Riccardo Guanà

When to operate on ovarian cysts in children

Abstract Background: Ovarian cysts are rare conditions in the pediatric age group. They are characterized by different clinical presentations and by the need to establish adequate type and timing of treatment in order to prevent complications, such as ovarian necrosis after torsion and infertility. The diagnostic approach should differentiate benign occasional findings, such as follicular cysts, from neoplastic lesions, and functional cysts which can occur either isolated or in the context of McCune-Albright syndrome. Our aim was to review all the patients affected by ovarian pathologies seen in our department in the past 5 years, in order to establish a protocol for the correct management of these conditions. Methods: In the past 5 years we studied 133 patients diagnosed with ovarian lesion. We subdivided the patients into three groups according to age: group A (age <6 months: 66 cases); group B (pre-pubertal patients, age 7 months to 10 years: 10 cases); and group C (pubertal patients, age 11–14 years: 57 cases). We collected historical and clinical data and assigned specific cut-off values in order to perform statistical analysis (Fisher’s exact test) comparing the three groups. Results: Ultrasound examination proved to be indispensable in the early detection of ovarian lesions and for identifying the correct treatment. Cyst size, and moreover appearance with ultrasound, were important considerations when selecting the most appropriate therapeutic approach. The larger lesions were found in group B, giving symptoms that required hospitalization (pelvic pain, nausea and vomiting) and leading to increased incidence of postoperative complications. Laparoscopic surgery appeared to be safe and effective for the treatment of ovarian pathologies. Conclusion: The management of ovarian lesions in children must be based on a minimally invasive approach, based on the patient’s age and ultrasound findings. Treatment should be conservative, with ovariectomy undertaken only when essential. In adolescents, only cysts larger than 6 cm that do not resolve with estro-progestinic therapy within 6 months should be surgically approached; in infants, any signs of cyst complications must be addressed surgically.

The Complex Surgical Management of the First Case of Severe Combined Immunodeficiency and Multiple Intestinal Atresias Surviving after the Fourth Year of Life

Severe combined immunodeficiency (SCID) is a life-threatening syndrome of recurrent infections and gastrointestinal alterations due to severe compromise of T cells and B cells. Clinically, most patients present symptoms before the age of 3 months and without intervention SCID usually results in severe infections and death by the age of 2 years. Its association with intestinal anomalies as multiple intestinal atresias (MIA) is rare and worsens the prognosis, resulting lethal. We describe the case of a four year-old boy with SCID-MIA. He presented at birth with meconium peritonitis, multiple ileal atresias and underwent several intestinal resections. A targeted Sanger sequencing revealed a homozygous 4-bp deletion (c.313ΔTATC; p.Y105fs) in tetratricopeptide repeat domain 7A (TTC7A). He experienced surgical procedures including resection and stricturoplasty. Despite parenteral nutrition-associated liver disease, the patient is surviving at the time of writing the report. Precocious immune system assessment, scrutiny of TTC7A mutations and prompt surgical procedures are crucial in the management.

Laparoscopic management of ovarian cysts in peripheral precocious puberty of McCune-Albright syndrome

Ovarian cysts are common in peripheral precocious puberty in McCune-Albright syndrome (MAS). The clinical course of these cysts is unpredictable due to episodes of hyperestrogenism typical of MAS ovarian hyperfunction. In persistent and recurrent large ovarian cysts with sustained estrogen hypersecretion and relevant clinical disturbances (increased linear growth and bone age maturation, vaginal bleeding and psychological disturbances) treatment is mandatory. Experimental courses of estrogen-blocking drugs may have insufficient or nil therapeutic effects. In these cases and when molecular analysis is required to obtain MAS diagnosis as in isolated peripheral precocious puberty, surgery is the option. Laparoscopy minimizes surgical aggression and facilitates obtaining tissue samples for molecular analysis, and sometimes relieves hyperestrogenism with the excision of hyperactive ovarian areas. It can be conducted with trans-umbilical laparoscopic ovarian cystectomy (TULOC) before 3 years of age and with traditional techniques afterwards.

Surgical enucleation of testicular leydigioma in a young child: case report and literature review.

Abstract Background: The leydigioma is the most frequent interstitial neoplasm of the testis. Clinical symptoms of all leydigioma usually are isosexual precocious pseudo-puberty due to autonomous testosterone overproduction, suppressed gonadotropin levels and a testis mass. Nowadays its therapy is debated. Design and methods: We report a case of a young child treated in 2008 for a leydigioma of the testis with atypical clinical behavior. Testicular oncologic markers and hormonal profile were assessed. Results: The patient was treated successfully with organ-confined surgery. Conclusion: We want to confirm the safety of conservative surgery against radical orchiectomy and review the literature of the last decade on this debated matter.

Laparoscopic versus Open Surgery in Complicated Appendicitis in Children Less Than 5 Years Old: A Six-Year Single-Centre Experience

Introduction. Acute appendicitis is the most common surgical emergency in the pediatric population. The peak incidence occurs in the first decade of life, while it is uncommon to face appendicitis in children younger than 5 years of age. Laparoscopy is now demonstrated to be the optimal approach also to treat complicated appendicitis, but in very young children this standardized operation is not always easy to perform. Material and Methods. From January 2009 to December 2015 we operated on 525 acute appendicitis, with 120 patients less than 5 years of age. Results. 90 children had a complicated appendicitis (localized or diffuse peritonitis): 43 (48%) were operated on by open approach and 47 (52%) by laparoscopy. The overall incidence of postoperative complications was greater in the open appendectomy group (63% versus 26%) and all severe complications requiring reintervention (6% of cases: 3 postoperative abscesses resolved with ultrasound guided percutaneous abscess drainage; 1 tubal surgery for salpingitis; 1 adhesion-related ileus requiring relaparotomy) were mostly associated with open surgery. Conclusions. Laparoscopic surgery resulted as the best approach for treating complicated appendicitis also in younger children, with minor and less severe postoperative complications compared to open surgery.

Cystic intra-abdominal masses in children

Benign intra-abdominal cystic masses in infancy are fairly uncommon and their etiopathogenesis, histology and clinical presentation differ significantly. Our aim is to report our experience in their treatment in order to discuss the best diagnostic and treatment modality. The medical records of 5 children (2M, 3F) with cystic intraabdominal masses referred to our hospital between November 2012 and September 2016, were retrospectively reviewed. All patients underwent open surgery and subsequent histopathologic analysis. Different clinical presentations, localizations of the masses, diagnostic tools, surgical approaches, histological examinations and outcomes were reviewed. Patients mean age was 5.4 years (range: 8 months-9 years). Two patients presented recurrent abdominal pain and abdominal distension; 1 patient had a palpable mass discovered incidentally and 2 complained acute abdominal pain. Routine laboratory tests, tumor markers and abdominal ultrasound were immediately done in all patients. Three patients underwent MRI and 1 abdominal CT. At laparotomy 2 hepatic cysts, 2 mesenteric cyst and 1 retroperitoneal cyst were discovered. Histology reports described: 1 hepatobiliary cystadenoma, 1 benign hepatic hamartoma and 3 cystic lymphangiomas (1 retroperitoneal and 2 mesenteric). There were no major postoperative complications, deaths, or recurrences in our series (follow-up 3-24 months). Despite the rarity of these lesions, benign cystic abdominal masses in children are not so uncommon and should be considered as causes of acute abdominal pain. The differential diagnosis is not always possible preoperatively. In our series, radical excision of the lesions was possible in all cases, allowing reliable histological results and avoiding recurrences.

Biliary complications after hepatic trauma in children

Aim of the Study: In pediatric patients with liver trauma and hemodynamic stability, conservative treatment is acknowledged as the gold standard. Patients and Methods: We conducted a retrospective analysis of 116 consecutive pediatric patients (<14-year-old) observed at our institution for closed abdominal trauma from January 2010 to January 2016. Among these, 16 patients (13%) had hepatic trauma Grade II or more, according to Moore liver trauma injury score. Results: Only one patient underwent surgery for hemodynamic instability; all others children received conservative treatment according to the American Paediatric Surgical Association guidelines. Three patients had a biliary complication (2, 5%). two patients treated surgically by drainage insertion and one was managed conservatively. Conclusions: Biliary complications of liver trauma in children may require aggressive surgical approach in selective patients.

Gastrostomy Intraperitoneal Bumper Migration in a Three-Year-Old Child: A Rare Complication following Gastrostomy Tube Replacement.

Feeding gastrostomy is used worldwide for adults and children with feeding impairment to obtain long-term enteral nutrition. Percutaneous endoscopic gastrostomy insertion is considered the gold standard, but after the first months requires gastrostomy tube replacement with a low-profile button. The replacement is known as an easy procedure, but several minor and major complications may occur during and after the manoeuvre. We describe intraperitoneal bumper migration in a 3-year-old boy, a rare complication following gastrostomy tube replacement, and we discuss the recent literature regarding similar cases.