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Spontaneous intracranial hypotension is an uncommon etiology of secondary headaches in children. We report a unique case of a girl with kaposiform lymphangiomatosis who developed postural headaches and imaging features of spontaneous intracranial hypotension without a spinal extradural collection. The girl underwent dynamic computed tomography myelography which revealed a cerebrospinal fluid (CSF)-lymphatic fistula related to a lymphatic malformation associated with the right T10 nerve. She underwent surgical ligation of the CSF-lymphatic fistula, resulting in resolution of the headaches. Spinal CSF-lymphatic fistulas are rare and have previously been reported in two patients with Gorham-Stout disease. The current report suggests that patients with systemic lymphatic anomalies who develop postural headaches should undergo evaluation for spontaneous intracranial hypotension and a CSF-lymphatic fistula. If discovered, surgical ligation is a potential treatment.

Importance: Chylothorax is the most common cause of pleural effusion in neonates and relatively rare in children. It can cause significant respiratory morbidity. Many clinical entities may contribute to chylothorax. Objective: To investigate the causes and manifestations of chylothorax in infants and children in China. Methods: Case records of 107 cases with chylothorax seen in Beijing Children’s Hospital from 2007 to 2017 were retrieved and analyzed; follow-up was carried out by telephone. Results: Of 107 cases, 58.9% (63/107) were primary chylothorax (PC) and 41.1% (44/107) were secondary chylothorax (SC). Also, 36.4% (39/107) were neonatal chylothorax (NC) and 35.5% (38/107) were postoperative chylothorax. In PC with a verified lymphatic anomaly, there was one case of diffuse pulmonary lymphangiomatosis (DPL) and six cases of generalized lymphatic anomaly (GLA), which accounted for 6.5% (7/107) of cases. In most patients, chylothorax was alleviated by conservative treatment based on total parenteral nutrition (TPN); 13.1% (14/107) of cases needed further surgery. In NC, the median duration of TPN was 9 days, but 10 of 20 cases who improved had recurrence upon re-introduction of a fat-free diet, which was alleviated by further TPN. The duration of hospitalization was (23 ± 14) days for congenital chylothorax. Upon long-term follow-up, except for GLA and DPL, most patients were doing well without recurrence. Interpretation: NC and postoperative chylothorax are the common subtypes. TPN is effective for most patients. Despite a prolonged and fluctuating clinical course, most patients had a good long-term prognosis. Key words: Chylothorax; Etiology; Neonate; Postoperative; Lymphatic anomaly

Diffuse pulmonary lymphangiomatosis (DPL) is a rare lymphatic disease that can cause diverse respiratory symptoms. A 22-year-old man, whose chest CT had shown an abnormality for years, presented with acute respiratory failure due to the abrupt onset of hemoptysis. The diagnosis of DPL was confirmed by surgical lung biopsy and lymphangiography. Histopathological investigation showed dilated vascular and lymphatic vessels. DPL can cause acute and life-threatening symptoms during its chronic clinical course. A coexisting anomaly in the venous system may be present in DPL patients with hemoptysis.