Perioperative Management of a Patient with Hereditary Angioedema and Intestinal Obstruction Secondary to an Ileal Tumor: A Case Report
Hereditary angioedema (HAE) is a rare genetic disorder resulting from deficiency or dysfunction of the C1-esterase inhibitor (C1-INH, C1-inhibitor) protein. It manifests with recurrent cutaneous and submucosal edema involving the extremities, abdomen, and/or larynx. Abdominal edema mimics other acut...
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Surgical Society of Kenya
2025-01-01
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| Series: | The Annals of African Surgery |
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| author | Antony Peter Gatheru Anne Kasyoka Barasa Isaac Karogo Mwangi Stanley Ngare Dan Kiptoon Edwin Oloo Walong |
| author_facet | Antony Peter Gatheru Anne Kasyoka Barasa Isaac Karogo Mwangi Stanley Ngare Dan Kiptoon Edwin Oloo Walong |
| author_sort | Antony Peter Gatheru |
| collection | DOAJ |
| description | Hereditary angioedema (HAE) is a rare genetic disorder resulting from deficiency or dysfunction of the C1-esterase inhibitor (C1-INH, C1-inhibitor) protein. It manifests with recurrent cutaneous and submucosal edema involving the extremities, abdomen, and/or larynx. Abdominal edema mimics other acute abdominal conditions including intestinal obstruction, potentially prompting unnecessary surgery if misdiagnosed. Prompt treatment with C1-INH typically alleviates abdominal angioedema symptoms within 24 hours, while untreated cases resolve within 2–5 days. Persistence of abdominal symptoms warrants further evaluation for other etiologies of acute abdomen. Surgical procedures with tracheal intubation can induce life-threatening upper airway edema in HAE patients. We describe the successful management of a 41-year-old female with HAE presenting with features of intestinal obstruction, which only partially resolved with the administration of C1-INH and conservative bowel decompressive management. Imaging revealed a small bowel tumor necessitating surgical intervention. Pre-operative prophylactic C1-INH was administered, followed by open laparotomy under general anesthesia with endotracheal intubation. Although C1-INH concentrates are the principal treatment for abdominal HAE attacks, other causes of acute abdomen should be considered, particularly in cases of persistent symptoms, to guide appropriate management. This case highlights the importance of multidisciplinary collaboration and careful perioperative planning to optimize outcomes for HAE patients requiring surgery. |
| format | Article |
| id | doaj-art-692cba7d460d4b158a2f14e84eea2319 |
| institution | Kabale University |
| issn | 1999-9674 2523-0816 |
| language | English |
| publishDate | 2025-01-01 |
| publisher | Surgical Society of Kenya |
| record_format | Article |
| series | The Annals of African Surgery |
| spelling | doaj-art-692cba7d460d4b158a2f14e84eea23192025-01-28T15:34:55ZengSurgical Society of KenyaThe Annals of African Surgery1999-96742523-08162025-01-012212126http://dx.doi.org/10.4314/aas.v22i1.4Perioperative Management of a Patient with Hereditary Angioedema and Intestinal Obstruction Secondary to an Ileal Tumor: A Case ReportAntony Peter Gatheru0https://orcid.org/0009-0006-9360-5632Anne Kasyoka Barasa1https://orcid.org/0000-0002-5268-5983Isaac Karogo Mwangi2https://orcid.org/0009-0004-5803-4606Stanley Ngare3https://orcid.org/0009-0001-2523-2426Dan Kiptoon4https://orcid.org/0000-0001-5318-4171Edwin Oloo Walong5https://orcid.org/0000-0003-1579-1880Department of Anaesthesia, University of Nairobi, Nairobi, KenyaImmunology Unit, Department of Human Pathology, University of Nairobi, Nairobi, KenyaDepartment of Surgery, Kenyatta National Hospital, Nairobi, KenyaDepartment of Medicine, Kenyatta National Hospital, Nairobi, KenyaDepartment of Surgery, University of Nairobi, Nairobi, KenyaAnatomic Pathology Unit, Department of Human Pathology, University of Nairobi, Nairobi, KenyaHereditary angioedema (HAE) is a rare genetic disorder resulting from deficiency or dysfunction of the C1-esterase inhibitor (C1-INH, C1-inhibitor) protein. It manifests with recurrent cutaneous and submucosal edema involving the extremities, abdomen, and/or larynx. Abdominal edema mimics other acute abdominal conditions including intestinal obstruction, potentially prompting unnecessary surgery if misdiagnosed. Prompt treatment with C1-INH typically alleviates abdominal angioedema symptoms within 24 hours, while untreated cases resolve within 2–5 days. Persistence of abdominal symptoms warrants further evaluation for other etiologies of acute abdomen. Surgical procedures with tracheal intubation can induce life-threatening upper airway edema in HAE patients. We describe the successful management of a 41-year-old female with HAE presenting with features of intestinal obstruction, which only partially resolved with the administration of C1-INH and conservative bowel decompressive management. Imaging revealed a small bowel tumor necessitating surgical intervention. Pre-operative prophylactic C1-INH was administered, followed by open laparotomy under general anesthesia with endotracheal intubation. Although C1-INH concentrates are the principal treatment for abdominal HAE attacks, other causes of acute abdomen should be considered, particularly in cases of persistent symptoms, to guide appropriate management. This case highlights the importance of multidisciplinary collaboration and careful perioperative planning to optimize outcomes for HAE patients requiring surgery.https://www.annalsofafricansurgery.com/perioperative-management-of-a-patient-with-hereditary-angioedemahereditary angioedemasmall intestineintestinal obstructiongeneral surgerycase report |
| spellingShingle | Antony Peter Gatheru Anne Kasyoka Barasa Isaac Karogo Mwangi Stanley Ngare Dan Kiptoon Edwin Oloo Walong Perioperative Management of a Patient with Hereditary Angioedema and Intestinal Obstruction Secondary to an Ileal Tumor: A Case Report The Annals of African Surgery hereditary angioedema small intestine intestinal obstruction general surgery case report |
| title | Perioperative Management of a Patient with Hereditary Angioedema and Intestinal Obstruction Secondary to an Ileal Tumor: A Case Report |
| title_full | Perioperative Management of a Patient with Hereditary Angioedema and Intestinal Obstruction Secondary to an Ileal Tumor: A Case Report |
| title_fullStr | Perioperative Management of a Patient with Hereditary Angioedema and Intestinal Obstruction Secondary to an Ileal Tumor: A Case Report |
| title_full_unstemmed | Perioperative Management of a Patient with Hereditary Angioedema and Intestinal Obstruction Secondary to an Ileal Tumor: A Case Report |
| title_short | Perioperative Management of a Patient with Hereditary Angioedema and Intestinal Obstruction Secondary to an Ileal Tumor: A Case Report |
| title_sort | perioperative management of a patient with hereditary angioedema and intestinal obstruction secondary to an ileal tumor a case report |
| topic | hereditary angioedema small intestine intestinal obstruction general surgery case report |
| url | https://www.annalsofafricansurgery.com/perioperative-management-of-a-patient-with-hereditary-angioedema |
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