RESUMO
BACKGROUND: Prader-Willi syndrome (PWS) is a genetic disorder involving multiple systems, with an incidence of about 1/10000-25000. Ovarian torsion (OT) is not commonly found in children. Ovarian cyst acts as one of the primary factors resulting in OT. While ovarian cyst torsion with Prader-Willi Syndrome has not been reported before. CASE PRESENTATION: A 12-years old female was admitted to Emergency Department of our hospital with the chief complaint of abdominal pain. The outcomes of physical examination revealed the height of 150 cm, weight of 103 kg, BMI of 45.77 kg/m2. The patient manifested the special facial features, an obese body, with the abdomen distended into a spherical shape. The fat accumulation in the abdomen significantly embarrassed the palpation. The abdominal CT scan indicated a huge cystic mass in the abdominal cavity, sized about 138 mm × 118 mm. According to medical history, the patient was born with low crying and hypotonia, who has developed the uncontrollable eating behavior since 3-years old. These abnormalities led to a speculation of PWS syndrome, so a genetic test was performed and finally confirmed it, concluding a torsion of ovarian cyst with PWS. With the multidisciplinary consultation, a careful treatment strategy containing the control of blood pressure and blood sugar, coenzyme Q10 was administrated to nourish the myocardium and the application of Growth Hormone was developed. All the above preoperative treatments have brought great benefits to patients. Thus promising the successful completion of operation. The postoperative follow-up till now indicated that the abdominal incision was well healed, without operative complications. CONCLUSIONS: This may be the first case report. In the treatment of ovarian cyst torsion, PWS syndrome requires fully consideration, as the latter can lead to multisystem abnormalities, especially the relation to perioperative management, and even fatalities. Genetic testing should be conducted early when PWS was suspected, accompanied with adequate preparation for the perioperative period, the follow-ups of patients should be maintained for a long time after surgery.
Assuntos
Dor Abdominal , Cistos Ovarianos , Síndrome de Prader-Willi , Humanos , Feminino , Criança , Cistos Ovarianos/complicações , Cistos Ovarianos/cirurgia , Síndrome de Prader-Willi/diagnóstico , Síndrome de Prader-Willi/genética , Dor Abdominal/diagnóstico por imagem , Dor Abdominal/etiologia , Hipotonia Muscular , Obesidade , Hormônio do Crescimento HumanoRESUMO
Objective: In the present study, we report a retrospective analysis of 23 cases of conjunctival congestion after laparoscopic operation in children and try to explore the causes and intervention measures. Methods and Results: This is a retrospective, single-centre and observational study, and all patients with conjunctival congestion after laparoscopic operation admitted to our institution between August 2021 and December 2021 were included in this study. Records of 23 patients including 16 male patients and 7 female patients were retrospectively analysed. These patients were in the age group of 2-12 years. Their primary symptom was different degrees of conjunctival congestion, and the symptom onset was between 2 and 7 days after laparoscopic operation, including laparoscopic inguinal hernia repair, laparoscopic appendectomies, laparoscopic Meckel's diverticulectomy, laparoscopic removal of foreign body ingestions and laparoscopic choledochal cystectomy, and the duration of operations varies from 20 min to 255 min. The symptom disappeared from 5 to 21 days after the operation, and the duration of the symptom ranged from 2 to 14 days. A total of 1718 operations were performed, of which 461 were laparoscopic and 1257 were general operations, the incidence of conjunctival congestion after laparoscopic surgery was 23/461, and compared with 0/1257 after ordinary surgery, there was a significant difference between them. Of these 23 patients, 5 patients received no treatment and the other 18 patients were intervened with steroid-containing eye drops. Although eye drops containing steroids can significantly relieve eye discomfort, the duration of conjunctival congestion between the two groups (i.e. steroid-containing eye drop treated vs. non-steroid-containing eye drop treated) did not differ significantly. All patients recovered well. In the follow-up till the end of February 2022, no serious complications had occurred. Conclusion: Conjunctival congestion after laparoscopic operation is extremely rare in children, and the underlying causes are still unclear. We speculate that the pressure of pneumoperitoneum may be the main cause of this phenomenon. Symptoms may be self-limiting, and steroid-containing eye drops can relieve effectively the discomfort.