Nasal Pyriform Aperture Stenosis in a Newborn: When to Operate.

Congenital nasal pyriform aperture stenosis (CNPAS) is a very rare cause of neonatal respiratory distress and is often missed because of its rarity. It arises from the overgrowth of the nasal process of the maxilla. Maxillofacial CT scan findings of pyriform aperture width <11 mm in a full-term baby, median central incisor, triangular-shaped palate, and median palatal ridge confirm the diagnosis. We describe here a case of CNPAS admitted with respiratory distress that increased further on feeding. An infant feeding tube of size 6 was not negotiable through the nostrils. Resistance was appreciated at the inlet of the nostril. Maxillofacial CT showed pyriform aperture stenosis of 3.4 mm, suggesting CNPAS. The child could not be weaned off a high-flow nasal cannula despite conservative management with decongestants, steroids spray, dilatation, and stenting for 20 days. Subsequently, surgical widening of the nasal aperture by a sublabial approach was done. The child was discharged on the 10th postoperative day on full oral feeds. It is important to suspect CNPAS in neonates with respiratory distress where other common causes have been ruled out, as it can be treated by surgery in cases refractory to conservative management.

Laparoscopic choledochal cyst excision and biliary reconstruction in patients with previous surgery/ intervention: Feasibility and outcome.

INTRODUCTION: The aim of the study was to evaluate the feasibility and outcome of laparoscopic surgery in complicated choledochal cyst (CDC) with previous interventions (laparotomy or biliary drainage). PATIENTS AND METHODS: Patients with CDC who underwent surgery from July 2014 to July 2019 were evaluated. CDC without previous interventions (Group A) was compared with CDC that had previous interventions (Group B) to assess the feasibility and outcome of laparoscopic surgery. RESULTS: In 5 years' period, 38 patients were operated for CDC. The mean age was similar in both groups (3.78 ± 2.27 in Group A and 4.08 ± 2.73 in Group B). Out of six CDC with previous intervention (Group B), five patients were previously managed at other institutions as follows: (1) Laparoscopic cholecystectomy and endoscopic retrograde cholangiopancreatography (ERCP) stenting. (2) Laparotomy for biliary peritonitis and ERCP. (3) Percutaneous drainage of the large cyst. (4) Laparoscopic cholecystectomy. (5) ERCP stenting. (6) Percutaneous drainage for biliary ascites. All patients underwent laparoscopic CDC excision and hepatico-duodenostomy. The mean duration of surgery was 160.3 ± 17.22 in Group A and 169.2 ± 17.5 in Group B ( P = 0.258). None required intraoperative blood transfusion. None had a bile leak. Drain was removed at 4.47 ± 0.98 in Group A, while at 4.17 ± 0.75 days in Group B ( P = 0.481). There was statistically no significant difference in feed starting time or length of stay. In follow-up of 6 months-3 years, all patients are asymptomatic. CONCLUSIONS: Laparoscopy in complicated CDC with previous intervention is technically tedious but is feasible. The procedure is safe and delivers a good outcome.

Ruptured choledochal cyst: One-stage or two-stage, open or laparoscopic surgery?

Choledochal cyst (CDC) can present with complications such as cholangitis, perforation, pancreatitis and malignancy. Compared to the open technique for CDC, advantages of laparoscopic surgery are well known. Biliary peritonitis secondary to perforation/rupture of CDC is rare and is described in <2% of cases. In ruptured CDC, there are different approaches of management like peritioneal lavage, and biliary drainage by T-tube or Cholecystosomy/choledochostomy or endoscopic retrograde cholangiopancreatography stenting and definitive repair after 4-6 weeks as two-staged procedure. Laparoscopy is a procedure of technical challenge and complexity in ruptured CDC. We discuss here our experience of single-stage laparoscopic excision and biliary reconstruction in ruptured CDC. Child was stabilised by percutaneous drain and one-stage laparoscopic reconstruction was done on day 4, reducing the burden/morbidity of carrying biliary drain, length of stay and cost factor. To the best of our knowledge one-stage laparoscopy in ruptured CDC has not been reported earlier.

Refractory congenital chylous ascites: First report of fibrin glue and mesh application by laparoscopy.

Chylous ascites (CA) is a form of ascites having leakage of lipid-rich lymph into the peritoneal cavity, due to damage or obstruction in the lymphatic system. Aetiology of CA could be congenital or acquired. Primary lymphatic hypoplasia is seen commonly in children and presents with lymphoedema, chylothorax or CA. CA is initially treated conservatively with the aim to provide gut rest and decrease intestinal secretions. Surgical treatment is recommended if 1-2 months of conservative approach fails. The success of the operation depends on identifying the site of leakage of the lymphatic duct. Surgical options are ligation of leaking lymphatics, peritoneo-venous shunt, laparotomy and fibrin glue. Laparoscopy has been used for diagnosis but not for glue and mesh application in congenital CA where the lymphatic leak is unidentified. We present here the first experience of laparoscopic fibrin glue and mesh application in congenital CA with successful outcomes.

Thoracoscopic excision of double-pulmonary sequestration in left haemithorax, without anomalous blood supply (supply from pulmonary artery) in a neonate: First case.

Pulmonary sequestration (PS) is a rare anomaly. PS is a mass of abnormal pulmonary tissue that does not communicate with the tracheobronchial tree and is supplied by an anomalous systemic artery. Although aberrant systemic arterial supply is considered the key element to diagnose PS, rarely it can have arterial supply from the pulmonary artery as a spectrum of sequestration. Here, we present an unusual case of double (upper and lower) extralobar sequestration, present unilaterally (left haemithorax) in a neonate, without anomalous blood supply (supply from the left pulmonary artery and drainage into the superior and inferior pulmonary veins), not reported in literature earlier, to the best of our knowledge. In the present case, a child presented with respiratory discomfort at birth, requiring surgery due to inability to wean off respiratory support. It is important to be aware of this variant of sequestration spectrum. In a 18 days old child, both pulmonary sequestrations were resected thoracoscopically, making it a rare case, not described earlier in literature.

Giant Omphalocele Complicated by Postoperative Duodenal Obstruction.

Omphalocele is a congenital defect in the abdominal wall, usually treated at birth or within 1-2 years of life depending on condition of patient and size and contents of the defect. We repaired a giant omphalocele without mesh in a 9-year-old girl. She developed duodenal obstruction in the postoperative period requiring another laparotomy and duodeno-jejunostomy to bypass obstruction.

Facial swelling with atypical mycobacterial infection in immuno-competent patient.

A middle aged immune-competent woman presented with a facial swelling, which was excised. The histopathology showed it to be a non-caseating granuloma. PCR confirmed that the swelling was caused by mycobacterium other than tuberculosis (MOTT) infection. No relapse was seen on follow up even without chemotherapy.

Pseudoexstrophy with epispadias.

The exstrophy variants are uncommon anomalies. The variants of the exstrophy complex have all the stigmata of the classical exstrophy such as divergent recti, widened symphysis pubis, and low-set umbilicus; however, the urinary tract is intact to a varying degree. Pseudoexstrophy, an exstrophy variant, is very rarely associated with epispadias. We report an unusual case of pseudoexstrophy with epispadias, in whom the intact bladder was initially covered by a mucous membrane which later epithelialized. The epispadias was repaired using a penile disassembly technique with posterior and ventral placement of bladder neck and urethra. Although the urethral meatus was hypospadiac, the child had achieved dry intervals with occasional stress incontinence.

Meckel's diverticulum with segmental dilatation of the ileum: radiographic diagnosis in a neonate.

We report a neonate with segmental dilatation of the ileum and a Meckel's diverticulum that was diagnosed preoperatively on the plain abdominal radiograph.