El Haddar Zohair, Sbia Yassine, Rkain Maria, Benajiba Noufissa, Babakhouya Abdeladim
Department of Pediatrics, Mohammed VI University Hospital, Oujda, Morocco.
Faculty of Medicine and Pharmacy, Mohammed I University, Oujda, Morocco.
Ann Med Surg (Lond). 2021 Jul 29;68:102588. doi: 10.1016/j.amsu.2021.102588. eCollection 2021 Aug.
The primary intestinal lymphangiectasia is a rare exudative enteropathy of unknown etiology that affects the lymphatic system. It causes lymphedema and malabsorption syndrome by the escape of the lymph and its elements into the intestinal lumen.
A female patient, diagnosed at the age of 11 with Waldmann's disease, has initially manifested chronic diarrhea with a stature-ponderal delay at the age of 6 months old; she was treated for a long time as celiac disease patient. Edematous syndrome, chronic diarrhea, staturo-ponderal delay and asymmetric lymphedema of the upper limb are the main clinical symptoms in this case. In addition, the exclusion of secondary intestinal lymphangiectasia was important for the diagnosis. Before and during her follow-up, the patient presented two complications of the disease: warts and osteomalacia. The patient did not respond to treatment with the low-fat diet; therefore, the need to add treatment with octreotide was necessary, which has given quite pleasant results. Octreotide was the therapeutic choice to treat the patient as she was resistant to the appropriate regimen with clinical improvement; nevertheless, certain biological elements of lymphatic leakage persisted.
Waldmann's disease is rare. It can be responsible, besides the typical signs, for complications including warts and osteomalacia. The histopathological study of intestinal biopsies may be normal if lymphangiectasias are localized. The treatment is based on a nutritional diet associated with octreotide. During the patient's follow-up, the evolution after almost two years of the introduction of octreotide compared to the diet alone showed improved outcomes.
The treatment of Waldmann's disease is based on an adapted diet and octreotide. This case highlighted the importance of the long term follow-up in this disease.
原发性肠淋巴管扩张症是一种病因不明的罕见渗出性肠病，会影响淋巴系统。它通过淋巴及其成分渗入肠腔而导致淋巴水肿和吸收不良综合征。
一名女性患者，11岁时被诊断为沃尔德曼病，6个月大时最初表现为慢性腹泻并伴有身高体重发育迟缓；她长期被当作乳糜泻患者进行治疗。水肿综合征、慢性腹泻、身高体重发育迟缓和上肢不对称性淋巴水肿是该病例的主要临床症状。此外，排除继发性肠淋巴管扩张症对诊断很重要。在随访前及随访期间，该患者出现了该病的两种并发症：疣和骨软化症。患者对低脂饮食治疗无反应；因此，需要加用奥曲肽进行治疗，且已取得了相当不错的效果。奥曲肽是治疗该患者的首选药物，因为她对适当的治疗方案耐药但临床症状有所改善；然而，仍存在某些淋巴渗漏的生物学指标。
沃尔德曼病很罕见。除了典型症状外，它还可能导致包括疣和骨软化症在内的并发症。如果淋巴管扩张局限，肠道活检的组织病理学研究可能正常。治疗基于营养饮食联合奥曲肽。在患者随访期间，与单纯饮食治疗相比，引入奥曲肽近两年后的病情进展显示出更好的结果。
沃尔德曼病的治疗基于调整后的饮食和奥曲肽。该病例突出了这种疾病长期随访的重要性。
