutaneous drainage constitutes the mainstay of treatment, whereas open surgical drainage should be reserved for selected cases.How to cite this article: Waghmare M, Shah H, Tiwari C, Khedkar K, Gandhi S. Management of Liver Abscess in Children: Our Experience. Euroasian J Hepato-Gastroenterol 2017;7(1):23-26.

Management of Traumatic Liver and Bile Duct Laceration.

Posttraumatic major bile leak in children is uncommon, with few cases reported in the literature. These injuries are seen in high-grade liver trauma and are difficult to diagnose and manage. We describe a 7-year-old boy with grade IV hepatic trauma and bile leak following blunt abdominal trauma. The leak was successfully managed by percutaneous drainage and endoscopic retrograde cholangiopancreatography (ERCP) stenting of the injured hepatic duct. How to cite this article: Tiwari C, Shah H, Waghmare M, Khedkar K, Dwivedi P. Management of Traumatic Liver and Bile Duct Laceration. Euroasian J Hepato-Gastroenterol 2017;7(2):188-190.

Mayer-Rokitansky-Kuster-Hauser syndrome associated with rectovestibular fistula.

A female neonate with two openings in the introitus and an absent anal opening at the anal site presents a diagnostic challenge. Mayer-Rokitansky-Kuster-Hauser (MRKH) syndrome associated with rectovestibular fistula, though rare, should be kept in mind as a differential diagnosis of this presentation. We present such a case in a one-year-old female child with MRKH syndrome and rectovestibular fistula.

Cysts of Gastrointestinal Origin in Children: Varied Presentation.

PURPOSE: Abdominal cysts of gastrointestinal origin are rare. Their rarity and varied clinical presentations make their pre-operative diagnosis difficult. METHODS: Fourteen patients with histological diagnosis of cysts of gastrointestinal origin admitted between 2009 and 2015 were retrospectively analyzed with respect to age, sex, clinical presentation, diagnostic modality, site and type of cyst, management, outcome and follow-up. RESULTS: The mean age at presentation was 4 years and there were six males and eight females. Abdominal pain was the most common presenting symptom. Five patients had an acute presentation-three had distal ileal mesenteric cysts and two had ileal duplication cyst sharing a common wall with ileum. Six patients presented with chronic abdominal pain and lump-three patients had omental cysts and three had mesenteric cysts-two of these in distal ileum and one in sigmoid colon. Two patients presented with antenatally diagnosed palpable abdominal lump. One had a mesenteric cyst of the ileum and the other had a distal ileal duplication cyst which required excision with resection and anastomosis. One patient had an atypical presentation. He was a known case of sickle cell trait and had presented with vague abdominal pain, recurrent cough and multiple episodes of haemoptysis over a period of one year. At laparotomy, gastric duplication cyst was found which was excised completely. Histopathology confirmed the diagnosis. CONCLUSION: Cysts of gastrointestinal origin are rare and have varied presentation. Surgical excision is the mainstay of treatment. The results and prognosis are good.

Mesenteric cyst(s) presenting as acute intestinal obstruction in children: Three cases and literature review.

BACKGROUND AND OBJECTIVES: Mesenteric cysts are rare in paediatric age group and usually present as asymptomatic abdominal lumps. Acute presentations are uncommon and their preoperative diagnosis is difficult. DESIGN AND SETTINGS: This is a retrospective observational study describing three children with mesenteric cysts who presented with symptoms and signs of acute intestinal obstruction. PATIENTS AND METHODS: Three children with mesenteric cysts who presented with acute abdomen are described as per their age, presenting symptoms and signs, investigations, management, and outcome. RESULTS: The three children presented in emergency with symptoms and signs of acute intestinal obstruction. On exploration, all had mesenteric cyst and were managed by deroofing, marsupilisation and excision. All patients recovered uneventfully. CONCLUSIONS: Presentation of mesenteric cyst as acute obstruction in paediatric age group is rare and preoperative diagnosis is difficult. The larger cysts are more likely to have an acute presentation.

Our experience with adnexal masses in the pediatric age group and review of literature.

BACKGROUND AND OBJECTIVES: Adnexal masses are rare in the pediatric age group. We present our experience with 20 patients with adnexal masses. DESIGN AND SETTING: This retrospective observational analysis was performed on 20 children with adnexal masses who were treated at our institute between May 2011 and November 2015. PATIENTS AND METHODS: Fifteen pediatric patients who were admitted between May 2011 and November 2015 were reviewed and retrospectively analyzed based on their age at the time of admission, their presenting complaints, clinical and radiological findings, tumor markers, management and follow-up. RESULTS: The patients' age at the time of admission ranged between 3 days and 12 years. Abdominal pain and lump were the most common presenting complaints.Four patients (20%) had antenatally diagnosed cystic ovarian lesions. On postnatal scan, two patients had a simple cyst measuring less than 6 cm, which resolved on follow-up ultrasound at 3 months. One neonate had a simple cyst, larger than 6 cm on postnatal scan, which was managed by marsupialization. One antenatally diagnosed patient had a dermoid cyst that required oophorectomy.Ten patients (50%) had dermoid cyst and underwent complete surgical excision of the mass. Based on histopathologic results, two of these patients had immature teratoma and required adjuvant chemotherapy (Bleomycin, Etoposide, and Cisplatin). The serum AFP levels of these patients were carefully monitored.One patient with bilateral ovarian cysts was diagnosed with Van Wyk-Grumbach syndrome, which resolved significantly after a 3-month regimen of thyroxin supplementation.Five patients presented with torsion and required emergency surgery-three had mature teratoma, one had an immature teratoma and one had large simple cysts. CONCLUSION: The majority of ovarian tumors are benign. Accurate staging, complete resection and chemotherapy for the treatment of malignant tumors have contributed to excellent survival rates in these patients.