Atypical facial clefts are rare anomalies that occur due to the failure of embryonic fusion of the branchial arches. The midline mandibular cleft or Tessier 30 cleft is one such rare anomaly. Such anomalies are diagnosed at birth and treated within a few months of age by establishing soft tissue and bony continuity, followed by orthognathic treatment in adulthood. It is very rare for such clefts to go untreated until adulthood. We present one such case report and our technique of management in a patient who presented to us at 25 years of age with a lower lip and mandible cleft with ankyloglossia, which was managed with a single-staged reconstruction.

Choledochal cysts are uncommon dilatations of the biliary tree. Giant choledochal cysts are those that exceed a maximum diameter of 10cm. Our case describes a female infant who presented to our paediatric surgery department with a three-day history of vomiting, abdominal distention, pale stool, and irritability. On palpation, she was found to have a large abdominal mass and the computed tomography (CT) scan showed a giant choledochal cyst. The patient underwent laparotomy with cholecystectomy, choledochal cyst drainage and complete excision, with hepaticojejunosotomy. At the last follow-up three years post-surgery, all growth parameters and liver enzymes were within normal ranges. To the best of our knowledge, this is the first documented case of a giant choledochal cyst in the paediatric Caribbean population.

UNASSIGNED: Hydatosis is a zoonotic parasitic disease caused by echinococcosis larval infection. South America, Africa, the Middle East, South Europe, India, and Australia are endemic to this disease. Splenic involvement is a rare and complicated hydatid disease presentation. A splenic hydatid cyst is an infrequent clinical occurrence, even in regions where the disease is endemic.
UNASSIGNED: A 16-year-old male, having a background of mild abdominal trauma and non-resolving dull abdominal pain attended a paediatric surgical outpatient office and following a thorough examination, was diagnosed with a giant solitary isolated splenic hydatid cyst. Subsequently, the patient received albendazole and underwent total splenectomy, necessitated by the considerable size of the cyst, classified as a giant.
UNASSIGNED: Splenic involvement of hydatid disease is a rare presentation (0.5-8%.). With the initial clinical finding often involving the accidental discovery of a palpable mass, the most frequently reported symptoms and signs include the presence of a palpable mass, fever, dull pain, or splenomegaly. Ultrasound and computed tomography are the most helpful tools for evaluating focal splenic diseases. The preferred treatment involves the use of antihelminthic drugs such as albendazole or mebendazole in conjunction with splenectomy. Total splenectomy is the preferred approach and is associated with decreased hospital stay, reduced healthcare costs, and a lower likelihood of recurrence.
UNASSIGNED: in endemic areas, in patients with splenic cysts, hydatidosis should be contemplated.

A neonate was born with a unique congenital lower limb dysmelia due to an abnormal presentation of amniotic band syndrome. An anomalous soft tissue tether from the plantar surface of the right foot to the right buttock caused extreme knee flexion, tibial rotation and malformation of the developing foot. This complex malformation required a multidisciplinary team (MDT) approach to decide between reconstruction and amputation. The band of tissue was released operatively at 73 days postdelivery, improving knee extension, and the tissue was banked on the thigh as a tube pedicle for future reconstruction. The patient underwent rehabilitation, which has been shown to be vital for synovial joint formation. At 18 months old, the decision was made to proceed with through-knee amputation and a prosthesis. The literature discussed shows the importance of an MDT approach in complex lower limb cases to give the best functional outcome for the patient.

BACKGROUND: Exomphalos anomaly is defined as the herniation of abdominal viscera into the base of the umbilical cord, with only a membranous sac covering these contents. It has an incidence of approximately 1 in 4000-6000 births. Management of exomphalos major (EM) remains controversial and limited, with very few studies to guide decision-making.
METHODS: This is a case series of four neonates with EM treated at a tertiary paediatric referral centre between 2018 and 2021 with a gradual compression dressing technique.
RESULTS: Four neonates were diagnosed with EM. The average gestational age was 38 + 5 (range 38 + 2 - 39 + 2), and the average birth weight was 3.1 kg (range 2.56 - 3.49 kg). The defect size ranged between 5 and 7 cm. All patients were commenced on gradual compression dressing between days 1 and 3 of life. Dressings were applied at the bedside in the general neonatal ward. The average time taken to reach full feeds was 1 week; only one patient required parenteral nutrition. Three underwent surgical repair at two and 16 weeks of age; one had delayed repair at the age of 1 year because of the COVID-19 pandemic. None required patch repair. None required prolonged ventilation after repair.
CONCLUSIONS: This case series describes a successful compression dressing technique that reduces sac content without the need for general anaesthetic or respiratory compromise, whereby simultaneous enteral feeding is tolerated.

A female full-term neonate, accompanied by her parents, was referred to the paediatric surgery department on the day of after birth. She presented with a 9 cm length pathological umbilical cord, of which the first 7 cm was red and wet, with ulceration, necrosis and healing areas. The patient never had a fever. Abdominal palpation showed no umbilical hernia and abdominal Doppler ultrasound was normal. After several days of disinfection, by biseptine antiseptic solution, and a monthly follow-up, most of the umbilical cord fell out. It only remained a 4 cm length navel consisting of 2 cm of excessive skin and 2 cm of mucous tissue. The lesion was surgically excised at 6 months old. The patient was discharged on postoperative day 1. The results of the histology confirmed the diagnosis of an epithelialised umbilical cord. The 1-month follow-up was uneventful.

Although mucoepidermoid carcinoma (MEC) is the most diagnosed malignancy of the salivary gland, it rarely localises to the bronchus, accounting for only 0.1-0.2% of all primary lung malignancies. Of those pulmonary MECs, most are found in segmental or lobar bronchi, and they are rarely found in mainstem bronchi, highlighting the novelty of this presentation for thoracic specialists. We present a case report of a seven-year-old female who underwent a carinal resection and a right upper lobectomy for the management of an endobronchial MEC causing right middle lobe (RML) obstruction. Intraoperatively, an exophytic mass originating from the junction of the right main bronchus and bronchus intermedius was identified, causing a partial obstruction of the RML bronchus. Frozen sections demonstrated clear margins and follow-up bronchoscopies have been unremarkable. Given their rarity, endobronchial MECs can be diagnostically difficult and cause uncertainty with respect to their management. Low-grade tumours have a much more favourable prognosis than their high-grade counterparts, with surgical resection being the gold standard of care. Therefore, the index of suspicion, time to diagnosis, and definitive treatment are critical to the outcome.

The authors describe a case of a male neonate with split notochord syndrome presenting with cervico-thoracic deformity, thoracic neuroenteric cyst, separate abdominal duodenal duplication cyst and concurrent intestinal malrotation. This combination of abnormalities is very rare. When these lesions are suspected, patients must be investigated carefully.This case is presented not only to recount an infrequent combination of structural abnormalities but also to raise awareness of the signs that should point to clinical suspicion and prompt diagnosis.Following surgical excision of the thoracic neuroenteric cyst, the patient has made a good recovery.

Scrotal epidermoid cysts are rare. Intratesticular epidermoid cysts are more common than extra scrotal cysts and are the most commonest benign tumors of the testicles. Midline scrotal raphe cysts are reported, but only a few have intrapelvic extensions deep into the pelvis. A nine-year-old boy presented with a painless scrotal swelling. Magnetic resonance imaging (MRI) of the pelvis confirmed the cystic nature with an extension of the swelling up to the base of the prostate. On surgical exploration, the cyst had a tapering stalk with cranial extension up to the base of the prostate. The patient underwent an excision of the cyst and made an uneventful recovery and was asymptomatic at the end of three months of surgery. The histopathology of the lesion was typical of an epidermoid cyst. Extratesticular scrotal epidermoid cysts with pelvic extension are a rarity with less than five cases reported in the literature. Our case stands to be the youngest reported case of a scrotal epidermoid cyst based on our knowledge. Scrotal epidermoid cysts are a very rare and benign entity, and upon recognition and confirmation of the extent of spread, extratesticular scrotal cysts can safely be removed. No other management may be required with no recurrences reported.

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